Investigating dysautonomia of Bradbury Eggleston syndrome: A case report

Soumayata Somendra,Kapil Gupta,Amit Tak,Jyotsna Shukla,Jitendra Gupta

doi:10.37897/rjn.2021.3.20

Abstract

This is a report of 65 years old man with recurrent episodes of dizziness while standing from supine position and history of constipation and nocturia since last 6 months. Patient disclosed no significant past history other than treated pulmonary tuberculosis 5 years back. The general mental state and vital parameters were within normal limits. Autonomic function tests and heart rate variability analysis measured autonomic reactivity and tone respectively. The tests revealed that autonomic dysfunction include severe sympathetic and mild parasympathetic involvement. After exclusion of other possible causes of orthostatic hypertension, diagnosis of pure autonomic failure also known as Bradbury Eggleston syndrome was established. Treatment with fludrocortisones and non-pharmacological interventions improved orthostatic tolerance.

Highlights

Dysautonomia is a condition characterized by orthostatic hypotension (OH), anhidrosis, impotence, sleep dysfunction, constipation and other gastro-intestinal symptoms [1]
In this report we investigated an unusual case of orthostatic hypertension and establish diagnosis of Pure Autonomic Failure (PAF) from exclusion and discussed role of autonomic function tests and heart rate variability in early diagnosis of the disease
The blood pressure (BP) was monitored in both arms with 130/80, 90/56 and 88/60 mm Hg in supine position, immediately after standing and after 3 minutes respectively

Summary

Introduction

Dysautonomia is a condition characterized by orthostatic hypotension (OH), anhidrosis, impotence, sleep dysfunction, constipation and other gastro-intestinal symptoms [1]. Pure Autonomic Failure (PAF) is an adult onset, progressive primary neurologic disorder of the autonomic nervous system and must be considered in the differential diagnosis of orthostatic hypotension [2]. Most adolescent patients with primary autonomic orthostatic hypotension have multiple system atrophy or PAF (Bradbury-Eggleston syndrome) [3,4]. In this report we investigated an unusual case of orthostatic hypertension and establish diagnosis of PAF from exclusion and discussed role of autonomic function tests and heart rate variability in early diagnosis of the disease.

Results

Conclusion