Polirradiculoneuropatías autoinmunes agudas

Abstract

Landry Guillain Barr Sthrol Syndrome (LGBS) is an acute autoimmune monophasic and selflimiting polyradiculoneuropathy affecting patients aged 19 to 59 years (1.7/100,000/year) more than below 18 year of age (0.8/100,000/year). Diagnostic criteria require: I. Progressive motor weakness of more than one limb, and II) absent muscle stretch reflexes. Features that strongly support the diagnosis are: 1. Cease of progression of weakness by 4 weeks, relative symmetrical involvement of the limbs, presence of mild sensory symptoms or signs, involvement of cranial nerves VII (50%) more than IX, X, III, IV and VI, recovery within 2 to 3 weeks after progression stop, presence of autonomic dysfunction, and absent of fever at onset; 2. Cerebral spinal fluid elevation of protein after first week of symptoms and less than 10 mononuclear leukocytes/mm3 except HIV seropositive patients (<50 cells/mm3), and 3. Nerve conduction slowing (<60% of normal ) or block at some point during the illness (80%), and increased distal motor latency up to 3 times above normal and F wave latency. There are four well defined clinical, pathological, neurophysiological and serological subtypes. Acute inflamatory demyelinating polyradiculoneuropathy (90%) and Miller Fisher syndrome (5%) are primarily demyelinating. Acute motor axonal neuropathy and acute motor sensory axonal neuropathy are primarily axonal (5%). Treatment includes supportive care and immunotherapy with high doses of intravenous IgG and plasmapheresis.