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

Authors: Kaffenberger, T.¹; Valko, P. O.¹; von Meyenburg, J.²; Baráth, K.²; Hewer, E.³; Heppner, F. L.; Jagella, C. E.¹; Horst, A.⁴; Siccoli, M. M.¹

Source: European Journal of Neurology, Volume 16, Number 2, February 2009 , pp. 278-281(4)

Publisher: Wiley-Blackwell

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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.

Keywords: brain magnetic resonance imaging; cerebral calcifications; cysts; leukoencephalopathy; magnetic resonance spectroscopy

Document Type: Short communication

DOI: http://dx.doi.org/10.1111/j.1468-1331.2008.02392.x

Affiliations: 1: Department of Neurology, University Hospital of Zurich, Zurich, Switzerland 2: Institute of Neuroradiology, University Hospital of Zurich, Zurich, Switzerland 3: Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland 4: Department of Internal Medicine, Hospital of Winterthur, Switzerland

Publication date: 2009-02-01