Publications (7) View all
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Article: Sturge-Weber Syndrome: Clinical Spectrum, Disease Course, and Outcome of 30 Patients.
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ABSTRACT: Sturge-Weber syndrome is a heterogeneous neurocutaneous syndrome with facial and leptomeningeal angiomas, glaucoma, seizures, stroke-like episodes, and mental retardation. The authors critically evaluated the clinical manifestations, outcome, and natural history in 30 patients with Sturge-Weber syndrome followed up from January 1985 to May 2010. Of the patients, 15 were males, age at diagnosis ranged from 1 month to 43 years. Typical port-wine stain nevus occurred in 26 (86%), it was bilateral in 2 (8%), and it was absent in 4 (4%). Nine patients had glaucoma (30%), 3 required surgery. Four had transient hemiparesis. All patients had seizures; they were well controlled in 22 (73.3%); in 8 they remained drug resistant. Three patients underwent surgery and became seizure-free. Of the 17 who had mental subnormality, 14 (82.4%) had seizure onset before 2 years. An early age at seizure onset and those with drug-resistant seizures had more severe degree of mental subnormality. Uncontrolled seizures, mental subnormality, visual handicap, and cosmetic disfiguration were the major impediments in life.Journal of child neurology 07/2012; · 1.59 Impact Factor -
Article: Posterior reversible encephalopathy syndrome revealing Takayasu’s arteritis
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ABSTRACT: Posterior reversible encephalopathy syndrome is a rare neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging predominantly affecting the posterior regions of the brain. We report a 10-years-8-months-old girl who presented with headache, multiple seizures, and altered sensorium. Her blood pressure was 130/100 mmHg and left brachial pulse was not palpable. CT scan brain showed typical non-enhancing hypodensities in bilateral parieto-occiptal lobes. Prompt treatment of the hypertension led to rapid reversal of neurological symptoms. CT aortogram revealed aortoarteritis with bilateral renal artery stenosis.The Indian Journal of Pediatrics 04/2012; 76(2):218-220. · 0.52 Impact Factor -
Conference Proceeding: Congenital Myasthenia syndrome: Natural history and long-term prognosis
Platform paper session , IANCON 2010, Trichy , India; 01/2010 -
Article: Congenital cutaneous candidiasis: a rare and unpredictable disease.
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ABSTRACT: Congenital cutaneous candidiasis (CCC) is an extremely rare disorder that presents within the first 6 days of life. The manifestations ranges from diffuse skin eruption without any systemic symptoms to respiratory distress, hepatosplenomegaly, sepsis, and death. We report a neonate who presented with generalized skin eruptions at birth, characterized by erythematous macules and papules. The eruption involved head, face, neck, trunk, and extremities. Candida albicans was demonstrated on direct KOH smear, skin biopsy. The disease implies a congenital intrauterine infection and is different from neonatal candidiasis, which manifests as thrush or diaper dermatitis. The infection is acquired from the maternal genital tract in an ascending fashion. Clinical features, direct smear examination of specimen, and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period. Topical antifungal therapy is sufficient unless systemic candidiasis is present. Prognosis for congenital cutaneous candidiasis is good.Indian Journal of Dermatology 01/2011; 56(1):92-3. -
Article: Posterior reversible encephalopathy syndrome revealing Takayasu's arteritis.
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ABSTRACT: Posterior reversible encephalopathy syndrome is a rare neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging predominantly affecting the posterior regions of the brain. We report a 10-years-8-months-old girl who presented with headache, multiple seizures, and altered sensorium. Her blood pressure was 130/100 mmHg and left brachial pulse was not palpable. CT scan brain showed typical non-enhancing hypodensities in bilateral parieto-occiptal lobes. Prompt treatment of the hypertension led to rapid reversal of neurological symptoms. CT aortogram revealed aortoarteritis with bilateral renal artery stenosis.The Indian Journal of Pediatrics 01/2009; 76(2):218-20. · 0.52 Impact Factor
