Autoimmune autonomic ganglionopathyとacute autonomic and sensory neuropathy

Abstract

Autonomic neuropathies may occur primarily or secondarily to various underlying diseases. Primary autonomic neuropathies are divided into pure autonomic neuropathy, autonomic neuropathy with sensory impairment, and autonomic neuropathy with sensory and motor impairment based on the concomitance or absence of sensory or motor dysfunctions. Autoimmune autonomic ganglionopathy refers to a pure autonomic neuropathy, which typically affects both cholinergic and adrenergic functions. About a half of the patients with autoimmune autonomic ganglionopathy are positive for anti-ganglionic acetylcholine receptor antibodies. The mode of progression widely ranges from acute to chronic, including that mimicking pure autonomic failure. The number of unmyelinated fibers in the sural nerve biopsy specimens tends to decrease with the duration of disease become longer. Immunomodulatory treatments are suggested to be effective for autoimmune autonomic ganglionopathy. Acute autonomic and sensory neuropathy is characterized by autonomic and sensory impairment without motor dysfunction that reaches its nadir within a short period of time mimicking the progression of Guillain-Barré syndrome. The monophasic clinical course and frequent presence of a history of antecedent infections suggests a participation of immune mechanisms. The initial symptoms are those related to autonomic disturbance or superficial sensory impairment, while deep sensory impairment accompanied by sensory ataxia subsequently appears in some patients. Sural nerve biopsy specimens reveal small-fiber predominant axonal loss, and autopsy cases show neuronal loss in the thoracic sympathetic and dorsal root ganglia. Hence, small neurons in the autonomic and sensory ganglia may be affected in the initial phase and, subsequently, large neurons in the sensory ganglia are damaged in acute autonomic and sensory neuropathy.