Paraneoplastic anti-CV2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy.
Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd brain protein belonging to the family of Ulip/CRMP proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study.
Antoine
J C
JC
Equipe d'Accueil 3063, Faculté de Médecine de Saint-Etienne, Service de Neurologie, H pital de Bellevue, France. 113464.3717@compuserve.com
Honnorat
J
J
Camdessanché
J P
JP
Magistris
M
M
Absi
L
L
Mosnier
J F
JF
Petiot
P
P
Kopp
N
N
Michel
D
D
D000368:Aged; D000369:Aged, 80 and over; D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D001369:Axons / Q000276:immunology*; D003711:Demyelinating Diseases / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D020364:Paraneoplastic Polyneuropathy / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D010525:Peripheral Nerves / Q000276:immunology*
D000888:Antibodies, Anti-Idiotypic