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Related Experiment Videos

Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy.

K C Gorson1, A H Ropper, D H Weinberg

  • 1Division of Neurology, St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Massachusetts 02135, USA.

Muscle & Nerve
|June 12, 1999
PubMed
Summary

A rare variant of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) can affect only the upper limbs, mimicking other neuropathies. This upper limb CIDP (UL-CIDP) may have a less favorable treatment response than generalized CIDP.

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Area of Science:

  • Neurology
  • Clinical Electrophysiology

Background:

  • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) typically presents with generalized symptoms.
  • Rare cases of CIDP involve focal or multifocal upper limb presentation (UL-CIDP).

Purpose of the Study:

  • To characterize the clinical and electromyographic (EMG) features of UL-CIDP.
  • To compare UL-CIDP with generalized CIDP (G-CIDP) and multifocal motor neuropathy (MMN).

Main Methods:

  • Retrospective review of 10 patients with UL-CIDP.
  • Comparison of clinical and EMG findings with G-CIDP and MMN patient cohorts.
  • Analysis of nerve conduction studies, including conduction block and sensory nerve involvement.

Main Results:

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  • UL-CIDP patients presented with numbness, paresthesias, weakness, and pain predominantly in the upper limbs.
  • Conduction block was common in motor nerves (68%), and sensory nerves were frequently abnormal (73%).
  • UL-CIDP was distinguished from MMN by sensory nerve abnormalities and absence of anti-GM1 antibodies.
  • No significant clinical or electrophysiological differences were found between UL-CIDP and G-CIDP, apart from regional onset.
  • Patients with G-CIDP showed a greater magnitude of recovery after treatment compared to UL-CIDP.

    • Conclusions:

    • UL-CIDP is a distinct variant of CIDP presenting with upper extremity sensorimotor symptoms.
    • UL-CIDP can mimic mononeuropathies but is distinguishable from MMN.
    • This variant may have a less favorable treatment outcome compared to generalized CIDP.