Abstract
The clinical manifestations and prevalence of von Hippel-Lindau syndrome (HLS) were reported from the Department of Medicine, Albert-Ludwigs-Universitat, Freiburg im Breisgau, Germany, and Department of Pathology, University of Zurich, Switzerland.
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
A dysplastic gangliocytoma was removed from the left cerebellar hemi¬ sphere
Summary
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. She exhibited multiple papules on her nose, cheek, forehead and ears and on the oral mucosa. A dysplastic gangliocytoma of the left cerebellar hemisphere was diagnosed at surgical resection of the tumor. Skin lesions included papules on the face, 1 infos, and on the gingiva and tongue Siblings of both patients showed the mucocutaneous lesions, thyroid disease, breast tumors, ovarian tumors, and neurological signs including macrocephaly, mental retardation, sei¬
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