Cranial nerve, spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy

• Published in: Practical Neurology Vol. 12; no. 1; pp. 68 - 69
• Main Authors: Shah, Sachit, Chandrashekar, Hoskote, Manji, Hadi, Davagnanam, Indran
• Format: Journal Article Book Review
• Language: English
• Published: London BMJ Publishing Group Ltd 01.02.2012; BMJ Publishing Group; BMJ Publishing Group LTD
• Subjects: Biological and medical sciences; Cervical Plexus - pathology; Cranial Nerves - pathology; Humans; Hypertrophy - etiology; Injuries of the nervous system and the skull. Diseases due to physical agents; Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - complications; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - diagnosis; Spinal Nerve Roots - pathology; Traumas. Diseases due to physical agents; Nervous system diseases; Chronic; Cranial nerve; Peripheral nerve disease; Inflammatory hypertrophy; Polyneuropathy; Spinal nerve; Spinal root
• ISSN: 1474-7758; 1474-7766
• DOI: 10.1136/practneurol-2011-000040

Comment Although observed in a variety of other conditions including hereditary neuropathies, neurofibromatosis, lymphoma, Refsum's disease, leprosy and amyloidosis, chronic inflammatory demyelinating polyneuropathy is one of the commoner causes of hypertrophic neuropathy on MRI and has been reported to be present in 11-57% of patients. 1 2 The hypertrophy appears to be related to the duration of disease and is thought to be secondary to repeated demyelination and remyelination, with Schwann cell proliferation and onion-bulb formation leading to increased nerve volume. 2 The hypertrophic changes have been reported to correlate with the site of conduction block on electrophysiology but not with time from last relapse, type of therapy or disability status. 2 3 Gadolinium enhancement of the nerve roots, with or without hypertrophy, is indicative of blood-nerve barrier breakdown and may suggest clinically active disease. 3 Although rare, massive enlargement of the nerve roots may even cause spinal cord compression. 4 Acknowledgement: Reviewed by John Winer, Birmingham, UK.