Central lesions in chronic inflammatory demyelinating polyneuropathy: An MRI study T.E. Feasby, MD; A.F. Hahn, MD; W.J. Koopman, RN; and D.H. Lee, MD

Article abstract-To determine the frequency of the possible association between chronic inflammatory demyelinating polyneuropathy (CIDP) and MS, we did magnetic resonance imaging (MRI) of the brain in 19 patients with CIDP. Only 1patient had clinical signs suggestive of central involvement. Seven of the 19 scans showed 2 or more brain “lesions.” In 1case the cause was an infarct and in 5 cases the patients were over 55 years of age and the lesions were not typical of MS. One 38-year-old patient had 2 small subcortical lesions. Typical MS lesions on MRI are uncommon in CIDP. NEUROLOGY 1990;40:476-478

Chronic inflammatory demyelinating.po1yneuropathy (CIDP) is regarded as restricted to the peripheral nervous system (PNS). The usual explanation for this localization is that the immune response in CIDP is directed against an antigen confined to the PNS, analogous to experimental allergic neuritis where lesionsin the P,-induced form of the disease are confined to the PNS where P2protein occurs. However, there are occasional pathologic and clinical reporW3 of the coexistence of CNS and PNS lesions in cases resembling CIDP. Two recent studies used magnetic resonance imaging (MRI) to identify brain lesions in patients with CIDP. Thomas et a14 reported 6 patients with the clinical features of both CIDP and MS; 5 patients who had MRIs all showed lesions typical of MS. Mendell et a15 did MRI on 16 patients with CIDP, 3 of whom had clinical features suggestive of MS. Six patients, including the 3 with “suspected” MS, had MRI lesions similar to those of MS. These studies imply that central demyelinating lesions may be common in CIDP, but each author acknowledged a selection bias in favor of CNS involvement in at least some cases. Consequently, the frequency of central lesions in CIDP cannot be estimated. To address this problem, we report a series of MRIs done on all our patients with CIDP seen in the last 8 years. Methods. Nineteen patients fulfilling the clinical criteria for the diagnosis of CIDP6were included. Monoclonal gammopathy was excluded. All had electrophysiologic studies of peripheral nerve conduction showing abnormalities typical of CIDP.7 Eight patients had pattern visual evoked response (VER) studies. Sixteen had biopsies of the deep and superficial peroneal or the sural nerves.

MRI was done on a General Electric Signa 1.5 tesla scanner. Five-mm-thick TI-weighted saggital images were obtained with T R 500 to 600 and TE 20 msec, a 24-cm field of view with 256 phase encoding steps and 2 averages. Five-mmthick T,-weighted axial images were used with T R 2,000 to 2,500 msec, with double echoes at T E 30 to 35 and T E 70 msec; the field of view was 20 to 24 cm and 128to 256 phase encoding steps were used. One or 2 averages were used. Slices were contiguous or separated by 2.5 mm in the axial plane.

Results. The l u a t i e n t s varied in age from 7 to 68 years at onset ( X = 39.5) and included 12 males. All fulfilled the clinical criteria for the diagnosis of CIDP.6 Sixteen also had a postural and intention tremor of the hands. CSF protein concentration was elevated in 16/18. Electrophysiologic studies revealed conduction velocity slowing in all cases and conduction block in 12/13 where this was examined adequately.8 Sensory potentials were absent in 8patients, decreased in amplitude in 10, and normal in 1. Biopsy of the deep and superficial peroneal nerves or the sural nerve was done in 16 patients; demyelination was found in 15/16 and mononuclear inflammation in 10/16. All patients had been treated previously, and most were being treated when MRI was done. Plasmapheresis had been given to 14, azathioprine to 12, and corticosteroids to all 19 patients. VERs were tested in 8 patients. All were normal except for 1 patient who showed a bilateral delay in conduction (110 and 114 msec). His ocular exam was normal, and he had no clinical signs of CNS lesions. The MRI scan of his head was normal. MRI was done 9 weeks to 17 years following the clinical onset of CIDP ( X = 5.5 years). Ten of 19

From the Department of Clinical Neurological Sciences (Drs. Feasby and Hahn, and W.J. Koopman),Victoria Hospital, and the Department of Radiology (Dr. Lee), University Hospital, University of Western Ontario, London, Ontario, Canada. Received July 19,1989. Accepted for publication in final form August 31,1989. Address correspondenceand reprint requests to Dr. Thomas E. Feasby, Department of Clinical Neurological Sciences, Victoria Hospital, 375 South Street, London, ON N6A 4G5, Canada. 476 NEUROLOGY 40 March 1990

Figure. Proton density scan‘(A) and T,-weighted scan (B) showing 2 small subcortical areas of increased signal in a 38-year-old woman.

