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A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman


Kaffenberger, T; Valko, P O; von Meyenburg, J; Baráth, K; Hewer, E; Heppner, F L; Jagella, C E; Horst, A; Siccoli, M M (2009). A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman. European Journal of Neurology, 16(2):278-281.

Abstract

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.

Abstract

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.

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12 citations in Web of Science®
15 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Neuroradiology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Neurology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:December 2009
Deposited On:14 Jan 2009 07:39
Last Modified:05 Apr 2016 12:49
Publisher:Wiley-Blackwell
ISSN:1351-5101
Publisher DOI:https://doi.org/10.1111/j.1468-1331.2008.02392.x
PubMed ID:19146646

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