NPD type A | Intermediate NPD, variant NPD B | NPD type B |
---|---|---|
Phenotype: Infantile onset of severe neurodegeneration with progressive psychomotor deterioration Natural history: uniform Prognosis: death typically by 3Â years of age | Phenotype: NPD B phenotype but also progressive neurologic findings including ataxia, variable degrees of developmental delay and peripheral neuropathy Natural history: variable Prognosis: patients live past early childhood, sometimes into adulthood | Phenotype: Chronic progressive multi-system disease with no or little neurologic involvement Natural history: variable Prognosis: variable (survival until the second to seventh decade of life) |