Bühlmann, Manuela

Fuhr, Peter

Kappos, Ludwig

Renaud, Susanne

Rüegg, Stephan

Steck, Andreas

RERO DOC Digital Library

J Neurol (2004) 251 : 1408–1410DOI 10.1007/s00415-004-0544-7Stephan J. RüeggManuela BühlmannSusanne RenaudAndreas J. SteckLudwig KapposPeter FuhrCervical dystonia as firstmanifestation of multiplesclerosisReceived: 24 February 2004Received in revised form: 10 May 2004Accepted: 17 May 2004Sirs: Cervical dystonia is very rarein patients with multiple sclerosis.A case is reported in which cervicaldystonia was the initial manifesta-tion of multiple sclerosis. Althougha simple coincidence of multiplesclerosis and cervical dystonia can-not be excluded, epidemiologicaldata and pathophysiological con-siderations make a causal correla-tion more likely. Multiple sclerosisas the cause of secondary cervicaldystonia may probably be under-recognized.Typical signs and symptoms ofthe first manifestation of multiplesclerosis (MS) include sensorydisturbances, motor paresis, opticneuritis, and eye motility disorders[5]. Sustained dystonia, unlikeparoxysmal movement disorders[1], is considered an extremely raremanifestation of MS [6, 9, 10, 13,15]. We describe a patient in whomcervical dystonia was the present-ing feature of MS.A 38-year old woman of Italianorigin presented with a ten monthhistory of cervical dystonia withcombined leftward rotation of 70degrees and rightward tilt of 40degrees, partially relieved bytouching the left cheek, but persist-ing during sleep. Transitory im-provement had been achieved withtiapride and biperiden. Three daysbefore admission she noted loss ofright retroauricular sensation. Onadmission, she was taking biperi-den 12 mg/d, tizanidine 6 mg/d, andclonazepam 1.5 mg/d for the dysto-nia, as well as citalopram 20 mg/dfor panic attacks. She had no his-tory of exposure to neurolepticdrugs prior to onset of dystonia,drug or alcohol addiction, perinatalasphyxia, trauma, arterial hyper-tension or diabetes. Her family his-tory did not reveal any patientswith movement disorders or psy-chiatric diseases.Neurological examinationshowed 12 points on the Tsui ratingscale [14] for cervical dystonia, andhypesthesia behind and below theright ear, most likely correspondingto segments C2 and C3, but noother pathological signs. Slit-lampexamination excluded Kayser-Fleischer rings. Blood tests showednormal results for cell counts,electrolytes, liver and renal func-tion, thyroid stimulating hormone,vitamin B 12, and ceruloplasmin.Enzyme-immuno-assays for herpessimplex virus, tick borne ence-phalitis, treponema, and borreliawere negative. Cell count (3.0 x 109(reference < 4.8 x 109)) and totalprotein content (354 mg/l (refer-ence < 480 mg/l)) of the cere-brospinal fluid were normal, butintrathecal IgG production wasshown by the presence of fouroligoclonal bands and an elevatedIgG Index (13.6, reference < 7.0).MRI of the cervical spine showedtwo non-enhancing lesions rightdorso-laterally at levels C3/4 andC2/3 (Fig. 1A). Cranial MRI re-vealed two right-hemispheric ovoidlesions in periventricular (Fig. 1Band C) and juxtacortical areas(Fig. 1D). Visual evoked potentialsof both eyes were within normallimits, but the side difference wasabnormal (Fig. 2A). Somato-sen-sory evoked potentials recordedafter stimulation of the left armshowed a markedly reduced ampli-tude of the potentials recordedfrom the scalp (Figs. 2B and C).A diagnosis of laboratory sup-ported definite MS (according toPoser’s criteria) or of MS (accord-ing to the new diagnostic criteriaby McDonald et al. [8], i. e., twoattacks, clinical evidence of onelesion, three MRI-detected lesionsconsistent with MS plus positiveCSF) was made. The patient im-proved after injection of botulinumtoxin A into the neck muscles com-bined with pulsed high-dose intra-venous methylprednisolone(500 mg daily for five days). Shereceived the last injection of botu-linum toxin 12 months after thefirst presentation. Fifteen and 27months after the first presentation,she showed only minimal signs ofcervical dystonia (both times 3points on the Tsui scale). Her med-ication at the last consultation con-sisted of tizanidine 6 mg/d, alpra-zolam 1 mg/d, paroxetine 40 mg/d,and mianserine 30 mg/d. Except fortizanidine, the other medicationswere for psychiatric symptoms.In most cases of focal dystonia,the cause remains unknown, lead-ing to a diagnosis of primarydystonia. In contrast, some clinical“red flags” were present in our caseand pointed to secondary focaldystonia. Sensory deficits in the C2and C3 dermatomes and persis-tence of dystonia during sleep weresuggestive of secondary focaldystonia. Paraclinical examinationssuch as imaging, electrophysiologi-cal and laboratory testing sup-ported the diagnosis of MS.We cannot exclude a coinci-dence of MS and focal dystonia inour case. However, the probabilityof such a coincidence is extremelylow (1: 3.500.000) according to re-ported prevalences of MS in Italy of50/100.000 [12], and of cervicaldystonia of 57/100.000 [3]. Further-more, the persistence of cervicaldystonia, also during sleep, is astrong argument for secondary,LETTER TO THE EDITORSJON 15441409Fig. 1 A: MRI of the cervical spine: T2-weighted ax-ial section at level C3/C4. Hyperintense lesion is pre-sent right dorso-laterally. B–D: MRI of the head.FLAIR sequences. Right-hemispheric ovoid lesions inperiventricular (B, C) and juxtacortical location (D).Note rightward head tilt due to dystonia. The lesionsdid not enhance with gadolinium in T1-weighted im-agesFig. 