22. European Stroke Conference 134 Interesting and challenging cases Multiple dysgenesis of brain supplying arteries in a patient with Osteogenesis imperfecta C. Krogias1, A. Labedi2, C. Lukas3, R. Gold4, S.H. Meves5 Dept. of Neurology, St. Josef-Hospital, Ruhr University Bochum, Germany, Bochum, GER-MANY1, Dept. of Neurology, St. Josef-Hospital, Ruhr University Bochum, Germany, Bochum, GERMANY2, Dept. of Radiology, St. Josef-Hospital, Ruhr University Bochum, Germany, Bochum, GERMANY3, Dept. of Neurology, St. Josef-Hospital, Ruhr University Bochum, Germany, Bochum, GERMANY4, Dept. of Neurology, St. Josef-Hospital, Ruhr University Bochum, Germany, Bochum, GERMANY5 Background: Osteogenesis imperfecta (OI) is a heterogeneous group of heritable connective tissue disorders char-acterized usually by fragile and brittle bones, blue sclera, dental malformations, deafness, and hy-perextensible ligaments. Rarely, this condition may cause abnormalities of vascular structure with carotid artery dissection or even rarer aplasia. Methods: Case report of a 46-year-old patient with osteogenesis imperfecta (OI) presenting with acute onset of right-sided hemiparesis and dysarthria. Despite existing hypertension no medication was taken. Not-icible was a blue-gray color of the sclera. Results: In addition to a small pontine ischemia, MRI revealed a bilateral aplasia of internal carotid arteries as well as a severe hypoplasia of right-sided vertebral artery (figure). Cranial MRA demonstrates a hypertrophied basilar artery. Bilateral prominent posterior communicating arteries supplied the mid-dle and anterior cerebral arteries territories. Conclusion: OI is a connective tissue disease, which may rarely cause abnormalities of vascular structure like dissections, fistulas and even rarer aplasias. The marked dysgenesis in the present case resulted in a single-vessel supply of the whole brain via a compensatory hypertrophied left vertebral and basilar artery. These cerebrovascular abnormalities remained asymptomatic for decades. 352 © 2013 S. Karger AG, Basel Scientific Programme 135 Interesting and challenging cases Reversible Cerebral Vasoconstriction Syndrome in a Patient with IgA Nnephropathy and Thrombotic Thrombocytopenic Purpura V.K. Sharma1, P.R. Paliwal2, H.L. Teoh3, B.P.L. Chan4 National University of Singapore, Singapore, SINGAPORE1, National University Health Sys-tem, Singapore, SINGAPORE2, National University Health System, Singapore, SINGAPORE3, Na-tional University Health System, Singapore, SINGAPORE4 Background and aim- Reversible cerebral vasoconstriction syndrome (RCVS) commonly presents with thunderclap headache and various neurological deficits. It is often associated with post-par-tum state or consumption of vasoactive agents. We describe a case of IgA nephropathy that devel-oped thrombotic thrombocytopenic purpura (TTP) and presented with thunderclap headache due to RCVS. Case Description- A 33years Chinese female, known case of IgA nephropathy and chronic renal fail-ure was admitted for thunderclap headache of new-onset. Headache woke her up from her sleep at night and also, she felt left sided numbness. She did not suffer from migraine in past. On arrival, she was fully conscious and had blood pressure 174/95mmHg. Neurological examination was unremark-able. Blood tests showed raised serum creatinine (852micromol/L- rapid rise from 260micromol/L 2-weeks ago; normal 45-90micromol/L), low platelet count (46000/microliter) and low Hemoglobin (7.2gm%). Her peripheral smear was suggestive of micro angiopathic hemolytic anemia. Plasma-pheresis was commenced but she became confused on day-2. Magnetic resonance (MR) imaging of brain was unremarkable. However, MR angiography showed long segments of narrowing of multi-ple intracranial arteries. No abnormal findings were noted in the cerebrospinal fluid (CSF). Transcra-nial Doppler ultrasonography (TCD) was performed daily to monitor blood flow in the intracranial arteries and demonstrated gradual normalization of flow velocities in affected arteries. Tests for various vasculitic markers were negative. We did not perform catheter angiography due to impaired renal functions. No arterial stenosis was noted on repeat MR angiography on day-12. Conclusions- Our case demonstrates the association of RCVS with IgA nephropathy and TTP. Thun-derclap headache, normal CSF analysis, negative vasculitic screen, rapid resolution of headache and normalization of flow velocities on serial TCD support the diagnosis of RCVS.
Karger_ESC London_2013
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