Neurology Board Review

Neurology Board Review

Question 1 • A 72 year old man presents with acute onset vertigo, nystagmus, dysphagia, and horners syndrome. The most likely diagnosis is?

Your Choices…. 1. Acute Labryinthitis 2. Benign paroxysmal positional vertigo 3. Lateral Medullary Infarction 4. Opthalmoplegic Migraine

Lateral Medullary Infarction! AKA Wallenberg Syndrome Ipsilateral face • Pain and Temperature • Dysphagia • Dysarthria • Nystagmus • +/- limb ataxia Contralateral Limbs • Pain and Temperature -Lateral Spinothalamic tract

Posterior Circulation Strokes • The 5 D’s of Brainstem • Dysphagia • Dysarthria • Diplopia • Dystaxia • Dizziness • Syncope/ Drop attack • Ipsilateral Face, Contralateral Extremity • Visual Field Deficits

Peripheral -Sudden -Tinnitus, Auditory -Severe n/v/dizzy -Horizontal Nystagmus -May be positional, recent infections Central -Insidious -No peripheral sx -Less severe n/v/dizzy -Vertical or Horizontal Nystagmus -Not positional, may have peripheral neuro deficits Vertigo

Question 2 A 74 year old female with history of DM, HTN, presents with 2 hours onset right face, arm > leg weakness with an associated right hemisensory deficit. No left sided deficits. No cranial nerve deficits. What is the most likely diagnosis? • Basilar Artery Occlusion • Subarachnoid Hemorrhage • Lacunar Infarction • Middle Cerebral Artery Occlusion • Posterior Cerebral Artery Occlusion

Middle Cerebral Artery Occlusion • Lateral parietal, temporal, and frontal lobes • Contralateral Motor/ and Sensory Face and Arm > leg • Ipsilateral Hemianopsia • Aphasia/ Dysarthria (left sided stroke) • Agnosia / Neglect, extinction of double stimulus (right parietal lobe)- timing!

CT Finding with MCA Occlusion • Hyperdense MCA sign • Loss of cortical ribbon • Sulcal Effacement • Obscuration of the grey/white junction

The Wrong Answers! • Basilar Artery Occlusion: Locked In • Subarachnoid Hemorrhage: HA • Lacunar Infarction: Pure motor or sensory • Posterior Cerebral Artery Occlusion: Primary visual disturbances

Question 3 • A 43 year old female presents to the ER with her husband. Her husband states that his wife has been having the worst headache of her life and is “a bit off”. On exam she uncomfortable and confused without focal motor or sensory deficits. A CT scan is obtained.

What is the most common etiology for the diagnosis revealed by the CT scan? 1. AVM 2. Cavernous Angioma 3. Mycotic Aneurism 4. Neoplasm 5. Saccular Aneurysm Question 3

Saccular Aneurysm • 80% of non-traumatic SAH are associated with saccular aneurysm • 5% of the population have aneurysms; increase risk of rupture includes- • Smoking • EtOH • Stimulant Abuse • Uncontrolled HTN

Subarachnoid Hemorrhage • Collection of blood in subarachnoid space • Secondary to trauma, ruptured aneurysm, AVM • 2-4% Patient visits for HA • 2-4% will have SAH; 12 % of pts with worst headache of life will have SAH, increases to 25% if abnormal neurologic exam • Headache 100%, Nausea and emesis 77%, focal deficits 64%, syncope 53%, neck pain 33%, photophobia, seizures in 25% of patients • 20-50% have prior warning headache “sentinel bleed” days to weeks prior

Cranial Nerve 6 (abducens) palsy; lateral rectus; ACOM • Cranial Nerve 3 (occulomotor) palsy; ptosis, medial, superior, inferior gaze, pupillary constrictors; PCOM • Subhyaloid Hemorrhage

Question 4 • An 84 year old man with h/o HTN, DM, AFIB on coumadin presents with left sided hemiparesis and left sided hemisensory changes with left sided neglect. He has a GCS of 15. Thirty minutes into his assessment his GCS falls to 11 with profound confusion. What is the most likely cause? • Anterior Cerebral Artery Embolism • Internal Capsule Intracerebral Hemorrhage • Posterior Cerebral Artery Rupture • Posterior Cerebral Artery Thrombosis • Vertebral Artery Occlusion

