Lhermitte-Duclos disease

Common Name(s)

Lhermitte-Duclos disease

Lhermitte-Duclos disease (LDD) is a very rare, benign (non-cancerous) brain tumor, called a dysplastic gangliocytoma of the cerebellum, that is characterized by abnormal development and enlargement of the cerebellum, and an increased intracranial pressure. LDD manifests most commonly in the third and fourth decades of life. Symptoms may include headache, nausea, cerebellar dysfunction, hydrocephalus, ataxia (problems with movement and coordination), and visual disturbances. Other features may include an enlarged brain (megalencephaly), hydromyelia, extra fingers or toes (polydactyly), partial gigantism, and/or a large tongue (macroglossia). Lhermitte-Duclos disease can occur as an isolated condition; it is also associated with a hereditary cancer syndrome called Cowden disease. Although the exact cause is unknown, mutations in the PTEN gene have been identified in some individuals with LDD.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Lhermitte-Duclos disease" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Lhermitte-Duclos disease" returned 18 free, full-text research articles on human participants. First 3 results:

Unusual presentation in adult medulloblastomas: imaging features mimicking cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease).
 

Author(s): Amey Savardekar, Pravin Salunke, Chirag K Ahuja, Swapnil Rane, Navneet Singla

Journal: Neurol India. ;60(5):555-7.

 

Last Updated: 8 Nov 2012

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Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) as a component of Cowden syndrome.
 

Author(s): Aparna Govindan, S Premkumar, Jacob P Alapatt

Journal: Indian J Pathol Microbiol. ;55(1):107-8.

 

Last Updated: 13 Apr 2012

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Bilateral Lhermitte-Duclos disease.
 

Author(s): Mustafa Bozbuga, Ilker Gulec, Hikmet Turan Suslu, Cicek Bayindir

Journal: Neurol India. ;58(2):309-11.

 

Lhermitte-Duclos disease (LDD) is a pathologic entity with progrediating, diffuse hypertrophy chiefly of the stratum granulosum of the cerebellum. Typically LDD is a unilateral lesion of the cerebellum or in vermis. Here we report a case of LDD with bilateral lesions of cerebellar ...

Last Updated: 28 May 2010

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Lhermitte-Duclos disease" returned 3 free, full-text review articles on human participants. First 3 results:

Lhermitte - Duclos disease with syrinx: case report and literature review.
 

Author(s): Khursheed Nayil, Maqbool Wani, Altaf Ramzan, Feroze Shaheen, Iqbal Lone, Abrar Wani

Journal: Turk Neurosurg. 2011 ;21(4):651-4.

 

Lhermitte-Duclos disease is typified by a hamartomatous lesion of the cerebellum. It is usually seen in females. The usual presentation is of raised intracranial pressure along with cerebellar signs.

Last Updated: 23 Dec 2011

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Recurrent Lhermitte-Duclos disease--case report.
 

Author(s): H Hashimoto, J Iida, K Masui, N Nishi, T Sakaki

Journal: Neurol. Med. Chir. (Tokyo). 1997 Sep;37(9):692-6.

 

A 43-year-old male presented with recurrent Lhermitte-Duclos disease (LDD), a rare pathological entity of the cerebellum of which the etiology is still controversial. He had undergone subtotal removal of a cerebellar lesion, misdiagnosed as a benign astrocytoma, 8 years previously. ...

Last Updated: 21 Nov 1997

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Association of Lhermitte-Duclos and Cowden disease: report of a new case and review of the literature.
 

Author(s): M Vinchon, S Blond, J P Lejeune, I Krivosik, P Fossati, R Assaker, J L Christiaens

Journal: J. Neurol. Neurosurg. Psychiatr.. 1994 Jun;57(6):699-704.

 

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 ...

Last Updated: 15 Jul 1994

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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