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OK, I think we're al now can uh one of the members of the audience just put on the chat if they can hear us all fine and see the slides? I just seems OK if you can't hear us or you can't see the slide, just put it in the chat and then we can er then we can sort that out. So thank you for joining us today and welcome to the first er teaching session. Uh So we're just gonna start immediately uh with stroke and then we're gonna go into demyelinating diseases. So, uh thanks to Shaha Sharif for presenting stroke. So, Hanna W um yeah, so I'll start off with stroke. Um So if we can go on to the next slide, please. OK. So um stroke by definition is a clinical syndrome consisting of rapidly developing um clinical signs of focal and potential, the er global disturbances of cerebral function. Um and these have to last for over 24 hours er or cause death er to be classified as a stroke. Um and there has to be no other apparent cause um other than that of a vascular origin um and the cerebral function issues occur when there's uh either blockage of blood flow in the brain or a bleeder that causes there to be parts of the brain that are starved of oxygen. Um And basically, it's a general term um that can be presented in two ways. So either ischemic stroke or hemorrhagic stroke. Um And additionally, we have ti A S as well or transient ischemic attacks er, that follow a similar pattern to these, but uh the signs and symptoms will resolve within 24 hours. Um And here you can see a quick little diagram um really simply explaining um the differences between the two or showing the differences between the two rather. Um So, yeah, um can we move on to the next slide, please? Ok. So um firstly, you have ischemic stroke um and this makes up the large majority of all strokes um that happen. So, um ischemic stroke can be caused by several potential mechanisms er that occur um that cause a reduction of blood flow to the affected brain region leading to inadequate tissue perfusion. Er And examples of these um mechanisms are listed below. So, firstly, embolisms, this is where an embolism that originates from somewhere else in the body, ie the heart or the lungs er can cause obstruction of the cerebral vessels, er causing hyperperfusion of a particular part of the brain. Um And then we have thrombosis, this is where a blood clot forms locally within the cerebral vessels itself. Um and this is often due to atherosclerotic plaque rupture. Er, next we have systemic hyperperfusion. Er this is where um the blood supply in the entire body is reduced and the entire brain is reduced. Um er this is usually secondary to systemic hypoperfusion such as er cardiac arrests. Um and then finally on the list, we have er cerebral venous sinus thrombosis. Um and this occurs when blood clots from the veins that drain into the brain um cause blood clots. Er This results in venous congestion causing tissue hypoxia. Um This is not an exhaustive list. There's more mechanisms as well, but these are some of the more common ones. Um So, yeah, can we go onto the next side, please? Right. So, in terms of hemorrhagic stroke, there's only two main subtypes really. So we have intracerebral um stroke, intracerebral hemorrhage and sub subarachnoid hemorrhage as well. So, intra intracerebral hemorrhage, as you can see here er occurs when there's bleeding um when the bleeding is due to a ruptured blood vessel, um this can be within the brain tissue or within the ventricles. Uh and then the subarachnoid hemorrhage occurs when there's bleeding outside of the brain between the pia matter and the arachnoid matter. Er this bleed can cause intracranial pressure and compression of the brain tissue and further cause disruption of blood flow. Uh and furthermore, the blood accumulation can also trigger um an inflammatory response causing further injury as well. Um so yeah, can move on to the next slide. Ok. So in terms of classification of stroke, um there are multiple different classifications of stroke um which will present in a different manner. Er but the classification of stroke is done based upon using the initial symptoms, using the Bamford classification system. Er and this system basically requires an assessment of three main things. Er So as you can see here, er one of the things is unilateral hemiparesis or hemisensory loss of the face, arm and leg. Um So this is basically saying paralysis of one of the limbs or the loss of ability to um have sensation in one of the limbs or the face of the arm. Er and then you have ho a homonymous hemin here and we have higher cognitive dysfunction such as dysphasia and visuospatial disorders as well. Uh So, by using this classification system, we can differentiate between total and partial anterior circulation stroke. Um So, firstly, for total um anterior circulation stroke, er, we'd need all three of these to be present. Um and total anterior circulation stroke, er is a stroke that basically affects the areas of the brain supplied by both the middle and the anterior cerebral arteries. Um and then moving on to the partial anterior circulation stroke, er, we'd only need two of these to be present. Um However, it must be noted that if the higher cognitive dysfunction is noted even by itself, that is enough to be classified as a um a partial anterior um circulation stroke. Um and then we also have a posterior circulation stroke. Um and um this this is when there is damage to the areas of the brain supplied by the posterior circulation. And this includes the cerebellum, the occipital lobe, the temporal, medial, medial temporal lobes, the thalamus and the brainstem. Er and for the diagnosis of a posterior circulation stroke, one of the following must be present. So, as we can see here, er cranial nerve palsy and a contralateral motor or sensory deficit, er bilateral motor slash sensory deficit, um conjugate eye movement disorders, um such as horizontal er gaze palsy, er cerebral dysfunction, such as vertigo, nystagmus, ataxia, er or isolated homonymous hemin. Um And you know, we can see the different supplies er of the brain and, and the diagram that sit there. Um And yeah, er can we move on to the next slide, please? Ok. Um So in terms of uh locating the lesion, uh so different strokes will have different presentations depending on where the lesion has occurred. So, um depending on where the lesion has occurred. So we'll run through the most um important slash um the more high yield examples first. Um so firstly, we have anterior cerebral artery stroke um and this causes er contralateral hemiparesis and sensory loss. Er and the lower extremities will be um will will be affected more than, than the upper. Uh and basically in simple words, this means that this is a that if a stroke occurs in the left anterior cerebral artery, uh it will cause sensory loss and paralysis of the right arm and leg. And notably the er legs will be more affected er than the arms. Um and then moving on, we have middle cerebral artery stroke and this presents in a similar manner as well. Uh So it'll cause contralateral hepar and sensory loss. But in this time, the upper limbs will be more affected than the lower limbs. Um A common pneum pneumonic often used in medical school for this is Mao. So ma A L er which basically means er middle arms, anterior legs. So ma A L um suggesting basically that the medial cerebral artery affects the arms more er and then the anterior affecting the legs more. Um So additionally, with er medial cerebral artery strokes, er we'll have contralateral mon homonymous hem anopia er without macular sparing as well. Um So, as you can see right underneath there, er right homonomous hemin. So a left middle artery er middle cerebral artery stroke would cause less. Um and then just to list below as well, we've got a diagram of what um macular sparing would look like as well. So as you can see, there's that little middle portion that's missed out um of where the the vision won't be affected. Ok. So, uh along with this, in terms of the middle cerebral artery stroke, um we might also notice aphasia uh in the patient which is a disorder of speech er, that can present in, in multiple different w ways depending on the severity of the stroke. Um it can present from mild to global aphasia, er with a patient struggling to speak in complete sentences or having issues with repeating speech, um issues with understanding others, er and also er producing understandable speech. Um so that's basically it for middle cerebral artery stroke. And then next we have er posterior cerebral artery stroke. So er this will present with contralateral homonymous hem anopia, but this time with macular sparing er and also with visual agnosia. So what this means is that the patient will struggle to recognize simple day to day objects simply by looking at them. Um and like I said, there, there's an example of what the er what the um homonymous hymn Noia would look like with mac macular sparing. Um And then next, we have a stroke that occurs in the branches of the posterior cerebral artery that supply the midbrain. Um This is of often known as Weber Syndrome as well. Er So this will cause um ipsilateral cranial nerve three palsy uh alongside contralateral weakness of the upper and lower limbs. Um So, cranial nerve three palsy will usually present as diplopia ptosis and afferent pupillary defect. Er and the eye will appear in the down and out position. Um So what this basically means is the diplopia refers to the double vision. The ptosis refers to the eye eyelid drooping. And the afferent pu defect suggests that the eye will dilate rather than constrict when um light is shone into it. Um and then moving on, we have er posterior inferior cerebral artery stroke. Uh This is often known as lateral medullary syndrome. Er and this will present with ipsilateral facial pain, er and temperature loss alongside contralateral limb and limb pain and temperature loss. Um and this type of stroke is also associated with Ipsilateral Horner syndrome. Um, and Horner Syndrome is basically when a patient presents with a constricted pupil, er, drooping eyelids and decreased sweating, known as anhydrosis. Um And then finally, some of the less common ones that will, that you'll see in exams um are your um anterior inferior cerebral artery stroke. Um and this basically causes symptoms that are very similar to Wahlen bg's um but also presents with facial paralysis and deafness on the ipsi lateral side. Er, and then finally, we have er basilar stroke, er, and the stroke basically causes a condition called locked in syndrome. Er, and this is when there's complete paralysis er below the eyes. So the patient will be able to blink, move the eyes up and down. Um but the patient will not be able to produce any movement such as chewing, swallowing, speaking, er, but