Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

Y4 Neurology Revision Tutorial Reka Kovacs 1. Cranial Nerve Disorders ** 2. Headache & Facial Pain Disorders *** 3. Cerebrovascular Diseases *** 4. Neurotrauma ** 5. Epilepsy Disorders *** Session Structure 6. Nutritional & Toxic Disorders ** 7. Peripheral Neuropathies *** 8. Infective/Inflammatory/Autoimmune Disorders **(*) 9. Neuro-degenerative Illnesses/Dementias *** 10. Functional Neurological Disorders ***THINK: WHERE, WHEN, WHY? • WHERE? • MOTOR à UMN, LMN, NMJ, MUSCLE • SENSORY à BRAIN, CORD, NERVE ROOT, NERVE • SYMPATHETIC/PARASYMPATHETIC • WHEN? • INSTANTANEOUS à VASCULAR, EPILEPTIC • MINUTES à MIGRAINE • HOURS/DAYS à INFECTIVE, INFLAMMATORY • YEARS à NEURODEGENERATIVE • WHY? • SURGICAL SIEVE! 1/10 Cranial Nerve Disorders III, VI & VII N Palsies **A 28-year-old man presents to the emergency department, having woken up and noticed the left side of his face was drooping. He had been completely fine the night before. He still has sensation in his face and can blink, swallow and speak as before. He hasn’t noticed any dizziness, weakness elsewhere and has not had any recent infections. He does however note that everything sounds a bit louder. On examination, he cannot lift his left eyebrow, there is obvious weakness on the left side of his mouth, otoscopy reveals a normal ear canal but gross testing of his hearing reveals hyperacusis of the left ear. What is the most likely diagnosis?A 28-year-old man presents to the emergency department, having woken up and noticed the left side of his face was drooping. He had been completely fine the night before. He still has sensation in his face and can blink, swallow and speak as before. He hasn’t noticed any dizziness, weakness elsewhere and has not had any recent infections. He does however note that everything sounds a bit louder. On examination, he cannot lift his left eyebrow, there is obvious weakness on the left side of his mouth, otoscopy reveals a normal ear canal but gross testing of his hearing reveals hyperacusis of the left ear. What is the most likely diagnosis? CNVII palsyCNIII Palsy • CN III is the oculomotor nerve. • Provides innervation to the extraocular muscles and pupillary constrictor muscles. • Efferent limb for the pupillary light reflex. • The muscles are the levator palpebrae superioris, inferior oblique, and superior, medial and inferior recti. CN III palsy causes a ‘down and out’ eye.CNIV Palsy • CN IV is the trochlear nerve. • Provides general somatic efferent to the extraocular superior oblique muscle. It assists in depressing and abducting the eye. • CN IV connects to the midbrain and is the only cranial nerve to leave the pontomesencephalic junction posteriorly. It passes through the superior orbital fissure of the skull. CN IV palsy causes inability to move eye down and out.CNVII Palsy • CN VII is the facial nerve. • It originates in the pontomedullary region, passes through the internal auditory meatus and exits through the stylomastoid foramen. • The facial nerve loops around the abducens nucleus. • Lower motor neurone facial nerve lesions cause upper and lower facial paralysis • Upper motor neurone facial nerve lesions cause lower facial paralysis only 2/10 Headache & Facial Pain Disorders Other Primary Headache Syndromes * Giant Cell Arteritis *A 22-year-old patient attends her GP clinic with a two-year history of headaches. Initially the headaches were each lasting a day and occurring only once a month, but they are now experiencing them every week. The pain is always on their left eye and forehead and they describe it as 9/10 severity with a throbbing character. They experience nausea during most of these episodes, and often has zig-zag lines across their vision for a few minutes prior to the onset of headache. They have no other medical history and their only regular medication is the COCP. What is the diagnosis?A 22-year-old patient attends her GP clinic with a two-year history of headaches. Initially the headaches were each lasting a day and occurring only once a month, but they are now experiencing them every week. The pain is always on their left eye and forehead and they describe it as 9/10 severity with a throbbing character. They experience nausea during most of these episodes, and often has zig-zag lines across their vision for a few minutes prior to the onset of headache. They have no other medical history and their only regular medication is the COCP. What is the diagnosis? Migraine with auraA 22-year-old patient attends her GP clinic with a two-year history of headaches. Initially the headaches were each lasting a day and occurring only once a month, but they are now experiencing them every week. The pain is always on their left eye and forehead and they describe it as 9/10 severity with a throbbing character. They experience nausea during most of these episodes, and often has zig-zag lines across their vision for a few minutes prior to the onset of headache. They