Neurology Board Review

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Neurology Board Review Jeffrey M. Gelfand, MD, MAS, FAAN Assistant Professor of Clinical Neurology, UCSF UCSF MS and Neuroinflammation Center Department of Neurology March 2018 Disclosures Dr. Gelfand
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Neurology Board Review Jeffrey M. Gelfand, MD, MAS, FAAN Assistant Professor of Clinical Neurology, UCSF UCSF MS and Neuroinflammation Center Department of Neurology March 2018 Disclosures Dr. Gelfand reports research support from Genentech and MedDay (both to UCSF). Dr. Gelfand serves as a steering committee member for a clinical trial sponsored by Genentech. Dr. Gelfand has received personal compensation for medical legal consulting from governmental and commercial entities for medical legal consulting. Dr. Gelfand receives grant support for training clinical fellows from the National MS Society. Case 1 - Weakness A 50 yo man reports leg weakness. On physical examination mental status normal. CN 2-12 normal. There is 4/5 power in the deltoids and iliopsoas (hip flexors), full distally. Reflexes are normal (2+). Babinski normal. Sensory exam is normal to LT, PP, temperature, vibration, position. Coordination normal. What is the neuroanatomical localization of his weakness? Case 1 - Weakness What is the localization of his weakness? A) Brain B) Brainstem C) Spinal Cord D) Nerve Roots E) Peripheral Nerves F) Muscle G) Neuromuscular junction Localizing weakness Upper Motor Neuron (UMN) Lower Motor Neuron (LMN) Neuromuscular junction (NMJ) Muscle Examples Brain, brainstem, spine Root, peripheral nerve Reflexes Brisk Decreased / absent Tone Spastic Decreased/ Flaccid RAM Slowed FFM/FT Variable (i.e. if severe distal weakness) Weakness Pattern Pyramidal Myotomal (variable) NMJ (i.e. MG Normal Normal Variable Variable, fatiguing Myopathy Normal Normal Variable but usually unaffected Usually Proximal Accompanying numbness CNS pattern (hemisensory); or spinal level Dermatomal or peripheral nerve No (as only motor!) Other signs +Babinski Atrophy Fatiguing, eye movements, bulbar No (as only motor!) Localizing Weakness - Pearls Pyramidal = corticospinal tract (Brain/spinal cord) preferentially affects extensors in UE and flexors in LE Bladder/bowel involvement -- think spinal cord, esp urinary urgency; can also have retention Sensory level think spinal cord Cranial neuropathies (CN 3-12) think brainstem; also with any crossed signs Facial weakness above pons (CN 7) or PNS equivalent Case 1 Weakness A 50 yo man reports weakness in his shoulders and legs. On physical examination there is 4/5 power in the deltoids and iliopsoas (hip flexors), full distally. Reflexes are normal (2+). Sensory exam is normal to LT, PP, temperature, vibration, position. He has been on chronic oral glucocorticoids for 5 years (for an inflammatory arthritis). An EMG-nerve conduction study is normal. Case 1 - Weakness What is the localization of his weakness? A) Brain B) Brainstem C) Spinal Cord D) Nerve Roots E) Peripheral Nerves F) Muscle G) Neuromuscular junction Additional Case Pearls: Myopathy, glucocorticoid induced. Usually normal EMG (due to selective Type 2 fiber atrophy) and normal CK. Clinical diagnosis. Should improve with minimizing glucocorticoid dose/exposure. Case 2 Numb Feet 65 yo man with numb feet x 5 years. The numbness involves both feet in a similar pattern, affects the entire foot top, bottom, sides and extends to just above the ankle. He doesn t remember exactly when it started, but it became more and more noticeable over time. He can still feel a little in his feet, but not as strongly. On exam there is numbness to LT and PP in the reported distribution. There is also decreased vibration sense in the toes and decreased position sense. Sways on Romberg. Reflexes are 1+ in the UE, absent knees and ankles (even