Lhermitte-Duclos disease
Encyclopedia
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. It was described by Jacques Jean Lhermitte and P. Duclos in 1920.
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.
It is important that only that part of the tumor (25-30%) is taken out so that there is no impact on neighboring areas.
Ganglioglioma
Ganglioglioma is a tumour that arises from ganglion cells in the central nervous system.The term "gangliocytoma" is sometimes equated with ganglioglioma. However, it is also sometimes equated with ganglioneuroma. In this context, the glial nature of the tumor is de-emphasized...
of the cerebellum, LDD) is a rare, slowly growing tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
of the cerebellum
Cerebellum
The cerebellum is a region of the brain that plays an important role in motor control. It may also be involved in some cognitive functions such as attention and language, and in regulating fear and pleasure responses, but its movement-related functions are the most solidly established...
, sometimes considered as hamartoma
Hamartoma
A hamartoma is a benign, focal malformation that resembles a neoplasm in the tissue of its origin. This is not a malignant tumor, and it grows at the same rate as the surrounding tissues. It is composed of tissue elements normally found at that site, but which are growing in a disorganized mass...
, characterized by diffuse hypertrophy
Hypertrophy
Hypertrophy is the increase in the volume of an organ or tissue due to the enlargement of its component cells. It should be distinguished from hyperplasia, in which the cells remain approximately the same size but increase in number...
of the stratum granulosum
Stratum granulosum
The stratum granulosum is a thin layer of cells in the epidermis. Keratinocytes migrating from the underlying stratum spinosum become known as granular cells in this layer...
of the cerebellum. It is often associated with Cowden syndrome
Cowden syndrome
Cowden syndrome is a rare autosomal dominant inherited disorder characterized by multiple tumor-like growths called hamartomas and an increased risk of certain forms of cancer....
and is pathognomonic
Pathognomonic
Pathognomonic is a term, often used in medicine, that means characteristic for a particular disease. A pathognomonic sign is a particular sign whose presence means that a particular disease is present beyond any doubt...
for this disease. It was described by Jacques Jean Lhermitte and P. Duclos in 1920.
Epidemiology
Lhermitte-Duclos disease is a rare entity; approximately 221 cases of LDD have been reported in medical literature. It's most common in the third and fourth decades of life.Clinical signs
Main clinical signs are:- headacheHeadacheA headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
- movement disorders
- tremorTremorA tremor is an involuntary, somewhat rhythmic, muscle contraction and relaxation involving to-and-fro movements of one or more body parts. It is the most common of all involuntary movements and can affect the hands, arms, eyes, face, head, vocal folds, trunk, and legs. Most tremors occur in the...
- visual disturbances
- abnormal EEGEEGEEG commonly refers to electroencephalography, a measurement of the electrical activity of the brain.EEG may also refer to:* Emperor Entertainment Group, a Hong Kong-based entertainment company...
- Diplopia
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
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.
External links
- MedPix: Lhermitte-Duclos — Radiology and Pathology