have no other medical history and their only regular medication is the COCP. What further investigations are indicated?A 22-year-old patient attends her GP clinic with a two-year history of headaches. Initially the headaches were each lasting a day and occurring only once a month, but they are now experiencing them every week. The pain is always on their left eye and forehead and they describe it as 9/10 severity with a throbbing character. They experience nausea during most of these episodes, and often has zig-zag lines across their vision for a few minutes prior to the onset of headache. They have no other medical history and their only regular medication is the COCP. What further investigations are indicated? NoneMigraine ± aura Incidence: Investigation: • approximately 1 in 7 people globally. • exam.osis based on clinical history & • mbetween ages 25-55., peak incidence • Investigations may include neuroimaging (MRI) to rule out secondary causes if red flags present. Pathophysiology: • No specific diagnostic test exists for • Complex! migraines. • Neurovascular disorder characterized by recurrent, throbbing headaches. • Isystem, cortical spreading depression, & neuroinflammatory processes. • Genetic predisposition, environmental factors, and neurotransmitter imbalances pathophysiology.o migraineMigraine ± aura Management: • Conservative: • Preventive Treatment: • Patient education on triggers, • Lifestyle modifications (e.g., stress headache diary, early recognition of management, regular sleep, attacks, and self-management hydration). techniques. • Prophylactic medications • beta-blockers • antiepileptic drugs e.g. topiramate, • Acute Treatment: sodium valproate • NSAIDs: aspirin, ibuprofen • antidepressants e.g. amitriptyline • Triptans e.g. sumatriptan • Antiemetics e.g. metoclopramide, prochlorperazine, domperidoneT ension Headaches Incidence: Management: • most common type of headache • Both genders are affected equally, peak • Conservative incidence between ages 20-50. • modifications and stress management techniques. Also consider medication- overuse! Pathophysiology: • bilateral, pressing/tightening pain, • Acute Treatment: usually mild-moderate intensity. • Paracetamol, NSAIDs • Stress, poor posture, and lack of sleep can trigger or exacerbate tension • Preventive Treatment: headaches. • Identifying and addressing triggers • CBT, biofeedback, physical therapy for Investigation: chronic tension headaches. • Clinical history & exam sufficient unless • Consideration of prophylactic red flags present e.g. sudden onset, focal medications (e.g., tricyclic neurological symptoms frequent or severe tension headaches.rCluster Headache Incidence: Management: • M:F ~ 2.5:1. • Patient education & counselling on • Peak onset between ages 30-50. recognising early symptoms, trigger • Associated with smoking avoidance, and lifestyle modifications. • Acute Treatment: Pathophysiology: • Triptans (SC or intranasal • severe, unilateral orbital/supraorbital/ sumatriptan) temporal pain with stabbing/burning/ piercing character • rebreather mask) oxygen via non- • activation of the trigeminal-autonomic reflex, hypothalamic dysfunction, and • Preventive Treatment: neurovascular mechanisms. • Verapamil = first-line preventive • May have triggers e.g. alcohol, sleep • Steroids (prednisolone) for short- disturbances, exercise, etc. term prophylaxis during cluster periods. • Lithium, occipital nerve blocks may be considered for refractory cases.T rigeminal Neuralgias Incidence: Management: • Rare, F>M • Patient education & counselling • peak onset typically between ages 50-70. • Acute medical treatment: Pathophysiology: • Anticonvulsant carbamazepine = • sudden, severe, unilateral facial pain first-line therapy for acute pain relief along one or more divisions of the and prevention of future attacks. trigeminal nerve (most commonly the • Baclofen or gabapentin may be used maxillary or mandibular divisions). as adjunctive therapy. • ? due to vascular compression of the trigeminal nerve root, leading to • Various surgical interventions: demyelination and hyperexcitability. • Microvascular decompression • Triggers such as light touch, chewing, or surgery talking can provoke paroxysmal pain. • Stereotactic radiosurgery (e.g., gamma knifeA 68-year-old man presents to his GP with a three-week history of progressively worsening headache, particularly over his left temple. He also reports fatigue, jaw pain exacerbated by chewing, and a low-grade fever. During the appointment, he reports a sudden ‘darkening’ and loss of vision in the L eye, which returns a few minutes later. Which of the following blood investigations is most likely to support the diagnosis? A) Serum electrolytes B) ESR and procalcitonin C) Thyroid function tests D) ESR and CRP E) Serum creatinineA 68-year-old man presents to his GP with a three-week history of progressively worsening headache, particularly over his left temple. He also reports fatigue, jaw pain exacerbated by chewing, and a low-grade fever. During the appointment, he reports a sudden ‘darkening’ and loss of vision in the L eye, which returns a few minutes later. Which of the following blood investigations is most likely to support the diagnosis? A) Serum electrolytes B) ESR and procalcitonin C) Thyroid function tests D) ESR and CRP E) Serum creatinineGiant Cell Arteritis Incidence: Management: • most common primary vasculitis in adults 1. High-dose glucocorticoids (prednisolone) ASAP • predominantly affects individuals over the age of 2. Ophthalmologic assessment 50; peak incidence in 70s to assess for ischemic complications & monitor • Women are affected 2-3 times more frequently for visual changes during Tx. than men. 3. Long-term glucocorticoid therapy: Pathophysiology: • Tapering doses for an extended period (often • Granulomatous inflammation of medium to large- several years), with monitoring sized arteries, particularly involving the temporal 4. Adjunctive therapy: arteries. • Methotrexate or other steroid-sparing agents Investigation: may be used as adjunctive therapy • Diagnosis based on clinical features and lab findings 5. Patient education & counselling (elevated ESR & CRP). • Confirmation often requires temporal artery biopsy. • Imaging studies e.g. ultrasound, magnetic resonance angiography (MRA), or PET can aid diagnosis & evaluation 3/10 Cerebrovascular Diseases TIA & Ischaemic Stroke *** Intracranial Haemorrhage (SAH, Intracerebral haemorrhage) ***A 65-year-old male presents to the emergency department with sudden onset left-sided weakness and facial droop starting 2 hours ago. He is unable to raise his left arm or leg and has difficulty speaking. On examination, there is weakness of the left arm and leg, left facial droop, and dysarthria. There is also decreased sensation on the left side of the body. Based on the patient's symptoms, where is the likely location of the brain lesion? A. Right frontal lobe B. Left parietal lobe C. Right occipital lobe D. Left temporal lobe E. Left frontal lobeA 65-year-old male presents to the emergency department with sudden onset left-sided weakness and facial droop starting 2 hours ago. He is unable to raise his left arm or leg and has difficulty speaking. On examination, there is weakness of the left arm and leg, left facial droop, and dysarthria. There is also decreased sensation on the left side of the body. Based on the patient's symptoms, where is the likely location of the brain lesion? A. Right frontal lobe B. Left parietal lobe C. Right occipital lobe D. Left temporal lobe E. Left frontal lobeA 65-year-old male presents to the emergency department with sudden onset left-sided weakness and facial droop starting 2 hours ago. He is unable to raise his left arm or leg and has difficulty speaking. On examination, there is weakness of the left arm and leg, left facial droop, and dysarthria. There is also decreased sensation on the left side of the body. What is the first line investigation indicated? A. MRI brain B. CT head C. Transcranial Doppler D. MRI spine E. Skull X-rayA 65-year-old male presents to the emergency department with sudden onset left-sided weakness and facial droop starting 2 hours ago. He is unable to raise his left arm or leg and has difficulty speaking. On examination, there is weakness of the left arm and leg, left facial droop, and dysarthria. There is also decreased sensation on the left side of the body. What is the first line investigation indicated? A. MRI brain B. CT head C. Transcranial Doppler D. MRI spine E. Skull X-rayThe CTH comes back à It has now been 3 hours since your patient’s onset of symptoms. What treatment should be initiated first? A. Warfarin B. Clopidogrel C. Nimedipine D. Alteplase E. IVIgThe CTH comes back à It has now been 3 hours since your patient’s onset of symptoms. What treatment should be initiated first? A. Warfarin B. Clopidogrel C. Nimedipine D. Alteplase E. IVIgIschaemic Stroke Most common type of stroke. Pathophysiology: Management: • disruption of blood flow resulting in tissue • Acute Tx: ischemia and infarction. • Causes include thrombotic or embolic • IV thrombolysis with tPA (= alteplase) within occlusion of cerebral arteries, often secondary 4.5 hours of symptom onset to atherosclerosis or cardiac embolism. • Endovascular thrombectomy for large vessel • Ischemic cascade leads to cellular injury, occlusions, if meeting specific criteria. inflammation, and ultimately, neuronal death. • Secondary Prevention: Investigation: • Antiplatelet therapy (aspirin + clopidogrel) • Manage modifiable risk factors: AF, • History & exam, Bamford/Oxford classification* hypertension, diabetes, hyperlipidemia, and smoking cessation. • non-contrast CT = first line imaging • Additional investigations include MRI, vascular • Stroke rehabilitation. imaging (CT angiography, MR angiography) • FBCs, coag, lipid profile, glucose coagulation profile, lipid profile, and glucose * geekymedics.com/stroke-classification/Haemorrhagic stroke – SAH vs ICH Subarachnoid haemorrhage Intracerebral haemorrhage • Incidence peaks between 40-60 years, slight F>M • Incidence peaks in over 65 years • Pathophysiology: Bleeding into subarachnoid • Pathophysiology: Spontaneous rupture of small space. Most commonly due to ruptured cerebral brain arteries or arterioles causing bleeding into aneurysms; less commonly arteriovenous brain tissue. Often due to HTN/ cerebral amyloid malformations, head trauma, bleeding disorders angiopathy, • LP if CT –tive/inconclusive à xanthochromia in • Diagnosis: non-contrast CT shows hyperdense CSF supports diagnosis. areas. • Management: • Management: • Acute: A-E, BP control, intracranial pressure • Acute: A-E, BP control, intracranial pressure monitoring. Nimodipine to prevent cerebral monitoring. vasospasm • Surgical: Evacuation for large hematomas or • Definitive: surgical clipping or endovascular ongoing bleeding; minimally invasive options coiling to secure ruptured aneurysm & available. prevent rebleed.Stroke is a clinical syndrome of presumed vascular origin characterised by rapidly developing signs of focal neurological deficit which lasts longer than 24 hours or leads to death. TIA is a temporary neurological dysfunction (lasting less than 24 hours). 4/10 Neurotrauma Head Injury (including) Post-Concussional Syndrome ** Spinal Injury **A 27 y/o man is brought to the ED after being involved in a high-speed motor vehicle accident. He was an unrestrained driver, and no airbags were deployed. He has multiple areas of abrasions, lacerations, and ecchymosis on his scalp and face. On neurological examination, he does not open his eyes to painful stimuli; he is intubated, and he withdraws his left side to pain. His right side is plegic. His right pupil is 3 mm and reactive to light and his left pupil is 8 mm and non-reactive. What is his GCS? A. 3 B. 15 C. 5 D. 6 E. 7A 27 y/o man is brought to the ED after being involved in a high-speed motor vehicle accident. He was an unrestrained driver, and no airbags were deployed. He has multiple areas of abrasions, lacerations, and ecchymosis on his scalp and face. On neurological examination, he does not open his eyes to painful stimuli; he is intubated, and he withdraws his left side to pain. His right side is plegic. His right pupil is 3 mm and reactive to light and his left pupil is 8 mm and non-reactive. What is his GCS? A. 3 B. 15 C. 5 D. 6 E. 7A 27 y/o man is brought to the ED after being involved in a high-speed motor vehicle accident. He was an unrestrained driver, and no airbags were deployed. He has multiple areas of abrasions, lacerations, and ecchymosis on his scalp and face. On neurological examination, he does not open his eyes to painful stimuli; he is intubated, and he withdraws his left side to pain. His right side is plegic. His right pupil is 3 mm and reactive to light and his left pupil is 8 mm and non-reactive. Which blood vessel is most likely to be injured? A. Middle meningeal artery B. Posterior cerebellar artery C. Superior sagittal sinus D. Internal carotid artery E. Cavernous sinusA 27 y/o man is brought to the ED after being involved in a high-speed motor vehicle accident. He was an unrestrained driver, and no airbags were deployed. He has multiple areas of abrasions, lacerations, and ecchymosis on his scalp and face. On neurological examination, he does not open his eyes to painful stimuli; he is intubated, and he withdraws his left side to pain. His right side is plegic. His right pupil is 3 mm and reactive to light and his left pupil is 8 mm and non-reactive. Which blood vessel is most likely to be injured? A. Middle meningeal artery B. Posterior cerebellar artery C. Superior sagittal sinus D. Internal carotid artery E. Cavernous sinusExtradural vs subdural haematomas • Extradural almost always arterial, classically due to injury of the middle meningeal artery • Subdural almost always venous due to tearing of subdural cortical bridging veins which extend to the dural sinuses.Extradural vs subdural haematomas Extradural haematoma Subdural haematoma • Typically young patient involved in a head • Typically elderly or young children strike during sport or motor vehicle accident • Due to falls, usually no clear history of trauma. • Patient may lose consciousness • In young children associated with non- transiently but regain a normal level of accidental injury consciousness (lucid interval); usually have an ongoing and often severe • Patient's level of consciousness gradually headache decreases with increasing mass effect • Over the next few hours, they gradually • Confusion is often seen in older patients. lose consciousness.Which structure is the green arrow pointing at? A. Pyramidal tract B. Cuneate fasciculus C. Spinocerebellar tract D. Lateral spinothalamic tract E. Rubrospinal tractWhich structure is the green arrow pointing at? A. Pyramidal tract B. Cuneate fasciculus C. Spinocerebellar tract D. Lateral spinothalamic tract E. Rubrospinal tractThese symptoms would suggest what type of lesion? A. Occlusion of the right anterior cerebral artery B. Right-sided hemisection of the spinal cord C. Peripheral neuropathy D. Parkinson's disease E. Complete transection of the spinal cordThese symptoms would suggest what type of lesion? A. Occlusion of the right anterior cerebral artery B. Right-sided hemisection of the spinal cord C. Peripheral neuropathy D. Parkinson's disease E. Complete transection of the spinal cord i.e. Brown-Sequard SyndromeWhich of the following clinical findings is most suggestive of lumbar radiculopathy compared to other differential diagnoses? A. Bilateral lower extremity weakness B. Sensory loss in a dermatomal distribution C. Pain aggravated by coughing or sneezing D. Absence of deep tendon reflexes E. Presence of saddle anaesthesiaWhich of the following clinical findings is most suggestive of lumbar radiculopathy compared to other differential diagnoses? A. Bilateral lower extremity weakness B. Sensory loss in a dermatomal distribution C. Pain aggravated by coughing or sneezing D. Absence of deep tendon reflexes E. Presence of saddle anaesthesia 5/10 Epilepsy Disorders Generalised & partial seizure forms of Epilepsy *** Status Epilepticus *A 6 y/o child presents at GP with repeated episodes where they become unresponsive and unaware of their surroundings. They have also been having memory issues, according to their parents and teachers. What is the most likely diagnosis? A. ADHD B. Focal Epilepsy C. Generalised Epilepsy D. Febrile seizures E. Vasovagal syncopeA 6 y/o child presents at GP with repeated episodes where they become unresponsive and unaware of their surroundings. They have also been having memory issues, according to their parents and teachers. What is the most likely diagnosis? A. ADHD B. Focal Epilepsy C. Generalised Epilepsy D. Febrile seizures E. Vasovagal syncopeSeizures vs Epilepsy Seizure: • specific focus spreading throughout the brain.inating from a • Most commonly manifests with motor or sensory symptoms and is often (but not always) associated with reduced consciousness levels. Epilepsy: • A disease of the brain where at least 1 of the following is tru1) At least two unprovoked (or reflex) seizures occurring >24 h apart 2) one unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years 3) diagnosis of an epilepsy syndrome.Epilepsy: Generalised vs partial seizures Focal/Partial Seizure Generalised seizure Can be aware or have impaired awareness (simple Vs Affect both cerebral hemispheres complex partial seizure) Originate from a specific focus in the brain. May progress to Motor onset: contralateral hemisphere (secondary generalisation) Tonic-clonic, myoclonic, atonic, Motor onset: Nonmotor onset: Automatisms, atonic, clonic, epileptic spasms, hyperkinetic, Absence myoclonic, tonic Nonmotor onset: Autonomic, Behaviour arrest, Cognitive (either negative features (e.g. impaired language), or positive features such as déjà vu, hallucinations, illusions, or perceptual distortions) Emotional (anxiety, fear, joy, or change in affect without subjective emotions), SensoryEpilepsy Ix & Tx Approach & Investigation: Management: • Don’t forget history (ideally a collateral)!! • Address triggers/lifestyle first! • Exclude potential causes: • Sodium valproate = first-line monotherapy for • Basic Obs, BM, FBC, U&E, Bone profile, generalised tonic-clonic seizures in: VBG, CT head, 12-lead ECG • males • girls aged under 10 years • Analyse seizure • females who are unable to have children. • Patient videos, electroencephalogram • Lamotrigine or levetiracetam = first-line (EEG), Video-telemetry monotherapy for generalised tonic-clonic seizures in: • Females able to have children • as a second line in above groups.Status Epilepticus Definition • ≥5 minutes of continuous seizure activity, or • ≥2 seizures without complete neurological recovery in between. • Refractory status epilepticus = continued seizures despite use of 2 anti-epileptic drugs (AEDs), one of which is a benzodiazepine. Triggers for seizures in patient with epilepsy: • Drug withdrawal, dose change or non-compliance • Intercurrent illness • Metabolic derangement e.g. hypoglycaemia • Drugs that lower seizure threshold e.g. tricyclic antidepressants, tramadol, some antibioticsStatus Epilepticus = medical emergency 6/10 Nutritional & Toxic Disorders Effects of Alcohol/drugs on the nervous system ** Vitamin Deficiencies and the Nervous System *failure. He has been responding well to diuresis. At home he regularly drank 60 units of alcohol a week. He suddenly becomes confused, with nystagmus and poor coordination. What treatment should be administered immediately? A. Thiamine C. Fluoxetine D. Calcitriol E. Furosemide F. IV fluidsfailure. He has been responding well to diuresis. At home he regularly drank 60 units of alcohol a week. He suddenly becomes confused, with nystagmus and poor coordination. What treatment should be administered