NEURORAD PPT SONALI
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Transcript of 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