with reinforcement). Negative Babinski. Feet are not arched. No other areas of anesthesia/decreased sensation. Rest of exam normal. PMH notable for DM Type 2 x 7 years, last HbA1c 9.0. On oral hypoglycemic agents, no insulin. No family history of neurological disease. Denies ETOH. What is the most likely cause of his numbness? A) Cervical Myelopathy B) Diabetic Distal Symmetric Polyneuropathy C) Diabetic lumbosacral radiculopathy D) Charcot Marie Tooth (inherited neuropathy) E) Leprosy Neuropathy What is the most likely cause of his numbness? A) Cervical Myelopathy B) Diabetic Distal Symmetric Polyneuropathy C) Diabetic lumbosacral radiculopathy D) Charcot Marie Tooth (inherited neuropathy) E) Leprosy neuropathy Case Pearls: - Diabetic neuropathy is the most common cause of polyneuropathy in the US (ETOH is #2). End-organ complication (DM1, DM2, even prediabetes). EMG-NCS with mixed axonal +/- demyelinating polyneuropathy. Distal or stocking-glove distribution of sensory loss (though usually starts with just the stocking component). Can also have burning, paresthesias (tingling) or bothersome tingling/sensations (dysesthesias) due to injury of small fiber unmyelinated sensory axons (i.e. painful diabetic neuropathy) worse at night. Can have autonomic dysfunction. Motor usually comes later and more severe. - Diabetic amyotrophy affects lumbosacral plexus (i.e. radiculopathy) + nerve acute, very painful focally and then weakness in the proximal leg, weight loss, autonomic dysfunction. Usually with new diagnosis Type 2 DM (or unaware Type 2 DM). Diabetes can also cause other patterns of PNS injury. - Leprosy is sometimes said to be the most common treatable cause of neuropathy in certain parts of the world. Usually occurs in patients with typical skin lesions but ~10% of cases are pure neuritic. Symmetric loss of pain/temperature and then light touch in temperature dependent cooler areas (i.e. earlobes, nose, dorsal hands/feet), direct invasion by M. Leprae. Case 3 Numb Feet 65 yo man with numb feet x 5 years, difficulty walking x 6 months and now using a cane. The numbness involves both feet in a similar pattern, affects the entire foot top, bottom, sides and extends to just above the ankle. He doesn t remember exactly when it started, but it became more and more noticeable over time. He can still feel a little in his feet, but not as strongly. Urinary urgency x 3 months. Legs feel a bit stiff. On exam there is numbness to LT and PP in the reported distribution. There is also decreased vibration sense in the toes and decreased position sense. Sways on Romberg. Reflexes are brisk (3+) in the UE and patellae, absent in the AJs. +Babinski. Tone is spastic in the legs. Spastic gait. PMH notable for DM Type 2 x 7 years, last HbA1c 9.0. On oral hypoglycemic agents, no insulin. No family history of neurological disease. Denies ETOH. What is the most likely cause of his numbness? A) Cervical Myelopathy B) Diabetic Distal Symmetric Polyneuropathy C) Diabetic lumbosacral radiculopathy D) Charcot Marie Tooth (inherited neuropathy) Localizing weakness Upper Motor Neuron (UMN) Lower Motor Neuron (LMN) Neuromuscular junction (NMJ) Muscle Examples Brain, brainstem, spine Root, peripheral nerve Reflexes Brisk Decreased / absent Tone Spastic Decreased/ Flaccid RAM Slowed FFM/FT Variable (i.e. if severe distal weakness) Weakness Pattern Pyramidal Myotomal (variable) NMJ (i.e. MG Normal Normal Variable Variable, fatiguing Myopathy Normal Normal Variable but usually unaffected Usually Proximal Accompanying numbness CNS pattern (hemisensory); or spinal level Dermatomal or peripheral nerve No (as only motor!) Other signs +Babinski Atrophy Fatiguing, eye movements, bulbar No (as only motor!) C3 Spinal cord C6 C7 What is the most likely cause of his numbness? A) Cervical Myelopathy B) Diabetic Distal Symmetric Polyneuropathy C) Diabetic