Limits...
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

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

License
getmorefigures.php?uid=PMC4664928&req=5

OMV033F2: 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).

Mentions: The patient progressed to respiratory failure and an autopsy was requested. At autopsy, the brain and spinal cord did not show pathologic changes. The spinal root ganglia and para-ganglion nerve roots, however, revealed marked acute demyelination and axonal degeneration (Fig. 2A–F). Infiltrating lymphocytes and macrophages extensively involved the dorsal root ganglia and proximal peripheral nerves as highlighted by CD68 and CD45 immunostains, respectively (Fig. 2G–I). By light microscopy, the musculature showed similar morphology to that of the prior muscle biopsy (Fig. 1E and F).Figure 2:


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

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

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

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

OMV033F2: 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).
Mentions: The patient progressed to respiratory failure and an autopsy was requested. At autopsy, the brain and spinal cord did not show pathologic changes. The spinal root ganglia and para-ganglion nerve roots, however, revealed marked acute demyelination and axonal degeneration (Fig. 2A–F). Infiltrating lymphocytes and macrophages extensively involved the dorsal root ganglia and proximal peripheral nerves as highlighted by CD68 and CD45 immunostains, respectively (Fig. 2G–I). By light microscopy, the musculature showed similar morphology to that of the prior muscle biopsy (Fig. 1E and F).Figure 2:

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