Document Detail

A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman.
MedLine Citation:
PMID:  19146646     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Leukoencephalopathy with cerebral calcifications and cysts (LCC) is a recently described, very rare entity, clinically characterized by progressive neurological deficits such as cognitive decline, epileptic seizures, pyramidal, extrapyramidal and cerebellar symptoms/signs. With the exception of two patients with adult onset, in all previously described cases symptoms onset occurred between early infancy and adolescence. RESULTS: We report a case of late onset LCC in a 59-year-old woman presenting with urinary and fecal incontinence and behavioural changes, then rapid progression with hemianopia, hemiparesis, ataxia and cognitive decline. Extensive work-up was performed, including brain magnetic resonance imaging, magnetic resonance spectroscopy, cyst fluid analysis and brain biopsy, confirming the final diagnosis of LCC. CONCLUSION: Our case supports the existence of a late onset adult form of LCC.
T Kaffenberger; P O Valko; J von Meyenburg; K Baráth; E Hewer; F L Heppner; C E Jagella; A Horst; M M Siccoli
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of neurology : the official journal of the European Federation of Neurological Societies     Volume:  16     ISSN:  1468-1331     ISO Abbreviation:  Eur. J. Neurol.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-01-16     Completed Date:  2009-04-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9506311     Medline TA:  Eur J Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  278-81     Citation Subset:  IM    
Department of Neurology, University Hospital of Zurich, Zurich, Switzerland.
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MeSH Terms
Age of Onset
Brain Diseases / pathology*,  physiopathology
Calcinosis / pathology*,  physiopathology
Cysts / pathology*,  physiopathology
Magnetic Resonance Imaging
Middle Aged

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