| Lhermitte-Duclos disease | |
|---|---|
| Classification and external resources | |
| OMIM | 158350 |
| MeSH | D006223 |
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum, LDD) is a rare, slowly growing tumor of the cerebellum, sometimes considered as hamartoma, characterized by diffuse hypertrophy of the stratum granulosum of the cerebellum. It is often associated with Cowden syndrome and is pathognomonic for this disease.[1] It was described by Jacques Jean Lhermitte and P. Duclos in 1920.[2]
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Lhermitte-Duclos disease is a rare entity; approximately 221 cases of LDD have been reported in medical literature.[3] It's most common in the third and fourth decades of life.
Cowden disease is caused by mutations of the PTEN gene.
Main clinical signs are:
Clinical signs may also go hand in hand with cowden's syndrome that includes multiple growths on skin. Headaches are very common. Though headaches might not be continuous, they come after some actions like food intake, lifting weights, up/down movements of the neck. Movement disorders typically make walking very difficult; one would find difficulty in placing successively steps on ground feel dizzy. Dizziness controls when one relaxes for a while; but starts again. Movement disorders also come with double vision. Double Vision is accompanied by headaches. For tumors in the lower part of the brain, the pain percolates to neck and upper back.
Indigestion and acidity are also important symptoms. During afternoons there might be a tendency of vomiting, and headaches might follow post vomiting.
On any of the conditions as mentioned above, an MRI should be done immediately to ascertain the size and position of the tumor, if any.
The tumor, though benign affects the functions of the brain. Its large size presses on the other vital organs in the brain, and squeezes the ventricles - this leads to the conditions as mentioned above.
Treatment involves surgery to understand the tumor better, the tumor in Lhermitte is mostly benign; once surgery is done; a part of tumor is taken out and given for Biopsy.
It is important that only that part of the tumor (25-30%) is taken out so that there is no impact on neighboring areas.
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