Abstract
Two unrelated patients with clinical characteristics of both Lhermitte-Duclos disease and the autosomal dominant Cowden disease are reported from the Department of Neurology, University Hospital, Leiden, The Netherlands.
Highlights
Lhermitte-Duclos disease and the autosomal dominant Cowden disease are reported from the Department of Neurology, University Hospital, Leiden, The Netherlands
A dysplastic gangliocytoma of the left cerebellar hemisphere was diagnosed at surgical resection of the tumor
The second patient had a history of tonic-clonic seizures in childhood, thyroid surgery at age 38 and he presented with cerebellar signs at 48 years of age
Summary
Two unrelated patients with clinical characteristics of both Lhermitte-Duclos disease and the autosomal dominant Cowden disease are reported from the Department of Neurology, University Hospital, Leiden, The Netherlands. Patient 1 was a mentally retarded woman with a large head, a high arched palate, dysarthria, mild cerebellar signs, and a single seizure at the age of 37 years. CT scan showed a nonenhancing mass in the cerebellar vermis and left cerebellar hemisphere, enlarged third and lateral ventricles, and a hypodense lesion in the centrum semiovale of the left cerebral hemi¬
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