Multiphasic Disseminated Encephalomyelitis Presenting As Alternating Hemiplegia

3tournal ofNeurology, Neurosurgery, and Psychiatry 1995;58:467-470 467 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.4.467 on 1 April 1995. Downloaded from SHORT REPORT Multiphasic disseminated encephalomyelitis presenting as alternating hemiplegia Sonia Khan, Basim A Yaqub, Charles M Poser, Saleh M Al Deeb, Saeed Bohlega Abstract but the MRI of the brain favoured the diag- Two cases of alternating hemiparesis are nosis of multiphasic disseminated encephalo- reported, one in a black Sudanese myelitis (MDEM). woman, the other in a Saudi man, who had two episodes of alternating hemiparesis separated in time by six and Case reports three years respectively. Based on the CASE 1 typical appearance of the MRI and the A 32 year old black Sudanese woman was results of brain biopsy, the diagnosis of admitted to the hospital because of sudden multiphasic disseminated encephalo- dysarthria. On the next day, she had a right myelitis was made rather than that of hemiparesis without impairment of con- multiple sclerosis. This entity is also dif- sciousness or seizures. Six years before, while ferentiated from recurrent disseminated living in the Sudan, she had suddenly had left encephalomyelitis, where the relapses sided hemiparesis and homonymous hemi- are symptomatically stereotyped al- anopia. The hemiplegia recovered completely though the appearance of the MRI is in one week, whereas the hemianopia per- similar and in which new lesions do not sisted. She was not diabetic or hypertensive, occur. Because of the unusual appear- and there was no history of cardiac disease or ance of these MRI lesions, brain biopsy is hyperlipidaemia. Systemic examination was often performed but recognising their normal. The neurological examination relevance should obviate that need. showed normal intellectual function, but she had slurred speech without aphasia. Other (7 Neurol Neurosurg Psychiatry 1995;58:467-470) findings included left homonymous hemianopia, right upper motor neuron facial nerve palsy, and slight deviation of the tongue to http://jnnp.bmj.com/ Keywords: multiphasic disseminated encephalomye- the right without wasting or fasciculations. Division ofNeurology, litis; multiple sclerosis; magnetic resonance imaging; She had upper motor neuron weakness of the Department of alternating hemiparesis with Neurosciences, right upper and lower limbs, hyper- Riyadh Military reflexia and Babinski sign. There was no limb Hospital, PO Box Hemiparesis in a young adult is a diagnostic ataxia or disequilibrium and all modalities of 7897, Riyadh 11159, challenge because of the many potential sensations were intact. She had a right hemi- Saudi Arabia S Khan causes. In developing countries, hemiparesis plegic gait. on September 25, 2021 by guest. Protected copyright. B A Yaqub in young adults is related to pregnancy, child- Complete blood count, sedimentation rate, S M Al Deeb birth, rheumatic heart disease, or infections' blood urea nitrogen, and serum electrolytes, Department of as opposed to that in western countries, liver function tests, and autoimmune screen Neurology, Havard Medical School, Beth where the predominant causes are atheroscle- for connective tissue disease, including lupus Israel Hospital, 330 rosis, cardiogenic embolism, blood disorders, anticoagulant and phospholipid antibodies, Brookline Avenue, and non-atherosclerosis vasculopathies. were all normal. Sickle cell screen was nega- Boston, MA 02215, the face is an uncom- USA Hemiparesis involving tive. The serum titres for brucella, syphilis, C M Poser mon presentation of primary demyelinating and hepatitis B were negative as was the Division of Neurology, disorders of the CNS and may cause diagnostic tuberculin skin test. A chest radiograph, ECG, Department of confusion, especially if the disease is mono- and transesophageal echocardiogram were Medicine, King Faisal phasic or if the relapse rate is low. It is rare in normal. Doppler examination of the neck ves- Specialist Hospital and Research Centre, definite multiple sclerosis.3 In low risk areas sels and four vessel cerebral arteriography P 0 Box 3354, Riyadh for multiple sclerosis, as in high risk coun- were normal. The CSF showed 2 white blood 11211, Saudi Arabia tries, other forms of primary demyelinating cells/mm3, total protein 0-26 g/l, glucose 3 S Bohlega diseases are seen, in particular postinfectious mmol/l, and an IgG index of 0-6. No oligo- Correspondence to: Dr Sonia Khan, Division of disseminated encephalomyelitis (DEM).4 clonal bands were seen. Tests for measles, Neurology, Department of We report two cases of alternating hemi- rubella, mumps, Epstein-Barr, and herpes Clinical Neurosciences, PO Box 7897, Riyadh 11 159, paresis in which stereotactic brain biopsy simplex