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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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 20 free, full-text research articles on human participants. First 3 results:

Lhermitte-Duclos disease.
 

Author(s): Sayg Uygur, Marina Coelho de Andrade, Carolina de Almeida Ito Brum, Andrea Lima Cruz Monerat, José Alberto Landeiro, Marcus André Acioly

Journal: Arq Neuropsiquiatr. 2014 May;72(5):392-3.

 

Last Updated: 27 May 2014

Go To URL
Lhermitte-Duclos disease. A case report.
 

Author(s): Andrea Giorgianni, Carlo Pellegrino, Alessandro De Benedictis, Anna Mercuri, Fabio Baruzzi, Renzo Minotto, Antonio Tabano, Sergio Balbi

Journal: Neuroradiol J. 2013 Dec;26(6):655-60.

 

Lhermitte-Duclos disease is a rare pathologic condition consisting of a dysplastic gangliocytoma of the cerebellum. Its association with phacomatosis and an autosomal dominant neoplastic syndrome, Cowden's syndrome is also known. Modern neuroimaging contributes to a correct diagnosis ...

Last Updated: 20 Dec 2013

Go To URL
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

Go To URL

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

Go To URL
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

Go To URL
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

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.