Idiopathic progressive hyalinizing and calcifying CNS vasculopathy.
We present a female adolescent with intermittently progressive neurologic deficits over a period of 7 years. Serial imaging studies showed a calcifying lesion with increasing enhancement, signal abnormalities from the subcortex to the medulla, and hemispheric volume loss. Stereotactic biopsies disclosed a hyalinizing and calcifying vasculopathy involving the penetrating vessels. The vasculopathy may result in ischemic lesions responsible for the severe deficits.
Moussouttas
M
M
Department of Neurology, Mount Sinai Hospital, New York, NY, USA.
Morgello
S
S
Geraci
A
A
Chan
B
B
Wallace
S
S
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D001921:Brain / Q000098:blood supply / Q000473:pathology*; D002114:Calcinosis / Q000473:pathology* / Q000503:physiopathology; D002561:Cerebrovascular Disorders / Q000473:pathology* / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D007839:Functional Laterality; D006429:Hemiplegia; D006801:Humans; D008279:Magnetic Resonance Imaging; D008526:Medulla Oblongata / Q000473:pathology; D008833:Microcirculation / Q000473:pathology