Internal Capsule Intracerebral Hemorrhage • Hemorrhagic transformation may occur during an apparent ischemic stroke • Sudden change in conciousness= ICH V.S posterior circulation CVA • Reversal of anticoagulation

Intracranial Hemorrhage • 8-13% of all strokes • 30 day mortality 44%, brainstem ICH 75% 24 hour • Only 20% of pts regain full functional independence • Increase incidence: AA, Asian, age >55, EtoH, Smokers • Trauma, HTN, altered homeostasis, hemorrhagic necrosis, venous outflow obstruction • Causes brain injury via: 1. Increased Intracranial Pressure 2. Increase edema, mass effect 3. Decrease perfusion to local and adjacent tissue 4. 35% ICH will expand sig (>33%) within 24 hours; majority within 6 hours

ICH • Basal Ganglia 40-50% • Lobar: 20-50% (esp young, increased sz activity) • Thalamus 10-15% • Pons 5-12% • Cerebellar 5-10% • Brain Stem 1-5% • Intraventricular Hemorrhage 1/3 BG • Volume= (a+b+c)/2

ICH GCS 3-4 2 5-12 1 ___________13-15 0 ICH Vol >30 1 ___________<30 0 IVH Yes 1 ___________No 0 Infratentoral Yes 1 ___________No 0 Age >80 1 ___________<80 0 0-6

Question 5 • A 45 year old male presents with nausea, emesis, and diarrhea. He is given 2 liters of IVF and 12.5mg of promethazine. 15 minutes later he is anxious and wants to leave the ED immediately. What is the diagnosis and management? • Anxiety or who cares. Let him go AMA • Is he tolerating PO? Give him some reglan and get him out. • I think he is delirious. Give him some haldol and call psych. • I think he is having a reaction to the med. Lets give him Prochlorperazine. Right? • I think he is having a reaction to the med. Lets give him some Benztropine.

Akathisia- benztropine • Acute distonic reaction marked by anxiety, restlessness • Other distonic rexns include torticollis • Associated with high potency antipsychotic (haldol), and any dopaminergic medications (promethazine, metoclopramide, prochlorperazine) • Treatment includes anti-cholinergic medications such as diphenhydramine and benztropine (not to use in kids less than 3)

A 65 year old male with DM, HTN, BPH, recent diagnosis of sciatica p/w 2 days of progressive difficult ambulation with worsening back pain radiating down to left leg. Exam is noteable for hyporeflexia with downgoing toes, +4/5 lower extremity strength, saddle paresthesia, and deminished rectal tone. 1. Stroke 2. Sciatica 3. Cauda Equina Syndrome 4. Acute back pain 5. Spinal Abcess Question 6

Ca, Infiltrative, Sarcoidosis, Trauma, Infectious, Ank Spon Pain, radicular Weakness- variable Hyporeflexia v.s spinal Saddle sensory changes Overflow incontinance urine/stool Cauda Equina Syndrome

MRI or CT Myelography Neurosurgical consultation Steroids + RT- randomized controled high dose, non-radnomized low dose; end treatment and 6 months in ability to ambulate Radical ressection + RT Cauda Equina Syndrome

Sciatica Radicular Pain Lateral or post leg to foot Straight leg raise (10-60), crossed Numbness, no weakness NSAIDS Epidural Abcess Staph (MRSA) 63%; Gram Neg, Strep, Anaerobes, TB (potts) Multiple levels Epidurals, Surgical, IVDU, Cryptogenic DM, ETOH, HIV Pain, Fever, Weakness MRI/ CT w/ gadolinium Surgical Decompression /Aspitation Abx: Nafcillin (Vanc)+Flagyl+ Ceftazidime or Cefotaxime Other options

Progressive neurological findings Constitutional symptoms (fever) History of traumatic onset History of malignancy Age 18 years or 50 years IVDU Chronic steroids HIV Osteoporosis Pain > 6 weeks *American college of radiology “Red Flags” Should I get the imaging….?

