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Sensory CIDP presenting as cryptogenic sensory polyneuropathy
Russell L Chin1, Norman Latov, Howard W Sander
1Weill Medical College of Cornell University, Department of Neurology, New York, NY 10022, USA. ruc9002@med.cornell.edu
Journal of the Peripheral Nervous System : JPNS
|September 15, 2004
Summary
Chronic inflammatory demyelinating polyneuropathy (CIDP) can mimic cryptogenic sensory neuropathy. Sural nerve biopsy is crucial for diagnosing sensory CIDP in patients with progressive sensory loss and normal or axonal electrophysiology.
Area of Science:
- Neurology
- Neuroscience
- Clinical Medicine
Background:
- Cryptogenic sensory neuropathy is often diagnosed when no cause is found for distal sensory neuropathy with minimal electrodiagnostic abnormalities.
- Some patients diagnosed with cryptogenic sensory neuropathy may actually have sensory chronic inflammatory demyelinating polyneuropathy (CIDP).
Purpose of the Study:
- To report that chronic inflammatory demyelinating polyneuropathy (CIDP) can present as cryptogenic sensory neuropathy.
- To highlight the diagnostic utility of sural nerve biopsy in identifying sensory CIDP.
Main Methods:
- Retrospective review of eight patients diagnosed with CIDP via sural nerve biopsy.
- Analysis of clinical presentation, electrodiagnostic studies, and nerve biopsy findings.
- Evaluation of response to IVIg therapy.
Main Results:
- All patients presented with sensory neuropathy (numbness, paresthesias, large fiber sensory loss) and normal muscle strength.
- Electrodiagnostic studies showed minimal abnormalities or axonal changes.
- Sural nerve biopsies confirmed chronic myelinopathy with segmental remyelination (13-40%).
- Four patients treated with IVIg showed improved sensation and gait.
Conclusions:
- Sensory CIDP can be misdiagnosed as cryptogenic sensory polyneuropathy due to normal or axonal electrophysiologic features.
- Sural nerve biopsy is recommended for progressive, predominantly large fiber sensory neuropathies of unknown cause.
- Early diagnosis of sensory CIDP allows for therapeutic intervention with potential for improvement.