How rare is Lhermitte-Duclos?

How rare is Lhermitte-Duclos?

Epidemiology. Lhermitte–Duclos disease is a rare entity; approximately 222 cases of LDD have been reported in medical literature. Symptoms of the disease most commonly manifest in the third and fourth decades of life, although it may onset at any age.

What is cerebellar dysplasia?

Disorganized development of the cerebellum causes cerebellar dysplasia in which the shape and “texture” of the cerebellum appear abnormal due to the presence of abnormal folia pattern or gray matter nodular heterotopia.

What is Lhermitte Duclos Syndrome?

Summary. Listen. 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.

Is the dysplastic gangliocytoma of the cerebellum benign?

Conclusions: Dysplastic gangliocytoma of the cerebellum is of benign behaviour and its incidence is extremely rare. The disease should be considered when confronted with a young adult presenting with clinical signs of progressive mass effect in the posterior fossa.

Can a gangliocytoma be removed after re-growth?

Complete resection (removal) of supratentorial gangliocytomas can be achieved in more than 75% of cases. Even after subtotal (partial) resection, clinically relevant re-growth of the tumor is rare. Therefore, although total resection is optimal, the outlook is reportedly good even after subtotal resection.

How old do you have to be to have gangliocytoma?

Gangliocytoma is a rare type of central nervous system (CNS) tumor made up of mature neurons. Gangliocytomas may occur in all age groups but most often occur in people between the ages of 10 and 30.

What are the signs and symptoms of gangliocytoma?

Signs and symptoms may depend on the tumor’s location and may include seizures (most commonly); increased brain pressure; endocrine disorders; and focal symptoms. Gangliocytomas are generally slow-growing and usually do not become malignant. Treatment involves surgical removal of the tumor.