Which of the following pretreatment patient characteristics has been associated with an increased risk of intracerebral hemorrhage following treatment with TPA for acute ischemic stroke? Advanced Age Increased NIHSS Isolated global aphasia Major surgery within 14 days Rapid improvement of neurological signs Question 7

Increased Stroke Severity • Increase stroke severity via NIHSS and increasing radiographic signs of infarct size on CT are two independent predictors of ICH after TPA

Tissue plasminogen activator for acute ischemic stroke. The National Institute of Neurological Disorders and Stroke rt-PA Stroke Study Group. NEJM. 1995 333:1581-1587. • Double-blind, randomized, placebo controlled • Pts tx with rTPA are 30% more likely to have minimal to no disability at 3 months compared to standard care • Increase risk of symptomatic ICH (6.4%) with increasing NIHSS • American Heart Association, American Academy of Neurology, ACEP (if system in place)

Inclusion Criteria Age > 18 Diagnosis of stroke with measurable deficit Time of onset < 3 hours before treatment will begin Relative Contraindications Major surgery or serious trauma within 2 weeks Only minor or rapidly improving stroke sx History of GI or GU hemorrhage within 21 days Recent arterial puncture as non-compressable site Glucose >400, <50 Post MI pericarditis Patient with observed seizure at time of stroke onset Recent Lumbar Puncture Exclusion Criteria Evidence of ICH on CT History of ICH or AVM Suspected SAH with normal CT Active internal bleeding Platelets < 100,000 Heparin within 48 hours with an elevated PTT Current use of oral anticoagulant with PT> 15sec SBP > 185 or DBP >110 at time treatment is to begin Within 3 months any intracranial surgery, serious head injury, or previous stroke (not TIA) tPA

Question 8 • A 32 year old man who lives in New England presents complaining of bilateral leg weakness. His symptoms began with paresthesias in his toes followed by progressive weakness in both legs. Cranial nerve exam is normal. Motor s 3/5 in both legs, 4/5 both arms and sensation to light touch is mildly decreased in both legs. DTR’s are absent in both legs and +1 in b/l arms. What is the most likely diagnosis? 1. Lambart-Eaton Syndrome 2. Familiar periodic paralysis 3. Guillan Barre Syndrome 4. Myasthenia gravis 5. Tick paralysis

Guillain-Barre Syndrome • Immune-mediated; motor, sensory, and autonomic dysfunction • GBS the most common cause of acute flaccid paralysis in the United States • Pure motor and motor + sensory subtypes. • 40-80% seropositive for Campylobacter jejuni • Haemophilus influenzae, Mycoplasma pneumoniae, and Borrelia burgdorferi. CMV, EBV, HIV • 85% of pts with normal recovery 6-18 months

Guillain-Barre Syndrome • Ascending weakness from proximal thighs to trunk and upper extremities • Cranial nerves, respiratory muscles (1/3rd) • Paraesthesias distal to proximal, Proprioception, sensory • Autonomic dysfunction; HR, BP, Temp, Fecal and urinary retention

Guillain-Barre Syndrome • Clinical diagnosis supported by: • Elevated or rising protein levels on serial lumbar punctures (90% pts) 1-2 weeks • CSF pleocytosis in HIV associated • Cauda Equina nerve roots enhance in 85% • ABG and FVC to assess respiratory function, intubate for ventilatory failure • IVIG and plasma exchange tx

Others Myasthenia gravis -Autoantibodies against post-synaptic Ach receptors -Bulbar sx initialy- ptosis, diplopia, dysphagia, 1% resp -Descending weakness -Thymoma 10-15% -Sx improve with rest Lambart-Eaton Syndrome -Autoantibodies against voltage gated calcium channels in pre-synaptic motor nerve terminal -Proximal lower extremity weakness (up from chair), months -Less common bulbar findings -Highly associated with cancer (50-70%) -Sx improve with movement

Others Familial periodic paralysis -AD, variable penetrance -Chanelopathy resulting in inexcitability of Na/Ca channels leading to periodic flacid paralysis -Hyperkalemic and Hypokalemic subtypes -Worsened by heat, stress, high carbohydrate meals Tick paralysis -Caused by neurotoxin from salivary gland -Ascending paralysis 1-2 weeks -Ataxia variant -Rock Mountain wood tick (Dermacentor andersoni) and American dog tick (Dermacentor variabilis)

A 25 year old male presents with 1 day of severe right sided head and neck pain with blurred blurred vision. He states he went to his chiropracter in the morning before symptom onset. On exam he has right sided miosis and ptosis with normal motor function and sensory function. What is his most likely diagnosis? Right brainstem cva Cluster Headache Bells Palsy Tick Bite Carotid artery dissection Question 9

Unilateral facial/neck/orbital pain Hypoageusia Transient blindness, amaurosis fugax 50% w/ partial horners syndrome- miosis, ptosis, no anhydrosis 25% pulsitle tinnitus Neck swelling, bruise May progress to CVA with dense hemiparesis Trauma Chiropractic manipulation Sports, yoga CTD HTN Smoking Oral contraceptives Carotid Artery Dissection

