Question:

What differentiates CADASIL from Binswanger disease?
1. Bimhemispheric white matter signal change on MR imaging.
2. Abnormal lactate and pyruvate serum levels.
3. Periventricular ovoid configuration white matter signal change.
4. Hypertension.
5. Familial history.





Answer:

The correct answer for the question "What differentiates CADASIL from Binswanger disease?" is:

4. Hypertension.



Explanation
1. Although typical features of white matter T2 signal abnormality by location in CADASIL can suggest the diagnosis in an appropriate clinical setting, Binswanger disease also has bihemispheric white matter T2 signal change.  [A characteristic MRI imaging feature to CADASIL is the presence of T2 hyperintensity white matter lesions in the anterior temporal poles and external capsules. Associated white matter hyper intense T2 signal is commonly seen in the periventricular and deep white matter. Additional typical features include focal or subcortical white matter increased T2 signal changes in the superior frontal lobes. These lesions are associated with true restricted diffusion in the acute phase with no associated with enhancement after gadolinium administration. In Binswanger disease, focal increased white matter T2 signal change is identified in the deep grey matter structures, corona radiate and centrum semi ovale with identification of true restricted diffusion in the acute phase.]

2. This is the pathognomonic feature of MELAS.  [MELAS is a maternally transmitted inherited disorder that is readily diagnosed with abnormal serum pyruvate and lactate levels.]

3. These are MR imaging features of multiple sclerosis.  [Ovoid configuration white matter T2 signal abnormality distributed in a periventricular distribution pattern is typical for multiple sclerosis, with active lesions demonstrating enhancement with or without increased true diffusion signal.]

4. [The clinical presentation and course of the disease bears a striking resemblance to Binswanger disease (Chronic hypertensive encephalopathy). CADASIL is however familial with no history of hypertension or other vascular risk factors.]

5. Binswanger disease in non-familial.  [CADASIL is familial with no history of hypertension or other vascular risk factors.]



From the manuscript:
Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL
Radiology Case. 2013 Mar; 7(3):8-15


This article belongs to the Neuro section.




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From the manuscript

Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL

Free full text article: Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL

Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common form of hereditary cerebral angiopathy. We present a case in which a pattern of diffusion signal change compatible with bihemispheric acute watershed infarcts occurred in a symptomatic patient demonstrating global hypoperfusion. To our knowledge, watershed infarcts in the clinical presentation of CADASIL have not been previously described.






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1. Cerebral autosomal dominant arteriopathy with subcortical infracts and leukoencephalopathy

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