Intrathecal synthesis of the anti-Hu antibody in patients with paraneoplastic encephalomyelitis or sensory neuronopathy: clinical-immunologic correlation.
We studied the intrathecal synthesis of the anti-Hu antibody (also called type 1 antineuronal nuclear autoantibody) in 14 patients with isolated paraneoplastic subacute sensory neuronopathy (SSN) and 16 with paraneoplastic encephalomyelitis (PEM). Patients with PEM had higher anti-Hu titers in the CSF (p = 0.003) but not in the serum than those with SSN. Only one patient (7%) with SSN had a positive intrathecal anti-Hu antibody synthesis whereas this was present in 14 (88%) of the 16 patients with PEM (p < 0.0001). The correlation between the intrathecal production of anti-Hu antibodies and PEM supports the role of autoimmune mechanisms in the pathogenesis of PEM. The absent intrathecal synthesis of anti-Hu antibodies in patients with SSN suggests easier accessibility of the systemic immune reaction to the sensory neurons probably due to the partial absence of blood-nerve barrier in the dorsal root ganglia.
Vega
F
F
Neurology Service, Hôpital de la Salpêtrière, Paris, France.
Graus
F
F
Chen
Q M
QM
Poisson
M
M
Schuller
E
E
Delattre
J Y
JY
D000368:Aged; D000906:Antibodies / Q000134:cerebrospinal fluid*; D001327:Autoimmune Diseases / Q000276:immunology; D051959:ELAV Proteins; D004679:Encephalomyelitis / Q000134:cerebrospinal fluid / Q000276:immunology*; D005260:Female; D005727:Ganglia, Spinal / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000134:cerebrospinal fluid / Q000276:immunology*; D009422:Nervous System Diseases / Q000276:immunology*; D009474:Neurons; D010257:Paraneoplastic Syndromes / Q000134:cerebrospinal fluid / Q000276:immunology*; D016601:RNA-Binding Proteins / Q000134:cerebrospinal fluid / Q000276:immunology*; D012678:Sensation Disorders / Q000276:immunology*
D000906:Antibodies; D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins