Acute Disseminated Encephalomyelitis in a Child Following Plasmodium vivax Malaria.

Acute Disseminated Encephalomyelitis (ADEM) is a multifocal, monophasic, acute demyelinating disease of the brain and spinal cord, which is commonly preceded by viral infections and occasionally bacterial infections or immunizations. Its occurrence following malarial infection, especially Plasmodium vivax Malaria is very uncommon. We report an 11-year girl who presented with clinical features of encephalopathy and generalized convulsions, 10 days following complete recovery from the Plasmodium vivax Malaria. Diagnosis of ADEM as a complication of Plasmodium vivax Malaria was made based on acute onset of neurological events, characteristic findings on Magnetic Resonance Imaging (MRI) of brain and prompt response to corticosteroid therapy. Follow-up MRI, 6 months after discharge, showed complete resolution of change found on the initial MRI. To the best of our knowledge, only two such cases have been reported in the English literature till date.

Acute disseminated encephalomyelitis: an uncommon presentation of hyper IgE syndrome.

The hyper-immunoglobulin E (IgE) syndrome (HIES), also known as Job's syndrome is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses of skin, recurrent cyst-forming pneumonia, and an elevated serum IgE level of > 2000 IU/ml. Although, most cases are sporadic, families with autosomal dominant (AD-HIES) and recessive (AR-HIES) traits have been reported. Very few articles were published previously on central nervous system abnormalities with definite neurologic manifestations which may vary from partial facial nerve paralysis to hemiplegia in children but Acute Disseminated Encephalomyelitis (ADEM) in a child with HIES hitherto has not been reported. Here we describe a 5-year-old male child with HIES who presented with neurologic manifestations of ADEM.

Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature.

Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.

Rheumatic valvular insufficiency in Bardet-Biedl syndrome: a case report.

Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder with multisystem affection. Cardiac Involvement, when present is usually in the form of congenital heart disease. However, valvular involvement due to acquired heart disease is extremely unusual. Herein, a 10-year-old boy of Bardet-Biedl syndrome is reported who presented with multiple rheumatic valvular regurgitation.

Overlap of sturge-weber syndrome and klippel-trenaunay syndrome.

Sturge-Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel-Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge-Weber syndrome in combination with Klippel-Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes.