Suvasini Sharma

Primary tubercular osteomyelitis of the sternum

Tubercular involvement of the sternum, leading to osteomyelitis, is a rarely described entity even in countries where tuberculosis is endemic. Presentation in pediatric age group is even more uncommon. We describe a 12 year old girl who presented with a mass over the manubruim sterni and fever. CT chest demonstrated a soft tissue mass in the anterior mediastinum, eroding the cortex of the manubrium. Tubercular etiology was suggested by presence of epithelioid granulomas and acid fast bacilli in the Ziehl-Neelsen staining of the aspirate from the lesion. the patient responded well to antitubercular treatment.

Evaluation of a parent-based behavioral intervention program for children with autism in a low-resource setting.

Background: Intensive behavioral intervention programs are recommended for children with autism. However, in resource-constraint settings, such programs are largely inaccessible, and there is an urgent need for development of low-cost interventions. Aim: To evaluate the efficacy of a parent-based behavioral intervention program in Indian autistic children. Materials and Methods: Sixteen children with autistic disorder undergoing the intervention for at least six months were enrolled. The mean development, social, expressive, and receptive language quotients, and the Childhood Autism Rating Scale (CARS) and Autism Behavior Checklist (ABC) scores were compared before and after the intervention. Results: The average duration of therapy was 19.5±11.78 months. There was a significant improvement in the development quotient (P=0.015), social quotient (P=0.004), expressive language quotient (P=0.03), CARS (P=0.001), and ABC (P=0.014) scores. Conclusion: Parent-based behavioral intervention programs have a promising role in management of children with autism in resource-constraint settings.

Cutaneous tuberculosis mimicking sporotrichosis.

The authors describe an unusual presentation of lupus vulgaris in a 5-year-old boy. The lesions had a linear arrangement with proximal spread mimicking sporotrichosis. Histopathology of the lesions revealed tuberculoid granulomas. Tubercular etiology was confirmed by the demonstration of acid fast bacilli in the smears from the regional lymph node aspirate stained with Ziehl Neelsen stain, and growth of Mycobacterium tuberculosis in the aspirate culture. The patient showed marked improvement of his lesions on anti-tubercular treatment.

Acute cysticercal meningitis—Missed diagnosis

Neurocysticercosis is the commonest CNS parasitic disease worldwide but cysticercal meningoencephalitis is relatively rare, especially in Indian patients. We herein report a girl with cysticercal meningitis that was initially not suspected and later diagnosed on the basis of cerebrospinal fluid (CSF) eosinophilia. The need for CSF examination with wright-giemsa staining to avoid missing CSF eosinophilia is discussed.

Atlantoaxial dislocation in a child with generalized primary dystonia.

We describe a 10-year-old boy with generalized dystonia who developed spastic quadriparesis within 1 month of dystonia onset. On neuroimaging, a craniovertebral junction anomaly with atlantoaxial dislocation and compressive myelopathy was present. We postulate that dystonia involving the neck led to atlantoaxial dislocation in this child with a craniovertebral junction anomaly.

Spastic paraplegia in a 4-year-old boy

Sjogren-Larsson syndrome (SLS) is an autosomal recessive disorder caused by the deficiency of fatty aldehyde dehydrogenase activity. It comprises a clinical triad of congenital ichthyosis, spastic paraplegia, and mental retardation. Seizures occur in 40% of patients. Some patients with SLS also exhibit minor features such as a pattern of glistening white dots in the fovea, defective development of tooth enamel, joint hyperextensibility, kyphoscoliosis, defective sweating and short stature. Brainstem auditory, visual, and short latency somatosensory evoked potentials may also be impaired in these children. The imaging findings of SLS consist of T2-hyperintense signal in the deep white matter with sparing of the subcortical U fibers, as seen in our patient. Myelination is not complete until 20 years of age in some subjects, and at age 4, incomplete myelination may also be present in the trigonal areas and the periventricular region in the frontal lobe. The basic defect of SLS is impairment in fatty alcohol oxidation because of deficient activity of alcoholnicotinamide adenine dinucleotide oxidoreductase (alcohol-NAD+ oxidoreductase), specifically the fatty aldehyde dehydrogenase component. As a consequence of this enzyme deficiency, long chain alcohols accumulate, which may lead to alteration of the epidermal water barrier and increased transepidermal water loss,

Megalencephalic leukoencephalopathy with subcortical cysts

Megalencephalic leukoencephalopathy with subcortical cysts is a progressive condition that affects brain development and function. Individuals with this condition typically have an enlarged brain (megalencephaly) that is evident at birth or within the first year of life. Megalencephaly leads to an increase in the size of the head (macrocephaly). Affected people also have leukoencephalopathy, an abnormality of the brains white matter. White matter consists of nerve fibers covered by a fatty substance called myelin. Myelin insulates nerve cells (neurons) and promotes the rapid transmission of nerve impulses. In megalencephalic leukoencephalopathy with subcortical cysts, the myelin is swollen and contains numerous fluid-filled pockets (vacuoles). Over time, the swelling decreases and the myelin begins to waste away (atrophy). Individuals affected with this condition may develop cysts in the brain; because these cysts form below an area of the brain called the cerebral cortex, they are called subcortical cysts. These cysts can grow in size and number.