Clinicopathological features of acute autonomic and sensory neuropathy

Abstract

Acute autonomic and sensory neuropathy is a rare disorder that has been only anecdotally reported. We characterized the clinical, electrophysiological, pathological and prognostic features of 21 patients with acute autonomic and sensory neuropathy. An antecedent event, mostly an upper respiratory tract or gastrointestinal tract infection, was reported in two-thirds of patients. Profound autonomic failure with various degrees of sensory impairment characterized the neuropathic features in all patients. The initial symptoms were those related to autonomic disturbance or superficial sensory impairment in all patients, while deep sensory impairment accompanied by sensory ataxia subsequently appeared in 12 patients. The severity of sensory ataxia tended to become worse as the duration from the onset to the peak phase of neuropathy became longer (P<0.001). The distribution of sensory manifestations included the proximal regions of the limbs, face, scalp and trunk in most patients. It tended to be asymmetrical and segmental, rather than presenting as a symmetric polyneuropathy. Pain of the involved region was a common and serious symptom. In addition to autonomic and sensory symptoms, coughing episodes, psychiatric symptoms, sleep apnoea and aspiration, pneumonia made it difficult to manage the clinical condition. Nerve conduction studies revealed the reduction of sensory nerve action potentials in patients with sensory ataxia, while it was relatively preserved in patients without sensory ataxia. Magnetic resonance imaging of the spinal cord revealed a high-intensity area in the posterior column on T(2)*-weighted gradient echo image in patients with sensory ataxia but not in those without it. Sural nerve biopsy revealed small-fibre predominant axonal loss without evidence of nerve regeneration. In an autopsy case with impairment of both superficial and deep sensations, we observed severe neuronal cell loss in the thoracic sympathetic and dorsal root ganglia, and Auerbach's plexus with well preserved anterior hone cells. Myelinated fibres in the anterior spinal root were preserved, while those in the posterior spinal root and the posterior column of the spinal cord were depleted. Although recovery of sensory impairment was poor, autonomic dysfunction was ameliorated to some degree within several months in most patients. In conclusion, an immune-mediated mechanism may be associated with acute autonomic and sensory neuropathy. Small neuronal cells in the autonomic and sensory ganglia may be affected in the initial phase, and subsequently, large neuronal cells in the sensory ganglia are damaged.

Autoimmune autonomic neuropathies: time to look beyond autoimmune autonomic ganglionopathy

“…It has been reported after infections and typically presents as an acute-onset syndrome with significant sensory deficits (including lack of pain and temperature sensation, lack of proprioception, and severe sensory ataxia) combined with neuropathic pain and severe autonomic failure (including orthostatic hypotension, atonic bladder, and severe gastroparesis). These patients also have characteristic hyperintensities in the dorsal columns on spinal magnetic resonance (MR) imaging as well as very low plasma norepinephrine levels [4]. Although the patients of this series reported by Golden, Bryarly, and Vernino seem to have a predominantly subacute course with no changes on spinal MR imaging, their clinical phenotypes are remarkably similar to those of patients with AASN [5].…”

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

“…Additional manifestations that are acquired in the fi rst week or two include erectile dysfunction, urinary frequency, urgency, and retention; vasomotor instability with acrocyanosis; and reduced salivation, lacrimation, or sweating. Sensation is normal initially but most patients develop various degrees of sensory impairment without motor dysfunction [ 230 ] . The sensory symptoms tend to be segmental and asymmetrical, often associated with pain.…”