What is hypothalamic hamartoma? What are its symptoms and how is it treated?

Below, find frequently asked questions about hypothalamic hamartoma:

What is hypothalamic hamartoma?

Gelastic epilepsy typically is the end result of an unusual brain tumor called hypothalamic hamartoma. These typical seizures consist of a variety of types; most notably uncontrollable laughing spells. The hamartoma itself is generally considered benign and does not need treatment from a tumor perspective. Other associated problems with these lesions include developmental delay, behavioral problems and endocrinological disorders.

What are the symptoms of hypothalamic hamartoma?

An aura is usually the first symptom experienced during seizures. The type of symptom experienced depends upon the part of the brain that is affected first. Some of the typical auras include episodes of "déjà-vu" (a sense that one has been in a certain situation before), an uncomfortable abdominal sensation of “rising," numbness or tingling, unexplained tastes or odors or sometimes even a “hard to describe feeling.” Auras usually occur in patients with partial epilepsy, although not all patients with partial epilepsy experience auras.

How is hypothalamic hamartoma treated?

Treatment to date has revolved around the use of anti-convulsant medications. This has led to disappointing results and surgical options have been explored. Options tried in the past include surgical resection, stereotactic radiation, radiofrequency ablation, and vagal nerve stimulation. Results have not been well documented in the medical literature, but the two most promising treatment options include microsurgical resection and stereotactic radiosurgery.

Microsurgical resection has been reported by few centers and techniques are varied. A recent series in Australia suggests the transcallosal, transforniceal approach to the hypothalamic tumor may yield better results than other more traditional approaches which are considered risky, secondary to the deep location of the tumor. This newly described technique involves a small craniotomy on top of the head with an approach to the tumor in between the two hemispheres of the brain. The tumor is then finally approached and resected through the roof of the third ventricle. Despite initial promising results, it remains too early to know the long term efficacy of resection and or disconnection of the tumor from the underlying hypothalamus. Early results suggest a seizure free rate of 50-70 percent, which is clearly better than other options.

What are the surgery risks for hypothalamic hamartoma?

Besides the usual risks of infection and bleeding, surgery in, and about, the third ventricle/hypothalamus area carries significant neurologic risks. These include hydrocephalus, stroke and death. Risks specific to the hypothalamus itself include eating disorders, aggression, trouble with regulating sodium balance in the body, level of consciousness and wakefulness difficulties, and memory disturbances. Memory disturbances appear to be the most commonly seen post-operative deficit and usually are transient.

What treatments for hypothalamic hamartoma does Cleveland Clinic offer?

At Cleveland Clinic, the pediatric epilepsy and neurosurgical epilepsy programs along with endocrinology and neuroradiology have begun a comprehensive program to treat this difficult clinical disorder. Surgery is the first step in achieving a better quality of life for this disabling problem.

Reviewed by a Cleveland Clinic medical professional.

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