A viral panel including CSF Varicella Zoster Virus (VZV) IgG, IgM, and PCR was negative. multisystem disease.1, 2 While ischemic stroke has been reported in patients with POEMS3, the cause of stroke in these patients is unclear, and there are very few reports of stroke as the presenting manifestation of POEMS.4 It is important to recognize ischemic stroke as a presenting manifestation of POEMS since it could help physicians diagnose and treat POEMS early to prevent progression. Case The patient was a 32 year-old right-handed man with no known prior medical history who was in his usual state of health until 2 months prior to presentation, when he started having right retro-orbital headaches and nasal congestion. He was diagnosed with sinusitis and treated with antibiotics and over the counter medications including pseudoephedrine and sympathomimetic nasal sprays. A few weeks later, he presented to the emergency room after developing several episodes of transient right monocular blindness without any other associated symptoms. He had a 10-pack year smoking history but no alcohol or illicit drug use. His medications included MLN8054 the pseudoephedrine and other sympathomimetic nasal sprays. General examination was unremarkable and his blood pressure was 120/70 mm Hg. Neurological examination, including full fundoscopic exam and visual acuity, was normal. His diagnostic evaluation included brain magnetic resonance imaging (MRI) that showed multiple small infarcts in the right corona radiata in the internal borderzone territory (Figure 1) and magnetic resonance angiography (MRA) of the head and neck that showed reduced flow in the right supraclinoid internal carotid artery that was confirmed by conventional angiography (Figure 1). Due to the headaches and history of sympathomimetic use, he was diagnosed with reversible cerebral vasoconstriction syndrome and discharged home on aspirin and verapamil. Open in a separate window Figure 1 Upper row: Diffusion weighted imaging MRI sequence (left) showing acute infarcts (arrow) in the right corona radiata and cerebral angiogram (right) showing narrowing of the right supraclinoid internal carotid artery and a pseudoaneurysm of the right middle cerebral artery (arrow). Lower row: Diffusion weighted imaging MRI sequence showing an acute infarct in the left corona radiate (arrow) and magnetic resonance angiography (right) showing reduced flow in the left middle cerebral artery (arrow). Three months later, he presented with right hand numbness, right facial paresis, and dysarthria. Brain MRI showed multiple small infarcts in the left corona radiata and MRA showed reduced flow in the left middle cerebral artery (Figure 1). A vasculitic process of the central nervous system was suspected. His diagnostic evaluation included an extensive panel Rabbit Polyclonal to KANK2 of inflammatory, rheumatological, and autoimmune laboratory tests which were all non-revealing (Table 1). Cerebrospinal fluid (CSF) analysis showed normal cell count and glucose, and a mildly MLN8054 elevated protein at 74 mg/dL. A viral panel including CSF Varicella Zoster Virus (VZV) IgG, IgM, and PCR was negative. Due to concern for CNS vasculitis, the patient underwent brain, leptomeningeal, and temporal artery biopsies that were negative for any inflammatory process but showed thickening of blood vessels. He was diagnosed with biopsy-negative CNS vasculitis and treated with intravenous methylprednisolone 1 gram per day for 5 days followed by a slow taper of oral prednisone over 6 months. Table 1 Laboratory, radiological, and pathological findings in our patient over the course of his illness thead th align=”left” rowspan=”1″ colspan=”1″ Months from presentation /th th align=”left” rowspan=”1″ colspan=”1″ 5 months /th th align=”left” rowspan=”1″ colspan=”1″ 11 months /th th align=”left” rowspan=”1″ colspan=”1″ 42 months /th /thead Hemoglobin14 mg/dL12 mg/dL11 mg/dLPlatelets550,000/L700,000/ L900,000/ LUrinalysisnormalnormalproteinuriaESR6 mm/hr64 mm/hr88 mm/hrRheumatoid Factor11.1 IU/mL42.3 IU/mLAnti-nuclear antibodynegativenegativeCSF WBC1 per mm3CSF Protein74 mg/dLCSF VZV IgG and PCRnegativeCSF VDRLnegativeBrain BiopsyThickening of br / blood vessels, br / negative for br / inflammationSPEPLow IgA lambdaCT Chest/Abdomen/PelvisMild hepato-splenomegaly br / Splenic infarcts br / Mediastinal and MLN8054 carinal br / lymph nodesHepatoplenomegaly and br / mild ascites and br / bilateral pleural br / effusions.Transthoracic echocardiogramnormaldilated right ventricle br / with normal function br / and a small br / circumferential br / pericardial effusion with br / thickened pericardiumFine needle aspiration Lymph br / nodePolymorphous population br / of small lymphocytesBone.
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