The spectrum of immune-mediated autonomic neuropathies: insights from the clinicopathological features: Table 1

Abstract

Although autonomic neuropathy may occur as a secondary consequence of various diseases, other patients without any obvious underlying diseases show profound autonomic dysfunctions from the early phase of the disease. These idiopathic or primary cases 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. The discovery of the antiganglionic acetylcholine receptor antibody suggested the involvement of immune mechanisms in idiopathic cases, especially in those cases with pure autonomic neuropathy. The ability to test for the presence of this antibody has significantly expanded the concept of autonomic neuropathy to include cases with a chronic progressive course that mimics pure autonomic failure. Recent work based on the antiganglionic acetylcholine receptor antibody has established autoimmune autonomic ganglionopathy as an isolated nosological entity. Other forms of primary autonomic neuropathies include acute autonomic and sensory neuropathy and acute autonomic sensory and motor neuropathy, although the nosological relationship of the latter to Guillain-Barré syndrome should be discussed. Although the possibility of infectious, metabolic or toxic aetiologies should be carefully excluded in these forms of autonomic neuropathy, the monophasic clinical course and the presence of antecedent infections suggest the involvement of immune mechanisms similar to Guillain-Barré syndrome. Neuronopathy in the autonomic ganglia is considered to be a common pathology in these autonomic neuropathies. In addition, clinically significant autonomic neuropathy may be associated with pre-existing immunological diseases such as paraneoplastic syndrome and Sjögren's syndrome. An overlap with autoimmune autonomic ganglionopathy has been suggested in these settings.

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

“…Although the level of SS-A antibodies was slightly increased in our case, tests for anti-SS-B antibodies were negative. The autonomic impairment seen in cases of Sjögren syndrome is usually chronic, different from that observed in our case (14). Although the patient complained of dry eyes and dry mouth, these symptoms can also be associated with autonomic failure.…”

“…This case report suggests that ganglionopathy participates in the pathogenesis of typical cases of AASMN (14). Autonomic neuropathies may be primary or occur secondary to underlying diseases such as paraneoplastic syndrome, connective tissue diseases and celiac disease (14). Although the level of SS-A antibodies was slightly increased in our case, tests for anti-SS-B antibodies were negative.…”

“…Neuronal cell loss in the dorsal root ganglia was shown in one autopsy case that involved profound dysautonomia as the initial manifestation and the development of weakness several days later (23). This case report suggests that ganglionopathy participates in the pathogenesis of typical cases of AASMN (14). Autonomic neuropathies may be primary or occur secondary to underlying diseases such as paraneoplastic syndrome, connective tissue diseases and celiac disease (14).…”

“…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).…”

Atypical presentation of Non-Hodgkin Lymphoma (NHL): a case report

“…La simmetria delle manifestazioni cliniche, la natura prevalentemente sensitiva e l'interessamento delle fibre di piccolo calibro (assenza di potenziali corticali registrabili dopo stimolo laser) potrebbero deporre per un quadro di polineuropatia sensitiva [3,4]. Tuttavia le neuropatie associate a NHL presentano più frequentemente caratteristiche similari a quelle osservabili nelle neuropatie di natura demielinizzante/infiammatoria (con riduzione delle velocità di conduzione) [5,6], con una minore probabilità di presentare le caratteristiche di una neuropatia assonale sensitiva o una neuronopatia (danno a carico del ganglio sensitivo) [5,6].…”