Director, Neurology Clerkship Associate Professor of Neurology I hope you find this file helpful for student preparation for the Neurology Shelf Exam.  The audio for the last 9 slides will be completed shortly, however for your information, the slides present topics I discuss with students during the shelf review session. Sincerely, Heather Anderson, MD Director, Neurology Clerkship Associate Professor of Neurology Bulbar weakness DDx: ALS, MG or other neuromuscular junction abnormalities Facial pain Trigeminal neuralgia – “shock-like”, 1 trigeminal distribution, precip: eating, talking, chewing, touching Tx: carbamazepine Facial palsy Bell's Palsy Ramsey Hunt (Herpes Zoster – Acyclovir) Lyme disease (rash, may have meningitis, other CN palsies, later may have encephalopathy) Low sensitivity of serology in 1st few wks. ELISA 1st → confirmatory western blot. Tx doxy, rocephin

Neuro-ophthalmology Optic neuritis Amarosis fugax Amblyopia Diplopia Afferent pupillary defect Amarosis fugax Amblyopia Diplopia Ptosis Optic neuritis Sudden, reduced vision in the affected eye Loss of color vision Pain with eye movement APD – optic nerve or severe retinal disease Amarosis fugax – Temporal arteritis, carotid stenosis Case: Pt with hx of episodes of left sided weakness and veil over right eye – which carotid? (right) Amblyopia – partial or complete loss of vision in 1 eye (congenital abnl development). Causes = strabismus (crossed eyes) Case: History of poor vision in 1 eye since birth in a pt with strabismus Diplopia Determine direction which provokes the symptoms CN 3 palsy – on forward gaze, eye is down and out Is the pupil involved (dilated) or spared Involved could be PCOM aneurysm, compressing parasympathetic fibers Case: Sudden onset of double vision, pain over right temple in a patient with a history of HTN, DM. On exam – mild ptosis on R, failure of R to adduct and impaired upgaze on R, pupils 3mm sluggish CN 4 – depression of eye primarily on adduction palsy – diplopia better on tilt opposite, worse on tilt same Ptosis – MG, CN III, Horner’s Case: Ptosis on the left, left pupil smaller than the right but reactive – exam otherwise normal Horner syndrome – sympathetic chain (brainstem stroke, tumor in lung apex, carotid dissection)

Other Cranial Nerve Disorders Bulbar weakness Facial pain Facial palsy Bulbar weakness DDx: ALS, MG or other neuromuscular junction abnormalities Facial pain Trigeminal neuralgia – “shock-like”, 1 trigeminal distribution, precip: eating, talking, chewing, touching Tx: carbamazepine Facial palsy Bell's Palsy Ramsey Hunt (Herpes Zoster – Acyclovir) Lyme disease (rash, may have meningitis, other CN palsies, later may have encephalopathy) Low sensitivity of serology in 1st few wks. ELISA 1st → confirmatory western blot. Tx doxy, rocephin

Vertigo Meniere’s Benign positional vertigo Labyrinthitis Aminoglycoside-induced neurotoxicity Cerebellar hemorrhage Vertebrobasilar TIA Meniere’s disease Episodes of vertigo, nausea, vomiting w/ diminished hearing and tinnitus Tx: Restrict salt/caffeine/tobacco, use of diuretics, meclizine, benzos Acoustic neuroma – unsteadiness, less commonly true vertigo (slow growing so gradual asymmetries in vestib fxn) Benign positional vertigo - Dix-Hallpike (Epley or Barany maneuvers) Recurrent episodes of vertigo lasting ≤ 1 minute, may be associated with nausea and vomiting Typically recur periodically for weeks to months without therapy Provoked by looking up while standing or sitting, lying down or getting up from bed, and rolling over in bed Sxs may wax and wane over time; often sudden remissions, only to have the episodes recur at a later date May have evidence of prior inner ear damage Labyrinthitis - prolonged, severe episodes of vertigo accompanied by nausea, vomiting, and disequilibrium Aminoglycoside-induced neurotoxicity, cisplatin-based chemotherapeutics. Case: Ill pt who develops deafness, tinnitus, balance, vertigo Cerebellar hemorrhage abrupt onset bioccipital HA, n/v, vertigo, loss of balance, worse with coughing/straining Vertebrobasilar TIA Case: 8-10 min episode of vertigo/swaying, diplopia, facial numbness, hemisensory loss, Wallenburg

