Indolent anti-Hu-associated paraneoplastic sensory neuropathy.
Paraneoplastic sensory neuropathy (PSN) usually runs a subacute progressive course, leaving the patient with severe sensory dysfunction in weeks to months. We describe five patients with PSN, high titers of anti-Hu antibodies (type 1 antineuronal nuclear autoantibodies), and an indolent clinical course. The patients had a median age of 55 years (range, 41 to 72). Four had small-cell (3) or undifferentiated large-cell (1) lung cancer. Patients presented with mild, asymmetric sensory symptoms; in two, the neuropathy was predominant in the arms. Two patients also had a visceral neuropathy causing gastrointestinal dysfunction. The PSN was stable or progressed very slowly without treatment for a median of 18 months (range, 5 to 32) and remained so after treatment with immunoglobulins (1 patient), chemotherapy (3), or both therapies (1). All patients were ambulatory, leading an independent life up until the time of the last visit or until death from the tumor (2 patients). The median follow-up was 36 months (range, 22 to 52). A paraneoplastic origin should be considered in patients with mild, very slowly progressive sensory neuropathies.
Graus
F
F
Service of Neurology, Hospital Clinic i Provincial, Barcelona, Spain.
Bonaventura
I
I
Uchuya
M
M
Valls-Solé
J
J
Reñé
R
R
Leger
J M
JM
Tolosa
E
E
Delattre
J Y
JY
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D018287:Carcinoma, Large Cell / Q000503:physiopathology; D018288:Carcinoma, Small Cell / Q000503:physiopathology; D004576:Electromyography; D005500:Follow-Up Studies; D006624:Hippocampus / Q000276:immunology; D006801:Humans; D008175:Lung Neoplasms / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009475:Neurons, Afferent / Q000502:physiology; D010257:Paraneoplastic Syndromes / Q000276:immunology / Q000503:physiopathology*; D010523:Peripheral Nervous System Diseases / Q000276:immunology / Q000503:physiopathology*
D001323:Autoantibodies