Sympathetic fibers run upwards vis cervical spine ganglia Bifruncate at division of CC to IC and EC (sweat glands) Innervate pupilary dilators (dilation lag) and lids Migraine, Brainstem CVA, Pancoast tumor, brachial plexus trauma, Lung lesion (TB, HMX), neuronal lesion Horners Syndrome

Angiography gold standard MRA optimal if available CT angiogram evolving, esp for trauma pts Anticoagulation with heparin Neurosurgical consultation Diagnosis and Treatment

A 43 year old male presents to the emergency room with 2 hours onset decreased movement of right side of face, ear pain, and thinks he might have had spoiled milk with his cereal this am because it tasted funny. What is the least important question for the diagnosis? When was the milks expiration date? Can he move his forehead? Does he have a history of migraine? Does he have clustered vesicles about the ear? Does he have peripheral motor weakness? Question 10

Facial Nerve CN 7 palsy Upper and lower facial weakness Post auricular pain Hyperacusis (stapedius) Hypoageusia (ant 2/3 tongue) Decreased lacrimation 30% pts w/ Crocodile tears, dysagusia, partial paralysis; 80-90% without sig deficit Bells Palsy- Not spoiled milk.

Causes HSV 1,2 VZV Mycoplasma pneumoniae Borrelia burgdorferie HIV (b/l) Adenovirus coxsackievirus Ebstein-Barr virus Hepatitis A, B, and C Cytomegalovirus Treatment Prednisone 60mg/day X 7 days Acyclovir 800mg 5X/day for 7 days Valacylovir 1000mg TID for 7 days Artificial Tears Bells Palsy

Bells Palsy- Treating Ourselves? • Prednisone treatment for idiopathic facial paralysis (Bell's palsy). N Engl J Med 1972 Dec 21; 287(25): 1268-72; 89% pred, 64% placebo • Cochrane Database 2002- Corticosteroids for Bell's palsy (idiopathic facial paralysis). No sufficient support for steroids • Cochrane Database 2004- randomized(?) trials of acylovir or valtrex with or without steroids for treatment of bells palsy ; insufficient evidence for support of antiviral medications • Valacyclovir and prednisolone treatment for Bell's palsy: a multicenter, randomized, placebo-controlled study; Otol Neurotol.2007 Apr;28(3):408-13. N=221; 6-8% improvement in severity and complete remission

Ramsey-Hunt Syndrome • Herpes Zoster Oticus; HSV1, HSV2, VZV • Triad of auricular pustules, ear pain, ipsilateral facial paralysis • +/- Hypoaguseia and hyperacusis • Worse prognosis

Question 11 • 38 y/o female with a history of epilepsy presents with multiple seizures without return to consciousness for 30 minutes. Her finger stick is 100 and her blood ICON is negative. The patient has been given 4 mg of ativan X2 but continues to seize. What is your next step? • 4 mg Midazolam • 8 mg Ativan • Vitamin B6 • Fosphenytoin load • Succinylcholine and etomidate with ETT

Fosphenytoin Load • Status Epilepticus • 30 minutes of seizure activity without return of consciousness • If seizure >4-5 minutes consider status; neuronal injury- must wake up! • Non-convulsive- EEG! • Treatment of status based on universal guidelines and institutional protocol • Treatment and investigation parallel

1/3rd new onset 1/3rd epilepsy 1/3rd: Idiopathic Hyper/hyponattremia Hypercalcemia Hypoglycemia CVA Trauma Infectious Mass HE Toxins INH Tricyclics (AVR, QRS) Theophylline Cocaine Sympathomimetics Alcohol withdrawal Organophosphates (strychnine) DM medications (glucose) Status Epilepcitcus

Status Epilepticus 1st Line: Ativan 4 mg over 2 minutes q5 min X2 • If no access 20mg diazepam pr, 10mg midazolam IM • 2nd Line: IV Fosphenytoin (20mg/kg at 150mg/min; may add 10mg/kg) • May give IV Keppra, Valproic Acid, Phenobarbitol if pt is on it • 3rd Line: Pentobarbitol, Intubation with continuous drip of midazolam or propofol • Other: Vitamin B6 (70mg/kg up to 5 )

Neurology Board Review