Alpers disease

Below you will find more information about Alpers disease from Medigest. If you believe that you are suffering from any of the symptoms of Alpers disease it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Alpers disease and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Alpers disease comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Named after Bernard Jacob Alpers, Alper's disease is a progressive degenerative disease that affects the central nervous system. Also called progressive sclerosing polidystrophy, progressive infantile poliodystrophy, progressive neuronal degeneration of childhood and Alpers' syndrome, this autosomal recessive disorder only occurs in infants and children, sometimes in siblings.

Treatment

Currently, there is no available cure for Alpers' disease and no treatments to slow down its progression. Anticonvulsants are used for seizures. Physical therapy can be implemented to maintain muscle tone and relieve limb stiffness. Unfortunately, diagnosis for patients with Alpers' disease is poor. Majority of the patients die within the first 10 years of their lives. The most common cause of death is liver failure, next is cardiorespiratory failure.

Symptoms and Signs

The first symptoms of Alpers' disease include seizures and failure to meet proper development. These symptoms usually occur during the first year of an infant's life, but can also appear in children aged 5 years old. Primary symptoms of Alpers' disease include progressive mental retardation, developmental delay, low muscle tone, progressive dementia and limb stiffness that may lead to quadriplegia. When seizures occur, the child may experience epilepsia partialis continua, a seizure consisting of repeated muscle jerks. A few cases of optic atrophy may occur, some even lead to blindness. Others experience deafness. Many patients of Alpers' disease suffer from liver impairment that leads to liver failure. Unfortunately, physical signs of liver problems may not be present.

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