Immunologic Aspects of Neurological and Neuromuscular Diseases

Clinical Features THIS DISEASE is pathologically characterized by cellular infiltration of the peripheral nervous system with lymphocytes and macrophages and by destruction of myelin (Fig 16-1). It is clinically characterized by subacute onset of weakness ranging from slight ataxia to total paralysis of motor and, occasionally, cranial nerves. Although subacute in nature, the disease may reach its peak of activity within 24 hours in some cases or smoulder at moderate intensity for two to three weeks. Proximal weakness usually occurs before distal weakness, and there is a tendency to symmetrical involvement. The seventh cranial nerve is almost universally affected. Tendon reflexes are usually abolished; sensory loss is variable and may be absent. Autonomic function may also be compromised, and many patients may have marked fluctuations in BP and cardiac arrhythmias that can cause death. Diagnostic criteria have been established for the disease. (See Asbury et al,