patients had normal MRIs and 2 patients had a single small subcortical area of high signal intensity on the T,-weighted scans. Neither of these 2 patients was considered likely to have MS on MRI criteria? Seven patients had multiple MRI lesions. One scan had t h e appearance of an old infarct. Five other patients with multiple lesions were over age 55 when scanned. The “lesions” in each of t h e 5 were mainly subcortical, not periventricular, and were typical of those commonly found in older control subjects.lOJ1One patient, age 38, had 2 small subcortical lesions on her MRI (figure). She had no clinical signs or symptoms of CNS involvement or laboratory evidence of MS.

numbness from the right groin to the right costal margin. Examination showed mild thinning of hand and foot muscles, increased tone in the left quadriceps muscle, and normal strength. Pinprick sensation was reduced in his feet and over the right lower abdomen and back from T-8 to T-12.Vibratory sensation was reduced in his fingers and below his knees. His tendon reflexes were 1+ in his arms, 2 + at his knees, and barely present at his ankles. He had bilateral Babinski signs. A myelogram and spinal and head MRL were all normal. Visual and auditory evoked responses were normal and CSF did not show oligoclonal banding. He responded symptomatically to treatment with prednisone. Examination 6 months later was unchanged except that the patch of abdominal numbness had disappeared.

Case report. Only 1patient had clinical features suggestive

Discussion. This study shows that, in an unselected series of well-documented cases of CIDP, CNS lesions typical of MS are not common on MRI. We did find MRI evidence of small subcortical “lesions” in 6 patients, 5 of whom were over 55 years of age. Fazekas et all1 studied older normal controls by MRI and found that 44% over the age of 50 had white matter “lesions.” They compared these MRI abnormalities with those found in a series of older MS patients and developed criteria for reliably differentiating these MRI findings

of a CNS lesion, possibly due to MS. At age 24, this man noted numbness in his feet and hands which spread slowly up to his hips and shoulders over 8 months. He developed mild weakness of his legs so that he could not run. Examination showed normal cranial nerves, mild thinning of distal limb muscles, normal muscle tone, and mild distal weakness. He had reduced vibratory and pinprick sensation in his hands and feet and areflexia. His plantar reflexes were downgoing. CSF protein level was 700 mg/l. Nerve conduction studies showed features of demyelination, including slow conduction velocities (median and peroneal motor = 35 m/sec) and conduction block. The deep and superficial peroneal nerve biopsy showed both demyelination and remyelination. He was treated with prednisone and showed modest improvement. Over the next 6 years he had 5 mildincreases in his distal numbness and tingling, responding on each occasion to treatment with prednisone and once to azathioprine. On 1 occasion he also had a patch of subjective numbness over his left chest. Seven years after the initial symptoms, he developed increased numbness in his feet, decreased strength and coordination in his legs, numbness of his right hand, and a patch of

in the normal elderly from those of MS.They required 2 of t h e following 3 criteria: at least 1 lesion >6 mm, a lesion abutting the ventricular bodies, and an infratentorial lesion. Applying these MRI criteria, none of our 5 patients over age 55 could be classified as having MS lesions. Subcortical lesions of this sort can be seen in MS patients but are not d i a g n o ~ t i c .T~h e 2 small subcortical lesions seen in a 38-year-old patient were not typical of MS but suspicion was raised because of her age. There were no other laboratory or clinical features of MS. March 1990NEUROLOGY 40 477

There has been continuing controversy over the apparent restriction of lesions to the PNS in cases of human demyelinating neuropathies, principally CIDP and acute Guillain-BarrB syndrome (GBS). The reported “exceptions” to this rule fall into the categories of (1) cases of CIDP where “MS lesions” have been demonstrated clinically, pathologically, or by diagnostic techniques; (2) cases of MS where PNS abnormalities have been detected pathologically or physiol o g i ~ a l l y ~ , (3) ~ J ~cases - ~ ~ where ; CNS and PNS lesions have developed simultaneously3J8; and (4) cases of acute GBS with CNS lesion^.^^-^^ The 1st category deserves further comment. The lack of typical MS lesions on MRI in our series of 19 patients with CIDP contrasts with 2 other MRI studies in CIDP,4s5 each reporting evidence of central lesions. However, the method of case selection in those studies was quite different. Thomas et a14 studied 6 patients with clinical features of both MS and CIDP; thus, it was not unexpected that the 5 cases scanned showed MRI “lesions” typical of MS. Mendell et a15 found positive scans in 6 of 16 CIDP patients; 3 of the MRI-positive patients also had clinical features of MS, leaving 3 MRI-positive cases of the remaining 13 with apparently no CNS signs or symptoms. The advanced age of these 3 latter patients (70, 77, and 79 years) suggests that the MRI lesions might be due to processes other than demyelination, such as cerebrovascular disease.gPakalnis et a12*reported 18patients with CIDP, 9 of whom had VER abnormalities (prolonged latency). Most of these cases were included in the report of Mendell et al.5 Four patients with abnormal VERs had no clinical signs of CNS disease. CSF protein electrophoresis studies were not reported. Rubin et aP reported a patient with CIDP confirmed by nerve biopsy who developed clinical signs of MS several years later. There is evidence of the clinical coincidence of MS and CIDP from the published reports reviewed here. This suggests a greater than chance occurrence of these 2 conditions. However, our study indicates that most patients with CIDP do not have evidence of MS and that the concurrence of these 2 diseases is uncommon.

