London, United Kingdom 2013 12 Interesting and challenging cases Atypical presentation of Superficial Siderosis successfully treated with iron chelation S. De Mercanti1, M. Iudicello2, M. Clerico3, F. Piazza4, M. Gibbin5, F. Longo6, L. Durelli7 San Luigi Gonzaga Hospital, Neurological Division, Orbassano, ITALY1, San Luigi Gonzaga Hospital, Neurological Division, , ITALY2, San Luigi Gonzaga Hospital, Neurological Division, Or-bassano, 3, San Luigi Gonzaga Hospital, Neurological Division, Orbassano, 4, San Luigi Gonzaga Hospital, Neurological Division, Orbassano, 5, San Luigi Gonzaga Hospital, Hematological Pediat-ric Division, Orbassano, 6, San Luigi Gonzaga Hospital, Neurological Division, Orbassano7 Superficial siderosis (SS) is a disorder resulting from chronic or intermittent bleeding into the sub-arachnoid space, with hemosiderin deposition in subpial layers of CNS. Patients develop debilitating symptoms typically including slowly progressive cerebellar gait ataxia and sensorineural hearing loss; less common symptoms are dementia, episodic and orthostatic headache, seizures, urinary in-continence and cognitive impairment. The origin of the haemorrhage is often not found. CSF anal-ysis usually shows xanthrochromia and elevated erythrocyte count; MRI display hypointensity on T2 weighted imaging. Our case concerns with a 31-year-old woman who experienced a concussed head injury at the age of 12 years after a road accident. Two years later she developed a daily severe headhache, initially diagnosed as migraine. Soon, along with headaches, occurred several episodies of diffuse hypertonia anf fixed gaze with loss of consciousness, moreover peristent hearing loss. EEG showed slow diffuse abnormalities, mostly in the right temporo-parietal side. MRI showed cerebellar hemosiderin deposition. CSF analysis demonstrated micro-bleeding, consistent with SS diagnosis. Regardless of extensive investigations, the source of the hemorrhage remained unclear. The patient started therapy with carbamazepine, but, despite this, the symptoms soon deteriorated clinically, radiologically and electroencephalographically. At 21 years the clinical picture was com-plicated by thrombosis of the left transverse sinus. The patient did not received blood thinning drugs due to bleeding high risk, she started therapy with diuretics and placed a CSF-peritoneal shunt. Af-ter that migraine disappeared. At 26 years she started high dose iron chelation (deferiprone), soon complicated by arthralgias. At 31 years she restarted a lower deferiprone dose (20 mg per kg for 4 consecutive days a week), with good clinical outcome (neurological evaluation with ICARS=3), and without progression or adverse events. E-Poster Session Red Cerebrovasc Dis 2013; 35 (suppl 3)1-854 273 11 Interesting and challenging cases ARTERIAL ISCHEMIC STROKE IN MULTIPLE MYELOMA A. MUBASHIR1, L. AL-DHAHIR2 Queen hospital, LONDON, UNITED KINGDOM1, Queen hospital, LONDON, UNITED KING-DOM2 We report case of arterial ischemic stroke in the presence of multiple myeloma. CASE REPORT: A 71 yr old male woke with right side weakness, right facial droop and slurred speech. Patient had type 2 diabetes and hypertension. On admission his vitals signs were stable, alert and MTS 10/10. Respiratory and cardiovascular examinations were unremarkable. GCS 15/15, right upper and low-er limb weakness 4/5, right upper motor neuron facial palsy with dysarthria. All other cranial nerves were intact, there was no sensory deficit present, and reflexes were normal and up-going plantar on the right. INVESTIGATIONS: CT head showed a right corona raidata infarction and lytic lesions in the skull bone, which first raised the suspicious of multiple myeloma. ECG and echocardiography were normal. Hb 9 g/dL, Ca 2.85 mmol/L , ESR 101 mm/h, CRP 33mg/L. Plasma electrophoresis was elevated for IgM and urine was positive for Bence Jones protein. Bone marrow biopsy was positive for plas-ma cells which confirmed the diagnosis. Bone scan was showed a lytic lesion in the right femur, CT chest /abdomen/ pelvis showed bilateral pulmonary embolism. TREATMENT: Treated with therapeutic Clexane for pulmonary embolism and commence on course of Melphalan and high dose of steroids for multiple myeloma. Had right femur fixation to prevent pathological fracture and was able to mobilize independently at the time of discharge. He was started on warfarin after 2 weeks. DISCUSSION: Our case report shows a case of arterial ischemic stroke in multiple myeloma pa-tients which is uncommon presentation in multiple myeloma patients. It raises the issue of primary prophylaxis of stroke in patient with hyper viscosity status such as multiple myeloma.This need fur-thure large scale studies to support this hypothesis.
Karger_ESC London_2013
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