the patient will be able to read, listen and respond to stimuli as well as comprehend it and process the information. Uh next slide, please. Ok. Uh So next we have the um acute management of of stroke. So firstly, we can use the Rossia um scoring system which is shown on your left hand side there. Er And anything above zero would make you suspicious o of a stroke. Uh So first thing you want to do when you're suspicious of a stroke is firstly rule out uh hypoglycemia as this can sometimes present with uh symptoms very similar to focal neurology. Er So this can be done easily with a, with a bedside er BM. Um So then the, the next part of it is to determine whether this is a ischemic stroke or a hemorrhagic stroke. Um And in order to basically perform a non contrast ct um and then depending on the type of stroke, it is the management will change. So firstly, if we discuss ischemic stroke, um so a stat dose of 300 mg of aspirin is given, er then the further further management of the patient depends on the duration of the symptoms. So firstly, if a patient presents er within six hours, the patient can be offered er thrombectomy er which is basically er a mechanical removal er of the clot. Um but in order for this to be offered the treatment, um but in, in order for this treatment to be offered, the occlusion must be in the proximal anterior circulation um which will be which will be proved by an mm mra of the brain. Ok. But, um, the six hour, um, symptoms, time is not a complete contraindication if the patient presents after six hours, um, it's more advised. But, er, even if a patient presents outside of the six hour window, er, and if the surgeons believe that there is a chance to salvage um brain tissue, um, because of the infarct is limited, they might still opt for er thrombectomy. Um And then additionally, if the patient presents within 4.5 hours of the symptoms, starting uh thrombolysis can be performed with the use of out of place. Um And for this to be performed, there's a huge list of contraindications um that are, that are present, I'll read out some of them, er but there are others as well that must, that you must be aware of. So, some of the contraindications are active bleeding, er previous intracranial hemorrhage, previous gi bleeds in the last three weeks or current viruses, er and pregnancy as well. Um And then moving on to hemorrhagic strokes. Um So firstly, it's, it is uh empirical to er reverse the anticoagulation if a patient is on anticoagulation. Um and then refer to neurosurgery. Um So, in terms of anticoagulation reversal, a commonly asked question is uh the reversal of warfarin. Er So, in order for this to be done successfully, um in terms of an intracranial bleed, the patient should be given IV Vitamin K alongside prothrombin complex as well. Uh And the next likely. Ok. Um So that's the acute management of stroke done. And then we're on to the er secondary management er of stroke um post stroke. So after the acute scenario is dealt with, it's extremely important to provide secondary prevention of stroke. So firstly, all patients should be placed on er antiplatelet therapy and the standard treatment is clopidogrel, 75 mg daily. Um but uh aspirin, 75 mg daily with modified release um uh with 200 mg twice daily may also be used if the clopidogrel is not being able to be tolerated by the patients. Um and um modified release er dial um 200 mg twice daily may be used if both clopidogrel and aspirin are contraindicated and can't be tolerated. Um And er then finally, aspirin se 75 mg daily may be used if both clopidogrel and modified release um are contra or contraindicated or cannot be tolerated. Um And then, so that's the antiplatelet therapy done and then moving on to the lipid modification um drug treatment uh unless contraindicated treatment with high intensity statins such as atorvastatin er will be offered at the diagnosis of ischemic stroke or tia A by secondary care. Uh And then uh the next management is the anti hypertensive drugs. So, treatment of anti er treatment for hypertension following stroke or ti A should be initiated by secondary care following diagnosis and may include things like thiazide like diuretics, long acting calcium channel blockers, er, angiotensin converting enzyme inhibitors and angiotensin two receptor blockers. Er, blood targets for people. BP targets for people with cardiovascular disease are the same as uh the ones for people without cardiovascular disease. Um, except for people with severe bilateral carotid artery stenosis for whom a systolic BP target of 100 and 40 to 100 and 50 is, er, is appropriate. Um And then finally, in terms of stroke management, uh this is not really secondary care but should be done acutely. Um Just forgot to mention it earlier, but all patients that present with a stroke should be assessed for safe swallowing of fluids and solids before any oral intake is had. So this should be done within 72 hours of admission um of the patient into, into the hospital. Um and patients that are deemed unsafe for swallow due to fears of aspiration should have a nasogastric tube placed as a first line mechanism for adequate nutrition and next time, please. Ok. So we just have a few two SBA S here. Um If you guys want to read the SBA um and have like a minute to read it and answer the question and then I can present the answer. We'll give about 30 seconds for you guys to read it and have a think and then we'll give the answer. Yeah. Are you happy to share the answer? Now, share Yeah. Um Yeah. So er the answer would be uh b so as we can see there, the ac A supplies the medial portions of the frontal lobe and the superior medial parietal lobe, er, which are involved in the lower limb supply. Er, and therefore, occlusion of this artery will cause contralateral lower limb weakness. Um The MTA supplies frontal, temporal and parietal lobes, er including the areas responsible facial throat and hand slash arm innervation. Er Hence, the occlusion causes speech impairments, contralateral weakness and hemiparesis er of the lower contralateral face. Um This is, this is forehead bearing due to bilateral innervation of the er frontalis muscle indicating an upper motor neurone lesion caused by stroke. Um and then the PCA supplies the occipital lobe which is involved in visual processing. Hence, er PCA strokes um can present with acute vision loss, um memory impairment due to um hypocal infarction as well. Uh and then move on to the next slide. Uh And then the last SBA you have to share the answer. Yeah. Ok. So the answer is Berber's syndrome. Um And this patient presented with cranial L3 palsy and right sided hemiparesis. Uh This is consistent with uh a midbrain stroke, er named be syndrome as we discussed earlier. Um and to rule out the the other causes. So, for example, Horners syndrome does not cause weakness, although it will cause theos um natural med would cause aps, lateral facial pain, uh apy lateral facial um loss of temperature, pain, sensation, and contralateral uh limb loss of temperature and pain sensation. Um and then er lateral pontine syndrome or an anterior inferior cerebral artery stroke would present with to lateral facial weakness, loss of temperature and pain sensation, uh contralateral limb weakness, uh and loss of temperature and um pain sensation. And the patient would also have deafness. Um And finally, we can rule out locked in syndrome as this would cause complete paralysis below the eyes. Uh But clearly this patient is able to move despite being weak. Yeah, great. Thank you very much, Jha. Uh We'll go on to ka now. Uh So kita you're ready to present. Uh Yeah, no problem. Are you gonna keep control then? So, yeah, I'll keep control of the slide and then just let me know when you want to. Uh So my teaching session is gonna be on neuromuscular disorders today. Um And can we go to the next slide, please? Uh And to understand what we are talking about on neuromuscular disorders. I thought I'd just include a definition. These are a wide range of diseases affecting the peripheral nervous system, which consists of all motor and sensory nerves that connects the brain and spinal cord to the rest of the body. And progressive muscle weakness is a pred is predominant condition in these disorders. So, when we're thinking about our um neuromuscular disorders, we're thinking mainly in the region of the peripheral nervous system. Er, and that's why the symptoms are fairly easier to recognize once you understand where it is they're coming from. But to understand that we first need to understand the musc, er, the nervous system, especially the nervous system that controls muscles. So, Morgan, can you go? So, our motor control is, it, it is mainly controlled by our cortical spinal tract and this starts in our precentral gyrus located in our frontal lobe And as it moves down, it forms, uh it goes into our, to our internal capsule, er, moves down our brain and goes into our cerebral peduncles, uh peduncle in the midbrain, er it then moves further down and then decorates at the medulla. Now you notice here that some of the tracts actually go uh carry on and decussate at the level um of the spinal er muscle. Uh that is the anterior corticospinal tract. This mainly controls er central muscles such as those of the trunk and the, the chest. You don't really need to worry about those. The majority of tracts in the corticospinal tract are controlled by. Um uh so the these are the ones that control our limbs. So our arms and our leg movements, the the majority of these are from the lateral corros spinal tract which decussate at the level of the medulla. Er and that's the one that you're mainly be testing when you're doing things like your upper limb and lower limb, uh neurological examination. So, as you can see, there's a Decca at the third pyramids and then it goes down all the way to the level of the skeletal muscle at the spine uh where we'll then get our second or in urine. So Morgan, can you move on to the next slide, please? So when you're thinking about how these nerves move through the spine, sorry, I thought, sorry about the quality here. It looked a lot better. I'm sure it will when you get the slides. But um when you're looking at how these these nerves or these nerve tracts move through the spine, it's important to know generally where in the tract, uh each, you know uh where in the um the spinal cord, each tract moves. So when you think about our lateral cord, the spinal tract, the one that controls our limb movements, uh it sits here in the lateral side. Uh and you can see and green in the in the slightly more clear image. Um and then your dorsal column which controls your light touch, vibration, pro reception is in the back. And you also have your um spinothalamic tract as well, which is for pain temperature, discriminative touch