Guillain-Barré syndrome mimics primary biliary cirrhosis-related myopathy

Abstract

Guillain-Barré syndrome (GBS) is an immune-mediated disorder characterized by acute polyneuropathy, ascending paralysis and post infectious polyneuritis. Two-thirds of patients present with a history of recent upper respiratory tract or gastrointestinal infection. The clinical history, neurologic examination and laboratory assessment allow for a straightforward diagnosis in the majority of cases. However, primary biliary cirrhosis (PBC) is known to cause clinically detectable muscular weakness. It is therefore critical to differentiate between PBC-associated muscular weakness and GBS-induced paralysis. Here, we report a patient with a longstanding history of PBC who developed progressive weakness and respiratory failure due to GBS, which clinically mimicked PBC myopathy. This is the first reported association between GBS and PBC.

Figure 1:

Muscle biopsy during life (A–D) and at autopsy (E and F). (A) Anterior thigh biopsy shows numerous fibers with decreased caliber and angulated fibers (H&E, ×10). (B) Individual myocyte necrosis and myophagocytosis are evident (H&E, ×20). (C) CD68-positive macrophages engulfing necrotic myocytes (×20). (D) CD45 immunostain is negative, illustrating the absence of myositis (×10). (E) Angulated fibers with decreased caliber are seen in the diaphragm (H&E, ×10). (F) High power view of individual myocyte undergoing myophagocytosis (×40).

Figure 2:

Spinal root ganglia and nerve roots showing marked acute demyelination and axonal degeneration. (A) Nerve root showing axonal degeneration (H&E, ×20). (B) LFB stain demonstrates reduced myelination (×20). Neurofilament immunostain highlights axonal loss and degenerative changes (×20). (D) Spinal ganglia with abundant acute inflammation. (E) LFB stain shows severe loss of myelination (×20). (F) Neurofilament immunostain highlights severe axonal degeneration (×20). (G) CD45-positive lymphocytes infiltrating the nerve root are seen. (H) Extensive CD45-positive lymphocytes involving the spinal ganglia. (I) Macrophages extensively involving the dorsal root ganglia (×20).