TABLE 26.4.
Type I: Non-Neuronopathic | Type II: Acute Neuronopathic | Type III: Subacute Neuronopathic | |
---|---|---|---|
Age at presentation | Childhood/adulthood | Infancy | Childhood/adulthood |
Hepatosplenomegaly | + → +++ | + | + → +++ |
Skeletal abnormality | – → +++ | – | ++ → +++ |
CNS disease | – | +++ | + → +++ |
Life span | 6–80 + years | <2 years | 2–60 years |
Ethnicity | Ashkenazi Jews | Panethnic | Panethnic, Swedes |
CNS, Central nervous system.
Note: Absence and severity of features are indicated by – to +++.