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


CONCLUSIONS
Our case supports the existence of a late onset adult form of LCC.
Kaffenberger
T
T
Department of Neurology, University Hospital of Zurich, Zurich, Switzerland.
Valko
P O
PO
von Meyenburg
J
J
Baráth
K
K
Hewer
E
E
Heppner
F L
FL
Jagella
C E
CE
Horst
A
A
Siccoli
M M
MM
D017668:Age of Onset; D001927:Brain Diseases / Q000473:pathology* / Q000503:physiopathology; D002114:Calcinosis / Q000473:pathology* / Q000503:physiopathology; D003560:Cysts / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged