Lhermitte Duclos Disease in the Absence of Cowdens - A Varied Presentation
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منابع مشابه
Lhermitte Duclos Disease in the Absence of Cowdens - A Varied Presentation
Background: Lhermitte-Duclos is a rare hamartomatous lesion of the cerebellar cortex with both neoplastic and hamartomatous features. Also known as dysplastic cerebellar gangliocytoma, they usually occur in the setting of Cowden’s syndrome, an autosomal dominant condition characterized by multiple hamartomas and neoplastic lesions in skin and internal organs. Although enlargement of the interna...
متن کاملLhermitte - duclos Disease :
Lhermitte-Duclos disease (LDD) is a benign neoplasm of posterior fossa, involving cerebellum. It is also known as dysplastic cerebellar gangliocytoma. It is not a true neoplasm but a hamartoma. It can be either isolated finding or associated with Cowden (multiple hamartoma syndrome). adults.
متن کاملLhermitte-Duclos disease.
Lhermitte-Duclos disease was first described in 1920 in the literature under the names of Purkinjeoma, granular cell hypertrophy of the cerebellum, hamartoma of the cerebellum, dysplastic gangliocytoma, ganglioneuroma, and gangliomatosis of the cerebellum. Patients tend to be young adults and may present with signs of cerebellar dysfunction or increased intracranial pressure secondary to obstru...
متن کاملLhermitte-Duclos disease.
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) is a rare benign cerebellar mass of unknown etiology which is characterized by enlargement of the cerebellar folia. Despite the controversy regarding its pathogenesis, imaging and histopathological findings are rather typical. A 17-year-old female presented with a 2-year history of progressive headaches and gait imbalance. Cr...
متن کاملLhermitte – Duclos Disease in a Young Adult: Rare Entity
Lhermitte - Duclos disease also called dysplastic gangliocytoma of cerebellum is an extremely rare cerebellar lesion which share features of both malformation and neoplasm. The usual presentation is of raised intracranial pressure along with cerebellar signs. We report a case of 23 year male who presented with headache & diplopia. MRI was suggestive of the diagnosis. Subtotal excision of th...
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ژورنال
عنوان ژورنال: Journal of Neurological Disorders
سال: 2016
ISSN: 2329-6895
DOI: 10.4172/2329-6895.1000260