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Focal upper limb demyelinating neuropathy

P K Thomas¹, D Claus, A Jaspert

¹Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, UK.

Brain : a Journal of Neurology

|June 1, 1996

Summary

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Chronic inflammatory demyelinating polyneuropathy (CIDP) can present focally in the upper limbs with diverse motor and sensory symptoms. Early diagnosis and appropriate treatment, such as immunoglobulin therapy, are crucial for managing this condition.

Area of Science:

Neurology
Clinical Neuroscience

Background:

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
Focal presentations of CIDP, particularly in the upper limbs, are less common and can mimic other conditions.

Observation:

Nine patients with chronic upper limb demyelinating neuropathy were studied.
Manifestations varied, including purely motor, mixed motor-sensory, and predominantly sensory involvement.
Symptoms were often unilateral (seven patients) and sometimes associated with muscle hypertrophy or swelling.

Findings:

Seven patients showed reduced nerve conduction velocity and conduction block, consistent with focal CIDP.
Nerve biopsies confirmed demyelination in two cases.
Treatment response varied; seven patients improved, but one with monomelic motor neuropathy deteriorated with corticosteroids before improving with immunoglobulin therapy.

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Implications:

This study highlights the diverse clinical presentations of focal CIDP in the upper limbs.
Recognizing these varied patterns is essential for accurate diagnosis and timely intervention.
Intravenous immunoglobulin therapy may be a valuable treatment option for specific CIDP presentations.