Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome.

Unlabelled: Dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contraction causing repetitive twisting movements and abnormal postures. Status dystonicus (SD) is an enigmatic disease of cryptic etiology. We hereby report a child with West syndrome (WS) who went on to develop SD following intramuscular adrenocorticotropic hormone (ACTH) injection.

Does ensuring optimum vitamin D levels result in early resolution of neurocysticercosis?

Background: Neurocysticercosis is a leading cause of acquired epilepsy. Calcified granulomas are known to cause seizure recurrence. Researchers have reported that vitamin D deficiency is associated with brain calcification and reduction in calcification occurs with vitamin D receptor agonist calcitriol through upregulation of SLC20A2.

Arterial Ischemic Stroke in a Child with Internal Carotid Artery Hypoplasia and Protein S Deficiency.

Congenital heart diseases, hemolytic anemia, collagen vascular diseases, and neurometabolic disorders are known to be associated with the development of arterial ischemic stroke (AIS) in children. However, not all the children with the aforementioned conditions develop AIS. Hence, the possibility of interplay of various predisposing factors in children with inherent underlying medical conditions seems likely.

Pediatric Tubercular Meningitis: A Review.

Childhood tuberculosis (TB) has a high incidence and prevalence in developing countries like India with tubercular meningitis (TBM) being the most common cause of death. Most cases of TBM are diagnosed late when despite adequate therapy; morbidity and mortality continue to remain high. This review aims to provide a pragmatic approach at dealing with cases of tubercular meningitis in children including clinical features, laboratory and radiological criteria, treatment options and prognostic implications.

Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India.

Survival and outcomes have improved considerably among patients with juvenile dermatomyositis (JDM) in the west. However, mortality continues to be high in the developing world. There is paucity of literature on this aspect of JDM from developing countries.

Subacute sclerosing panencephalitis in a toddler: changing epidemiological trends.

Subacute sclerosing panencephalitis (SSPE) is a devastating "slow virus" brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2-10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline.

Kawasaki disease and the emerging coronary artery disease epidemic in India: is there a correlation?

Although Kawasaki disease (KD) is now being increasingly reported from India, the vast majority of children with KD are still not being diagnosed and treated. A recent study from Chandigarh has shown that the incidence of KD is at least 4.54/100,000 children below 15 y of age.

Syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia caused by mutations in SLC30A10, a manganese transporter in man.

Environmental manganese (Mn) toxicity causes an extrapyramidal, parkinsonian-type movement disorder with characteristic magnetic resonance images of Mn accumulation in the basal ganglia. We have recently reported a suspected autosomal recessively inherited syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia in cases without environmental Mn exposure. Whole-genome mapping of two consanguineous families identified SLC30A10 as the affected gene in this inherited type of hypermanganesemia.

Is Kawasaki disease incidence rising in Chandigarh, North India?

Objective: To estimate incidence of Kawasaki disease (KD) over time among children in the city of Chandigarh, North India.

Patients And Methods: We analysed records of all children with KD below 15 years of age at the Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, from January 1994 to December 2008. Diagnosis and treatment of KD were based on American Heart Association criteria.

Spontaneous hematomyelia in a child with hemophilia A: a case report.

Hemorrhagic complications in patients with hemophilia have been occasionally reported in the spinal column and the spinal cord. Treatment is based on prompt replacement therapy as the occurrence and development of neurologic dysfunction are related to the length of time between the onset of symptoms and the factor replacement. We report case of a 7-year-old hemophilic boy who presented with flaccid paraparesis resulting from thoracic hematomyelia.

Kawasaki disease and Henoch Schonlein purpura: changing trends at a tertiary care hospital in north India (1993-2008).

The objective of the study was to evaluate the epidemiologic trends (1993-2008) in Kawasaki disease (KD) and Henoch Schonlein purpura (HSP) among children as seen in a tertiary care north Indian hospital. A hospital-based retrospective chart analysis of all the patients diagnosed with KD and HSP in our pediatric rheumatology clinic from January 1993 to December 2008 was performed. The diagnosis of KD and HSP was based on the American Heart Association and American College of Rheumatology criteria respectively.

Anomalous bilateral lateral rectus muscles and anterior polar cataract with dysmorphic features.

Congenital anomalies, such as absence, hypoplasia, bifurcation, or reduplication of various extraocular muscles, including lateral rectus muscles have been reported previously. We report a 5-year-old girl who had posteriorly malinserted lateral rectus at 15 mm from the limbus in the right eye with multiple associated ocular and systemic congenital malformations.

Red cell distribution width (RDW) in the diagnosis of iron deficiency with microcytic hypochromic anemia.

Objective: To study the utility of red cell distribution width (RDW) in the diagnosis of iron deficiency among children with microcytic hypochromic anemia.

Methods: 151 children (6 months-12 years) with microcytic (MCV<75 fl) anemia were classified into iron deficient (IDA) and non-iron deficient anemia (non-IDA) on the basis of serum ferritin and total iron binding capacity (TIBC). RDW values were obtained on an automated hematology analyzer.

Strategies for minimizing corticosteroid toxicity: a review.

Glucocorticoids (GCs) are used commonly for the treatment of various pediatric inflammatory and autoimmune diseases. Although potent and generally effective, they are not without risks for producing serious adverse effects, especially when used in high doses for prolonged periods of time. For proper use of systemic glucocortcoids, a basic knowledge of the pharmacology, clinical usage guidelines, and adverse reactions of these agents is imperative.