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Guillain-Barré syndrome mimics primary biliary cirrhosis-related myopathy.

Munday WR, DiCapua D, Vortmeyer A, Gomez JL - Oxf Med Case Reports (2015)

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology , Yale School of Medicine , New Haven, CT , USA.

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.

No MeSH data available.


Related in: MedlinePlus

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).
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OMV033F1: 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).

Mentions: Laboratory investigations revealed an elevated creatine kinase (CK) of 608 U/l (24–170 U/l). Serum myoglobin was significantly elevated at 3850 µg/l (<30 µg/l). Nerve conduction studies showed evidence for a myopathy and no evidence for a neuropathy or disorder of the neuromuscular junction. Cerebrospinal fluid protein was not elevated. Muscle biopsy revealed fibers with decreased calibers including angulated fibers and occasional fascicular atrophy (Fig. 1A). There was occasional fiber necrosis and myophagocytosis (Fig. 1B). CD68 immunostain highlighted the myophagocytosis of necrotic muscle fibers (Fig. 1C), whereas CD3 showed scattered positive T cells (Fig. 1D) suggesting a limited inflammatory process. There was muscle fiber splitting and occasional fiber regeneration.Figure 1:


Guillain-Barré syndrome mimics primary biliary cirrhosis-related myopathy.

Munday WR, DiCapua D, Vortmeyer A, Gomez JL - Oxf Med Case Reports (2015)

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).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4664928&req=5

OMV033F1: 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).
Mentions: Laboratory investigations revealed an elevated creatine kinase (CK) of 608 U/l (24–170 U/l). Serum myoglobin was significantly elevated at 3850 µg/l (<30 µg/l). Nerve conduction studies showed evidence for a myopathy and no evidence for a neuropathy or disorder of the neuromuscular junction. Cerebrospinal fluid protein was not elevated. Muscle biopsy revealed fibers with decreased calibers including angulated fibers and occasional fascicular atrophy (Fig. 1A). There was occasional fiber necrosis and myophagocytosis (Fig. 1B). CD68 immunostain highlighted the myophagocytosis of necrotic muscle fibers (Fig. 1C), whereas CD3 showed scattered positive T cells (Fig. 1D) suggesting a limited inflammatory process. There was muscle fiber splitting and occasional fiber regeneration.Figure 1:

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology , Yale School of Medicine , New Haven, CT , USA.

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.

No MeSH data available.


Related in: MedlinePlus