that sits in the front. And it's important to know that because you can also have vascular changes and vascular problems that can lead to problems in these tracts and being able to, to know where these tracts run in the spinal cord can have be localized lesions later on. So if M Morgan, if we can move on to the next slide, when we're thinking about our motor neuron, they have dedicated the level of the medulla, they come down to the level of the, the muscle. And that's our first order in urine. So the first order in urine is always the first neuron. The first order in urine in our cortical spinal tract is our upper motor neuron, the second or the urine. So here you can see at the ventral root uh which sits inside the anterior horn of the spinal cord. You can see it um decussation, so sorry, not decussation, a connection between the the first order and the second order neuron. The second order neuron moves from the spinal cord into your muscle fibers. A key difference of the motor tract and the sensory tract is you can see your dorsal root ganglion sits outside of the spinal cord. So you can actually get upper motor neuron sensory problems. Upper second order sensory problems from a dorsal ganglion problem. But you can also get um upper motor neuron problems uh from a spinal injury with your and lower motor neurine problems from spinal injury as well because your second or neuron, which is your lower motor neuron still sits inside the spine. Uh And that's quite an important distinguishing because when you think about the peripheral nervous system, some of the lower motor neuron signs that you'll see can still originate from the spinal cord themselves. And so it's still important to exclude or include spinal pathologies. Uh when you're looking at a patient that has presented with what could be a neuromuscular disorder. So, moving on to the next slide, the the first condition that we wanted to talk about was motor neurone disease. It's very common. This is a disease of the motor neurons, hence the name uh and that's quite important in the patient's presentation because we can now exclude sensory neurons. So, in this uh condition, it's quite characteristic for there to be no sensory involvement because it's again, it's a disease of the motor neurons that kind of helps guide your history and can help your diagnosis particularly when you a question of a patient that's presenting with motor neuron or motor problems. And there are various patterns of motor neurone disease with a S being the most common. Uh and some other ones that you may have heard of is progressive muscular atrophy and bulbar palsy. Um We may do a bit more of a teaching session on these later on, but the most common one being A S is the one I we wanted to focus on in this session. Uh And the course of motor neurone disease is unknown. It's suggested that there are certain genetic predispositions uh that may actually lead to sudden degeneration of the motor neurons and it's fairly random. Uh and it presents with both upper and lower motor neurons. So you're thinking of your first mo neuron in your brain and your second or urine that goes to your peripheries. So you're looking at upper and motor neuron signs. Uh but you can have isolated upper motor neurine signs and isolated lower motor urine signs. So it's important not to exclude it if you have signs of just one. So if we move on to the next slide, uh we wanna look at the key features and because we don't know the cause we can't really stop it and we don't know where it is that we're attacking and it's a genetic problem. So the key things in the history that will tell you is asymmetric limb weakness and that's because the, the degeneration is random, it doesn't actually follow a particular pattern, follow a particular structure. And because we still don't know too much about it, we can't actually predict where it is that we're going to see the damage. So, asymmetric limb weakness is quite a characteristic sign, particularly in A LS. And again, you get those upper and lower motor neuron signs because the degeneration can happen in any of the nerves on the cord of spinal tract, fasciculations are a lower motor neuron sign. Uh and these are quite common, particularly on the tongue. These can be quite common in patients that have motor neurone disease. And again, because it's a disease of the motor neurons, you are not going to have any sensory signs. Um doesn't necessarily suggest that you can't have any sensory pathology. But in motor neurone disease, you're not going to see any sensory signs, particularly when they're presenting your history in your examination. Ok. Um In your exams, sorry, not your examination. Um And again, this is degenerative problem that tends to happen older. Uh So you're not going to really see it in patients younger than 40. And there is sometimes a family history uh of motor neurone disease. It doesn't actually typically run through families. Uh So this isn't as important, but it's quite important to note a family history of motor neurine disease. As we do think there's a genetic aspect of it. And again, there are some closely related conditions such as frontotemporal dementia. If you move on to the next slide, sorry, it's like a tendon, so on. Er, and think about general symptoms. Again, these are all gonna be disorders of motor neurons in, in very random places you won't have all of these. Uh you may have some, you may have all of them and as you progress, you tend to find that the list starts to build and build and build. So you get weakness in ankles or legs, uh you get slurred speech and you get weak grip, muscle cramps and twitches, you may get weight loss. This is actually typically from disuse atrophy where you are actually can't use your muscles. And so they start to break down and that's where the weight loss comes from. You may get difficulty, er, stopping yourself laughing in inappropriate situations. They actually lose control of your muscles and your ability to regulate. Even if you don't find it funny anymore, you find it difficult to stop. And again, this kind of ties into uncontrollably crying, you may not, you may be upset but you actually have difficulty controlling the muscles that may stop you from doing this and then uncontrollable, excessive yawning and difficulty breathing. So you can see there's a lot of dysregulation of muscular activity involved in this. Ok. Um Oh, that's a good question from Feda. So I'll stop for a second. So fas asks, why do you get reduced reflexes in lower motor urine and brisk reflexes in upper motor neuron. So how it actually works is our lower motor neuron is the one that attaches to the muscle itself, right? And so this is the one that's gonna give the muscle, it's it's action. Our upper motor neuron is the one that sends a signal from the brain when the brain has decided it wants to move to the lower motor neuron. So the upper motor neuron is more of a regulatory neuron. Ok. So it actually both inhibits and excites the second order or lower motor neuron. So when you get in upper motor neuron lesions or disorders of the central nervous system, uh where your brain or your first order spinal neuron might be damaged is you lose your ability to regulate that second, lower motor neuron. And that means that you can actually over excite that second neuron. And that's why you get increased reflexes or brisk reflexes in upper motor neuron problems. Whereas if you have a lower motor neuron problem, this muscle is only d this nerve sorry is only designed to stimulate the muscle. If you get damage in your lower motor neuron, you then can't elicit your reflexes and therefore you get reduced reflexes. Does that help your understanding? Ok. It's, it's ok. Well, if you still need an explanation, I can carry on later, but we'll carry on with the presentation for now. So we can go to the next slide. So to diagnose motor neurone disease, there's no actual clinical diagnostic test. Um Oh brilliant. So what you need to do is actually exclude all of the other potential causes for problems that may present in a similar way. And so you do quite a lot of neurological tests such as nerve conduction studies, electromyograms. And in this case, nerve conduction studies in mo neurone disease will actually be normal. Um And um electromyograms will have a reduced number of action potentials plus increased amplitude. Don't worry too much if you remember that. Um But it's just one of the, the characteristic signs that you may see on an E MG. Um You do an MRI to exclude certain other differentials as well and lumbar puncture blood tests muscle biopsies aren't done often, but you can do them. Uh and sometimes gene testing is, as we mentioned, there is a genetic component to these uh conditions. And sometimes gene testing can also offer some, some to shed some light to a cause. But again, it's not a diagnostic test that we can do uh you know, regularly. So you wanna, it's more of a clinical diagnosis based on your judgment and you wanna make sure you've excluded all other potential other causes. So we go on to the next side, we'll kind of start to cover the treatment of motor neurone disease. And again, because we don't really know too much about it. There is no cure for motor neurone disease. So you're thinking about your conservative surgical and your um medical and your surgical management in terms of conservative management. Next slide, please. Uh We have a MD team, so you'll have an MDT for your motor neurine patients because they will need to be managed long term by multiple members of the healthcare team. Uh So you're thinking your doctor, of course, that would be you. In this case, the specialist nurse, physios, occupational health, speech and language, dieticians. You also want to get emotional support because it's something you can't get rid of. You wanna make sure that the patient is supported long term after your care. When we're thinking about our medical treatments, there is a drug. Sorry. Can we go to the next slide. There is a drug that can actually slow down the progression of uh motor neurine disease called Riluzol. It's the only licensed drug for er, slowing down the progression of motor neurine disease. But again, it doesn't treat motor neurine disease and then you sort of treat based on the patient's presentation and complications. So, if the patient has muscle cramps, you can use things like quiNINE um muscle stiffness, baclofen, dring, Hyden hydro bromide. There are also alternative options. There are communication aids and carbocysteine for swallowing difficulties. Again, this is all based off um you know, a clinical assessment, things like quiNINE have a lot of side effects, tinnitus, hearing loss, hearing and visual disturbances. So you kind of want to involve physiotherapists if you're going to include something like