Introduction: The anti-Hu antibody are associated with central nervous system paraneoplastic syndromes such as sensory neuropathy, cerebellar ataxia or limbic encephalitis. The isolated autonomic dysfunction as the presenting manifestation of anti-Hu antibody-related paraneoplastic syndrome is extremely infrequent.
Case report: A 68 year-old male presented with a two-month history of severe constipation following of orthostatic hypotension, impotence, urinary retention and intestinal pseudo-obstruction as manifestations of progressive dysautonomia. Four months after the onset of these symptoms, he developed paresthesias in the hands and feet, motor weakness in both upper and lower limbs and ophtalmoplegia. Anti-Hu antibodies were positive in serum and cerebrospinal fluid, although tumor was not found.
Conclusions: Progressive dysautonomia may result from a number of diseases. We encourage to consider also the possibility of anti-Hu antibody-related syndrome, despite the case that it is a very unusual isolated initial manifestation.