lumbosacral radiculopathy D) Charcot Marie Tooth (inherited neuropathy) E) Leprosy neuropathy Case Pearls: Cervical spondylosis with myelopathy (with cervical central canal stenosis). Most common cause of myelopathy in older adults ( age 55). Diagnosis is clinical, as 80% of ASYMPTOMATIC people 60yo have some degree of spondylosis on imaging; key is clinical correlation: need symptoms/signs to be clinically concerning. This patient has a superimposed mild diabetic polyneuropathy (thus the absent ankle jerk reflexes despite otherwise florid upper motor neuron signs. If take away the diabetic history, the numb feet could also be a partial sensory level from cervical myelopathy. Localization is key as an EMG-NCS would have shown polyneuropathy which is true but not the cause of his gait impairment! Case 4 32 yo woman presents with ascending numbness x 3 days, began in her feet, now up to her umbilicus, pins and needles and less sensation. Now having trouble walking x 1 day. Urinary retention What diagnostic test would be the next best step in her workup? A) MRI Brain B) MRI Cervical and Thoracic Spine C) MRI Lumbar Spine D) CT Thoracic Spine E) Electromyography and nerve conduction studies A) MRI Brain B) MRI Cervical and Thoracic Spine C) MRI Lumbar Spine D) CT Thoracic Spine Case 4 Acute myelopathy E) Electromyography and nerve conduction studies Pearls: -- This is an acute myelopathy (spinal cord localization). It is essential to evaluate for compressive causes (spondylosis, disc, epidural abscess, tumor). MRI is far superior to CT to image the spinal cord. A CT myelogram can be used to exclude compression if unable to get an MRI but won t be able to image the cord for pathology. -- Spinal lesions are often partial (i.e. can have a thoracic sensory level from a cervical lesion if only partially affected as opposed to complete transection) the localization should be the potential level OR ABOVE i.e. in this case T10 or above. If MRI T spine negative, must image higher! This is commonly not appreciated in clinical practice. -- MRI Lumbar spine images the conus and cauda nerve roots. It barely images the lower tip of the spinal cord. If considering a myelopathy affecting legs must get MRI of the T spine. The lumbar here is based on bone, not spinal cord (which ends at L2 in adults(!), thus the ability to get lumbar punctures safely distally. -- After imaging, next test would be a CSF exam. If inflammatory then myelitis (autoimmune, infectious). T2 T1 T1 Post-contrast Case resolution / Pearls: -- Inflammatory myelopathy (myelitis), in this case what turned out to be a new diagnosis of multiple sclerosis. Metabolic myelopathies (i.e. B12 deficiency affecting the dorsal column) are usually subacute and not acute like this. Hyperacute myelopathies can be ischemic. White matter lesions on MRI Multiple Sclerosis is a clinical diagnosis. Favor based on history of clinical attacks (relapses) and typical findings of white matter lesions on brain/spine MRI (esp periventricular/corpus callosal involvement). CSF shows oligoclonal bands in ~90% but not specific for MS (vs neuroinflammation generally). Broad ddx for white matter lesions including vascular, infections, migraine, etc. Brain MRI in MS Healthy 23 yo 21 yo with MS Case 5 Facial Weakness 25 yo woman comes to a same day urgent clinic visit complaining of facial weakness. Woke up with it and noticed when looked in the mirror right away. There is a severe paralysis of the right face that clearly involves the right forehead and right lower face. He cannot close his eye on the right (and when he tries to close the eye the eyeball rolls upwards) He reports that his face feels heavy and numb to him but formal sensory testing is normal to LT, PP. Sounds are a bit louder on the right (hyperacusis). No other CN or other abnormalities on exam. No vesicles in the ear. No other relevant