antibodies and for the presence of Saudi Arabia. showed primary demyelination. It was impos- fungal antigens were negative. An EEG Received 28 June 1994 sible to differentiate the illness from multiple showed focal slowing in the left frontoparietal and in revised form 3 October 1994 sclerosis on the basis of the history, the CSF and right occipital regions. Pattern shift visual, Accepted 19 October 1994 examination, and evoked potential studies somatosensory, and brainstem auditory 468 Khan, Yaqub, Poser, Al Deeb, Bohlega evoked potentials were normal. Computed metastasis were initially considered, but brain J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.4.467 on 1 April 1995. Downloaded from tomography and MRI of the brain showed biopsy showed inflammatory primary bilateral multiple, scattered enhancing nodu- demyelination (fig 2), so a diagnosis of lar lesions in the white matter (fig la-c). MDEM was made. She was given methyl- The diagnoses of multiple tuberculomas or prednisolone (1 g intravenously daily for five days), followed by a course of tapering oral steroids. After one week, the hemiparesis started to improve and the dysarthria disap- peared after 10 days. Four weeks later, the patient had fully recovered. The hemianopia from her previous episode had remained unchanged. The steroids were discontinued at the end of six weeks. Repeat brain MRI eight weeks after the onset of her illness showed considerable improvement in the white matter lesions (fig 1d), which no longer enhanced after injection of gadolinium. CASE 2 In June 1990, a previously healthy 29 year old Saudi man had acute right leg weakness, which progressed over a week to involve his right arm and face as well. He also noted numbness of his right leg. He complained of frontal headache, difficulty in concentrating, and mild weight loss. He had no seizures, fever, diplopia, or urinary difficulties. The neurological examination on admission showed mild dysnomia and right spastic hemiparesis affecting the leg more than the arm and face. There was subjective diminu- tion of pain, temperature, and vibratory sensations. A brain MRI showed two large lesions in the left subcortical white matter and a smaller one posteriorly on the left (fig 3a). There was little adjacent oedema and no mass effect. These lesions enhanced poorly after gadolinium injection. A left internal carotid arteriogram, an EEG, and visual and somatosensory evoked potentials were negative. Blood count, sedimentation rate, elec- Figure 1 Case 1 (a) Contrast enhanced brain CT: multiple enhancing nodules in both autoimmune screen http://jnnp.bmj.com/ cerebral hemispheres. There is dilatation of the atrium of the right lateral ventricle trolytes, for vasculitis, associated with a hypodense area in the parietotemporal region on the right characteristic of serological tests for borrelia, brucella, an old lesion. (b) MRI Tl weighted image after gadolinium injection showingfive syphilis, cryptococcus, toxoplasma, and HIV enhancing nodules in the left subcortical white matter. There is a large area of low intensity were all as were the tuberculin skin in the right parieto-occipital region indicating the site of an old, inactive lesion, which negative, probably occurred six years previously when the patient developed left hemianopia and test, chest radiograph, ECG, and abdominal hemiparesis. (c) T2 weighted MRI: multifocal high signal intensity lesions in the white ultrasound. A CSF examination showed 6 matter of both hemispheres. One of the lesions encroaches on the cortex on the left side. white blood a There is extensive white cells/mm,3 total protein of 0 37 matter high signal intensity in the right parieto-occipital region. a raised Note the sparing of the corpus callosum and the absence ofperiventricular lesions. (d) T2 g/l, IgG index of 1 64 (normal on September 25, 2021 by guest. Protected copyright. weighted MRI eight weeks later: there has been a considerable reduction of the size of the <0 7), IgG synthesis of 19 1 mg/24 hours left sided lesions while the one on the right remains unchanged. (normal 0-3-5), and three oligoclonal bands. Figure 2 Case 1: brain biopsy (a) Light microscopy. Perivascular oedema, inflammation, and demyelination (haematoxylin-eosin). (b) Electronmicrograph showing complete disorganisation of the myelin sheath (white curved arrow on top) and bare axons (white curved arrow and straight black arrow at the bottom). V* t Many macrophages contain myelin bodies within their cytoplasm (two straight white arrows at the bottom right). No viral particles were seen. Multiphasic disseminated encephalomyelitis presenting as alternating hemiplegia 469 enhanced faintly after gadolinium injection. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.4.467

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