A Rare Case of Guillain-Barré Syndrome With Severe Pandysautonomia

Abstract

Acute pandysautonomia is a rare disorder characterized by autonomic failure affecting sympathetic, parasympathetic, and enteric functions. We present a case of acute inflammatory demyelinating polyneuropathy (AIDP) with severe pandysautonomia in a young, otherwise healthy, female who presented with gastrointestinal symptoms and sensory demyelinating polyneuropathy, which progressively worsened and subsequently developed bladder dysfunction and orthostatic hypotension. We discuss the challenges with diagnostic workup as well as the challenges we encountered as part of the management.

Keywords: Guillain-Barré; IVIG; demyelinating polyneuropathy; gastroparesis; nephrology; neurology; orthostatic hypotension; pandysautonomia.

Figure 1.

Skin lesion found over the right scapula.

Figure 2.

Electromyography showed severe sensory neuropathy and diffuse demyelinating neuropathy in bilateral superficial peroneal nerves and sural nerves, with normal nerve conduction studies in upper extremities. Also note the skin temperatures in extremities, which may associate with autonomic vascular compromise despite use of an α-adrenergic agonist. F waves also show significant latencies in conduction, bilateral tibial nerves greater than peroneal nerves.