PMH, social history, takes no medications. What is the most likely diagnosis? A) Acute ischemic stroke B) Bell s Palsy C) Ramsay-Hunt Syndrome D) Trigeminal Neuralgia Facial Weakness Case Conclusion A) Acute ischemic stroke B) Bell s Palsy C) Ramsay-Hunt Syndrome D) Trigeminal Neuralgia Case Pearls: -- Key localization pearl is that FOREHEAD MOTOR FUNCTION HAS BILATERAL INNERVATION FROM THE CNS Upper motor neuron facial weakness (i.e. strokes) cause lower facial weakness sparing the forehead. Lower motor neuron facial weakness (CN7 injury) causes hemifacial weakness involving the forehead. (Strokes may rarely cause LMN patterns of facial weakness by affecting the CN7 nucleus in the pons but there are usually other brainstem findings but important consideration if exam not clean or other symptoms/signs or risk factors). -- Bell s Palsy refers to idiopathic LMN facial weakness (thought to be from HSV reactivation in most cases). VZV reactivation in the geniculate ganglion can cause facial weakness (Ramsay-Hunt Syndrome), vesicles can be seen in the ext auditory canal). Lyme disease (secondary) is an important diagnostic consideration and can be unilateral or bilateral; sarcoidosis can also cause bilateral or unilateral or recurrent facial palsy. -- Treatment of Bell s Palsy should include an oral steroid course (may improve recovery modestly). Benefit of acyclovir/valacyclovir less clear (more recent RCTs show no benefit and but some earlier trials with steroid combo did) Case Series 6 Headache A 60 yo man comes to the ER complaining of the worst headache of my life. What historical feature would be most concerning for Subarachnoid Hemorrhage (SAH)? A) Severe head pain B) Maximal pain intensity within seconds at headache onset C) Photophobia D) Nausea Case Series 6 Headache What historical feature would be most concerning for Subarachnoid Hemorrhage (SAH)? A) Severe head pain B) Maximal pain intensity within seconds at headache onset Thunderclap headache C) Photophobia D) Nausea Pearls: Thunderclap refers to reaching maximal pain intensity within seconds/very quickly. At some point all migraineurs may have a headache that is their worst but migraine crescendos over time. CT is very sensitive for SAH; a CSF exam looking for xanthochromia (yellowing of blood in CSF from heme breakdown) and blood can still have a role when imaging is negative though CT sensitivity is getting better Migraine At least five attacks with: Headache attacks lasting 4-72 hr (untreated or unsuccessfully treated) Headache has at least two of the following four characteristics: unilateral location pulsating quality moderate or severe pain intensity aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs) During headache at least one of the following: nausea and/or vomiting photophobia and phonophobia Not better accounted for by another diagnosis. - With (25%) or without (75%) aura (visual, sensory, motor) - Chronic migraine if 15 days of migraine/months ICHD-3 Criteria Migraine treatment Acute (abortive) therapy First-line: NSAIDs (esp naproxen), acetaminophen Triptans (avoid if MI, stroke, uncontrolled HTN) Fluids Dopamine agonists (prochlorperazine, chlorpromazine) Avoid opiates Preventive therapy -- Consider if 4 HA days/month -- Many evidence-based options to consider Other Headache Pearls Cluster HA: Unilateral pain, esp men, autonomic symptoms, cluster over time in frequency and time of day, exceedingly painful; High flow O2 acutely; lithium, other med options Temporal arteritis: Formally consider for any new HA age 50 (but usually much older incidence); risk of vision loss (from ischemic optic neuropathy); elevated ESR; jaw claudication. Temp artery biopsy (but start steroids first empirically) Trigeminal Neuralgia: Unilateral facial pain, lancinating, electrical, can have triggers (i.e. touch, acidic foods), no true sensory loss (or else trigeminal