Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia

Abstract

Acute-onset and severe sensory and autonomic deficits with no motor dysfunction, typically preceded by a febrile illness, with poor recovery, and often fatal outcome are the hallmark features of acute sensory and autonomic neuronopathy (ASANN). Pathologically and electrophysiologically, ASANN is characterized by an extensive ganglionopathy affecting sensory and autonomic ganglia with preservation of motor neurons. Consequently, patients, usually children or young adult, develop acute-onset profound widespread loss of all sensory modalities resulting in automutilations, as well as autonomic failure causing neurogenic orthostatic hypotension, neurogenic underactive bladder, and gastroparesis and constipation. The diagnosis is clinical with support of nerve conduction studies and autonomic testing, as well as spinal cord magnetic resonance imaging showing characteristic posterior cord hyperintensities. Although the presumed etiology is immune-mediated, further studies are required to clarify the physiopathology of the disease. We here performed a systematic review of the epidemiology, pathophysiology, diagnosis, and management of ASANN, with three representative cases that recently presented at our clinic. All three patients had the typical clinical manifestations of ASANN but in different combinations, illustrating the variable phenotype of the disorder. Immunosuppression is seldom effective. Management options are limited to supportive and symptomatic care with the goal of minimizing complications and preventing death.

Gadolinium enhancement in cervical dorsal roots in a patient with acute autonomic and sensory neuropathy: a case report

“…This clinical manifestation suggests that the pathophysiology of AASN might be the same as in that of Guillain-Barré syndrome (GBS). In GBS, post-infectious immune-mediated mechanisms cause the disruption of the blood-nerve barrier leading to demyelination or axonal loss [ 1 , 8 ]. Our MRI with contrast findings suggest that inflammation in the dorsal roots, together with the posterior column of the spinal cord and cervical dorsal root ganglia, is one of the causes of sensory disturbance in patients with AASN.…”

“…Therefore, especially in the early stages of AASN, MRI with contrast facilitates the diagnosis of AASN. It is difficult for physicians to differentiate AASN from other mimicking disorders, such as sensory GBS, acute sensory ataxic neuropathy with antiganglioside antibodies, Sjögren disease or paraneoplastic neuronopathy because there are no specific biomarkers for AASN [ 1 , 8 ]. We considered sensory GBS or acute sensory ataxic neuropathy with antiganglioside antibodies but disregarded them because of the rarity of severe autonomic dysfunction in these diseases.…”

“…Acute autonomic and sensory neuropathy (AASN) is a rare disorder characterized by impaired autonomic and sensory functions, without motor impairment, that reaches its peak severity within a short period of time [ 1 ]. Although the relationship between AASN and Guillain-Barre syndrome has not yet been clarified, the progression of AASN symptoms is similar to that of Guillain-Barre syndrome [ 2 ].…”

Validation and Reliability of a Novel Vagus Nerve Neurodynamic Test and Its Effects on Heart Rate in Healthy Subjects: Little Differences Between Sexes

“…Notably, mechanical allodynia—which is a common symptom when nerves receive prolonged exposure to inflammatory cytokines ( Jensen and Finnerup, 2014 ; Beaulieu-Laroche et al, 2020 ; Bonet et al, 2021 )—provoked by the VN-NDT had the best test accuracy in detecting digestion alterations and burning sensation in the stomach. Indeed, gastrointestinal dysfunctions are very common in acute and chronic APNs ( Freeman, 2005 ; Oaklander and Nolano, 2019 ; Gutierrez et al, 2020 ; Marathe et al, 2020 ). Since the perioperative and postsurgery risks of cardiovascular side events ( Lankhorst et al, 2015 ; Ho et al, 2020 ; Malaty et al, 2021 ) are higher in post-COVID-19 patients and patients with APN, which are difficult to be studied, it is possible to adopt the VN-NDT as a sensitive, faster, and riskless screening test.…”

“…Here, we report for the first time that a sequence of neck movements can systematically affect HR, both in males and females, suggesting a key role of the stretch on the neck portion of the VN in HR modulation. Gutierrez and coworkers reported that a patient with acute sensory and autonomic neuropathy had her symptoms relieved by neck movements ( Gutierrez et al, 2020 ) which are included in the VN-NDT. Therefore, we can argue that studying the VN-NDT effects can be helpful in diagnosis and symptoms management in autonomic dysfunctions.…”