Acknowledgment We thank Ms. Mary Ann Leverton for preparation of the manuscript.

478 NEUROLOGY 40 March 1990

References 1. Schoene W, Carpenter S, Behan PO, Geschwind N. ‘Onion bulb’ formations in the central and peripheral nervous system in association with multiple sclerosis and hypertrophic polyneuropathy. Brain 1977;100:755-773. 2. Ro YI, Alexander CB, Oh SJ.Multiple sclerosis and hypertrophicdemyelinatingperipheral neuropathy. Muscle Nerve 1983;6312-316. 3. Rubin M, Karpati G, Carpenter S. Combined central and peripheral myelinopathy. Neurology 1987;37:1287-1290. 4. Thomas PK, Walker RWH, Rudge P, et al. Chronic demyelinating peripheral neuropathy associated with multifocal central nervous system demyelination. Brain 1987;110:53-76. 5. Mendell JR, Kolin S, Kissel JT, Weiss KL, Chakeres DW, Rammohan KW. Evidence for central nervous system demyelination in chronic inflammatory demyelinating polyradiculoneuropathy Neurology 1987;37:1291-1294. 6.Dyck PJ, Lais AC, Ohta M, J3astmn JA, Okazald H, Gmver RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-637. 7. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyneuropathy: a clinical and electrophysiological study of 91 cases. Brain 1978;110:1617-1630. 8. Brown WF, Feasby TE. Conduction block and denervation in Guillain-BarrL!polyneuropathy. Brain 1984;107:219-239. 9. Ormerod IE, Miller DH, McDonald WI, et al. The role of MRI imaging in the assessment of multiple sclerosisand isolated neurological lesions: a quantitative study. Brain 1987;1101579-1616. 10. Fazekas F, Chawluk JB, Alavi A, Hurtig HI, Zimmerman RA. MR signal abnormalities at 1.5T in Alzheimer’s dementia and normal aging. AJNR 1987;8421-426. 11. Fazekas F, OffenbacherH, Fuchs S, et al. Criteria for an increased specificity of MRI interpretation in elderly subjects with suspected multiple sclerosis. Neurology 1988;38:1822-1825. 12. RosenbergNL, Bourdette D. Hypertrophic neuropathy and multiple sclerosis. Neurology 1983;33:1361-1364. 13. Pollock M, Calder C, Allpress S. Peripheral nerve abnormalities in multiple sclerosis. Ann Neurol 1977;2:41-48. 14. Eisen A, Paty D, Hoirch M. Altered supernormality in multiple sclerosis peripheral nerve. Muscle Nerve 1982;5:411-414. 15.Eisen A, Yufe R, Trop D, Campbell I. Reduced neuromuscular transmission safety factor in multiple sclerosis. Neurology 1978;28598-602. 16. Weir A, Hansen S, Ballantyne JP. Motor unit potential abnormalities in multiple sclerosis: further evidence for a peripheral nervous system defect. J Neurol Neurosurg Psychiatry 1980;43:999-1004. 17. Weir AI, Hansen S, Ballantyne JP. The single fibre electromyographic jitter in multiple sclerosis. J Neurol Neurosurg Psychiatry 1979;42:1146-1150. 18. Lassmann H, Budka H, Schnaberth G. Inflammatory demyelinating polyradiculitis in a patient with multiple sclerosis. Arch Neurol 1981;3899-102. 19. Forrester C, Lascelles RG. Association between polyneuritis and multiple sclerosis.J Neurol Neurosurg Psychiatry 1979;42864-866. 20. Best PV. Acute polyradiculoneuritis associated with demyelinated plaques in the central nervous system: report of a case. Acta Neuropathol (Berl) 1985;67:230-234. 21. Pakalnis A, Drake ME, Barohn RJ,Chakeres DW, Mendell JR. Evoked potentials in chronic inflammatory demyelinating polyneuropathy. Arch Neurol 1988;45:1014-1016.

.

Central lesions in chronic inflammatory demyelinating polyneuropathy: An MRI study T. E. Feasby, A. F. Hahn, W. J. Koopman, et al. Neurology 1990;40;476 DOI 10.1212/WNL.40.3_Part_1.476 This information is current as of March 1, 1990 Updated Information & Services

including high resolution figures, can be found at: http://www.neurology.org/content/40/3_Part_1/476.full.html

Citations

This article has been cited by 5 HighWire-hosted articles: http://www.neurology.org/content/40/3_Part_1/476.full.html##oth erarticles

Permissions & Licensing

Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions

Reprints

Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus

Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1990 by Edgell Communications, Inc.. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.

Central lesions in chronic inflammatory demyelinating polyneuropathy: an MRI study.

To determine the frequency of the possible association between chronic inflammatory demyelinating polyneuropathy (CIDP) and MS, we did magnetic resona...
410KB Sizes 0 Downloads 0 Views