Acute and chronic neuropathies: new aspects of Guillain-Barre syndrome andchronic inflammatory demyelinating polyneuropathy, an overview and an update

During the last 15 years new information about clinical, electrophysiologic al, immunological and histopathological features of acute and chronic infla mmatory neuropathies have emerged. Thus, the Guillain-Barre syndrome (GBS) is no longer considered a simple entity. Subtypes of the disorder besides t he typical predominant motor manifestation, are recognized, i.e. a cranial nerve variant with ophthalmoplegia, ataxia and areflexia, an immune-mediate d primary motor axonal neuropathy (AMAN), and a motor-sensory syndrome (AMS AN). Also, the clinical pattern of GBS is related to preceding viral or bac terial infections. Two types of acute motor paralysis have been described, one with slow and incomplete recovery, another with recovery times identica l with acute inflammatory demyelinating polyneuropathy (AIDP). Histological ly, the first is characterized by Wallerian degeneration of motor roots and peripheral motor nerve fibres. in the latter anti-GM antibodies bind to th e nodes of Ranvier producing a failure of impulse transmission. Motor-point biopsies have shown denervated neuromuscular junctions and a reduced numbe r of intramuscular nerve fibres. Molecular mimicry has been postulated as a possible mechanism triggering GBS. Thus, in the cranial variant antibodies to ganglioside GQ1b recognizes similar epitopes on Campylobacter jejuni st rains and similar observations apply to anti-GM1 antibodies. Chronic inflam matory demyelinating polyneuropathy (CIDP) also has several different clini cal presentations such as a pure motor syndrome, a sensory ataxic variant, a mononeuritis multiplex pattern, relapsing GBS, and a paraparetic subtype. Each of the acute and the subtypes have different, more or less distinct, electrophysiologic and pathological findings. Instructive patient stories a re presented together with there electrophysiologic and biopsy findings. (C ) 1998 Elsevier Science Ireland Ltd. All rights reserved.