Journal of the Neurological Sciences 197 (2002) 85 – 87 www.elsevier.com/locate/jns Short communication Multiple sclerosis presenting as ataxic hemiparesis Mark J. Gorman* Department of Neurology, Wayne State University, 4201 St. Antoine 8D UHC, Detroit, MI 48201, USA Received 23 July 2001; received in revised form 3 January 2002; accepted 11 February 2002 Abstract Multiple sclerosis (MS) can present with many clinical pictures, but only rarely as a lacunar syndrome. The following case presentation is of multiple sclerosis with initial presentation as ataxic hemiparesis (AH), indistinguishable from the vascular syndrome. This case serves to illustrate that even classic lacunar syndromes can actually be the initial manifestation of multiple sclerosis and that other etiologies besides ischemic stroke need to remain in the differential diagnosis until a definitive has been concluded. D 2002 Elsevier Science B.V. All rights reserved. Keywords: Multiple sclerosis; Stroke; Lacunar syndrome; Ataxic hemiparesis 1. Introduction Despite a propensity for focal or multifocal neurological deficits that present insidiously, multiple sclerosis (MS) has been documented to present with initial episodes that resemble stroke, including a lacunar syndrome [1 – 3], although this is distinctly unusual. Other unusual presentations include paroxysmal attacks of brainstem dysfunction lasting just seconds or minutes without concomitant neurological findings. Transient dystonias and acute vertiginous attacks have also been reported [4]. The following is a case of MS that initially presented as an ataxic hemiparesis (AH), indistinguishable from the vascular syndrome. 2. Case report A 42-year-old right-handed female presented to the emergency department, 11 days after onset of flu-like symptoms (malaise, lightheadedness and anorexia), with complaints of lightheadedness, incoordination and difficulty in walking. She never had prior neurological symptoms and was released after an examination revealed no abnormalities. Two days later, she noted right-sided incoordination and a tendency to bite the right side of her tongue and cheek. Although no headache was noted, she did admit to fatigue and watery stools. She was taking no medications and stroke * Tel.: +1-313-577-1242; fax: +1-313-745-4216. E-mail address: mgorman@moose.med.wayne.edu (M.J. Gorman). factors were absent, save for a strong family history of atherosclerosis, including a brother who had myocardial infarction at the age of 30. General examination revealed an obese, but normotensive female. Mental status was normal and there was no dysarthria. Cranial nerve examination revealed saccadic pursuits to the left, diminished pinprick sensibility on the right side of the face, with accompanying increase in the right palpebral fissure and nasolabial fold flattening. Increased tone as well as mild weakness was noted in the right hand and finger extensors, triceps and grip. A right downward drift and increased checking response were noted. Mildly diminished pinprick sensation was found in a patchy distribution in the right leg. Increased right-sided deep tendon reflexes along with slowed and clumsy right hand movements. Dysmetria was found on right-sided finger-tonose testing. Her gait was wide-based and unsteady and she was unable to perform tandem walking. Some circumduction was noted, along with a tendency to hold the right arm in a flexed posture while ambulating. Extensive hypercoagulable work-up was unremarkable. MRI of the head revealed an area of increased signal on the proton density and T2-weighted images (Fig. 1) in the posterior limb of the left internal capsule and a smaller lesion adjacent to the right lateral ventricle, as well as a similar lesion in the right periventricular white matter (Fig. 2). MRA of the circle of Willis was normal. Slight gadolinium enhancement of the left thalamus and internal capsule was noted, indicating an acute lesion, and confirming its likelihood of causing the patient’s symptoms (Fig. 3). CSF 0022-510X/02/$ - see front matter D 2002 Elsevier Science B.V. All rights reserved. PII: S 0 0 2 2 - 5 1 0 X ( 0 2 ) 0 0 0 4 5 - X 86 M.J. Gorman / Journal of the Neurological Sciences 197 (2002) 85–87 Fig. 1. Transverse MRI, T2-weighted, showing a large high signal intensity