Anti-Hu antibody neuropathy: a clinical, electrophysiological, and pathological study

Clin Neurophysiol. 2005 Jan;116(1):28-34. doi: 10.1016/j.clinph.2004.07.012.


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.

Significance: New guidelines for the selection of patients for anti-Hu antibody test are recommended.

Publication types

  • Comparative Study

MeSH terms

  • Action Potentials / physiology
  • Action Potentials / radiation effects
  • Aged
  • Antibodies, Anti-Idiotypic / cerebrospinal fluid
  • Carcinoma, Small Cell / metabolism
  • Carcinoma, Small Cell / pathology*
  • Demyelinating Diseases / physiopathology
  • ELAV Proteins
  • Electric Stimulation / methods
  • Encephalomyelitis / metabolism
  • Encephalomyelitis / physiopathology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Muscle, Skeletal / physiopathology
  • Nerve Degeneration / physiopathology
  • Nerve Tissue Proteins / immunology*
  • Neural Conduction / physiology
  • Paraneoplastic Polyneuropathy / metabolism
  • Paraneoplastic Polyneuropathy / physiopathology*
  • RNA-Binding Proteins / immunology*
  • Reaction Time / physiology
  • Reaction Time / radiation effects
  • Retrospective Studies


  • Antibodies, Anti-Idiotypic
  • ELAV Proteins
  • Nerve Tissue Proteins
  • RNA-Binding Proteins