The spectrum of acquired demyelinating polyradiculoneuropathy.
Guillain-Barré Syndrome (GBS) is best viewed as a clinical syndrome which can have at least two clinical substrates: the commonest is an acquired demyelinating polyradiculoneuropathy and the other is an acute motor axonal neuropathy. An acute acquired neuropathy can also sometimes cause the Miller Fisher syndrome of ophthalmoplegia, areflexia and ataxia, and even more rarely a pure sensory neuropathy. The demyelinating form of GBS also forms one end of a spectrum which has Chronic Idiopathic Demyelinating Polyradiculoneuropathy (CIDP) at its other pole. CIDP usually produces a mixed sensory and motor, albeit predominantly motor deficit, but some patients have pure multifocal motor neuropathy (MMN) and other even less common patients have pure sensory CIDP. According to the definitions of international committees, the symptoms of GBS reach their nadir within 4 weeks and those of CIDP in not less than 8 weeks. The spectrum is completed by patients with a monophasic illness reaching its nadir in 4 to 8 weeks (subacute idiopathic demyelinating polyradiculoneuropathy (SIDP). In addition there are patients with discrete attacks of acute demyelinating neuropathy which have been called recurrent GBS. The differential diagnosis of acquired demyelinating neuropathy differs according to the position of the individual case on this spectrum. The pathogenesis of each case may depend on the nature of the autoantigen, the balance between T cell and antibody-mediated autoimmune mechanisms, and the tempo of the inflammatory process. These differences may explain the empirical observations that steroids are helpful in CIDP but not GBS. Removal of antibodies by plasma exchange or flooding the immune system with normal immunoglobulin is beneficial in GBS and some cases of CIDP.(ABSTRACT TRUNCATED AT 250 WORDS)
Hughes
R A
RA
Department of Neurology, UMDS, Guy's Hospital, London, UK.
D000208:Acute Disease; D001259:Ataxia / Q000503:physiopathology; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000503:physiopathology*; D006801:Humans; D016472:Motor Neuron Disease / Q000503:physiopathology; D009886:Ophthalmoplegia / Q000503:physiopathology; D011129:Polyradiculoneuropathy / Q000503:physiopathology*; D012678:Sensation Disorders / Q000503:physiopathology; D013577:Syndrome