neurorad ppt sonali

8/8/2019 NEURORAD PPT SONALI

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Sonali Patel

Rush Medical College, MS4


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Historyy 41 yo Caucasian female

y CC: episode of LUE weakness followed by L. facial

numbnessy ROS: also c/o nausea, dizziness, and photophobia;

denies any other neurologic complaints, any priorepisodes, or any headaches

y PMH: Epilepsy (last seizure 20 years ago), Ex smoker

y FH:y Mother: MS, Deceased 2/2 breast cancer

y Sister: MS, ? vasculitis


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Physical Examy No focal abnormalities

yNAD, AAOx3, following all commands at baseline

y CN II-XII intact

y Motor: normal tone , 5/5 throughout

y Sensation: intact to light touch & pinprick

throughouty Reflexes: +2 throughout, downgoing toes bilaterally

y Cerebellar: normal FTN, RAM normal

y Gait: Steady, within normal limits


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Labs and Imagesy CSF:

- Glucose: 58- WBC: 0

- Protein: 25- Myelin Basic Protein < 2.0- NO oligoclonal banding- IgG: 2.5, IgG%: 11, IgG index: 0.53- Pre-alb: 4, Alb: 59, Globulin: 37

y CBC, Chem 7, Ca, Mg, PO4, and Coags: NORMALy ESR: 15y ANA: negativey

Protein Electrophoresis: nL, no monoclonal paraproteins


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Differential Diagnosis

y Demyelinating Disease:

y

Multiple Sclerosisy Lyme disease

y Acute Disseminated Encephalomyelitis

yVasculitis

y Microvascular Diseasey CADASIL


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CADASIL MS


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Sonali Patel, MS4


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IntroductionyVery Rare

y Autosomal Dominant

y Mutation on NOTCH 3 gene on chromosome 19q12

y MeanAge at Onset of Symptoms: 46

y MeanAge at Death: 61


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Pathophysiologyy Notch 3 involved in cell fate of vascular smooth muscle

cells vessel development, vascular remodeling after

injury

y Accumulation of the ectodomain of the Notch 3receptor within blood vessels

y Normally cleavage ofNotch3 receptor results in largeextracellular and small intracellular fragment

y Mutation leads to arteriopathy


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y Widespread vasculopathy:y Non-arteriosclerotic and amyloid

angiopathy

y Affect the leptomeningeal and perforatingarteries, skin, and muscles

y Pathological hallmark: granularosmiophilic material (GOM) in the media

y Studies on brain perfusion and brainmetabolism:y Significant reductions of cerebral blood

flow, blood volume and glucose utilization

in 30s

U

nderlyingM

ech

anism


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Clinical Manifestations

y Recurrent ischemic events ( TIAor Stroke): 85%

y Cognitive Deficits: 60%

y Migraine with aura: 30%

y Psychiatric disturbances: 25-30%


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A

ge at Onset


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Clinical Manifestationsy CADASIL coma: up to 10%

y Seizures: 5-10%

y Overall course of CASASIL is highly unpredictable;

y Early onset = rapid progression

y Difficulty walking ~60 yoa

y Bedridden ~65 yoa

y Mean age of death ~65-71 yoa


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MRI

yThree common findings:

yAnterior temporal lobe and external

capsule hyperintensities: on T2 weightedsequences

ySubcortical lacunar lesions: best seen on

FLAIR imagesyCerebral microbleeds: small, round, dark

lesions on T2-weighted images


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MRI


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D

iagnosis


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y Support and counseling

y Symptomatic treatment: Migraine prophylaxis,antidepressants

y Risk reduction: Stroke prevention: antihypertensive

and lipid lowering agents (as indicated)

y Contraindicated: Triptans, Tricyclics, and angiography

Management


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QUESTIONS?


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y Dont be lazy

y Dont page radiology at 3 am for nonsense

y Dont order images ifI dont know whyIm ordering them

y Dont get imaging of the paranasal sinuses

y Dont ask to check for a bleed on a head CT due to a dropin Hgb

y Dont page the resident without seeing the patient and

ordering other necessary labsAnd finally

y Dont make the radiologist feel like theyre talking to a treestump

What I

Learned

neurorad ppt sonali

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