Autonomic dysfunction is frequent and disabling in non-paraneoplastic sensory neuronopathies

Author(s)

Muro Martinez, Alberto Rolim ^[1] ; Martins, Melina Pazian ^[1] ; Ribeiro de Rezende, Thiago Junqueira ^[1] ; Faber, Ingrid ^[1] ; Gonzalez Salazar, Carelis del Valle ^[1] ; Girotto Takazaki, Karen Antonia ^[1] ; Nucci, Anamarli ^[1] ; Franca, Jr., Marcondes Cavalcante ^[1]

Affiliation

^[1] Univ Campinas UNICAMP, Neuromuscular Div, Neurol Dept, Campinas, SP - Brazil

• Document type: Journal article
• Source: JOURNAL OF THE NEUROLOGICAL SCIENCES; v. 402, p. 111-117, JUL 15 2019.
• Web of Science Citations: 0

Abstract

Introduction: Sensory neuronopathies (SN) are characterized by asymmetric non-length dependent sensory deficits and sensory ataxia. Autonomic dysfunction in SN was not yet evaluated regarding its frequency, characteristics and relationship to sensory deficits. To address these issues, we performed a comprehensive clinical and neurophysiological evaluation of a large cohort of patients with non-paraneoplastic SN (np-SN). Methods: We enrolled 50 consecutive patients with npSN and 32 age/sex-matched healthy controls. They were clinically evaluated (SCOPA-Aut scale) and underwent neurophysiological autonomic assessment (quantitative sudomotor axon reflex test, heart rate variability and sympathetic skin response). Results: Mean age of patients was 50.9 +/- 10.3 years and there were 18 men. npSN patients had higher SCOPA-Aut scores than controls (26.63 +/- 12.72 vs. 12.66 +/- 9.11, p < .001). QSART was abnormal in 92% of the patients - sweat volumes in all examined sites were smaller among patients (p < .001). Cardiovascular autonomic neuropathy was more frequent in these patients as well (p < .001). Conclusion: Altogether our results suggest that autonomic dysfunction in distinct domains is frequent in npSN patients. These findings suggest that the clinical picture of npSN is related to a double neuronopathy: sensory and autonomic. (AU)

• FAPESP's process: 13/01766-7 - Sensory neuronopathies: investigation of new diagnostic methods, mechanisms of the disease and therapeutic strategies
• Grantee: Marcondes Cavalcante Franca Junior
• Support Opportunities: Research Grants - Young Investigators Grants
• FAPESP's process: 13/26410-0 - Clinical, immunological and neurophysiological characterization of sensory neuronopathies
• Grantee: Alberto Rolim Muro Martinez
• Support Opportunities: Scholarships in Brazil - Doctorate (Direct)