CLINICO-PATHOPHYSIOLOGICAL FEATURES OF ACUTE AUTONOMIC AND SENSORY NEUROPATHY - A LONG-TERM FOLLOW-UP-STUDY

We evaluated the clinicopathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed f or over 3 years. Signs of an autonomic disturbance including vomiting, anhidrosis, urinary disturbances, orthostatic hypotension and reduced coefficient of variation of the R-R interval on electrocardiography g radually improved about 1 year after onset. However, all three exhibit ed severe generalized sensory impairment for all modalities with the d evelopment of persistent sensory ataxia. No sensory nerve action poten tials could be elicited and no somatosensory evoked potentials could b e obtained. Sural nerve biopsy revealed severe axonopathy. In two pati ents, a high-intensity area was observed in the posterior column of th e spinal cord on T2-weighted axial magnetic resonance images. The lev el of neuron-specific enolase in cerebrospinal fluid was markedly elev ated in two patients, indicating spinal nerve root or sensory neuron d amage. Motor nerve function was well preserved in all patients, Our fi ndings suggests that the major lesion in patients with AASN, particula rly those with a sensory deficit, is present in the dorsal root gangli on neurons, that is there is a ganglioneuronopathy.