Ramsay-Hunt Syndrome Type II
Definition
Ramsay-Hunt syndrome type II is a very rare, progressive neurological disorder that causes epilepsy, tremor, mental impairment, and eventually death.
Description
Ramsay-Hunt syndrome type II begins in adulthood. It is a relentlessly progressive degenerative disease that culminates in death, characterized by Parkinson-like tremors, and muscle jerks (myoclonus).
Demographics
The average age of onset is about 30 years of age.
Causes and symptoms
Some cases seem to be caused by abnormalities of the mitochondria within the cell. Mitochondria are the cells' power stations. They are organelles within each cell that are responsible for producing energy.
Some cases of Ramsay-Hunt syndrome type II appear to be inherited in an autosomal dominant fashion, meaning that a child who has one parent with the abnormal gene has a 50:50 chance of inheriting the disorder. Other cases appear to be inherited in an autosomal recessive fashion, meaning that individuals who develop the disease have inherited defective genes from both parents.
Ramsay-Hunt syndrome type II begins as an intention tremor in the limbs, particularly the arms. An intention tremor is an involuntary shaking or trembling that occurs when an individual is attempting a purposeful movement; the tremor is not manifested when the individual is at rest. The intention tremor generally occurs in just one limb. Over time, the entire muscular system is affected. In addition to the tremor, individuals with Ramsay-Hunt syndrome type II experience sudden twitching or contraction of muscle groups, called myoclonus. Some individuals experience progressive hearing impairment. As the disease progresses, the individual experiences decreased muscle tone, increasing weakness, disturbances of fine motor control, difficulty walking, epilepsy, and (in some cases) mental deterioration. The disease usually progresses over the course of about 10 years, ultimately resulting in the death of the patient.
Diagnosis
An electroencephalogram (EEG) may reveal certain abnormalities of the electrical patterns in the brain. Muscle biopsy may or may not reveal mitochondrial abnormalities.
Treatment team
Ramsay-Hunt syndrome type II is usually diagnosed and treated by a neurologist. In an effort to maintain functioning as long as possible, other treatment members may include physical therapists, occupational therapists, and speech and language therapists.
Treatment
There is no cure for Ramsay-Hunt syndrome type II. Seizures may respond to antiseizure medications such as phenobarbital, clonazepam, or valproic acid. The involuntary muscle jerking (myoclonus) may decrease with such medication as valproic acid; benzodiazepines such as clonazepam; L-tryptophan; 5-hydroxytryptophan with carbidopa; or piracetam.
