Journal ofNeurology, Neurosurgery, and Psychiatry 1995;59:189-191 189

LESSON OF THE MONTH J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.189 on 1 August 1995. Downloaded from Chronic inflammatory demyelinating mimicking a syndrome

L Ginsberg, A D Platts, P K Thomas

Abstract showed a diffuse demyelinating polyneuropa- A patient with chronic inflammatory thy. She was treated with oral , demyelinating polyneuropathy (CIDP) with some improvement. In 1987, her back established by biopsy developed cauda recurred. There was a reduction in motor equina symptoms due to swelling of the nerve conduction velocity (25 m/s) and a CSF nerve roots in the lumbar spinal canal. protein concentration of 1 A-4 8 g/l, grossly Magnetic resonance imaging of the lum- enlarged nerve roots were seen in the lumbar bar spine showed profoundly thickened thecal sac at myelography. nerve roots from the level of the conus treatment was again beneficial. medullaris, filling the caudal thecal sac. In 1992, she experienced a severe recur- Immunosuppressant treatment produced rence of lumbar pain, worse with walking or partial clinical and radiological resolu- sitting for extended periods, and relieved by tion. This case shows that spinal rest in a standing position. She also described compressive syndromes may occur in numbness and burning paraesthesiae over the acquired hypertrophic neuropathies as anterior aspect of the left thigh, with mild well as in hereditary motor and sensory numbness distally in both lower limbs. There neuropathy and expands the range of the was no weakness, bladder and bowel function clinical presentation of CIDP. were normal, and there were no symptoms in the upper limbs or symptoms referable to the (J Neurol Neurosurg Psychiatry 1995;59: 189-191) cranial nerves. Examination showed normal cranial nerves and no abnormal motor signs. Tendon reflexes were absent apart from a Keywords: chronic inflammatory demyelinating sluggish right biceps jerk; the plantar polyneuropathy; lumbar spinal canal; magnetic reso- responses were flexor. There was mild subjec- nance imaging tive impairment for light touch and pinprick distally in all four limbs. Vibration sense and

joint position sense were normal. Peripheral http://jnnp.bmj.com/ Widespread nerve enlargement may occur nerves were not thickened. in leprosy, amyloid neuropathy, chronic Routine haematological and biochemical inflammatory demyelinating polyneuropathy investigations were normal with no evidence (CIDP), acromegaly, neurofibromatosis, of a serum paraprotein. An autoantibody Department of Clinical Refsum's disease, and hereditary motor and screen was negative. DNA analysis did not Neurosciences, Royal sensory neuropathy (HMSN) types I and III.' show the duplication on the short arm of Free Hospital School In hypertrophic neuropathy, enlargement of chromosome 17 associated with HMSN Ia. ofMedicine, London, nerve roots to on September 29, 2021 by guest. Protected copyright. UK the proximal has been reported Nerve conduction studies showed a gener- L Ginsberg produce spinal compression syndromes,2-14 alised sensorimotor neuropathy with patchy P K Thomas although this has generally been slowing (to 20 m/s motor nerve conduction University associated with the hereditary causes. 14 velocity) and conduction block. Magnetic res- Department of We describe a patient with CIDP who devel- onance imaging of the lumbar spine showed Clinical , Institute ofNeurology, oped a lumbar "spinal stenosis" syndrome that the theca was almost completely occu- London, UK due to swollen nerve roots within the caudal pied by enlarged nerve roots from T12 to L5. L Ginsberg theca. There was abnormal enhancement of the P K Thomas thickened nerve roots after intravenous Department of triamine Radiology, Royal Free gadolinium diethylene penta-acetic Hospital, London, UK Case report acid (DTPA) (figure A, B). A sural nerve fas- A D Platts A 44 year old woman presented with a 13 year cicular biopsy showed demyelination with Correspondence to: history of fluctuating low and sen- minor hypertrophic changes, moderate loss of Dr L Ginsberg, Department of Clinical Neurosciences, sory symptoms in the lower limbs. She had myelinated nerve fibres, and scattered infiltra- Royal Free Hospital School otherwise previously been healthy. Her par- tion with CD4 and CD8 lymphocytes. The of Medicine, Rowland Hill Street, London NW3 2PF, ents were first cousins but there was no family appearances were those of CIDP. UK. history of neuromuscular disease. At her origi- The patient was treated with 300 mg Received 10 February 1995 nal presentation in 1979, she reported low cyclosporin A twice daily, with relief of her and in revised form 2 May 1995 back pain, left sided , and sensory symptoms suggestive of lumbar spinal steno- Accepted 9 May 1995 symptoms in her left leg. Investigations sis. Repeat MRI one year after starting 190 Ginsberg, Platts, Thomas J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.189 on 1 August 1995. Downloaded from

