Abstract
OBJECTIVE: To report three patients with chronic inflammatory demyelinating polyneuropathy (CIDP) presenting with symptoms suggestive of cervical (one patient) and lumbar root disease. METHODS: Nerve conduction studies, EMG, and nerve biopsy were carried out, having found the nerve roots to be very enlarged on MRI, CT myelography, and at surgery. RESULTS: Clinically, peripheral nerve thickening was slight or absent. Subsequently one patient developed facial nerve hypertrophy. This was mistaken for an inner ear tumour and biopsied, with consequent facial palsy. Neurophysiological tests suggested a demyelinating polyneuropathy. Sural nerve biopsy showed in all cases some loss of myelinated fibres, inflammatory cell infiltration, and a few onion bulbs. Hypertrophic changes were much more prominent on posterior nerve root biopsy in one patient: many fibres were surrounded by several layers of Schwann cell cytoplasm. There was an excellent response to steroids in two patients but not in the third (most advanced) patient, who has benefited only marginally from intravenous immunoglobulin therapy. CONCLUSIONS: MRI of the cauda equina may be a useful adjunct in the diagnosis of CIDP.
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- AUSTIN J. H. Recurrent polyneuropathies and their corticosteroid treatment; with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone, and prednisone. Brain. 1958 Jun;81(2):157–192. doi: 10.1093/brain/81.2.157. [DOI] [PubMed] [Google Scholar]
- Antoine J. C., Baril A., Guettier C., Barral F. G., Bady B., Convers P., Michel D. Unusual amyloid polyneuropathy with predominant lumbosacral nerve roots and plexus involvement. Neurology. 1991 Feb;41(2 ):206–208. doi: 10.1212/wnl.41.2_part_1.206. [DOI] [PubMed] [Google Scholar]
- Barohn R. J., Kissel J. T., Warmolts J. R., Mendell J. R. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989 Aug;46(8):878–884. doi: 10.1001/archneur.1989.00520440064022. [DOI] [PubMed] [Google Scholar]
- Crino P. B., Grossman R. I., Rostami A. Magnetic resonance imaging of the cauda equina in chronic inflammatory demyelinating polyneuropathy. Ann Neurol. 1993 Mar;33(3):311–313. doi: 10.1002/ana.410330314. [DOI] [PubMed] [Google Scholar]
- De León G. A., Hodges F. J., 3rd Subarachnoid block and enlargement of the spinal canal in hypertrophic neuritis. J Neurol Sci. 1976 Jun;28(2):139–146. doi: 10.1016/0022-510x(76)90099-x. [DOI] [PubMed] [Google Scholar]
- De Silva R. N., Willison H. J., Doyle D., Weir A. I., Hadley D. M., Thomas A. M. Nerve root hypertrophy in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1994 Feb;17(2):168–170. doi: 10.1002/mus.880170206. [DOI] [PubMed] [Google Scholar]
- Dyck P. J., Lais A. C., Ohta M., Bastron J. A., Okazaki H., Groover R. V. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975 Nov;50(11):621–637. [PubMed] [Google Scholar]
- Ginsberg L., Platts A. D., Thomas P. K. Chronic inflammatory demyelinating polyneuropathy mimicking a lumbar spinal stenosis syndrome. J Neurol Neurosurg Psychiatry. 1995 Aug;59(2):189–191. doi: 10.1136/jnnp.59.2.189. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hawke S. H., Hallinan J. M., McLeod J. G. Cranial magnetic resonance imaging in chronic demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1990 Sep;53(9):794–796. doi: 10.1136/jnnp.53.9.794. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Krendel D. A., Parks H. P., Anthony D. C., St Clair M. B., Graham D. G. Sural nerve biopsy in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1989 Apr;12(4):257–264. doi: 10.1002/mus.880120402. [DOI] [PubMed] [Google Scholar]
- Matsuda M., Ikeda S., Sakurai S., Nezu A., Yanagisawa N., Inuzuka T. Hypertrophic neuritis due to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a postmortem pathological study. Muscle Nerve. 1996 Feb;19(2):163–169. doi: 10.1002/(SICI)1097-4598(199602)19:2<163::AID-MUS6>3.0.CO;2-C. [DOI] [PubMed] [Google Scholar]
- McCombe P. A., Pollard J. D., McLeod J. G. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110(Pt 6):1617–1630. doi: 10.1093/brain/110.6.1617. [DOI] [PubMed] [Google Scholar]
- Morgan G. W., Barohn R. J., Bazan C., 3rd, King R. B., Klucznik R. P. Nerve root enhancement with MRI in inflammatory demyelinating polyradiculoneuropathy. Neurology. 1993 Mar;43(3 Pt 1):618–620. doi: 10.1212/wnl.43.3_part_1.618. [DOI] [PubMed] [Google Scholar]
- Naganuma M., Doi S., Shima K., Matsumoto A., Tashiro K. [Chronic inflammatory demyelinating polyradiculoneuropathy associated with multifocal nerve hypertrophy--report of a case with MRI study]. Rinsho Shinkeigaku. 1991 Nov;31(11):1186–1191. [PubMed] [Google Scholar]
- Ormerod I. E., Waddy H. M., Kermode A. G., Murray N. M., Thomas P. K. Involvement of the central nervous system in chronic inflammatory demyelinating polyneuropathy: a clinical, electrophysiological and magnetic resonance imaging study. J Neurol Neurosurg Psychiatry. 1990 Sep;53(9):789–793. doi: 10.1136/jnnp.53.9.789. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rosenberg N. L., Bourdette D. Hypertrophic neuropathy and multiple sclerosis. Neurology. 1983 Oct;33(10):1361–1364. doi: 10.1212/wnl.33.10.1361. [DOI] [PubMed] [Google Scholar]
- Schoene W. C., Carpenter S., Behan P. O., Geschwind N. 'Onion bulb' formations in the central and peripheral nervous system in association with multiple sclerosis and hypertrophic polyneuropathy. Brain. 1977 Dec;100(4):755–773. doi: 10.1093/brain/100.4.755. [DOI] [PubMed] [Google Scholar]
- Thomas P. K., Walker R. W., Rudge P., Morgan-Hughes J. A., King R. H., Jacobs J. M., Mills K. R., Ormerod I. E., Murray N. M., McDonald W. I. Chronic demyelinating peripheral neuropathy associated with multifocal central nervous system demyelination. Brain. 1987 Feb;110(Pt 1):53–76. doi: 10.1093/brain/110.1.53. [DOI] [PubMed] [Google Scholar]
- Waddy H. M., Misra V. P., King R. H., Thomas P. K., Middleton L., Ormerod I. E. Focal cranial nerve involvement in chronic inflammatory demyelinating polyneuropathy: clinical and MRI evidence of peripheral and central lesions. J Neurol. 1989 Oct;236(7):400–405. doi: 10.1007/BF00314898. [DOI] [PubMed] [Google Scholar]