Anti-Hu antibody neuropathy: a clinical, electrophysiological, and pathological study.
OBJECTIVE
The objective is to report the clinical, electrophysiological, and histopathological features of 16 patients with anti-Hu antibody neuropathy.


METHODS
Clinical and electrophysiological data in 16 patients (11 females and 5 males) with positive anti-Hu antibody and nerve biopsy data in 9 cases were analyzed.


RESULTS
Cancer was detected in 11 patients, including 9 with small-cell lung cancer. Classical paraneoplastic subacute sensory neuronopathy (SSN) and/or encephalomyelitis (EM) was observed in 7 patients (44%), including 5 with SSN. The most common clinical feature was sensory-motor neuropathy (SMN), accounting for 50% of cases. Though sensory nerve conduction abnormality was the prominent feature in 14 (88%) cases, sensory and motor nerve conduction was abnormal in all cases. Motor nerve conduction findings were typical of axonal degeneration. The most common nerve conduction pattern was that of SMN, with a sensory neuronopathy pattern being observed in only 3 cases. Sural nerve biopsy in 9 patients showed axonal degeneration in all cases and inflammatory cells in 4 cases.


CONCLUSIONS
Classical sensory neuronopathy is rarer than expected, both clinically and electrophysiologically. Motor involvement is not uncommon and motor nerve conduction abnormality is frequently seen. A diverse clinical and electrophysiological, and histopathological spectrum was observed in this neuropathy.


CONCLUSIONS
New guidelines for the selection of patients for anti-Hu antibody test are recommended.
Oh
Shin J
SJ
Department of Neurology, University of Alabama at Birmingham, Veterans Affairs Medical Center, UAB Station, Birmingham, AL 35294, USA. shinjoh@uab.edu
Gürtekin
Yasar
Y
Dropcho
Edward J
EJ
King
Peter
P
Claussen
Gwendolyn C
GC
D000200:Action Potentials / Q000502:physiology / Q000528:radiation effects; D000368:Aged; D000888:Antibodies, Anti-Idiotypic / Q000134:cerebrospinal fluid; D018288:Carcinoma, Small Cell / Q000378:metabolism / Q000473:pathology*; D003711:Demyelinating Diseases / Q000503:physiopathology; D051959:ELAV Proteins; D004558:Electric Stimulation / Q000379:methods; D004679:Encephalomyelitis / Q000378:metabolism / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology; D009410:Nerve Degeneration / Q000503:physiopathology; D009419:Nerve Tissue Proteins / Q000276:immunology*; D009431:Neural Conduction / Q000502:physiology; D020364:Paraneoplastic Polyneuropathy / Q000378:metabolism / Q000503:physiopathology*; D016601:RNA-Binding Proteins / Q000276:immunology*; D011930:Reaction Time / Q000502:physiology / Q000528:radiation effects; D012189:Retrospective Studies
D000888:Antibodies, Anti-Idiotypic; D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins