Lhermitte–Duclos disease
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| Lhermitte-Duclos disease (LDD) | |
|---|---|
| Other names | Dysplastic gangliocytoma of the cerebellum (DGC) |
 | |
| Lhermitte-Duclos disease histology | |
| Specialty | Neuro-oncology |
Lhermitte–Duclos disease (LDD) (English:
Signs and symptoms
Main clinical signs and symptoms include:[citation needed]
Patients with Lhermitte–Duclos disease and Cowden's syndrome may also have multiple growths on skin. The tumor, though benign, may cause neurological injury including abnormal movements.
MICROSCOPY (lhermitte-duclos disease) 1>Enlarged circumscribed cerebellar folia 2>internal granular layer is focally indistinct and is occupied by large ganglion cells 3>myelinated tracks in outer molecular layer 4>underlying white matter is atrophic and gliotic
Pathophysiology
In Lhermitte–Duclos disease, the
Diagnosis
![[icon]](/File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by adding to it. (May 2017) |
Treatment
Treatment is not needed in the asymptomatic patient. Symptomatic patients may benefit from surgical debulking of the tumor. Complete tumor removal is not usually needed and can be difficult due to the tumor location.[citation needed]
Epidemiology
Lhermitte–Duclos disease is a rare entity; approximately 222 cases of LDD have been reported in medical literature.[4] Symptoms of the disease most commonly manifest in the third and fourth decades of life, although it may onset at any age.[5] Men and women are equally affected, and there is not any apparent geographical pattern.[5]
History
The disease was first described in 1920 by Lhermitte and Duclos.[5]
See also
- Multiple hamartoma syndrome
- List of cutaneous conditions
References
External links
- Lhermitte-Duclos syndrome at Who Named It?
- MedPix: Lhermitte-Duclos — Radiology and Pathology