Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-10235
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.
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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.
|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
|DDC:||610 Medicine & health|
|Deposited On:||14 Jan 2009 08:39|
|Last Modified:||02 Dec 2013 17:18|
|Citations:||Web of Science®. Times cited: 6|
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