this, uh and carbocysteine, acetylcysteine only we use for d secretions because they're only effective against that. So, you know, it's kind of a clinical diagnosis. It's a big decision and it's more management of any complications that come with motor neuron disease. Um All right, sorry, that's my mother calling me. Um Can we go to the next slide, please? And when you think about your surgical er treatment, you can't actually do anything surgically, but you may need surgical intervention particularly in the complications. So we think about degeneration of motor neurons, we can't regulate our ability to swallow or to breathe anymore. So you may need a gastrotomy too to ensure that the patient has a long term feeding method, uh and noninvasive ventilation. Unfortunately, this is where motor neurone disease tends to be kind of near its later stages because patients lose their ability to regulate their respiratory muscles, they lose their ability to breathe for themselves. And this can be often, you know, respiratory issues, recurrent respiratory infections can often be the cause of death in a lot of patients with motor neurone disease. So this is something that you wanna consider as well. Um OK. So that's motor neuron disease kind of quickly covered when you're thinking about multiple sclerosis, which is a a strong differential for this. We're looking at more chronic cell mediated autoimmune damage to our central nervous system. Ok. Uh And this is cell media damage to our brain and our spine, right? But don't forget that your lower order, your second order lower motor neuron or your second order neuron in the corticospinal tract also sits in the spine. So you can actually still have damage to your peripheral nervous system or your your lower motor neurons in a patient that has multiple sclerosis where you're only really getting attack to your central nervous system. So again, this leaves lesions on the on the white matter. Uh and this can lead scarring, hence the name multiple sclerosis. It's multiple neurons that are randomly being attacked by our own body. And the cause is is unknown, can sometimes be related to viral pathologies. But um for the most part, there is no particular cause. Uh it tends to occur in, in women three times more common than men. Uh and it tends to also occur between the ages of 2040. This is the, the mean age that it tends to, to start originating. And if we go to the next slide, uh when you think about your symptoms, because you're getting an attack of all the neurons in your central nervous system, you can have a whole broad range of symptoms. And that's why it can be quite difficult to diagnose something like multiple sclerosis. Uh However, the most common presentation, particularly on the first episode is optic neuritis. And if you haven't heard of it, you will essentially present with uh ophthalmoplegia, which is difficulty moving your eye and pain on moving your eye as well. You get reduced visual acuity. Um and there's a whole bit of other signs, you'll, you'll learn a bit more about it as you, as you study ophthalmology. But optic neuritis is quite a a telltale sign of multiple sclerosis because multiple sclerosis is the most common cause of optic neuritis. There are other causes as well, which you won't need to know about right now. Things like syphilis can cause optic neuritis and B12. You, you know, of course, you'd exclude them if you had a patient with optic neuritis. When you're looking at a patient with multiple sclerosis, if a patient comes in with these visual symptoms as eye pain. It can be quite a big indicator that this could be uh multiple sclerosis, particularly if they have, you know, problems with their limbs as well. Uh And again, think about sensory deficits, we can get pins and needles. Trigeminal neuralgia is quite common complications. Multiple sclerosis can cause trigeminal neuralgia. Um and you also can get la it's spine and this is pain when you flex your neck. Uh and this, this is quite a good test to actually do when you're suspicion of a suspicious of a patient with multiple sclerosis. And you're doing a cranial nerve, upper and neurological examination, you can ask them to flex their neck and they'll actually experience pain from this. Um And this can be quite a, a good indicator for multiple sclerosis. Um Then you have your motor neurons because it's in the central nervous system that's mainly being attacked. You're thinking mainly upper motor neurine problems. But again, you do still have those neurons, the second order neurons that sit inside the spinal tract that can still be attacked. So you can also get lower motor neuron signs as well. So you can get a mix of both. And when you're thinking about cerebellar problems, cos again, central nerve system attack, you can get ataxia and tremor thinking about your cerebellar functions and regulating movements and then a whole bunch of other random things that are also associated with damage to the central nervous system. So urinary, incontinence, sexual dysfunction, intellectual deterioration. So it you know, all of these again won't be present, but you'll start to build a picture of what could be happening. And through your investigation, diagnostic testing, you will arrive at an answer. So if we go to the next slide, please, um there are various patterns of progression again, sorry about this image, but I can talk you through it um when you're coming to multiple sclerosis. So going actually from the bottom of the image. So on our Y axis, we have increasing disability and on our x axis, we have the the time as, as things progress. OK. Uh And when you're thinking, we're going from the bottom graph. Now, this is the most common type of multiple sclerosis called relapsing remitting multiple sclerosis where you actually have, you can see a baseline of symptoms or baseline disability, then you'll have these attack, an attack which is quite acute in setting this attack will then drop off and then you'll return back to a baseline that's not quite as good as the one you're on and this carries on. And this is actually the most common form of progression for multiple sclerosis. The graph above that shows a primary progressive multiple sclerosis. Uh This can just progress, you know, as you can see, it just progresses naturally, slowly and steadily deteriorating in neurological function. The graph above that is secondary progressive and it's called secondary progressive is because you start off with relapsing, remitting first and you're carrying on as we expect you to. But after one of your attacks, for some reason, you then develop into progressive multiple sclerosis. Hence the name secondary progressive multiple sclerosis. Uh not understood why, but this is actually the second most common um type of multiple sclerosis as well. And that's sometimes something they have to ask you in your progress test. The last one, a little bit more rare is progressive, um progressive relapsing again. So you have that progressive style where you're steadily declining in neurological function, but you also have superimposed attacks on top of that as well. Quite rare. But again, when you think about the most common type is our relapsing, remitting. That's exactly how I expect the condition to carry on progressing. So we go to the next slide, please. When you're thinking about investigating a patient with MS, ignore that black square, it shouldn't be there. Um You want to get a, a general picture of what's going on inside the patient, but there is a diagnostic test that you can do in this case. So again, your cranial nerve examination fundoscopy, particularly if you're suspicious of optic neuritis, um you can actually see it sometimes do bear in mind. Optic neuritis can have a normal optic disc as well. Um But optic neuritis is essentially inflammation of the optic disc, um not optic disc, optic nerve, sorry, but you can have a normal optic nerve and optic disc on fundoscopy. So don't exclude optic neuritis because everything is normal. Uh You do ecg and you do bloods again, looking for those things that can cause optic neuritis. Um you know, b12 and folate syphilis are quite common ones as well. A vasculitis screen because things that can cause vasculitis can also cause similar symptoms. And here I've mentioned NM O this is called uh neuromyelitis ophthalmicus. It's a very common differential of optic neuritis in the sense that it actually present of multiple sclerosis, sorry, in the sense that it presents in a very similar way. This can also cause optic neuritis and can also cause peripheral nervous systems but has a different diagnostic test. So if you do get an exam question or an Iski station where you actually have a patient that could have on sclerosis, this is quite a useful differential for you to go for because it actually presents in almost exactly the same way. Uh the diagnostic test, however, or the, you know, the test of choice is uh MRI brain and spine. Um and you can use gadolinium. Gadolinium is a contrast which is taken up in inflammation. Again, bear in mind your central nerve system or your cells, um your sorry, your immune system is attacking your central nervous system. So you should see inflammation in certain parts of your brain and your spine. So Galin can be quite useful contrast, particularly if you're very suspicious of multi sclerosis and you see these things, Dawson's finger plaques. Uh I won't go too much into what exactly they are. But you, if you take a look at them after this, uh it, it, it becomes quite obvious but they're quite useful and they're very telltale of multiple sclerosis. And so is juxtaoral hyperintensity. If you move on to the next slide, we start thinking about the treatment for M oh, sorry. There are actually more special tests you can do. Um because of the way optic neuritis presents, it can present in a similar way to glaucoma. So you can do tonometry, uh which is a test that allows you to record intraocular pressure, sometimes done just to exclude against excluding other options. Lumbar puncture is very good and it's quite ano another useful test for multiple sclerosis. And the thing you're looking for in a lumbar puncture is a li Coron of glands and essentially all these are, are essentially molecules that are produced when the central nervous system is damaged or attacked when there's inflammation in the central nervous system. So multiple sclerosis will produce illegal Coronal bands, but it's not only multiple sclerosis that does. So, just because you see them in a lumbar puncture, it doesn't necessarily suggest you have multiple sclerosis. And again, other things that may suggest increased inflammation inside your nervous central nervous system, especially on our lung. Um Lumbar puncture is raised glucose, you may get increased protein and increased white cell bear in