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Signs & Symptoms of Alexander Disease

by Maria Tussing

Alexander disease is a rare disease that affects the nervous system and is part of a group of disorders called leukodystrophies. These diseases involved the destruction of myelin, the fatty covering that insulates nerve fibers and encourages the rapid transmission of nerve impulses. This results in the disruption of nerve impulses, impairing nervous system functions, according to the National Institute of Neurological Disorders and Stroke. Alexander disease is a progressive disease, meaning it gets worse over time, and there is no cure. Alexander disease is caused by a genetic defect, though not inherited. Rather the defect appears to occur spontaneously. There are three types of Alexander disease; infantile, juvenile and adult.
Infantile Alexander Disease

Alexander disease most commonly occurs in children under age two. This disease affects all parts of the body. In children with Alexander disease the brain is often enlarged, which can result in seizures, delayed development, brain and spinal cord dysfunction and convulsive disorders. Hydrocephaly, or "water on the brain," is actually an accumulation of fluid between the brain and the skull. This can result in unusually large head size and developmental defects. Since the brain is affected by this disease, it also affects all other parts of the body. The child may not gain weight and grow at expected rates. They may suffer from seizures, muscle spasms, difficulty walking and mastering speech and mental regression. Infantile Alexander disease is sometimes fatal before the child reaches a year old, but some children with this disease live for five to 10 years.

Juvenile Alexander Disease

Juvenile onset Alexander disease usually starts showing symptoms between four and 10 years of age. Children with this disease have difficulty talking and swallowing and are unable to cough. Extremities may become weak and the child may have muscle spasms, especially in the legs. Lack of coordination is typical. Children with this disease may suffer mental decline, but sometimes the mental faculties stay intact. They may also show signs of kyphoscoliosis, a front-to-back curvature of the spine.
Adult Alexander Disease

Adult onset Alexander disease is the rarest and usually most mild form of the disease. It may begin anytime from the late teens to much later in life. Symptoms in this form are often so mild that the disease isn't diagnosed until an autopsy is done. The adult form of this disease shares many symptoms with the juvenile type; difficulty swallowing, sleep disturbances and speech impairment. Older patients may also suffer from impaired coordination. The symptoms of the adult form of Alexander disease sometimes resemble those of a tumor, multiple sclerosis or Parkinson's disease.