Niemann-Pick
Alternate Names : Sphingomyelinase Deficiency (Type A Niemann-Pick Disease)
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Niemann-Pick Prognosis (Expectations)
Although Types A and B are both caused by the same enzymatic deficiency, the clinical prognosis for these two groups of patients is very different. Type A Niemann-Pick is a severe neurologic disease, which generally leads to death by 2 to 3 years of age.
In contrast, patients with Type B generally have little or no neurologic involvement and may survive into late childhood or adulthood. The underlying reason for this dramatic difference in the two forms of the disease is not really understood, and, at present, it is not possible to accurately predict the severity of the disease by enzyme testing.
A child showing signs of Type C before one year of age may not live to school age. Children showing symptoms after entering school may live into their mid to late teens, with few surviving into their twenties.
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