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  • Title: A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman.
    Author: Kaffenberger T, Valko PO, von Meyenburg J, Baráth K, Hewer E, Heppner FL, Jagella CE, Horst A, Siccoli MM.
    Journal: Eur J Neurol; 2009 Feb; 16(2):278-81. PubMed ID: 19146646.
    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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