A-Z of Neurological Conditions

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Name

Niemann-Pick Disease

Related to/Also known as

Juvenile dystonic lipidosis

Description

Niemann-Pick (NP) is an inherited metabolic disorder, in which harmful quantities of a fatty substance accumulate in the spleen, liver, lungs, bone marrow and, in some people, the brain.

Types

There are 4 types - A, B, C and D.

Type A, Infantile NP, has its onset in early infancy and results in early death.

Type B, Juvenile Non-neuronoplathic NP, involves enlargement of the spleen and liver, which characteristically occurs in pre-teens years.

Type C may occur in early life or teens, with enlargement of the spleen and liver, accompanied by progressive loss of speech, ataxia and other problems.

Type D - similar to type C, but specifically found in people of Nova Scotia ancestry.

Useful National contacts

Niemann-Pick Disease Group UK
Tel: 0191 415 0693
www.niemannpick.org.uk

Our resources

The library at Neurosupport has a range of reference resources on this condition and on a wide range of disability related issues.

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