Paroxysmal sympathetic hyperactivity and refractory hypotension in Guillain-Barré syndrome with autoimmune encephalitis: a case report and literature review

TL;DR

A rare case of Guillain-Barré syndrome combined with autoimmune encephalitis led to severe autonomic dysfunction, but the patient improved with integrated treatment over six months.

Contribution

Highlights the rare co-occurrence of GBS and AE, emphasizing the need for integrated therapy to manage autonomic complications.

Findings

The patient exhibited paroxysmal sympathetic hyperactivity and refractory hypotension during GBS and AE.

Integrated treatment with IVIG and PLEX led to successful weaning from ventilation and resolution of hypotension.

Severe autonomic dysfunction can occur at any stage of GBS combined with AE, requiring prolonged ICU care.

Abstract

Guillain-Barré syndrome (GBS) is an acute inflammatory peripheral nerve disorder mediated by autoimmune mechanisms. However, its co-occurrence with autoimmune encephalitis (AE) is rare. We present a 51-year-old man who initially presented with symmetrical numbness and weakness in all limbs, followed by hallucinations, behavioral abnormalities, and consciousness disturbances. Cerebrospinal fluid (CSF) analysis revealed protein-cell dissociation, indicative of GBS. Brain magnetic resonance imaging (MRI) showed abnormal signals in the splenium of the corpus callosum. Electromyography showed reduced amplitude in motor nerve conduction of bilateral common peroneal nerves and left tibial nerves. He developed respiratory distress, requiring tracheal intubation and mechanical ventilation. Finally, he was diagnosed with GBS combined with AE and received treatment with intravenous immunoglobulin (IVIG) and plasma exchange (PLEX). Subsequently, he experienced paroxysmal sympathetic hyperactivity (PSH) and refractory hypotension requiring vasopressor support. After comprehensive treatment, he was successfully weaned off the ventilator, and his refractory hypotension resolved after more than six months. This case illustrates that severe autonomic dysfunction can occur at any stage of GBS companied with AE. Furthermore, these patients often require prolonged ICU stays and experience slower recovery, but may still achieve a favorable outcome with appropriate integrated therapy.

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