neuropathy). Need to exclude a structural lesion (vascular loop, MS) with MRI. First line treatment with carbamazepine. Idiopathic Intracranial Hypertension (formerly called pseudotumor cerebri): Bilateral, can be positional (worse when laying flat), disc edema on funduscopy (KEY EXAM TO DO FOR HA EVALUATION!), associated with obesity, need to exclude other causes of intracranial hypertension (i.e. meningitis) Meningitis: New bilateral HA, stiff neck, may or may not have true meningismus (less common in adults and with aseptic meningitis, photophobia. If encephalopathy then meningoencephalitis. CSF exam to confirm. Also imaging. Dementia Ddx Pearls AD most common worldwide; amnesia; early AD can be monogenic and atypical phenotypes Vascular, also very common, can coexist, may be stepwise in decline DLB (alpha-synucleinopathy) -- Visual hallucinations, REM sleep behavior disorder, Parkinsonism, delirium (esp with antipsychotics FTD Apathy, poor judgement, language CJD (prion disease) archetypical rapidly-progressive dementia, myoclonus, ataxia, characteristic MRI findings (cortical ribboning on DWI, basal ganglia) NPH commonly tested in boards contexts with triad of dementia, magnetic gait, bladder and imaging hydrocephalus; in clinical practice can have congenital hydrocephalus and unclear benefit of shunting esp when dementia phenotype has been present for time Stroke secondary prevention pearls For most causes, antiplatelet superior ASA, clopidogrel or ASA/dipyramidol; if stroke on ASA then the latter Anticoagulation: Afib (increasingly recognized as cause of cryptogenic stroke, need for prolonged monitoring); cardioembolic (cardiac thrombus and possibly low EF and?pfo with ASD);?dissection (newer data that antiplatelet may be non-inferior); APLAS Carotid endarterectomy for symptomatic disease (70-99%); may have role for stenting but max medical management increasingly is better and better so balance against risk Seizure Pearls Goal of treating to minimum dose that treats the disease. If can achieve efficacy with monotherapy that is preferred but sometimes need multiple options. Role of levels: Compliance, rarely toxicity. Usually monitor clinically Seizure vs syncope (pearls are warning/aura and delayed coming to with sz vs syncope) Seizure Pearls Medication Pearls - Levitaracetam (renally cleared, no drug-drug interactions), po/iv - Lamotrigine (Rash, risk of Stevens Johnson) - Topiramate (cognitive side effects, kidney stones,?weight loss) - Zonisamide (weak carbonic anhydrase inhibitor, long acting) - Carbamazepine / Oxcarbazepine Hyponatremia, liver, can screen for Stevens Johnson risk with HLA-B* Lacosamide (less drug-drug interactions; oral and IV forms) - Felbamate Aplastic anemia - Rufinamide -- caution with short QT, adjunctive - Clobazam Stevens Johnson/TENS, TEN - Vigabatrin -- infantile spasms but risk of vision loss (retinal toxicity) Case Ascending Weakness 30 yo man with weakness in his feet, ascending to his arms. Tingling in feet and hands but normal LT/PP/Vibration. On exam reflexes are absent in the ankles. Weakness in shoulders and distal lower extremities CSF with normal cell count and glucose. Total protein elevated to 75. Case GBS Guillain-Barre Syndrome Acute Inflammatory Demyelinating Polyneuropathy Classically dropped reflexes and CSF with elevated protein and no pleocytosis (but exceptions very early). EMG-NCS can also be normal for the first several days to 2 weeks. Treatment with IVIG/PLEX (not steroids) Concern for respiratory failure (monitor MIF and FVC with RT), intubate if MIF -20 or FVC 1 DVT prophylaxis Cardiac monitoring (autonomic instability risk Neurology Board Review Jeffrey M. Gelfand, MD, MAS, FAAN Assistant Professor of Clinical Neurology, UCSF UCSF MS and Neuroinflammation Center Department of Neurology March 2018
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