Lhermitte – Duclos Disease in a Young Adult: Rare Entity
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Abstract:
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 the mass along with shunt drainage was performed. Post operative course was uneventful. Histopathological features confirmed the diagnosis.
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lhermitte – 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...
full textLhermitte-duclos Disease-intraoperative Cytological Findings of a Rare Entity
OBJECTIVE: A rare case of LDD confirmed by biopsy is being reported. Review of the published cases shows that the disease usually manifests by the signs of increased intracranial pressure with inconstant cerebellar symptoms. MRI is the imaging modality of choice by now. As such, clinical problems after gross total or complete removal have not been reported so far, but herein we stress the impor...
full textLhermitte-duclos Disease in a Young Adult Case Report
Dysplastic gangliocytoma of the cerebellum is a very rare lesion. The other names assigned to this pathology are Lhermitte-Duclos disease. ganglioneuroma. hamartoma of the cerebellum. purkengioma. granule cell hypertrophy or granulomolecular hypertrophy of the cerebellum. Only few cases have been reported in the literature (1-20).since the first report by Lhermitte and Duclos (11).Clinically. t...
full textLhermitte - 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.
full textLhermitte-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...
full textLhermitte-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...
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Journal title
volume 8 issue 3
pages 194- 198
publication date 2013-07-01
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