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

[sociallocker id=”615″]

  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.

[/sociallocker]