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A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman |
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| Authors: | T Kaffenberger P O Valko J von Meyenburg K Baráth E Hewer F L Heppner C E Jagella A Horst M M Siccoli |
| Institution: | Department of Neurology, University Hospital of Zurich, Zurich, Switzerland;;Institute of Neuroradiology, University Hospital of Zurich, Zurich, Switzerland;;Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland;;Department of Internal Medicine, Hospital of Winterthur, Switzerland;;and Department of Neuropathology, Charité, Universitätsmedizin Berlin, Germany |
| 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 |