| Neurofibromatosis type I associated multiple sclerosis. | |
| | |
MedLine Citation:
|
PMID: 21591539 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
|
Neurofibromatosis (NF) type I is a common autosomal dominant disease that principally affects the skin and peripheral nervous system. Neurofibromatosis type I associated multiple sclerosis is a very rare condition. A 28-year old NF1 man developed progressive spastic-ataxic gait, left side dysmetria, right internuclear ophthalmoplegia, spastic dysarthria. MRI of the brain depicted Dawson finger appearance demyelination of the corpus callosum and other multifoci demyelinating lesions typical for MS. CSF revealed high CSF protein with negative oligoclonal band. Visual evoked potential showed prolonged P100 latency, abnormal waveform and temporal dispersion bilaterally. The syndrome partially responded and stabilized with corticosteroid. Six months later progression of the syndrome characterized by paraparesis, bilateral cerebellar hemispheric syndrome and bilateral internuclear ophthalmoplegia occurred. Repeated MRI revealed more extensive white matter lesions extended into centrum semiovale. The progressive syndrome did not respond to corticosteroid. Primary progressive multiple sclerosis was diagnosed. Only thirteen cases with NF1 and multiple sclerosis have been described in the literature. The association has been hypothesized to be related to mutations in the neurofibromin protein or oligodendrocyte-myelin glycoprotein (OMgp) gene. |
| | |
Authors:
|
Hemmarin Pipatpajong; Kammant Phanthumchinda |
Related Documents
:
|
7955609 - Asymmetrical osteoarthritis in a patient with ehlers-danlos syndrome and poliomyelitis. 11843319 - Ehlers-danlos syndrome type iv with few extrathoracic findings: a newly recognized poin... 5308459 - Gastrointestinal complications of the ehlers-danlos syndrome. 3710179 - Homograft of preserved sclera for post-traumatic scleral staphyloma in ehlers-danlos sy... 21459689 - Thyroide hormone resistance syndromes. 21324829 - Acth-independent macronodular adrenal hyperplasia (aimah) associated with insulinoma. 21798029 - Evaluation of natural language processing from emergency department computerized medica... 19128929 - Management of median arcuate ligament syndrome: a new paradigm. 16274859 - Pisiform ligament complex syndrome and pisotriquetral arthrosis. |
Publication Detail:
|
Type: Journal Article |
Journal Detail:
|
Title: Journal of the Medical Association of Thailand = Chotmaihet thangphaet Volume: 94 ISSN: 0125-2208 ISO Abbreviation: J Med Assoc Thai Publication Date: 2011 Apr |
Date Detail:
|
Created Date: 2011-05-19 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 7507216 Medline TA: J Med Assoc Thai Country: Thailand |
Other Details:
|
Languages: eng Pagination: 505-10 Citation Subset: IM |
Affiliation:
|
Division of Neurology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Lactic acidosis associated with severe neuromuscular weakness and stavudine therapy.
Next Document: Management of a large, recurrent simple cyst of the liver by total excision: a case report.