3 research outputs found

    Post Herpetic Anti-NMDA- Receptor Encephalitis in an 18-month-old Infant

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      Herpes simplex encephalitis (HSE), caused by herpes simplex virus type 1 (HSV-1), is the most common cause of severe sporadic encephalitis worldwide.HSE is occasionally accompanied by the recurrence of clinical symptoms that usually occur a few weeks following the initial infection. According to recent studies, the recurrence can be due to a secondary autoimmune mechanism rather than the virus invasion.One of the most common etiologies for autoimmunity is Anti-NMethyl D-Aspartate receptor encephalitis. This disorder is a treatable autoimmune encephalitis manifesting as movement disorder or neuropsychological involvemen

    Homocystinuria: A Rare Disorder Presenting as Cerebral Sinovenous Thrombosis

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    How to Cite This Article: Eslamiyeh H, Ashrafzadeh F, Akhondian J, Beiraghi Toosi M. Homocystinuria: A Rare Disorder Presenting as Cerebral Sinovenous Thrombosis. Iran J Child Neurol. Spring 2015;9(2):53-57.AbstractObjectiveHomocystinuria is an inborn error of amino acid metabolism caused by cystathionine beta-synthase deficiency that affects methionine metabolism. The clinical features are heterogeneous ranging from mental retardation, ectopia lentis, and osteoporosis to vascular events such as deep vein thrombosis,   sagital sinus thrombosis, and myocardial infarction. Cerebral sinovenous thrombosis (CVST) is an unusual disorder in children and requires prompt and accurate management. Some causal factors for thedevelopment of CVST differ between children and adults. The majority of cases with CSVT are found to have an underlying cause for thrombosis like dehydration, infections, prothrombotic and hematologic disorders, malignancy and trauma.Although homocystinuria is usually associated with ischemic strokes, CVST as initial clinical presentation of homocystinuria is rare in children.In this article, we presented a 10-year old boy with seizure, hemiparesis, and ataxia due to CSVT caused by homocystinuria

    Multiple supratentorial and infra -tentorial cavernous hemangiomas in a 5 year–old girl

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    Cavernous hemangiomas (CHs) are vascular structures comprising of clusters of abnormally dilated blood vessels. It is estimated to occur this anomaly in approximately one out of every 500-600 people,.  Individuals often show the first sign of cavernous hemangiomas in their second or third decade of life therefore, the presentation of this disorder is not common in children, here we presented a 5 year-old-girl who developed abruptly nearly fixed right sided eyes deviation and incoordination after a short course of viral infection. In physical examination, she had left peripheral facial palsy in addition to right eye oculomotor nerve and left abducens nerve palsy.  A computed tomography (CT) scan of her brain revealed hemorrhages in posterior aspect of the pons and some areas of hemispheres in different stages. Following brain magnetic resonance imaging, multiple popcorn ball low-signal T2* lesions with both supra- and infra tentorial location with marked peripheral hypo intensities were seen. These findings were in favor of multiple cavernous hemangiomas of the brain. The novelity of our case was due to observing cavernous angiomas in both supra and infra tentorial spaces in pediatric age
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