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Acute/subacute Neuro-Behcet’s disease

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33-year-old African–American male had an episode of headache followed by self-limiting diplopia and dysconjugate gaze (inward rotation of left globe). CT head was reportedly normal, and ophthalmoplegic migraine was considered… Click to show full abstract

33-year-old African–American male had an episode of headache followed by self-limiting diplopia and dysconjugate gaze (inward rotation of left globe). CT head was reportedly normal, and ophthalmoplegic migraine was considered at that time. Six months later the patient presented with left facial weakness, slurred speech, and leftsided incoordination with gradual onset and progressive worsening over a week. There was no history of recent infection or immunization. MRI showed extensive asymmetric mesodiencephalic junction lesion with enhancement involving the right basal ganglia, thalamus, midbrain, and pons (Fig. 1). Autoimmune, paraneoplastic, and infectious work up were negative. Neoplastic and inflammatory conditions were considered in the differential. Patient’s symptoms improved spontaneously without treatment and biopsy was deferred. Another month later the patient presented with worsening ataxia, dysarthria, new right-sided weakness, and difficulty in swallowing. Repeat MRI demonstrated near complete resolution of the right mesodiencephalic junction lesion and interval development of a similar left-sided lesion involving the left basal ganglia, thalamus, midbrain, and pons (Fig. 1). The spontaneous improvement of the right-sided signal changes and enhancement essentially excluded neoplastic conditions. CSF studies, paraneoplastic panel, autoimmune, and infectious workup were again unrevealing. The patient was Fig. 1 MRI of acute/subacute Neuro-Behcet’s disease. Axial T2weighted fluid attenuated inversion recovery (a), and axial postcontrast T1-weighted (b) images show hyperintense asymmetric right mesodiencephalic junction lesion with characteristic sparing of the red nucleus (arrow in a). There was significant craniocaudal extension (not shown). Postcontrast enhancement may be present (arrow in b) in acute lesions mimicking a tumor. Axial T2-weighted fluid attenuated inversion recovery (c), and axial postcontrast T1weighted (d) images through the same level 1 month later without intervening treatment show interval spontaneous near complete resolution of the right mesodiencephalic junction signal changes and enhancement, and interval development of new similar vasogenic edema and enhancement in the left mesodiencephalic junction. The left red nucleus is spared (arrow in c) & Marinos Kontzialis [email protected]

Keywords: acute subacute; behcet disease; mesodiencephalic junction; subacute neuro; neuro behcet; junction

Journal Title: Acta Neurologica Belgica
Year Published: 2017

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