A Tl weighted spin echo images of the lumbar spine. (A) sagzttal, (B) axial L314 both post-gadolinium pretreatment, showing the thecal sac filled with abnormal enhancing enlarged nerve roots, virtually obliterating the CSF space from Tl2 to L5. (C) axial L314 post- gadolinium post-treatment (one year with cyclosponrn A), showing persistent thickening oflumbosacral nerve roots but abnormal enhancement no longer apparent. suggested .'5 Finally, the response to immunosuppressant treatment, both clinically and in terms of resolution of immunosuppressant treatment showed that the abnormal enhancement on MRI, was con- the spinal canal remained occupied by abnor- sistent with CIDP rather than HMSN. mal soft tissue from the lower thoracic region Immunosuppressant treatment was chosen to L5/S1 compatible with persistent thicken- in 1992, as opposed to a further course of cor- ing of the lumbosacral nerve roots. Abnormal ticosteroids, as these had previously only pro- response, and also in view of enhancement of these roots after intravenous duced a partial http://jnnp.bmj.com/ gadolinium DTPA was no longer apparent, the patient's borderline hypertension and however (figure C). moderate . In the absence of muscle weakness or severe sensory loss, treatment with plasma exchange or high dose intra- Discussion venous immunoglobulin was judged inappro- Differentiating between CIDP and HMSN is priate and cyclosporin A was thought to be a common diagnostic problem in clinical neu- more rapidly acting and effective than azathio- rology. In this patient's case, a diagnosis of prine. 16 on September 29, 2021 by guest. Protected copyright. HMSN had previously been entertained In view of the relative predilection of the despite the negative family history, partial neuropathological changes of CIDP for the response to corticosteroids, and raised CSF proximal portions of nerves,'7 it is perhaps protein concentration. This may in part have surprising that there is little published refer- been because clinical presentations similar to ence to CIDP, as opposed to HMSN, produc- the patient's, with a syndrome ing spinal compression syndromes. Indeed, due to swollen nerve roots in the lumbar the absence of palpable peripheral nerve theca, have only previously been described in thickening in our patient in the context of detail in the context of hereditary hyper- such pronounced spinal root enlargement trophic neuropathies. '4 The detailed investiga- would have been unexpected in HMSN. tion of this patient, in particular the 1992 The bias in the medical literature towards of nerve conduction and biopsy findings, HMSN rather than CIDP as a cause pointed towards CIDP, however, as the cor- spinal compression may in part stem from the rect underlying diagnosis. Further support time when these conditions were nosologically was provided by the imaging. Thus although less distinct, patients being given the generic the actual nerve enlargement was non-spe- diagnosis of "progressive hypertrophic inter- cific, pathological enhancement of the roots stitial neuropathy of Dejerine and Sottas".' after intravenous gadolinium DTPA, indica- Many of the case reports of spinal compres- tive of breakdown of the blood-nerve barrier, sion syndromes from that era, and indeed Chronic inflammatory demyelinating polyneuropathy mimicking a syndrome 191