mind, you can still see these on the lumbar puncture and they can still suggest inflammation. Um Then there's another special test called visual evoked potentials. Uh Essentially all this is is you place a Nero at the front of your head and the back of your head, you shine a light into the patient's eye and you record the speed of the impulse from the front to the back. And of course, you'll be expecting a reduced speed in a patient who has multiple sclerosis, who has central nervous system damage inside their brain. If we go to the next slide, we start thinking about the treatments for multiple sclerosis. Uh sorry, the diagnosis I haven't diagnosed the patient yet. Um Diagnosis is based off the mcdonald's criteria. So you can do all of these things and you can, you know, have these findings and be confident you have a patient who looks like he has multiple sclerosis, but you actually still need dissemination in space and time. And all this means is that you need to have symptoms at two different dates on multiple different dates, hence dissemination in space and multiple different parts of your body. And that's um and that's dissemination in space. So I may have said that the wrong way round, but you get what I mean. So you need to have symptoms at different dates and in different parts of your body to suggest that there's multiple sclerosis bear in mind that the most common form of progression is that relapsing, remitting. So we need to have a previous episode where the patient has mentioned, actually, I did actually have, you know, problems with my vision difficulty moving my arm. This time, they presented with bila um or bilateral limb weakness or something like that. Ok. So you're looking at two different attacks and in multiple different parts of the body and that's what's required to actually properly diagnose multiple sclerosis. And that's your mc mcdonald's criteria. And now we can go on to management again for this one, no cure. But again, you can still manage these patients conservatively, conservatively, medically and surgically when you're thinking about your conservative treatment. Next slide, please. Um we still have our MDT er and you know, staying team will be roughly involved because these patients will still be deteriorating in neurological function. And again, again, with your motor, there is you're gonna start losing control of your ability to, to actually breathe that eat. And this is very important and these patients, it may also be quite important to get VD prophylaxis so that they may not be mobile, particularly during some of their flare ups and um Vitamin D as well if they haven't been out. Er, but that's just something again for your is D. Uh and when you think about your medical managements in an acute flare up of multiple sclerosis. So again, when you're thinking about back to that, that graft where you have your relapsing, remitting symptoms in a relapse where you now have actually very severe symptoms after you've been steady on a baseline for a while, you need to use steroids. So the steroid of choice is methylprednisolone or, or IV tend to prefer oral because again, it's more convenient to just pop a pill in and, you know, instead of setting up an IV for a patient and it's cheap for the NHS as well. Um Economy is very important in the NHS and sustainability. Um If you are going to give a steroid orally though, always cover with a PPI because steroids can also increase your risk of peptic ulcers. So um very important whenever you give um oral steroids uh to cover patients with A PP as well. And then when you think about long term after they come back back to their baseline, OK. My slides have disappeared. I see Morgan has two. Yeah. The, the Wi Fi is not looking very good and they can you, can you see the slides now? Um Oh, no, sorry. I just II had the slide up. So I was gonna start sharing them myself. There you go. Can you see them now? Uh Yes, yes, we have. Um OK. So I'll carry on and I'm thinking long term again, there's nothing more you can do. There's no curative treatment, but there are these things called disease modifying treatments and these are essentially just immunosuppressants and things like Alemtuzumab oli, you know, very fancy names. But, you know, you just have to learn these and these are the common ones that you'll see. And again, if you're gonna give a patient an immunosuppressant and make sure they don't have any underlying infections or any problems. So you do a blood drawn a viral screen before starting these and you have to monitor these patients IgG G and white cell count levels. As if they start to drop, you need to drop your, your dosage because obviously your, your these patients are becoming too immunosuppressed. And when you think about surgical managements, again, not much you can do surgically. Uh you know, when thinking about progression, can we go to the next slide, please? Thinking about progression. Again, if you look at the bottom, these patients can still have breathing difficulties, can still have dysphasia. So, noninvasive ventilation and gastrotomy is still important. But there are certain, you know, procedures that you can actually do for certain situations for certain certain symptoms. So deep brain stimulation can be quite useful for tremors and stiffness. Um thing, a procedure called a rhizotomy can and trigeminal neuralgia. Uh And essentially, this is just removing the fingers of the trigeminal nerve so that you don't have the pain caused by trigeminal neuralgia anymore. Back of the fe palm essentially is just surgically inserted in the back of your head and left. There can be quite useful for patients with spas, spasticity and hemato hematopoietic stem cell transplant. Very risky rare treatment. But it is quite effective. Essentially, what we do is take the patient's stem cells out. You treat the patient, give the patient chemotherapeutics to kill off their internal immune system and then you give them your treated immunosuppressants. Um Oh, sorry, they're treated stem. So essentially their new immune system should be treated and shouldn't attack their central nerve system anymore. Of course, because you're immunosuppressing the patient. They have to be isolated the whole time during this procedure. But it's actually been shown to be very effective with about 95% of patients. Actually, in five years having no symptoms with MS. This is again a conversation you can have with your patients something long term. It is quite a rare procedure, but it is actually still an option that is also offered in the UK as well. So I've gone to the next slide. Now, we are gonna kind of turn our attention to the neuromuscular junction. So you have your second order neuron and it attaches to whatever muscle, the muscle groups that it, that it's, you know, designed to go to. And when you think about a Neom Muar junction, you have, you know your sodium channel, you know, bringing your, your neuron down, then you have calcium influx, which then releases acetylcholine, er which is your neuromuscular neurotransmitter. And these acetylcholine receptors on the muscle will then respond to this. So where a problem can occur on this when thinking about a neuromuscular disorder is myasthenia gravis. So if you go to the next slide, please, myasthenia gravis is essentially an autoimmune mediated attack of acetyl codeine receptors. So these receptors that sit on the muscle, so you're still releasing acetylcodeine as you should. But but because these um these antibodies are sitting inside and occupying these acetyl codeine receptors, you can't actually get to that acetylcodeine that your neuron is releasing to the muscle. So this can lead to degenerative weakness and long term muscular problems. Uh and it's associated with Thymomas um and other autoimmune disorders because it's an autoimmune mediated problem. Things like pernicious anemia, thyroid, rheumatoid sle are quite common. And as you know, if you have one autoimmune condition, you are actually predisposed to getting other autoimmune conditions. And as for all autoimmune conditions, they are more common in women as well. Uh And this is another one of those cases with myasthenia gravis. Er and essentially this is what's happening here. Um you have on the left image, it's it's perfectly normal acetylcholine is released and it attaches to your acetylcholine receptors on the muscle and the muscle contractions triggered. And in myasthenia gravis, these auto these antibodies are attached to the receptors. And for that reason, acetylcholine can't attach. So if we go on to the next slide, thinking about that, there are certain key features er that we get in myia gravis, that are kind of characteristic trace functions. And one of the main ones is that it affects extraocular muscles. And you can get diplopia with these patients. These patients pain of double vision protecting and looking in certain directions. You get proximal muscle weakness, which is quite an important distinguishment because with something like peripheral neuropathies, you may get distal muscle weakness, which may be ascending a proximal muscle weakness. You know the muscles of your, your face, your neck and your limb girdle tend to be more affected or affected. First in myasthenia gravis, yeps, which is just drooping of your eyelid, dysphasia with difficulty swallowing. And an important characteristic side of my gravis is that the weakness worsens on increased movement and is relieved by rest. And that's because you are releasing the acetylcholine and because there aren't as many receptors you run out of receptors. And so your weakness worsens. But as you rest, the, the reuptake of acetylcholine is, you know, increased and is taken back to your neurons. And so you actually have er more receptors that are available than be um accessed. So these patients will typically tell you that their symptoms are worse throughout the end of the day because they actually uh they are running out of receptors as they carry on using those muscles. So when we're again, looking at uh diagno diagnosing it or investigating um it, we tend to use antibodies, you can see antibodies in blood So you do blood tests, anti acetylcodeine antibodies uh are the most common antibody seen in my gravis. 