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.

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

 

 

General Support Organizations

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

Brain MRI features in Lhermitte-Duclos disease.
 

Author(s): Wladimir Bocca Vieira de Rezende Pinto, Paulo Victor Sgobbi de Souza

Journal: Arq Neuropsiquiatr. 2014 Aug;72(8):645.

 

Last Updated: 7 Aug 2014

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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

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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

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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 8 free, full-text review articles on human participants. First 3 results:

[The successful treatment of a patient with Lhermitte--Duclos disease (A case report and literature rewiew)].
 

Author(s): V N Shimanskiy, V V Karnaukhov, L V Shishkina, E V Vinogradov

Journal: Zh Vopr Neirokhir Im N N Burdenko. 2015 ;79(4):78-83.

 

Lhermitte--Duclos disease is a rare autosomal dominant inherited disorder characterized by the loss of the normal cerebellar cortex architecture and hamartoma formation in the cerebellar hemispheres. Most commonly, this disease manifests in the third and fourth decades of life. Approximately ...

Last Updated: 4 Nov 2015

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Lhermitte-Duclos Disease Treated Surgically in an Elderly Patient: Case Report and Literature Review.
 

Author(s): Hiroaki Matsumoto, Hiroaki Minami, Yasuhisa Yoshida

Journal: Turk Neurosurg. 2015 ;25(5):783-7.

 

A 75-year-old man with Lhermitte-Duclos Disease (LDD) manifesting as progressive headache is presented. Magnetic resonance imaging demonstrated a right cerebellar mass lesion with the characteristic "tiger-striped appearance". A mild mass effect was evident at the medulla oblongata, ...

Last Updated: 7 Oct 2015

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Similar MR imaging characteristics but different pathological changes: a misdiagnosis for Lhermitte-Duclos disease and review of the literature.
 

Author(s): Shengyue Huang, Guobin Zhang, Junting Zhang

Journal:

 

Although previous reports purpored that the unique magnetic resonance imaging (MRI) features of Lhermitte-Duclos disease (LDD) obviates the need for biopsy, we have made a misdiagnosis of LDD which has an indistinguishable imaging appearance. We present a patient who suffered from ...

Last Updated: 11 Aug 2015

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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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