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274 Scientific Programme 22. European Stroke Conference © 2013 S. Karger AG, Basel 14 Interesting and challenging cases Ischemic stroke due to systemic amyloidosis Y. Beckmann1, F. Gelal2, Ö. Göçmez3 Department of Neurology, Katip Çelebi University Atatürk Training and Research Hospi-tal, Izmir, TURKEY1, Department of Radiology, Katip Çelebi University Atatürk Training and Research Hospital, Izmir, TURKEY2, Department of Neurology, Katip Çelebi University Atatürk Training and Research Hospital, Izmir, TURKEY3 We describe a case with primary systemic amyloidosis who presented features of central neurolog-ical involvement and the sudden unconsciousness due to ischemic stroke as the first defining mani-festation. Case report A previously healthy 35-year-old man suffered from sudden generalized intense headache, vomit-ing and loss of consciousness. On arrival he was somnolent, dysarthric and dysphagic Neurological examination showed multidirectional nystagmus, impaired gag reflex, left ataxic hemiparesis. Vital functions were normal. He had no premorbid medical and family history. DWI-MRI showed small lesions on the left half of medulla oblongata, left cerebellar hemisphere, right parietal cortex and right side of corpus callosum. A cranial MRI 1 day later showed enlargement of medullary lesions as well as appearance of several new lesions in the left cerebellar hemisphere. In MRA thrombosis of left vertebral artery was demonstrated. Nine days later, MRI showed involvement of both posterior fossa and supratentorial structures to a greater extent (Figure 1, 2). On the third day, he developed bilateral pretibial edema and urine analysis had proteinuria. A re-nal biopsy demonstrated deposition of amyloid in the glomeruli, leading to a definite diagnosis of secondary amyloidosis (Figure 3). Despite treatment with melphalan and prednisone, he died four weeks later. Discussion We report here a patient who presented ischemic stroke because of large vessel atherosclerosis as the first manifestation of systemic amyloidosis. Although peripheral neuropathy is a well known com-plication of systemic amyloidosis, the true incidence of central nervous system involvement insys-temic amyloidosis is unknown. The purpose of this present study is to describe a patient with unusu-al symptoms as primary manifestation of amyloidosis, something that has been very rarely reported elsewhere in medical literature. Amyloidosis should be considered among the differential diagnoses of acute stroke syndromes especially in the absence of any risk factors for stroke. 13 Interesting and challenging cases How an untreated sore throat can lead to catastrophic cerebral infarction: a case of Lemierre’s syndrome S.K. Gill1, A. Bhagat2, D.M. Collas3 West Hertfordshire Hospitals NHS Trust, Watford, UNITED KINGDOM1, West Hertfordshire Hospitals NHS Trust, Watford, UNITED KINGDOM2, West Hertfordshire Hospitals NHS Trust, Watford, UNITED KINGDOM3 BACKGROUND A fit 22-year old female student with 1 week’s sore throat presented due to left eye swelling, head-ache, fever, lethargy, dysphagia and dyspnoea. Apart from a morning-after pill she had taken no drugs and had no past medical history EXAMINATION Alert, febrile T38.8C, neck stiffness, cervical lymphadenopathy, left parotid swelling, proptosis and chemosis with discharge; no neurological deficit except partial ophthalmoplegia due to orbital oede-ma INVESTIGATIONS CT LUNGS: bilateral patchy cavitating lesions; NECK: tonsillar abscess constricting oropharynx, enhancing cervical lymph nodes, parotid swelling. Left jugular vein occluded. CT BRAIN: cavern-ous sinus thrombosis, normal brain parenchyma, no venous sinus thrombosis MICROBIOLOGY - negative swabs, urine, blood, and tonsillar aspirate. CRP302, platelets 38, WBC 22, prothrombin 15.3s, ALT 169 DIFFERENTIAL DIAGNOSIS Initially glandular fever then meningitis, Strep septicaemia or toxic shock syndrome were revised when the cavitating lung lesions and pharyngitis with jugular occlusion and proptosis suggested Le-mierre’s syndrome and cavernous sinus thrombosis PROGRESS Despite multiple antibiotics, platelet transfusion then anticoagulation she became obtunded with in-creasing proptosis, fixed L pupil, papilloedoma and haemodynamic instability. Both carotid arteries were narrowed, compressed or thrombosed, leading to bilateral hypoxic brain damage and massive bi-hemispheric infarction culminating in death in 5 days. Autopsy showed pus in both cavernous sinus and in thrombosed jugular veins CONCLUSION Lemierre’s syndrome (1936) is a rare, little-known, potentially lethal complication of oropharyngeal sepsis. Fusobacterium necrophorum, a normal part of oral flora, becomes invasive and propagates proximally (septic emboli to the lungs), retrogradely (cavernous thrombosis) and locally (the carotid sheath). Increased awareness promotes earlier recognition, use of antibiotics and anticoagulation, and may improve survival


Karger_ESC London_2013
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