Seizures DDx for causes of seizures Metabolic Drug-induced Structural Developmental Tumor, abscess, etc. Trauma, previous stroke, other injury Quick review of additional material since seizure material has been provided elsewhere Epilepsy = >1 unprovoked seizure Electrolytes Hypoglycemia, hypo Ca, Mg, hypo or hypernatremia Hyponatremia could be caused by SIADH (head trauma) – low serum osm & Na, high urine osm Case: Pt has been ill, dehydrated → seizure. What test? Draw sodium level Auras olfactory, auditory, or gustatory hallucinations, deja vu, fear = temporal lobe Herpes favors temporal lobe Thrombotic thrombocytopenic purpura Can cause seizure (2º to hemorrhage, uremia, hyponatremia, fever). Anemia, low platelets, elevated creatinine, purpura

Multiple Sclerosis Demographics Diagnostic features of MS INO Transverse myelitis Urinary incontinence – detrusor hyperreflexia (upper motor neuron lesion) Multiple episodes of neurologic deficit over space and time Treatment – A question which has shown up on the shelf exam in the past is the long-term management of MS. If they ask the question (they might not ask it anymore), but the answer they were looking for is interferon beta. This is not a complete answer since other classes are available for long-term management, so hopefully the question is no longer being asked Demographics - Young woman (20’s-30’s) INO (internuclear ophthalmoplegia) failure of ipsi eye to adduct with nystagmus of contra eye Lesion is in ipsi MLF Transverse myelitis Case: 1 week hx of progressive sensory loss to T10 level, for example, w/ accompanied urinary urgency and freq, nocturia, perception of a tight band-like sensation around midabdominal region Can occur anywhere in spinal cord (T10, aching b/t shoulder blades (lower cervical or upper thoracic) Consider demyelinating lesion if history of previous neurologic event (optic neuritis, etc)

Parkinson’s Plus Syndromes Progressive supranuclear palsy Lewy body dementia Corticobasal degeneration Multiple system atrophy PSP No voluntary vertical eye movements, but preserved Doll’s eyes Diffuse brainstem disease, neuronal loss, NFTs, gliosis in reticular formation & ocular motor nuclei. Early pathology is primarily in midbrain, leads to early vertical eye movement abnormalities Characteristic exam findings in addition to eye findings: Gait instability, rigidity Lewy Body Dementia Fluctuations in cognitive function Hallucinations Parkinsonism Falls, syncope Sensitivity to neuroleptics Corticobasalganglionic degeneration Shy-Drager (Multiple system atrophy)

Other Movement Disorders Opisthotonos Opisthotonos (only the back of the head and the heels would touch the supporting surface) dystonic rxn from Compazine (prochlorperzine) or other phenothiazines or antipsychotics – Tx = Benadryl (anticholinergic)

Toxins Organophosphate poisoning Anticholinergic syndrome Toxicity Carbon monoxide poisoning Medication side effects Case: Farm worker develops SOA, n/v, blurred vision, salivation, fasciculations = organophosphate poisoning. Tx = atropine, remove clothing Pralidoxime binds organophosphate-inactivated acetylcholinesterase, but no demonstrated benefit, and its use may be harmful Case: Pt taking a new medication, develops tachycardia, dry mouth, flushed skin = anticholinergic syndrome Tx = physostigmine Any subacute change in neurologic fxn within 1 month of Rx change (especially AEDs) – consider Rx AE Case: 45 yo man with long-standing hx szs → after a recent doctor’s appt, develops bilateral nystagmus and ataxia on f-to-n, wide-based, neuro exam o/w nl What to ask? Was there a change in medication? Increased Dilantin dose recently → Dilantin toxicity Case: 40 yo man restoring an old home, wood-burning stove → disorientation, confusion, severe headache, pulse ox 100%. Carbon monoxide poisoning Check carboxyhemoglobin level Tx = hyperbaric oxygen Case: 35 yo man with schizophrenia, taking clozapine, Prilosec, simvastatin, and aspirin. He develops fever, malaise, severe sore throat = agranulocytosis from clozapine Case: 20 yo man suddenly develops chest pain, tachycardia, elevated blood pressure, and pupils are equal and reactive = cocaine

Conversion Disorder Discussion of features characteristic of Pseudoseizures (non-epileptic events) Pseudocoma Pseudoweakness Etc. Hoover’s sign Hand over face Collapsing or giveaway weakness Tunnel vision Sensory loss that stops exactly at midline

Meningitis Diagnosis Treatment Etiologies Rifampin – prophylactic therapy against Neisseria meningitides Ceftriaxone – acute tx for N meningitides Physical exam findings – headache, neck stiffness, fever, photophobia, n/v Could be viral or bacterial Review CSF lab results If there are seizures in the presentation, think HSV