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31 YEAR OLD FEMALE

1.    What is the Dx? A.    Rasmussen Encephalitis B.    Chronic Ischemic White Matter Disease C.    PRES D.    CADASIL 2.    What is the “C...

1.    What is the Dx?
A.    Rasmussen Encephalitis
B.    Chronic Ischemic White Matter Disease
C.    PRES
D.    CADASIL

2.    What is the “Classic History” ?
A.    Gelastic Seizures
B.    Precocious Puberty
C.    Migraines
D.    Ascending Paralysis

3.    White matter involvement where is “Classic” ?
A.    It classically spares white matter
B.    Temporal Lobes
C.    Parietal Lobes
D.    Occipital Lobes

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  1. What is the Dx?
    D. CADASIL
  2. What is the “Classic History” ?
    C. Migraines
  3. White matter involvement where is “Classic” ?
    B. Temporal Lobes

CADASIL
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy).
Essential Trivia:

  • It’s an inherited condition (hence the name). It typically presents between age 30-40.
  • The history of migraines (sometimes with aura) is classic. Depression and focal neurologic defects can also occur. For the purpose of multiple choice, I’d go with migraine.
  • Lots of things involve the frontal white matter. However, involvement of the temporal white matter -especially when severe in a 30 year old should make you say CADASIL.
  • The other classic finding is relative sparing of the occipital subcortical white matter and U-Fibers.
  • The MRA is usually normal, with minimal if any atherosclerosis.

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