Alexander Disease

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Alexander disease is a rare and often fatal neurodegenerative disorder characterized by Rosenthal fibers forming in the astrocytes of the brain that primarily occurs in infants and children.

Alexander disease has been divided into three forms  based on age of onset and type of symptoms: infantile, juvenile, and adult forms.

Alexander disease caused by a dominant mutation in the glial fibrillary acidic protein (GFAP) gene on chromosome 17.

Symptoms vary between the three forms of the disease, in infantile form which is must occur during the first two years of life. There are both mental and physical developmental delays, followed by the loss of developmental milestones, an abnormal increase in head size, and seizures. An onset of juvenile form is between the ages of two and thirteen and present with the complaints of excessive vomiting, difficulty swallowing and speaking, poor coordination and loss of motor control. Adult forms are rare. The symptoms mimic the symptoms of Parkinson’s disease or multiple sclerosis.

A diagnosis of Alexander disease is based on genetic test, radiologic findings and brain biopsy (demonstration of Rosenthal fibers), results in an individual who has symptoms suggestive of this condition.

There is no cure for this disease. Treatment, which is symptomatic and supportive like’s nutritional support, antibiotic, anti-epileptic drugs and surgical intervention may be recommended.

Alexander disease is generally one of regression and progressive neurologic degeneration. Prognosis of this disease is not good and affected individual live and where form on to 10 years of age .

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