immediately? A. Thiamine C. Fluoxetine D. Calcitriol E. Furosemide F. IV fluids Wernicke’s encephalopathyVitamin deficiencies causing neuropathy 7/10 Peripheral Neuropathies Common Mononeuropathies (including Carpal Tunnel syndrome) *** Polyneuropathy **Which structure is compressed in Carpal Tunnel Syndrome? A. Median Nerve B. Radial Nerve C. Axillary Nerve D. Lateral Cord E. Ulnar NerveWhich structure is compressed in Carpal Tunnel Syndrome? A. Median Nerve B. Radial Nerve C. Axillary Nerve D. Lateral Cord E. Ulnar NerveWhich of the following is a characteristic clinical finding of peroneal nerve palsy? A. Weakness of plantar flexion B. Sensory loss over the medial aspect of the thigh C. Difficulty in dorsiflexion and eversion of the foot D. Pain radiating down the posterior thigh to the calf E. Loss of sensation over the lateral aspect of the foot and dorsum of the toesWhich of the following is a characteristic clinical finding of peroneal nerve palsy? A. Weakness of plantar flexion B. Sensory loss over the medial aspect of the thigh C. Difficulty in dorsiflexion and eversion of the foot D. Pain radiating down the posterior thigh to the calf E. Loss of sensation over the lateral aspect of the foot and dorsum of the toesDiabetic Polyneuropathy • Affects up to 50% of diabetic patients. • Hyperglycaemia leads to microvascular changes, oxidative stress, nerve damage. • Investigation: • Clinical assessment: Sensory loss, neuropathic pain, motor weakness. • Neurological examination: Vibration, light touch, temperature sensation, ankle reflexes. • Electrophysiological studies: Nerve conduction studies, electromyography. • Blood tests: HbA1c, renal function, vitamin B12. • Management: • Glycaemic control. • Pain management: Tricyclic antidepressants, gabapentinoids, topical agents. • Symptomatic treatment: Orthotic devices, physical therapy, foot care. • Cardiovascular risk management. • Regular follow up & monitoring 8/10 Infective/ Inflammatory/ Autoimmune Disorders MS *** Infection Other disorders *A 20 –year-old student is brought to A&E by a flatmate with a one-day history of flu-like symptoms with photophobia, neck stiffness, and headache with a GCS of 13. On examination he has a temp of 39.2℃ and HR 124bpm, Kernig’s sign positive with no other focal neurological signs. PEARL.You suspect meningitis, so you do a lumbar puncture, and send the CSF off for tests. The results: • Fluid appears cloudy • CSF glucose 2.0 mmol/L.20-0.45) • Serum glucose 5.2 mmol/L • WCC 95% neutrophils, 5% lymphocytes What is the most likely causative organism? A.Bacterial meningitis, in particular Neisseria meningitides B.Bacterial meningitis, in particular Staphylococcus aureus C.Viral meningitis, in particular enterovirus E.Viral meningitis, in particular herpes virusYou suspect meningitis, so you do a lumbar puncture, and send the CSF off for tests. The results: • Fluid appears cloudy • CSF glucose 2.0 mmol/L.20-0.45) • Serum glucose 5.2 mmol/L • WCC 95% neutrophils, 5% lymphocytes What is the most likely causative organism? A.Bacterial meningitis, in particular Neisseria meningitides B.Bacterial meningitis, in particular Staphylococcus aureus C.Viral meningitis, in particular enterovirus E.Viral meningitis, in particular herpes virusA 35-year-old female patient is experiencing progressive lower limb spasticity (with a wide-based gait) along with an increasing sense of incoordination and urinary urgency. You learn that two years ago the patient had an episode of temporary blindness in one eye, which her GP had diagnosed as an optic neuritis. What is the most likely diagnosis?A 35-year-old female patient is experiencing progressive lower limb spasticity (with a wide-based gait) along with an increasing sense of incoordination and urinary urgency. You learn that two years ago the patient had an episode of temporary blindness in one eye, which her GP had diagnosed as an optic neuritis. What is the most likely diagnosis? Multiple sclerosisMultiple Sclerosis • Peak onset typically aged 20 to 40 years, F>M • immune-mediated inflammation, demyelination, and neurodegeneration in the brain and spinal cord. • Lesions are disseminated in time and space • MRI à McDonald criteria • LP à CSF-specific oligoclonal bands • Management: • Corticosteroids = first-line treatment for acute relapses e.g. high dose methylprednisolone • Beta-interferons = first-line disease- modifying therapies (DMTs) for relapsing- remitting MS. • Emerging immunotherapies e.g. monoclonal antibodies targeting CD20 = ocrelizumabA 15-year-old male presents to the emergency department with progressive weakness and tingling sensations in his lower limbs. He reports that these symptoms started a week ago and have gradually worsened, now affecting his arms and face. On examination, he is found to have bilateral lower limb weakness, diminished reflexes, and ascending paralysis. He denies any significant medical history or recent illness. What is the most appropriate initial