A 49-year-old Japanese male with Lhermitte-Duclos disease subsequently developed a very rare association with Cowden's disease. Partial tumor removal established the diagnosis of Lhermitte-Duclos disease. Follow-up examinations discovered the presence of Cowden's disease. Long-term follow-up of patients with Lhermitte-Duclos disease is essential to identify signs of Cowden's disease, which carr...

Lhermitte-Duclos disease is a rare entity, and its pathological features are unique. Pathological findings are characteristic of the disease, with global hypertrophy of the cerebellum, coarse gyri, and the typical "inverted cortex" pattern. Several associated lesions were noted in many patients with Lhermitte-Duclos disease. It is only recently that an association between Lhermitte-Duclos disea...

Lhermitte Duclos disease also called dysplastic gangliocytoma of cerebellum is an extremely rare cerebellar neoplasm. It usually presents with raised intracranial pressure along with cerebellar signs. We report a rare case of Lhermitte Duclos disease of a 20 years male who presented with signs & symptoms of raised intracranial tension. CT features were suggestive of Lhermitte-Duclos disease. Su...

Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare cerebellar benign tumor with characteristic appearance of thickened cerebellar folia giving a laminated or striated appearance, quite diagnostic of the condition. We had seen a patient with medulloblastoma with imaging findings suspicious for thickened cerebellar folia reminiscent of Lhermitte-Duclos disease....

The expression of neurofilament (NF) proteins was examined in the surgical specimen from a 42-year-old woman with Lhermitte-Duclos disease. Hypertrophic granule cell neurons of the dysplastic tissues were reactive with monoclonal antibodies, including antibodies to each of the three human NF subunits. Furthermore, antibodies to dephosphorylation-dependent epitopes on NF proteins stained the cel...

Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, was first diagnosed by doctors Lhermitte and Duclos in 1920. In subsequent years, the disease was described by many researchers: Bielschowsky and Simons in 1930, Christensen in 1937, Duncan and Snodgrass in 1943, Ambler in 1969, and Padber in 1991. Each of the authors referred to this disease in a different way: diffus...

Since 1920, when Lhermitte and Duclos first described a tumorlike abnormality of the cerebellum, which has come to bear their name, there has been an increasing number of reports of this rare entity [1-10]. Most cases have not had radiographic correlation , but there are several reported cases in which CT was part of the pretreatment evaluation [2-7], and one case was demonstrated on low-field ...

A case of surgically confirmed Lhermitte-Duclos disease demonstrated contrast enhancement on MR. Histologic examination verified corresponding increased vascularity in the molecular layer and adjacent leptomeninges.

Cowden syndrome is an autosomal dominant condition of multiple hamartomas. Patients with this phakomatosis have an increased risk of breast cancer and thyroid tumours. Lhermitte-Duclos disease is usually a sporadic condition of cerebellar ganglion cell hypertrophy, ataxia, mental retardation, and self-limited seizure disorder. We describe a three generation family with Cowden syndrome and Lherm...