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A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59‐year‐old woman
Author(s) -
Kaffenberger T.,
Valko P. O.,
Von Meyenburg J.,
Baráth K.,
Hewer E.,
Heppner F. L.,
Jagella C. E.,
Horst A.,
Siccoli M. M.
Publication year - 2009
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2008.02392.x
Subject(s) - medicine , leukoencephalopathy , magnetic resonance imaging , ataxia , pediatrics , cognitive decline , brain biopsy , radiology , pathology , dementia , psychiatry , disease
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.