lesion involving left thalamus and posterior limb of the internal capsule. A small lesion is also found adjacent to the posterior horn of the right lateral ventricle. had normal glucose, protein and white blood count, but an IgG index of 1.3 (normal 0.3 – 0.7) and four oligoclonal bands were noted. Visual evoked potentials were normal. Transthoracic echocardiogram revealed mild left ventricular hypertrophy and Holter monitoring for 24 h did not find any significant abnormalities. Serological testing found a posi- Fig. 3. T1-weighted MRI with gadolinium enhancement at the same level as that in Fig. 1. Enhancement can be seen in the area of the left thalamus and posterior limb of the internal capsule. tive antinuclear antibody (1:320– 1:640), but negative rheumatoid factor and an erythrocyte sedimentation rate of 12 (normal 1 –20). A diagnosis of laboratory-supported definite multiple sclerosis was made. Treatment with high dose intravenous corticosteroids was initiated and then converted to oral after 5 days. Clinical improvement ensued and at 6 weeks followup, she had a return of normal strength, but mild residual dysmetria, hyperreflexia and clumsiness remained. Extended follow-up revealed intermittent symptoms of fatigue and hand numbness, without neurological findings. The patient has done well a year on interferon. 3. Discussion Fig. 2. Transverse T2-weighted MRI revealing a similar lesion in the right periventricular white matter. Ataxic hemiparesis is a well-known lacunar syndrome, usually associated with small, deep infarction in the posterior limb of the internal capsule or in the basis pons (although other localizations have reported [3– 6]). Localization to the thalamocapsular region is more likely, in this case, on clinical grounds because of the sensory changes [5], and also based on the enhancement noted on the MR scan. Initial descriptions attributed this syndrome predominantly to arteriolar lipohyalinosis and atheromatous plaque [8], but the syndrome has been well documented to be caused by atheroma of the parent artery overlying the mouth of the penetrating arteriole [8]. Although debate continues, there seems ample evidence that at least some lacunar syndromes are caused by cardioembolism [9] and large artery disease M.J. Gorman / Journal of the Neurological Sciences 197 (2002) 85–87 [10]. Rare etiologies have included cerebral cysticercosis [11], trauma [6], hemorrhage [12] and tumor [7]. While there have been a few reported cases of ataxic hemiparesis due to a demyelinating lesion of multiple sclerosis [2], the clinical details of such a case have not been presented. It is possible that this case could be one of acute disseminated encephalomyelitis (ADEM), although the clinical picture of ADEM is usually more severe and involves headache, fever, nausea, vomiting, confusion, delirium and even coma [13]. Multiple sclerosis, while generally thought of as a ‘‘white matter’’ disease, actually involves some gray matter in the large majority of cases. Since myelin surrounds the axons throughout their extent except for the most proximal segment, myelin pathological changes can be found within collections of neurons in gray matter structure [14]. Acute multiple sclerosis can present as a ‘‘gray matter’’ syndrome. It is usually distinguishable from acute stroke by a slightly slower onset and a detailed exam may reveal subtle signs of dysfunction correlating to other locations, reflecting ‘‘silent’’ lesions of multiple sclerosis. This case serves to demonstrate that while infarction can explain the majority of cases of ataxic hemiparesis, demyelinating disease, as well as other causes in the differential should remain in consideration until appropriate clinical and laboratory determinations have been completed. Acknowledgements I wish to thank Steven R Levine, MD for his assistance in reviewing this manuscript. 87 References [1] Anzalone N, Landi G. Non ischaemic causes of lacunar syndromes: prevalence and clinical findings. J Neurol Neurosurg Psychiatry 1989;52:1188 – 90. [2] Lazzarino LG, Martinelli A, Nicolai A. Lacunar syndromes due to non ischaemic causes: prevalence and clinical findings. Acta Neurol 1993;15:401 – 8. [3] Luijckx G-J, Spaans F, Boiten J, Lodder J. 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