| Cerebellar leukoencephalopathy: most likely histiocytosis-related. | |
| | |
MedLine Citation:
|
PMID: 18936429 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
BACKGROUND: Histiocytosis, both Langerhans and non-Langerhans cell type, can be associated with cerebellar white matter abnormalities, thought to be paraneoplastic. The associated clinical picture consists of ataxia, spasticity, and cognitive decline. Hormonal dysfunction is frequent. MRI shows cerebellar white matter abnormalities, as well as brainstem and basal ganglia abnormalities. This so-called "neurodegenerative syndrome" may occur years before or during manifest histiocytosis and also years after cure. We discovered similar MRI abnormalities in 13 patients and wondered whether they could have the same syndrome. METHODS: We reviewed the clinical and laboratory information of these 13 patients and evaluated their brain MRIs. Seven patients underwent spinal cord MRI. RESULTS: All patients were isolated cases; 10 were male. They had signs of cerebellar and pyramidal dysfunction, behavioral problems, and cognitive decline. MRI showed abnormalities of the cerebellar white matter, brainstem, basal ganglia, and, to a lesser extent, cerebral white matter. Three patients had spinal cord lesions. Three patients had laboratory evidence of hormonal dysfunction. No evidence was found of an underlying metabolic defect. In two patients biopsy of nodular brain lesions revealed histiocytic infiltrates. CONCLUSIONS: Considering the striking clinical and MRI similarities between our patients and the patients with this neurodegenerative syndrome in the context of proven histiocytosis, it is likely that they share the same paraneoplastic syndrome, although we cannot exclude a genetic disorder with certainty. The fact that we found histiocytic lesions in two patients substantiates our conclusion. Patients with cerebellar white matter abnormalities should be monitored for histiocytosis. |
| | |
Authors:
|
M S van der Knaap; W F M Arts; J Y Garbern; G Hedlund; F Winkler; C Barbosa; M D King; A Bjørnstad; N Hussain; M K Beyer; C Gomez; M C Patterson; P Grattan-Smith; M Timmons; P van der Valk |
Related Documents
:
|
10938609 - Intrathecal baclofen alleviates autonomic dysfunction in severe brain injury. 2702569 - Subacute brain atrophy after radiation therapy for malignant brain tumor. 18854439 - Reduced gray matter volume in the frontotemporal cortex of patients with early subacute... 18633179 - Brain perfusion and cognitive function changes in hypertensive patients. 9210989 - Psychomotor agitation following gabapentin use in brain injury. 8856789 - Severe stealth virus encephalopathy following chronic-fatigue-syndrome-like illness: cl... 16737989 - Computed tomography and pulmonary function abnormalities in sickle cell disease. 15259889 - Recall and recognition are equally impaired in patients with selective hippocampal damage. 18394439 - Anti-cardiolipin antibodies and endothelial function in patients with coronary artery d... |
Publication Detail:
|
Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
|
Title: Neurology Volume: 71 ISSN: 1526-632X ISO Abbreviation: Neurology Publication Date: 2008 Oct |
Date Detail:
|
Created Date: 2008-10-21 Completed Date: 2008-11-06 Revised Date: 2009-08-12 |
Medline Journal Info:
|
Nlm Unique ID: 0401060 Medline TA: Neurology Country: United States |
Other Details:
|
Languages: eng Pagination: 1361-7 Citation Subset: AIM; IM |
Affiliation:
|
Department of Child Neurology, VU University Medical Center, Amsterdam, The Netherlands. ms.vanderknaap@vumc.nl |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Cerebellar Ataxia / complications, diagnosis, pathology Cerebellar Diseases / complications, diagnosis*, pathology Child Female Histiocytosis / complications, diagnosis*, pathology Humans Magnetic Resonance Imaging / methods Male Posterior Leukoencephalopathy Syndrome / complications, diagnosis*, pathology Retrospective Studies |
| Comments/Corrections | |
Comment In:
|
Neurology. 2009 Aug 11;73(6):488; author reply 488-9
[PMID:
19667329
]
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report.
Next Document: Invited commentary: cross-cultural influences on sleep--broadening the environmental landscape.