management for this patient? A. High-dose corticosteroids B. Intravenous immunoglobulin (IVIg) C. Plasma exchange (plasmapheresis) D. Empiric antibiotics for suspected infection E. Supportive care and close monitoringA 15-year-old male presents to the emergency department with progressive weakness and tingling sensations in his lower limbs. He reports that these symptoms started a week ago and have gradually worsened, now affecting his arms and face. On examination, he is found to have bilateral lower limb weakness, diminished reflexes, and ascending paralysis. He denies any significant medical history or recent illness. What is the most appropriate initial management for this patient? A. High-dose corticosteroids B. Intravenous immunoglobulin (IVIg) C. Plasma exchange (plasmapheresis) D. Empiric antibiotics for suspected infection E. Supportive care and close monitoringGuillain-Barré syndrome Incidence: . • Rare; may vary by geographic region and seasonality, with Management: jejunised risk following infections such as Campylobacter • Supportive Care: • Close monitoring for respiratory and Pathophysiology: autonomic dysfunction, with early • immune-mediated demyelination or axonal injury of initiation of mechanical ventilation if peripheral nerves, resulting in ascending symmetric muscle required. weakness and paralysis. • Molecular mimicry between microbial antigens and • Immunomodulatory Therapy: peripheral nerve components triggers an aberrant immune response, leading to nerve damage. • Ifirst-line Tx to accelerate recovery and Investigation: reduce disease severity. • Dprogressive weakness, areflexia, and sensoryng rapidly abnormalities. • Rehabilitation: • optimise functional recovery and prevent • Ccell counts may show elevated protein levels with normal complications e.g. contractures, pressure ulcers • Electrophysiological studies e.g. nerve conduction studies subtype can help confirm the diagnosis and classify 9/10 Neuro-degenerative Illnesses/Dementias Idiopathic Parkinson’s Disease *** Vascular Dementia **** Other Neuro-degenerative Illnesses/Dementias *Which of the following best describes a key pathological hallmark of Alzheimer's disease? A. Amyloid plaques composed primarily of tau protein aggregates. B. Neurofibrillary tangles formed by the accumulation of beta-amyloid protein. C. Disruption of the blood-brain barrier leading to neuroinflammation. D. Degeneration of cholinergic neurons in the basal forebrain. E. Accumulation of beta-amyloid protein and tau protein hyperphosphorylation.Which of the following best describes a key pathological hallmark of Alzheimer's disease? A. Amyloid plaques composed primarily of tau protein aggregates. B. Neurofibrillary tangles formed by the accumulation of beta-amyloid protein. C. Disruption of the blood-brain barrier leading to neuroinflammation. D. Degeneration of cholinergic neurons in the basal forebrain. E. Accumulation of beta-amyloid protein and tau protein hyperphosphorylation.Which of the following best describes a characteristic feature of Huntington's disease? A. Loss of dopaminergic neurons in the substantia nigra. B. Accumulation of Lewy bodies in the brainstem. C. Expansion of CAG trinucleotide repeats in the HTT gene. D. Deficiency of the enzyme lysosomal alpha- galactosidase A. E. Formation of Pick bodies in the cerebral cortex.Which of the following best describes a characteristic feature of Huntington's disease? A. Loss of dopaminergic neurons in the substantia nigra. B. Accumulation of Lewy bodies in the brainstem. C. Expansion of CAG trinucleotide repeats in the HTT gene. D. Deficiency of the enzyme lysosomal alpha- galactosidase A. E. Formation of Pick bodies in the cerebral cortex.A 76-year-old R-handed man is referred to the Neurology clinic by his GP. His primary concern is progressive worsening of his mobility over the course of the last 12 months. On further questioning he describes getting increasingly slow, and difficulty getting his balance. He also has a tremor in his right hand, and his handwriting has gotten smaller. What is the most likely diagnosis?A 76-year-old R-handed man is referred to the Neurology clinic by his GP. His primary concern is progressive worsening of his mobility over the course of the last 12 months. On further questioning he describes getting increasingly slow, and difficulty getting his balance. He also has a tremor in his right hand, and his handwriting has gotten smaller. What is the most likely diagnosis? Parkinson’s DiseaseIdiopathic PD Bradykinesia = hallmark, plus at least one of • Resting tremor (4-6 Hz) - often asymmetrical, “pill-rolling” • Rigidity – “lead pipe”, “cogwheel” • Postural instability PD pharmacological Tx • L-Dopa = first line. • Dopa-decarboxylase inhibitor often co-administered (e.g. carbidopa, benserazide) • particularly for younger patients or those with mild symptoms. Can also be used asatments, adjunctive therapy to L-Dopa in later stages of the disease. • Adjuvant