DIAGNOSTIC SLIDE SESSION 2013
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Transcript of DIAGNOSTIC SLIDE SESSION 2013
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DIAGNOSTIC SLIDE SESSION 2013
CASE 6
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Disclosure Statement
• Nothing to disclose
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Clinical Summary
• A 30 year old man with recent treatment for Streptococcal pharyngitis developed weakness of the left upper and lower extremities and left facial droop.
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MRI Preoperative
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Biopsy 1
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Biopsy 1
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CD68
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CD3 CD20
CD3
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Diagnosis?
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Behçet Disease • Idiopathic disorder classified as
vasculitis• Common in Turkey and Middle East – Silk Road Disease
• Clinical features– Recurrent oral and genital ulcers – Uveitis– Erythema nodosum– Folliculitis– Meningitis or central nervous system
vasculitis (Neuro-Behçet)
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Neuro-Behçet DiseaseClinical features • Headache • Behavioral changes• Hemiparesis
MRI• Scattered contrast enhancing lesions in basal ganglia, brainstem, or
internal capsule
Neuropathological Findings• Perivascular infiltration of inflammatory cells, with or without necrosis
– Mononuclear– Polymorphonuclear– Rare eosinophils
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Follow-up
• Treated with Cellcept – Symptom free for four years
• Serial MRIs stable
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Final Diagnosis
• Behçet Disease –with Neuro-Behçet presentation
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References
• Taskapilioglu O, Seferoglu M, Akkaya C, Hakyemez B, Yusufoglu C, Basak AN, Gundogdu A, Bora I. AN, Gundogdu A, Bora I. Delayed diagnosis of a neuroBehçet patient with only brainstem and cerebellar atrophy: literature review. J Neurol Sci. 2009;277(1-2):160-3.
• Borhani Haghighi A, Sharifzad HR, Matin S, Rezaee S. The pathological presentations of neuroBehçet disease: a case report and review of the literature. Neurologist. 2007;13(4):209-14.
• Arai Y, Kohno S, Takahashi Y, Miyajima Y, Tsutusi Y.Autopsy case of neuro-Behçet's disease with multifocal neutrophilic perivascularinflammation.Neuropathology. 2006;26(6):579-85.