Peripheral neuropathy in late-onset Krabbe disease: report of three cases.

[krabbe disease]

Late -onset Krabbe disease may have variable misleading clinical manifestations and be a puzzling problem for physicians . We report clinical and peripheral nerve studies of three patients with adult-onset Krabbe disease . Two cases had a predominantly spastic paraparesis ; in one case , the symptoms mimicked a cerebrovascular disorder . Predominantly , demyelinating neuropathy was observed in one case and axonal neuropathy in two cases . In all cases , no typical intracytoplasmic inclusions were found . These observations suggest that peripheral neuropathy in adult-onset Krabbe disease has variable clinical and pathological characteristics , different from those described in the classic form .