J Korean Neurol Assoc.
1988 Jun;6(1):91-96.
A Case of cerebrotendinous Xanthomatosis Associated with Cerebral Infarction
- Affiliations
-
- 1Department of Neurology, National Medical Center, Seoul, Korea.
- 2Department of Radiology, College of Medicine, Yonsei University.
Abstract
- Cerebrotendinous xanthomatosis-is a very rare, automsomal recessive disorder which characterized by xanthomatous deposits in multiorgan system. The main clinical features include pyramidal, cerebellar and brain stem dysfunction, juvenile cataracts, dementia and xanthomas, especially on Achilles tendon and other tissues. We experienced a patient, 43 year old male who showed cataract, mild cerebellar dysfunction and multiple xanthomas as well as recently developed right hemiparesis. The tendinous xanthomatosis was confirmed pathologically and the brain CT scan showed cortical atrophy, demyelination and multifocal cerebromalacia, which correlated with neurologic manifestation of this patient and with neuropathologic findings of cerebrotendinous xanthomatosis. The EEG showed generalized slowing of background activities.
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- A Case of Cerebrotendinous Xanthomatosis
- Cerebrotendinous Xanthomatosis With Spinal Cord Syndrome
- Sonographic Findings of Polyneuropathy Associated With Cerebrotendinous Xanthomatosis: A Case Report
- Normolipemic Tendinous and Tuberous Xanthomatosis: One Case Report
- A Case of preeumect Cerebrotedinous Xanthomatosis