medications include MAO-B inhibitors, COMT inhibitors, and anticholinergics 10/10 Functional Neurological Disorders Specific types of Neurological functional disorder **Ms. Smith, a 35-year-old woman, presents to the emergency department complaining of weakness in her legs. She reports that the weakness started suddenly this morning when she woke up and has progressively worsened throughout the day. She denies any recent trauma or injury to her legs. Upon further questioning, she mentions that she has been under a lot of stress at work lately and has been feeling overwhelmed. She states that she has been experiencing similar episodes of weakness intermittently over the past few months, but they usually resolve on their own within a few hours. On examination, Ms. Smith demonstrates weakness in both legs with intact sensation and reflexes. Hoover’s test is positive. There are no other neurological deficits noted. What is the most likely cause of Ms. Smith's leg weakness? A. Guillain-Barré Syndrome B. Multiple Sclerosis C. Functional Neurological Disorder D. Lumbar Disc Herniation E. Amyotrophic Lateral Sclerosis (ALS)Ms. Smith, a 35-year-old woman, presents to the emergency department complaining of weakness in her legs. She reports that the weakness started suddenly this morning when she woke up and has progressively worsened throughout the day. She denies any recent trauma or injury to her legs. Upon further questioning, she mentions that she has been under a lot of stress at work lately and has been feeling overwhelmed. She states that she has been experiencing similar episodes of weakness intermittently over the past few months, but they usually resolve on their own within a few hours. On examination, Ms. Smith demonstrates weakness in both legs with intact sensation and reflexes. Hoover’s test is positive. There are no other neurological deficits noted. What is the most likely cause of Ms. Smith's leg weakness? A. Guillain-Barré Syndrome B. Multiple Sclerosis C. Functional Neurological Disorder D. Lumbar Disc Herniation E. Amyotrophic Lateral Sclerosis (ALS)Ms. Smith, a 35-year-old woman, presents to the emergency department complaining of weakness in her legs. She reports that the weakness started suddenly this morning when she woke up and has progressively worsened throughout the day. She denies any recent trauma or injury to her legs. Upon further questioning, she mentions that she has been under a lot of stress at work lately and has been feeling overwhelmed. She states that she has been experiencing similar episodes of weakness intermittently over the past few months, but they usually resolve on their own within a few hours. On examination, Ms. Smith demonstrates weakness in both legs with intact sensation and reflexes. Hoover’s test is positive. There are no other neurological deficits noted. What imaging is needed?Ms. Smith, a 35-year-old woman, presents to the emergency department complaining of weakness in her legs. She reports that the weakness started suddenly this morning when she woke up and has progressively worsened throughout the day. She denies any recent trauma or injury to her legs. Upon further questioning, she mentions that she has been under a lot of stress at work lately and has been feeling overwhelmed. She states that she has been experiencing similar episodes of weakness intermittently over the past few months, but they usually resolve on their own within a few hours. On examination, Ms. Smith demonstrates weakness in both legs with intact sensation and reflexes. Hoover’s test is positive. There are no other neurological deficits noted. What imaging is needed? NoneFND • Accounts for up to 20% of neurology clinic presentations. Management: • = neurological symptoms • MDT approach: • Caused by a PROBLEM with the FUNCTIONING of the nervous system • Gold standard = biopsychosocial model • A “software” issue of the brain, not the hardware of care, involving collaboration between (as in stroke or MS) neurology, psychiatry, & allied health • With positive diagnostic features typical of FND professionals. • Cexperiences themdifficulties for the person who • Symptom-focused interventions e.g. - https://neurosymptoms.org/ motor-retraining exercises • CBT, physiotherapy, OT, SALT Investigation: • Patient education & counselling: • NICE guidelines emphasise importance of thorough • Providing patients with information clinical assessment to establish a diagnosis of FND disorders.entiate it from other neurological about FND, validating their experiences, • Neuroimaging and other investigations may be and offering reassurance indicated to rule out structural brain abnormalities • Good patient counselling affects or other medical conditions masquerading as FND. outcomes! Key Points • Where, when, why – use surgical sieve • OSCEs: Examiners can’t read your mind! Maximise score by following a structure • https://geekymedics.com/cranial-nerve-exam/ • Work out an order that works for you e.g. ToPCaRS