Pandysautonomia: clinicopathological. Report of 5 cases

Five cases of pandysautonomia were reported. Pupils, salivary and sweat glands, gastrointestinal tract, urinary bladder and heart were impaired in all cases, indicating that the peripheral parts of both sympathetic and parasympathetic nervous systems were involved, especially the cholinergic post ganglionic efferent fibers. Besides, two cases showed sensory impairment of the distal parts of the extremities, slight atrophy of small hand muscles and weak or absence of deep tendon reflexes. Three cases showed elevation of CSF protein without cellular reaction. Sural nerve biopsy was performed in 3 cases. Neuropathological changes included the reduction of myelinated neurofibers, atrophy of Schwann's cells and the presence of degeneration products and myelin-like structures. One case also revealed small vasculitis among neurofascicles. It seems that the disease entity is a result of autoimmune dysfunction. The prognosis of pandysautonomia in the present group was good, four cases have some improvement during 1-4 years of follow-up study.