85 to 9% of cases in those 10% of cases that don't have this antibody anti muscle specific tyrosine kinase antibodies is seen in 40% and can be quite a useful secondary test if you're still suspicious of myosin and gravis. But I haven't, haven't confirmed it with the antibody. Um Creatine kinase can also be normal in these patients as well despite their inability to use their muscles. Um because they're not contracting their muscles, it's also creating time as you'd expect to be normal. But the single most sensitive test is actually a single fiber E MG. Er and it's very effective and actually it's um use if you don't detect Myosin and gravis using this test, it's highly suggestive that the problem itself is not myotonia gravis, then you do a ct thorax to look for Thymomas, which are strongly associated with my, your gravis. And this isn't common, but you can still do it 10 test. And what they do is they administer IV edrophonium and this would temporarily reduce the muscle weakness and the symptoms of myasthenia gravis in patients. But because it's got a lot of risks, particularly cardiovascular risks, they don't use it very much in practice anymore. Uh If we move on to the next slide, when you're managing this, because you have um you know, a reduction in receptors you need to increase the amount of time that acetylcholine can spend in the neuromuscular junction. So you give them long term acetyl um acetylcholinesterase inhibitors, acetylcholinesterase is the enzyme that breaks down acetylcholine in the neuromuscular junction. So, by inhibiting this enzyme, you can increase the amount of acetylcholine that sits in the junction and hopefully, you know, improve the symptoms of myasthenia gravis. Um And if this doesn't work immunosuppressants, of course, this is autoimmune mediated. So it's your immune system, you can give immunosuppressants such as prednisoLONE or azaTHIOprine cycloSPORINE, mycophenolate mofetil are alternatives, but they tend to be the one predniSONE tends to be the more preferred one. And then surgery is thymectomy is strongly related with um myasthenia gravis and can be related with the new um antibodies that are produced in myasthenia gravis. And if we go to the next slide, please, so we also have what's called myasthenic crisis. And essentially, there's just the worsening of your myasthenia gravis to the point where you have respiratory failure that requires intubation and mechanical ventilation. And this is so severe that you actually need to just uh clear out the immune system. So to do this, you need to use plasmapheresis and IV immunoglobulin. And that's what I viv stands for. This, essentially just replaces your immune system to a more effective one and stops this um really bad attack. And of course, you know, supportive care. So, a condition that's closely related to mysia Gravis. Uh also the Neom Muar Jack is Lambert Edon and Myasthenic Syndrome. Or you may also have heard of it as Lambert Eaton Syndrome. And this is essentially a paraneoplastic syndrome, most, most commonly related to small cell lung cancer, but can be related to other cancers such as lymphoma. And it can also occur independently as an autoimmune disorder as well. And essentially all this is is an autoimmune attack of the synaptic voltage gated calcium channels in the per um peripheral nervous system. And if we think back to that image, the voltage gated calcium channels, you need voltage g calcium channel influx in order to release our neurotransmitter er of acetylcholine into the neuromuscular junction. So you're gonna have reduced release of acetylcholine because of this autoimmune attack on these voltage gated channels. And since you um next slide please, this is what it looks like. So this antibody here, although it looks like it's attached to the receptor on the right image. So on the left image is what's normal, you get calcium influx and that calcium influx in er promotes these signals to elite acetylcholine. When you have that antibody attached to that calcium channel, you get reduced calcium influx and so you get reduced acetylcholine release into the space right and you go to the next slide, this again like myasthenia gravis has very similar symptoms that you can follow. So you can get limb girdle weakness. So this tends to be the lower limb first. And it also tends to be proximal weakness, such as in myasthenia gravis. You can get hyperreflexia, autonomic symptoms such as dry mouth impotence and difficulty urinating. You can get ophthalmoplegia and ptosis. But these aren't as common in lambda eaten. Whereas ptosis is quite a characteristic sign of myasthenia gravis. And what's actually very, very different about these two is that the patients with Lambert Eaton syndrome will have increasing strength with repeated muscle contraction. Whereas myasthenia gravis patients will have decreasing strength or at least weakening with repeated muscle contraction. And that's quite an important clinical sign that we can use to differentiate between the two as well as signs that are suggestive of a potential underlying neoplasm in patients with Lambert Eaton syndrome. And if we turn to the next slide, please, and when it comes to investigations again, you wanna differentiate between mice and you gravis, you can repeat those same tests. They'll be on the previous slide. If you wanna look back at them, you do an E MG, which is very important in this patient because you also see incremental responses to repetitive electric stimulation. Again, that tells you that the muscle is actually getting better repetitive stimulation, characteristic sign of Lambda Eaton syndrome. And you also want to look for the cancer because that's very important because to actually get rid of this, the treatment is removing the underlying cancer itself because it is a paraneoplastic problem. So, you know, again, we thinking about management, we want to treat the cancer. We want to use immunosuppressants particularly if it gets too, too. Um, the, the symptoms are getting too, too worse, too difficult to manage. So, prednisoLONE azaTHIOprine in this case are the preferred immunosuppressants. There is a, a trial drug to actually help with the symptoms and it's called 34 diaminopyridine. It's not actually clinically licensed yet, but the trials are looking very good. And you know, for some of you who maybe, you know, in year two or year one, you may actually see this. Uh it's still in, in practice as regularly when for patients who have Lad syndrome. Uh but again, if things are also getting difficult to manage immunosuppressants, IV IG and plasmapheresis can be useful just to give a boost to your immune system temporarily and to kind of relieve those symptoms. Um And this is again, this is just a, a picture taken from patients who were trialed on a 34 diaminopyridine. And you can actually see increased acetyl codeine um release on that third image right at the end with the drug. So it is quite effective. Ok. So now we have looked at problems at new muscular junction with the problems in the central nervous system. The the last real place you can get real problem with a um neuromuscular disorder is the motor neuron itself, which is consistent of your motor neuron and your myelin. Ok. So when we're going to the next slide. The main problem that I think is quite important for you guys uh is um the plantation on this is, you know, varies whatever you hear. But uh guillain-barre syndrome or GBS, um and this is immune mediated demyelination of the peripheral nervous system. And if you think about the name demyelination, you are removing myelin from these patients and so you're slowing down impulses. Um but you can get types of G BSS that can actually attack the axon itself and can stop impulses. Um But the most common type is demyelination. So don't worry too much about it. I'll speak a bit more about types of GBS later on. Uh the most common trigger by far is Campylobacter judging. Uh and this is a enteric bacterium that can cause infectious diarrhea, uh particularly from raw poultry or raw chicken. Um So you have to be very careful. Patients will typically give you a history of, you know, eating out or they had undercooked or raw chicken somewhere and then they had diarrhea, usually nonbloody, usually self limiting, but, you know, 12 weeks later, they suddenly presented with this week in their arm, um and legs. So that's quite a characteristic side can also be mycoplasma pneumonia, a form of atypical pneumonia. So these patients can also present with chest symptoms. So you're looking for a previous infection because it's an immune mediated problem. Uh Other viruses such as CMV, EBV and zika are actually common causes of GBS as well. But when you're thinking about the most common cause Campylobacter quite easily will be quite, it'll be quite obvious in the history or history taking in the patient. Um And if we go to essentially the, the reason it happens is because there's a cross reaction with ganglioside antibodies which are very similar to the antibodies uh used to attack Campylobacter. Right? So when you're thinking of key features, we have back and leg pain, uh which is only in about 65% of patients, we have progressive symmetrical limb weakness of all the limbs and this is classically ascending. So it starts off in your legs and then as your legs starts to get weaker, it rises up your legs and then can spread into your arms. And there's a characteristic sign of GBS as well. Um You can have reduced or absent reflexes because it's attacking your second order neuron. So these are going to be lower motor neuron signs that you're producing. Um And then you may have some mild or sensory signs such as distal paresthesia as well. Uh go to the next slide. Um There are certain other features of GBS, you know, again, it's a progressive condition that can cause a whole lot of symptoms. So you don't really need to know what you need to be able to do is recognize it in either a question or in a patient. It again, that history of gastroenteritis, you have muscle weakness, you can have cranial involvement. So, diplopia, bilateral, facial nerve palsy, uh oropharyngeal weakness. You have autonomic problems. Again. Um urinary retention, diarrhea, and papilledema as well because you also tend to find reduced CSF resorption. Uh and that can lead to raised intracranial pressure as well. Uh And when we come to diagnosing it, can we go to the next slide, please? Um Oh, this is actually quite a common variant. Uh Sorry. Yes, this is quite a common variant of GBS. It's called Miller Fisher syndrome. And it classically presents with the triad of ophthalmoplegia, a reflex and ataxia. But you also have your broad GBS symptoms as well. And they do quite, they do like asking a lot of questions about that as well, particularly in our progress test. So do watch out for that one and watch out for that triad. Uh And you're still gonna have a similar history uh in this patient as well. Uh And it also presents with descending paralysis rather than ascending. So this will be weakness that starts in your shoulders and then spreads down again. These are the different types of GBS, the far left one is our classic sensory motor problem. And then you can also have pure motor para um paraparetic. So that's what I mean. There's a lot of different types of GBS don't get too bogged down on the different types. The two main ones you really need to know