Clinico-pathophysiological features of acute autonomic and sensory neuropathy: A long-term follow-up study

Abstract

We evaluated the clinico-pathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed for 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 gradually improved about 1 year after onset. However, all three exhibited severe generalized sensory impairment for all modalities with the development of persistent sensory ataxia. No sensory nerve action potentials could be elicited and no somatosensory evoked potentials could be obtained. Sural nerve biopsy revealed severe axonopathy. In two patients, a high-intensity area was observed in the posterior column of the spinal cord on T2*-weighted axial magnetic resonance images. The level of neuron-specific enolase in cerebrospinal fluid was markedly elevated in two patients, indicating spinal nerve root or sensory neuron damage. Motor nerve function was well preserved in all patients. Our findings suggests that the major lesion in patients with AASN, particularly those with a sensory deficit, is present in the dorsal root ganglion neurons, that is there is a ganglioneuronopathy.

Sensory neuropathies including painful and toxic neuropathies

“…Fig.1Differential diagnostic approach to sensory neuropathies according to the underlying pathophysiological process (* prominent sensory ataxia, # sometimes including autonomic involvement, AASN acute autonomic and sensory neuropathy[93], CIDP…”

Differential Recovery in Cardiac and Vasomotor Sympathetic Functional Markers in a Patient with Acute Autonomic Sensory and Motor Neuropathy

“…In this patient, dysautonomia was observed in both the peripheral sympathetic and parasympathetic nervous systems. From the viewpoint of autonomic neuropathy with a monophasic clinical course similar to GBS, studies have described at least three major groups based on concomitant sensory motor involvement (13,14): (1) pure autonomic neuropathy (15)(16)(17), (2) acute autonomic and sensory neuropathy (13,18,19) and (3) AASMN (3-6). The nosological entity of AASMN should be considered carefully because autonomic dysfunction has been frequently reported in patients with GBS (14, 20-22).…”

Guillain-Barré Syndrome and Related Disorders

“…The CSF protein concentration is usually mildly elevated. Combined sensory and autonomic GBS variants are probably common [ 219,231 ] .…”