[Progressive dysautonomia as initial manifestation of anti-Hu antibody-related syndrome]. / Disautonomía progresiva como presentación del síndrome anti-Hu.

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.

[Solitary costal plasmacytoma: a rare cause of subacute demyelinating polyradiculoneuropathy]. / Plasmocitoma solitario costal: causa excepcional de polirradiculoneuropatía desmielinizante subaguda.

INTRODUCTION: The solitary plasmacytoma (SP) is a non-frequent, localized variant of multiple myeloma with a single bone lesion and earlier appearance. Polyradiculoneuropathy is the most frequent neurological manifestation. We report the case of a young male who developed a subacute demyelinating polyradiculoneuropathy as the initial symptomatology of a costal SP, which constitutes an extremely infrequent association, given age and site of the lesion. CASE REPORT: A 32-year old, previously healthy, man presented with a one month history of progressive symmetrical paraparesis and paresthesias in feet. The neurological examination revealed 4/5 paraparesis, global arreflexia and hypopalesthesia in legs. Cerebrospinal fluid examination revealed elevated proteins (83 mg/dl) with normal cell count and glucose content. Investigations showed high levels of beta(2)-microglobulin (3 mg/l), and a monoclonal IgG lambda gammapathy. The chest X-ray and thoracic CT revealed an osteolytic lesion in the left third rib. Nerve conduction studies showed sensitive and motor polyneuropathy with secondary axonal degeneration. Bone marrow biopsy was normal. Second and third left ribs were excised, revealing a pathologic diagnosis of plasmacytoma. The patient became asymptomatic after corticosteriod administration lesion excision and local radiotherapy. Conclusions. Subacute demyelinating polyradiculoneuropathy can be due to multiple causes. SP can be one of its etiologies and occur at infrequent ages and localizations.