about are your classic sensory motor and your Miller Fisher syndrome. They're the most commonly asked about ones. So don't worry too much about all the other types of GBS that you can get. Er, and of go to the next slide when investigating GBS, again, there's anti gangliocyte antibodies in your blood. The most useful anti GM one is available in like 25% of patients but there are other types of anti gangliocyte antibodies. Um And when you're looking at your Miller Fisher variant, anti GQ one B is quite common. Uh Again, there's no, you know, fancy eye remembering it, you just have to kind of know it. Lumbar puncture will reduce raise protein, a normal white cell, there's raised protein because you have inflammatory attack on your peripheral nervous system. So you increase the protein but there's no actual infection. So your white cell count should remain relatively normal. Uh And then you also do nerve conduction studies and you'll see a decrease in motor nerve conduction velocity. Again, bear in mind you have demyelination. So you're slowing down the conduction of more of the motor nerve. So that's quite used for an effective test. Um And then you can have all these other signs you don't really need to know about. But GBS um when it comes to treating GBS, go to the next slide, please. Um Con conservative supportive care. First line treatment is IV immunoglobulin. You can use plasmapheresis as Well, so again, just boosting up our immune system, er, and ensuring that your cells can, will stop attacking you. Bear in mind that I viv only reduces the severity of uh GBS. It doesn't actually treat it. So it reduces the severity but won't reduce the length of time that you suffer from GBS. But most people recover in about 6 to 12 months. Again, this does vary in patient to patient. Some people recover quicker, some people recover longer. Some people never recover. Um So that's something too important important to remember and there's no surgical treatments for this. So we go to the next slide. Finally, we can get to our SBA S. So uh what I'll do, I'll just read them out and I'll give you guys a second to answer. So a 30 year old female came into the emergency department presented with new onset double vision fatigue and arm weakness. She noticed her weakness gets worse, particularly towards the end of the day and that she's been feeling more short of breath after minimal exercise. Recently on examination, you noticed that she had di diplopia. Uh what's the most sensitive test uh for the single, most likely diagnosis in this patient? OK. Yep, we've skipped the next question. Um So the answer is single fiber electromyogram. Bear in mind it's 92 to 100% effective. Some people do fall into the trap of going anti acey codeine antibody because that's probably the one you may have heard of protected, you know, coming through medical school, but the most sensitive test is single fiber electromyogram. Er, and again, do remember that if you can't find er signs of Myia gravis on a single fiber electromyogram, the chances are it's not myasthenia gravis. Um Can we go to the next slide, please? And that's just an explanation of what's going on there and the next slide. So next uh again, I'll read that out for you. The 39 year old female was admitted to the neuro Neurology ward following an episode of severe eye pain and neurological deficit. She describes the previous episode six months earlier, which was slightly similar but less severe. After several tests were performed, a diagnosis of multiple sclerosis was confirmed. The patient was concerned about what this will mean for her future and how this will progress. What's the best description for the most common type of progression seen in MS? OK, a few more seconds and then we'll get the answer. Oh, never mind. Um So here we have unpredictable attacks which may or may not leave permanent damage. So do you remember the most common type? We go to the next slide, please. The most common type is relapsing, remitting. Also, uh something to note down is that relapsing, remitting MS doesn't always leave damage so you actually can return back to the same baseline, but you tend to deteriorate step wise as you progress through time. So yes, that was the answer for that one. And for the last question, a so that's a bit of a long stem, 40 year old male, 42 year old male came into the neurology outpatient department presenting with frequent falls, inability to swallow properly for the last three days. And double vision with blurring patient had noticed the force prior to his visual symptoms, he had bilateral ptosis, abnormal lateral gaze in both eyes and a weak hand grip on asking him to walk. Oh, on asking him to walk in a straight line, he failed to do so. Uh On further examination, you could elicit no reflexes and found some distal limb paresthesia. He has no past medical history other than a recent episode of gastroenteritis which resolved three days ago. Uh it was resolved in three days, two weeks prior, given the findings was the single most likely diagnosis. Let me know when you want me to put the answer. OK. No. So you're gonna just go back through those examination findings and yeah, make your interpretation of what you think might be going on. OK. Um Get the answer, please, Maureen. So this is actually a case of Miller Fisher syndrome. Uh And if we go to the next slide to explain why. So this patient again had a history of gastroenteritis. Uh you know, a few weeks prior suggestive of GBS bear in mind, there are different types of GBS as well. Uh So the patient presented with abnormal lateral gaze in both eyes, which also had diplo bum no reflexes and difficulty walking. This is consistent with a triad of a variant of GBS Miller Fisher syndrome where you have ophthalmoplegia, a reflex and ataxia. So do watch out for that trap. They do sometimes like to put down questions. But yeah, all done. And that's me done for this session. Yeah. Thank you very much. That was a really good uh really good presentation. Good questions. We're just gonna switch over uh to cranial nerve defects. Now I'm gonna invite Chris as a speaker. Uh Let's see. Just gonna have a changeover of speakers right now. Oh, sorry. Uh uh Sharing my screen uh my colonoscopy. I can uh II can do the uh slide if you want Chris. Uh Can you do this slice? I can do the ones. OK. Yeah, that would be great. Can you see it there? Yeah, I can see the size. Yeah, great. Thank you. So I'm gonna be cover, I'm gonna be co covering a cranial nerve uh revision and any kind of pathologies along with it. Um The first two slides are just for a small revision if um to understand the function of cranial nerves. Uh as you guys probably know of the first one is olfactory, which one which is responsible for the smell. Second one is uh optic which is responsible for vision. And then the third one is oculomotor which is responsible for eye movement, pupil constriction, eye opening ation. And then the first one is also for eye movement, clear. The first one is facial, so it's responsible for facial sensation, muscle mastication. And then the sixth one is abducence which is also a responsible eye movement. Explain it please. Thank you. Uh And the seventh one is um oh sorry. The fifth one is tral, I'm sorry. Uh Seventh one is facial and is responsible for muscle, facial expression. Eighth one is uh bibio cochlear which is responsible for sharing your balance. Ninth one is Gonal, it's responsible for swelling, salivation and taste sensation. 10th 1 is also responsible 10 taste sensation. Uh It's also responsible for uh to cough reflex as well. 11th 1 is accessory which is responsible for uh sternocleidomastoid and um uh so turning your head and with and trapezia as well. Uh The 12th 1 is to it responsible for tongue. Yeah, side please. So this is the more of the important bit and I'll be covering the 3rd, 4th and 6th nerve palsy. So there is no palsy, which is all uh motor neuro palsy. It's commonly caused by uh posterior communicating artery aneurysm or it can also be caused by cavernous sinus, any cavernous sinus problems such as infection, tumor infiltration by like pituitary, adenoma or thrombosis. The 4th and 6th are rare. Uh more rare and less common. Uh They can be caused by diabetes, mellitus or by trauma to the eye. And the common symp uh signs that you see for omo or palsy is that they are looking down and out. They usually have double vision uh ptosis, which is uh dripping on the eyelid and proptosis, which is popping out of the eye and fixed and dilated pupils. Uh The pupil involvement is more commonly seen in what's called a surgical uh uh OK. Motor neurop palsy rather than medical. OK. Motor neuro palsy. But um so like for tumors, tumor compression and um for uh damage to the OK, with the nerve, false nerve, uh false nerve palsy would present with hypermetra, which is uh when your eye is looking up and out and double vision is usually worse on vertical plane. So that's usually a total sign of false ner palsy and six nerve palsy. Your eye usually looks uh inwards. So they're adopted a le rest and the, the double vision is also usually worse on a horizontal plane as well. Next slide, please. So another important um uh I think the cover for eye um would be internuclear, ophthalmoplegia. This is not necessary cranial nerve pathology, but it's important to know and it's com it's good to be able to determine if it's ne uh cranial uh oculomotor tro abducent nerve palsy versus in internuclear oia, it's usually caused by a lesion and uh M LF which is median longitudinal fasciculus, which is responsible for controlling the horizontal gaze. It's usually caused by uh MS or some kind of cerebral vascular accident. You will present with uh impaired abduction during the uh horizontal gaze. And you can also present with nystagmus on the abducting eye and they can uh reai they can still retain the ability to s uh see nearby target. This can be on one side or on both sides. Uh So the imaging in this case would be MRI if there is a some kind of um brainstem lesion and CSF or O LP, if you suspect um uh MS and UCO clue bands in LP, um management is dependent on what the cause is identified next slide, please. So a question is 80 year old. Uh The first question is eight year old male presents to um emergency department with one hour history of sudden onset double vision. Uh after he hit himself against the lamppost on physical examination, he reports a double vision which is worse when looking down and gets better when tilting uh his head to the left side. And there is some bruise around his eyes, which of the following is the most accurate critical diagnosis. We'll give you guys 30 seconds to choose an answer. So the answer for uh for the first question is um a which was right, sorry. Uh Going back to slide, I think it was right. Uh Was it uh trochlear