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Published on: November 9, 2017
Amyloid neuropathy mimicking chronic inflammatory demyelinating polyneuropathy
Stephane Mathis1, Laurent Magy, Laho Diallo
1Department of Neurology, CHU Poitiers, University of Poitiers, Poitiers, France.
Muscle & Nerve
|December 23, 2011
Summary
Amyloid neuropathy can mimic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Early identification of amyloid neuropathy is crucial, especially in CIDP patients unresponsive to treatment, to ensure appropriate care.
Area of Science:
- Neurology
- Peripheral Nervous System Disorders
- Amyloidosis
Background:
- Amyloid neuropathy is a rare condition characterized by progressive sensory loss and autonomic dysfunction.
- It can be misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Purpose of the Study:
- To highlight the diagnostic challenges in differentiating amyloid neuropathy from CIDP.
- To emphasize the importance of considering amyloidosis in non-responsive CIDP cases.
Main Methods:
- Case series of 5 patients with amyloid neuropathy misdiagnosed as CIDP.
- Review of clinical presentation, electrodiagnostic studies, CSF analysis, and nerve biopsy findings.
- Genetic analysis for transthyretin mutations and assessment for acquired amyloidosis.
Main Results:
- All 5 patients initially diagnosed with CIDP showed no improvement with immunomodulatory treatment.
- Nerve biopsies confirmed amyloid deposits.
- Transthyretin (V30M) gene mutation identified in 3 patients; 2 had acquired amyloidosis.
Conclusions:
- Patients with suspected CIDP unresponsive to treatment require evaluation for alternative diagnoses like amyloid neuropathy.
- Careful assessment for autonomic involvement is essential in diagnosing amyloid neuropathy.
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