Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Chronic acquired neuropathies can present with diverse clinical and pathological features.
• Distinguishing variants of demyelinating polyradiculoneuropathies is crucial for effective management.
• Autonomic nervous system involvement can complicate the diagnosis of polyneuropathies.

Observation:

• A case of chronic acquired neuropathy primarily affecting sensory and autonomic nerves was investigated.
• Electrophysiological studies revealed demyelinating polyradiculoneuropathy with axonal degeneration.
• Rectal mucosa biopsy showed depletion of postganglionic noradrenergic fibers, indicating autonomic dysfunction.

Findings:

• The patient exhibited a demyelinating polyradiculoneuropathy with significant axonal loss and autonomic denervation.
• Intravenous immunoglobulin and corticosteroid therapy led to symptom improvement and electrophysiological recovery.
• The observed features suggest a potential novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Implications:

• This case expands the understanding of CIDP variants, highlighting potential autoimmune targets beyond myelin.
• Autonomic dysfunction, including noradrenergic fiber depletion, should be considered in CIDP diagnoses.
• Successful treatment with immunomodulatory therapies underscores the inflammatory basis of this neuropathy.