nerve palsy, I think? Um Yeah. R right. Trocar and palsy. And the reason for that is that um both, both C ND were present with. Um OK, no more palsy would present with headache, photophobia, diplopia and would typically have C NS uh CN S3, pre palsy presentation. Um E would present with painful eye and pitting. The eye means ocular trauma, which is likely and because it gets better when you're looking at the left side is most likely a right-sided lesion rather than left side. So the answer is right-sided uh totally there pal next side, please. So the next question is 42 year old female presents with a background of multiple sclerosis and intends uh the Neurology Clinic for followup on physical examination uh when performing the age test and looking to her right. Uh The patient is unable to abduct her left eye and nystagmus is noted in the right eye on abduction, abduction, sorry. And uh which best option, this describes the abnormality. Let's go to the next slide. So A and B um left fa is left uh frontal eye field that would present with patient looking towards the left side, towards the side of the lesion. And then B which is uh uh uh pontine reticular formation. I think the region on that would result in patient looking away from the uh from the side of the lesion. Um and essentially the symptoms and signs much of much of was internuclear ophthalmoplegia. So it's either the right or left and you can uh uh you can determine that. And um that because the patient is numbers uh has an ability to adopt her left eye and the SAGS is on the right eye. You can ad dot uh you can deduct that. It's most likely d that's correct expat. Yeah. So the next uh pathology I will be covering is fragile neuralgia. It's a chronic pain condition. Uh You can see it in uh more in the female population and over the age of 50 it can be either idiopathic or secondary to things like malignancy, ventricule, uh uh A BM SMS, sarcoidosis Lyme disease. Uh It's usually a unilateral facial pain, uh described as shooting, stabbing, debilitating is usually triggered by a light touch, uh eating wind, bro, blowing into your face. Next page for me. All right. Thank you. Uh So it's usually a clinical diagnosis. But if you want to exclude any A BMS or malignancy, you can use newer imaging. So the management is usually a uh for medical management. It's usually a um oh, previous life. Sure. It's usually a epileptic um drugs uh like carBAMazepine, which is the first line. And if that doesn't work, you can use phenytoin and lamoTRIgine and surgical would be if they, if these medical uh management doesn't work and the patient, um patients still get severe symptoms, then they would be put on either a microvascular decompression when they change the location of blood vessel. So it doesn't irritate the trigeminal nerve or they can inject alcohol or they can remove the cause, which is either a tumor or a VM. There's also other rarer types of neuralgia like gross neuralgia or hospital neuralgia, but it's not as important. So, and we can move on uh next slide, please. So the next one is facial nerve palsy. Uh it's caused by dysfunction of the facial nerve. It can be, it's most, mostly unilateral, but it can be s uh bilateral as well. It's usually caused by a, some kind of CS involvement. So, either a stroke which is causing a lesion in the motor cortex or area connecting the motor cortex to the uh facial nerve nuclei. It can be caused by acoustic neuroma or otherwise called a vestibular senoma. It can also be caused by trauma or cystic systemic infection or inflammation like dot Lyme's disease or sarcoidosis. It can also be caused by Lanze Hunt syndrome, uh which is caused by uh vasa zoster virus and uh more than your rights multiplex as well. So, presenting complaint would be uh unilateral um or sometimes bilateral facial weakness or paralysis, difficulty closing the eye hyper ees because of um because it allows for dampening of the sounds, uh altered sense of taste because it supplies the uh uh an two third with the tongue, uh decreased sensi uh salivation and lacrimation because it surprises uh submandibular and um and other uh um uh sorry glands, uh loss of nasal labial fo folds which is the fold around your um face exci please. So there's also Bell's palsy, which is idiopathic uh facial norm known palsy. The causes are not very well known and uh they hypothesized that it's most likely caused by HSV one reactivation. It's often acute and unilateral, it affects the f forehead but not as uh doesn't affect the muscle mastication or extra ocular muscles. It can also cause uh otalgia as well. Um And it, it pre pretty much displays similar symptoms to a normal uh some uh facial neuron palsy. Next slide, please. Uh So the investigation is mainly clinical and um the management, it would be dependent on what the diagnosis is. So, for things like uh Ro Hunt syndrome, you would use a antiviral agent like Aciclovir for anti uh for Lyme's disease, you would probably prescribe a antibiotics like doxycycline and for acoustic neuromas or trauma, you would give a surgical intervention and you could uh offer them rehabilitation to restore facial muscle mu function as well. And um for Bell's Palsy under the guideline, you give corticosteroid, um 50 mg, prednisoLONE OD for 10 days and plus minus a uh acyclovir next slide, please. So, um, so next, we're gonna cover ischemic stroke, affecting cranial nerves. Um So there are like around three strokes that affects uh cranial nerve nuclei. So, the first one would be basilar artery stroke, uh which can affect the um uh OK, uh cranial nerve nuclei and it can cause lots of horizontal eye movements. Um The next one would be lateral pontine syndrome, uh where they affect the um 7th and 8th nerve nuclei. So they can present with pause of the face, altered taste sensation, decrease salivation and acclimation. So the last one is Willen Perry Syndrome or otherwise called a lateral majority syndrome. They can affect the nucleus and which is connected to the cranial nerve, um nuclear 10th and 11th and 12th. So they can present with dysphagia, hoarseness of voice, absent, gag reflex. Uh Next slide, please. Uh This is a bit more niche but uh I think it would be good to know. There's a bul of palsy and pseudo of P palsy, which is um one of those things that can affect um your 10th, 11th and 12th, uh near New Guy. Uh There's characteristic symptoms seen in bell palsy. It's a lower motor neurone lesion affecting the cranial nerve or cranial nerve nuclei. It's usually caused by brainstem stroke or some tumors or a LS autoimmune disease like GBS as well. They usually present with absent g reflex, uh wasted, wasted or fuss. They can present with absent jerk reflex. Um They essentially present with uh no uh with a lower motor neuron sign. Whereas pseudo bubble palsy, they are upper motor neuron lesion affecting the what's called the corticobulbar tract running from the motor cortex to the cranial neuron. It can cause by virus. Things like a bilateral internal capsule, uh capsule stroke, or they can also be caused by degenerative causes like M ND, uh progressive sub nuclear palsy. They can also be affected by autoimmune disease such as MS. They have the opposite, almost the opposite signs. Uh as the bulbo palsy, they have increased gag reflex, increased jo jerk reflex. Uh They have a spastic dys ostria which is uh when they uh have difficulty talking. Um They also interestingly present with emotional lability. So they're not express they're not. Um So their family might notice that they, they have change in personality or they're not acting normal next slide piece. So these are the last two SBA S um A 71 year old man presents to his GP uh with ringing in his ears upon asking. Uh He mentions that he has experienced similar episodes before and also has um felt as though his world is spinning on examination. He has left sided facial droop, impaired hearing on the left side and otoscopy uh reveals a Visar rash on his a auditory canal. What is the most likely diagnosis next slide, please? So, because the patient presents with tinnitus vertigo, facial palsy, herpetic rash, uh on their ears and some hearing loss on the examination. Uh So uh can you go back to your previous life? So Bell's palsy would just present with uh facial uh facial droop. Uh it wouldn't cause any kind of uh hearing loss. So, a is not likely orthodoxy would cause a hearing loss but wouldn't cause facial droop, meniere's disease. Uh would cause uh tinnitus hearing loss as well as a um uh oral fullness, but it's not consistent with what's described here. So, no, d um he would present with hearing loss, uh vertigo, he uh as well as um as well as uh um sorry, as well as um hearing loss of sorry, hearing loss, vertigo. And uh they can also uh describe tinnitus as well. But in this case, it's not very likely because the patient has herpetic rash, which is more consistent with uh Ramsay Han syndrome. So e is the correct answer. Next question. Sorry. So the last question is 26 year old female presents is referred to the neurology clinic with a two week history of dysphagia, difficulty speaking, emotional ability. A few months ago, she has a self-limiting episode of phlegm weakness and urinary incontinence. On physical examination. There is slurred, speech, spastic tongue and brisk jaw reflex. Which of the following is a pathophysiological mechanism best which best explains the underlying cause ever. So, the answer for this would be c the reason is that um this is um yeah, uh if you could go back, sorry. Uh If uh so the reason why it's the case is because uh this is a common presentation of pseudobubo palsy. And so B is more myasthenia gravis. So B is not the option D is a, wouldn't present with this kind of symptoms. So this is not the case. Um E is Wallenberg uh syndrome, I think. So. Uh this is not also consistent with what's presented here. So it's either a uh A or C and um and because this patient has had um other, other previous episodes as well, it's most likely a caused by MS. And so it's most likely c it's a demyelination on cortical bulbar uh tract which is causing uh pseudobulbar palsy. And yeah, that's if you could go to the slide at the end. Uh So thank you for coming to our session, teaching session today. Um uh I hope you guys fill in the feedback form and uh please follow us on Instagram and Facebook and I hope to see you guys all in the next teaching session. Yeah, thank you to everyone that's going today. Uh If there's any questions just put in the chat, er, or just email us or add us on Instagram, I put the Instagram link and the er and the email at the top of the chat. If you guys want to join Instagram or just email us a question or query, you're more than welcome to. We're gonna stay around for five minutes. 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