subsequently, may have been examples of 7 Hinck VC, Sachdev NS. Myelographic findings in hyper- trophic interstitial . AJR Am J Roentgenol 1965; J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.2.189 on 1 August 1995. Downloaded from CIDP, as nerve biopsy abnormalities were 95:947-8. non-specific, and a family history of neurolog- 8 Bellon EM, Kaufman B, Tucker ME. Hypertrophic neuropathy: plain film and myelographic changes. ical disease was often absent247lolsl3 or at Radiology 1972;103:319-22. best questionable.5 811 14 Our patient shows 9 Kremenitzer M, Ager PJ, Zingesser LH. Myelographic evidence for enlargement in a case of definitively that spinal compression syn- Charcot-Marie-Tooth disease. Neuroradiology 1976;11: dromes may occur in acquired hypertrophic 165-7. 10 Hammerschlag SB, Adelman LS, Marcus EM, Wolpert neuropathies as well as in HMSN and SM. Cervical myelographic changes in hypertrophic expands the spectrum of the clinical presenta- interstitial polyneuropathy. Ann Neurol 1977;2:83-4. 11 Carlin L, Biller J, Challa V, Riela A. Hypertrophic tion of CIDP. neuropathy with spinal cord compression. Surg Neurol 1982;18:237-40. 12 Miura T, Hirabuki N, Imakita S, Harada K, Kawai R, We thank Dr R H M King and Miss J Workman for assistance Mitomo M, Takahashi M. Radiological findings in a with the histopathological studies. case of Charcot-Marie-Tooth disease. Br . Radiol 1985;58:1017-20. 13 Morano JU, Russell WF. Nerve root enlargement in Charcot-Marie-Tooth disease: CT appearance. 1 Thomas PK, Lascelles RG, Stewart G. Hypertrophic Radiology 1986;161:784. neuropathy. In: Vinken PJ, Bruyn GW, eds. Handbook of 14 Rosen SA, Wang H, Comblath DR, Uematsu S, Hurko 0. clinical neurology, Vol 21, system disorders and atrophies. Compression syndromes due to hypertrophic nerve roots Amsterdam: Elsevier/North Holland, 1975:145-70. in hereditary motor sensory neuropathy type 1. 2 Bruns G. Zur Kenntnis der hypertrophischen Neuritis Neurology 1989;39:1173-7. (Roussy-Corril). BeitrPathAnat 1951;111:407-18. 15 Crino PB, Grossman RI, Rostami A. Magnetic resonance 3 Roger H, Poursines Y, Gallais P, Roger J. Polynevrite imaging of the cauda equina in chronic inflammatory hypertrophique de l'adulte (nonfamiliale) avec para- demyelinating polyneuropathy. Ann Neurol 1993;33: paresie spasmodique. Rev Neurol 1952;86:695-8. 311-3. 4 Lewtas NA, Dimant S. The diagnosis of hypertrophic 16 Pollard JD. Chronic inflammatory demyelinating polyneu- interstitial polyneuritis by myelography. J Fac Radiol ropathy. In: McLeod JG, ed. Bailliere's clinical neurology: 1957;8:276-9. inflammatory neuropathies. London: Bailliere Tindall, 5 Symonds CP, Blackwood W. Spinal cord compression in 1994:107-27 hypertrophic neuritis. Brain 1962;85:251-60. 17 Prineas JW. Pathology of inflammatory demyelinating 6 Andermann F, Lloyd-Smith DL, Mavor H, Mathieson G. neuropathies. In: McLeod JG, ed. Bailliere's clinical Observations on hypertrophic neuropathy of Dejerine neurology: inflammatory neuropathies. London: Bailliere and Sottas. Neurology 1962;12:712-24. Tindall, 1994:1-24.

A note on Claude Bernard-Horner's syndrome 1 Bernard C. Sur les effets de la section de la portion continuedfrom page 188 cephalique du grand sympathique. Comptes Rendus. Societi de Biologie, Paris 1852;4:168-9. 2 Pourfour du Petit F. Memoire dans lequel il est demonstre acquired a lifelong interest in eye diseases. He worked que les nerfs intercosteaux fournissent des rameaux qui with von Graefe who became his close friend. In 1862 portent des esprits dans les yeux. Histoire de l'Academie Royal des Sciences, Paris, Mimoires 1727;1-19. he was appointed Professor of ophthalmology in his 3 Mitchell SW, Morehouse GR, Keen WW. Gunshot wounds home town. Apart from his studies of cervical sympa- and other injures to nerves. Philadelphia: Lippincott, thetic paralysis, he described a man with red-green 1864. 4 Hare ES. Tumour involving certain nerves. London colour blindness who had transmitted the disorder to Medical Gazette 1838;23:16-8. his male grandchildren through an unaffected daugh- 5 Homer F. Ueber eine Form von Ptosis. Klinische ter: establishing sex linked transmission. Monatsblatter fur Augenheilkunde 1869;7:193-8, trans-

JMS PEARCE lated by JF Fulton.6 http://jnnp.bmj.com/ 304 Beverley Road, 6 Fulton JF. Homer and the syndrome of paralysis of the Analby, Hull HU10 7BG, UK cervical sympathetic. Arch Surg 1929;18:2025-39. on September 29, 2021 by guest. Protected copyright.