2 A: Visual evoked potentials. The latency ofcomponent P100 on both sides (right: 99.0 ms; left:108.0 ms) is within the range of normal values, butthe side difference (9.0 ms, normal < 6.0 ms) ispathological, with prolongation of recording afterstimulation of the left eye. The upper two traces: av-eraged curves of 200 recordings each. Lower traces:average of the two curves. L denotes recording afterstimulation of the left eye; R, after stimulation of theright eye. B and C: Somato-sensory evoked poten-tials: Recordings after stimulation of the right mediannerve at the wrist (B); of the left median nerve at thewrist (C). The top traces represent the recordingsfrom the scalp, the bottom traces, from the processusspinosus C6. The reference was at Fz. Latencies of P15and N20 are within normal limits, without patholog-ical side differences. Components N9, N11, and N13after stimulation of the left arm can not be identified(probably for technical reasons in the presence ofdystonia); the potentials recorded from the scalpshow a markedly reduced amplitude (diminution ofamplitude N20-P25 by 86 %; normal < 45 %)ABCAC DB1410i. e., in this case MS-related, dysto-nia [11]. Thus, we propose thealteration of central processingproduced by an MS lesion as themore likely explanation for thecervical dystonia in the presentcase [7]. Anecdotal reports de-scribe torticollis resulting fromdifferent cervical lesions [2, 4] andthe association of spinal MSplaques with torticollis [6] andhand dystonia [15]. However, dys-tonia was not the first manifesta-tion of MS in any of these reports.To conclude, MS may probablybe an underdiagnosed cause ofsecondary focal dystonia, and con-versely, secondary focal dystoniamay herald multiple sclerosis andshould be included in the differen-tial diagnosis of patients present-ing with this clinical feature.References1. Berger JR, Sheremata WA, Melamed E(1984) Paroxysmal dystonia as the ini-tial manifestation of multiple sclerosis.Arch Neurol 41:747–7502. Cammarota A, Gershanik OS, Garcia S,Lera G (1995) Cervical dystonia due tospinal cord ependymoma: involvementof cervical cord segments in the patho-genesis of dystonia. Mov Disord 10:500–5033. Epidemiological Study of Dystonia inEurope (ESDE) Collaborative Group(2000) A prevalence study of primarydystonia in eight countries. J Neurol247:787–7924. Fujimoto N, Nabatame H, NakamuraK, Konishi T, Kamiya Y (1991) Spinalepidural abscess as the cause of torti-collis – diagnosis by magnetic reso-nance imaging, (article in Japanese).Rinsho Shinkeigaku 31:49–535. Keegan BM, Noseworthy JH (2002)Multiple Sclerosis. Annu Rev Med53:285–3026. KlostermannW, Vieregge P, Kompf D(1993) spasmodic torticollis in multi-ple sclerosis: significance of an uppercervical spinal cord lesion. Mov Disord8:234–2367. LeDoux MS, Brady KA (2003) Sec-ondary cervical dystonia associatedwith structural lesions of the centralnervous system. Mov Disord 18:60–698. McDonald WI, Compston A, Edan G,et al. (2001) Recommended diagnosticcriteria for multiple sclerosis: Guide-lines from the international panel onthe diagnosis of multiple sclerosis.Ann Neurol 50:121–1279. Rozza L, Bortolotti P, Sica A, Weronig S,Orrico D (1993) Kinesigenic dystoniaas the first manifestation of multiplesclerosis with cervical and brainstemlesions. Eur Neurol 33:331–33210. Svetel M, Sternic N, Filipovic S, KosticV (1997) Spasmodic torticollis associ-ated with multiple sclerosis: report oftwo cases. Mov Disord 12:1092–109411. Svetel M, Ivancovic N, Marinkovic J,Jovic J, Dragasevic N, Kostic VS (2004)Characteristics of dystonic movementsin primary and symptomatic dysto-nias. J Neurol Neurosurg Psychiatry75:329–33012. Tassinari T, Parodi S, Badino S, VercelliM (2001) Mortality trend for multiplesclerosis in Italy (1974–1993). Eur JEpidemiol 17:105–11013. Tranchant C, Bhatia KP, Marsden CD(1995) Movement disorders in multi-ple sclerosis. Mov Disord 10:418–42314. Tsui JKC, Eisen A, Stoessel AJ, Calne S(1986) Double blind study of botu-linum toxin in spasmodic torticollis.Lancet 328:245–24715. Uncini A, Di Muzio A, Thomas A, Lu-garesi A, Gambi D (1994) Hand dysto-nia secondary to cervical demyelinat-ing lesion. Acta Neurol Scand 90:51–5516. Yücesan C, Tuncel D, Akbostanci MC,Yücemen N, Mutluer N (2000)Hemidystonia secondary to cervicaldemyelinating lesions. Eur J Neurol7:563–566St. J. Rüegg, MD · M. Bühlmann, MD · S. Renaud, MD · A. J. Steck, MD · L. Kappos, MD · P. Fuhr, MDDepartment of NeurologyUniversity HospitalPetersgraben 44031 Basel, SwitzerlandTel.: +41-61/265-4167Fax: +41-61/265-5638E-Mail: pfuhr@uhbs.chThis case report was presented as a posterat the Annual Meeting of the MovementDisorders Society, held in Miami, Novem-ber 7–11, 2002.

Cervical dystonia as first manifestation of multiple sclerosis

http://doc.rero.ch/record/312240/files/415_2004_Article_544.pdf

Cervical dystonia as first manifestation of multiple sclerosis

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library