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 -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

Vertigo 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

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.

Question 3 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

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 5-12 1 ___________13-15 0 ICH Vol >30 1 ___________<30 0 IVH Yes 1 ___________No 0 Infratentoral Yes 1 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) 2 minutes to work, sx gone in 15

Question 6 1. Stroke 2. Sciatica 3. Cauda Equina Syndrome 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

Cauda Equina Syndrome 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

Other options Epidural Abcess Sciatica Radicular Pain 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 Sciatica Radicular Pain Lateral or post leg to foot Straight leg raise (10-60), crossed Numbness, no weakness NSAIDS

Should I get the imaging….? 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”

Question 7 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

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

Double-blind, randomized, placebo controlled 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)

tPA Exclusion Criteria Evidence of ICH on CT History of ICH or AVM 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)

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)

Question 9 Right brainstem cva Cluster Headache Bells Palsy Tick Bite 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

Carotid Artery Dissection 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

Horners Syndrome 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

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

Question 10 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?

Bells Palsy- Not spoiled milk. 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 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- 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

Status Epilepcitcus Toxins INH Tricyclics (AVR, QRS) Theophylline 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 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 )

Question 12 A 35 year old female 1 week post-partum presents with 1 day of severe headache, nausea and vomiting. She is slightly confused and lethargic. She is afebrile, normo-tensive, with a negative UA. Given the clinical picture, what is the treatment of choice? PCC or FFP Emergent Craniotomy Serial lumbar punctures Magnesium Sulfate IV Heparin

Heparin, Venous Sinus Thrombosis Headache, nausea, emesis, ams, focal deficits; pesudotumor cerebri Women, peripartum, hypercoaguable states, systemic inflammatory conditions CT head, MRV Atypical ischemic or hemorrhagic region Tx: Heparin

Question 13 A 70 year old male presents to the ER with weakness in the leg upon waking this morning. His exam shows left leg 2/5 strength with ataxia of limb, 4/5 left arm strength, no facial droop. He keeps asking what time it is. Where is his lesion? Middle Cerebral Artery Anterior Cerebral Artery Posterior Cerebral Artery Basilar Artery Carotid Artery

Anterior Cerebral Artery Stroke Affects medial parietal, temporal, and frontal lobes Contralateral Motor and Sensory Leg > face and arm Dis-inhibition, perseveration, primitive reflexes

Basilar Artery Stroke Bilateral sx Coma Locked in syndrome

Question 14 A 23 year old patient presents is BIBEMS being bagged with a GCS of 3. His friend is with him and states that while doing “a lot” of cocaine his friend developed severe headache with sudden loss of conciousness. Which of the following considerations in further management is incorrect? Pretreat with lidocaine and consider fentanyl and vecuronium Do not allow single episode of hypoxia or hypotension Hyperventilate to pC02 25-30 Raise head of bed to 30 degrees Consider manitol or hypertonic saline for deterioration in neurologic status

Maintain pCO2 between 35-40, not any lower! Pretreatment Oxygen NRB Lidocaine 1.5mg/kg 3 minutes before Fentayl 2ug/kg Vecuronium .01mg/kg (De-fasciculating Dose) Intubation by most experienced MD; single episode of hypoxia associated with poor outcome Ventilation *Short term hyper-ventilation for nerologic deterioration *Maintain pCO2 35-40 *Long term hyper-ventilation not Rx

Management of elevated ICP CPP=MAP-ICP Maintain cerebral perfusion Do not lower BP by > 20% General rule is to maintain systolic between 160-180 A single hypotensive episode is assoicated with worse outcomes Tx hypotension with IVF Treatment of Increased ICP includes: -Mannitol -Raise Head of bed 30 D -Hypertonic Saline (future) -Hyperventilation -Surgical evacuation

Question 15 Hypoglycemia Metabolic Derangement Migraine CVA A 45 year old inmate with no pmhx presents with 1 hour of headache, right leg and arm paralysis, left forearm numbness, third right toe numbness, and a voice in his head telling him that he is hungry. Which of the following must you concsider in your differential? Hypoglycemia Metabolic Derangement Migraine CVA All of the above

All of the Above! Hypoglycemia (may be focal) Seizure, Todds paralysis (may last 24 hours) CNS infection Bells Palsy (forehead affected) Other Metabolic derangement Migraine (focal deficits possible) Conversion disorder Malingering Lower CNS lesion, trauma Toxic

THE END THANK YOU! Please also read -Parkinsons -Dimentia -Delerium -Multiple Sclerosis -Everything else!

Question 16 ? If you want more… A 22 year old female presents with double vision. The symptoms disappear with either eye is covered. Extraoccular movements are intact when tested individually. On conjugate gaze testing there is nystagmus in the left eye and limited adduction in the right eye. What is the most likely cause? 1. Dislocated Lense 2. Tertiary neurosyphilis 3. Internuclear Opthalmoplegia 4. Sixth Nerve palsy 5. Third Nerve palsy

Internuclear Opthalmoplegia Occurs due to disruption in the medial longitudinal fasciculus (MLF) Corrdinates conjugate eye movements Most commonly due to MS MS occurs in young women; deficits vary anatomically and temporally Each gaze center passes inputs to ipsilateral cranial nerves and sends a contralateral input through MLF to CN 3. Lesions can be unilateral or bilateral

Diplopia Monocular Refractive error Dislocated lenses Iridodialysis Malingering Binocular CN palsies Brain lesions HTN crisis Cocaine Wernicke’s SLE Retro-orbital mass/hematoma Binoccular= disappears when either eye is covered