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Published on: May 11, 2022
Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis
Ken-ya Murata1, Hiroshi Ishiguchi, Ryuki Ando
1Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama, Wakayama 641-8510, Japan.
Summary
We present a rare case of chronic inflammatory demyelinating polyneuropathy linked to asymptomatic primary biliary cirrhosis (PBC). This highlights the importance of investigating PBC in patients with specific neurological symptoms.
Area of Science:
- Neurology
- Hepatology
- Immunology
Background:
- Primary biliary cirrhosis (PBC) is a chronic liver disease often associated with autoimmune conditions.
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder affecting peripheral nerves.
Observation:
- A patient presented with neurological symptoms suggestive of CIDP, including slow nerve conduction velocities and sensory deficits.
- Liver biopsy revealed asymptomatic PBC, with minimal clinical or biochemical signs of liver disease.
- Electrophysiological studies showed significant nerve conduction abnormalities, including conduction blocks.
Findings:
- The patient was diagnosed with CIDP in the context of previously undiagnosed, asymptomatic PBC.
- This case underscores the rare but significant association between these two conditions.
- Asymmetrical sensory-dominant neuropathy with high IgM titers warrants investigation for anti-mitochondrial antibodies and potential PBC.
Implications:
- Early detection of asymptomatic PBC is crucial in patients with unexplained demyelinating neuropathies.
- This association broadens the clinical spectrum of PBC complications.
- Differentiating demyelinating neuropathy associated with PBC is vital for appropriate patient management.
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