Clinical pearls in pediatric neurology.

The authors present three neurological cases (common and uncommon), that have important management implications. The specific diagnosis can be suspected clinically if the clinician is aware of the entities. Besides clinical clues, the recognition of important findings in MRI brain is highlighted.

CNS vasculitis and stroke in neonatal lupus erythematosus: a case report and review of literature.

Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has been described as transient vasculopathy only. We describe a 2-months-old girl who presented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations.

Acute axonal polyneuropathy following honey-bee sting: a case report.

Hymenoptera stings lead to a myriad of neurologic manifestations by the mechanism of immediate or delayed hypersensitivity reactions. The more common form of polyneuropathy associated with these stings is the acute inflammatory demyelinating type. We describe a 6-year-old girl, who developed progressive, symmetrical, ascending weakness within 3 days after a bee sting.

Childhood Guillain-Barré syndrome subtypes in northern India.

This prospective cross-sectional study was conducted at a tertiary care research centre in North India to describe the frequency and clinical characteristics of subtypes of childhood Guillain-Barré syndrome. Among the 68 children enrolled, 65 were finally diagnosed with Guillain-Barré syndrome (median age, 60 months); 45 (69%) were boys. The most common subtype was acute motor axonal neuropathy in 27 patients (41.

Noninvasive screening for preclinical atherosclerosis in children on phenytoin or carbamazepine monotherapy: a cross sectional study.

Purpose: This study was carried out to compare the carotid Intimal Media Thickness (IMT), and endothelial function using brachial flow mediated dilatation (FMD), in Epileptic children (6-12 years) on phenytoin (PHT) or carbamazepine (CBZ) monotherapy for ≥18 months with a control group of children.

Methods: In this cross-sectional study 30 children (aged 6-12 years) on PHT monotherapy and 28 children on CBZ monotherapy were compared with an equal number of apparently healthy age and sex matched children unexposed to antiepileptics. Fasting lipids, sugar, Hs-CRP levels and ultrasonographic assessment of carotid IMT and endothelial function using brachial FMD were conducted.

Proximal Myopathy: A Rare Presentation of Celiac Disease.

Celiac disease presenting as proximal myopathy is rarely seen, particularly in children. We report a 5-year-old girl who presented with bilateral lower limb weakness and on examination had proximal myopathy. She also had florid rickets and short stature.

Use of the modified Atkins diet for treatment of refractory childhood epilepsy: a randomized controlled trial.

Purpose: The aim of this study was to evaluate the efficacy of the modified Atkins diet in a randomized controlled trial in children with refractory epilepsy.

Methods: Children aged 2-14 years who had daily seizures despite the appropriate use of at least three anticonvulsant drugs were enrolled. Children were randomized to receive either the modified Atkins diet or no dietary intervention for a period of 3 months.

Efficacy of modified constraint induced movement therapy in improving upper limb function in children with hemiplegic cerebral palsy: a randomized controlled trial.

Purpose: The objective of this randomized single blind (outcome assessor) controlled trial was to evaluate the efficacy of 4 weeks of modified constraint induced movement therapy (mCIMT) in improving upper limb function in 3-8 years old children with hemiplegic cerebral palsy.

Methods: Thirty-one children were randomly assigned to receive the mCIMT (N=16) with conventional therapy or conventional therapy alone (N=15). Children were evaluated three times (at enrollment, follow up at 4 weeks and 12 weeks).

Detection of Mycobacterium tuberculosis GlcB or HspX Antigens or devR DNA impacts the rapid diagnosis of tuberculous meningitis in children.

Background: Tuberculous meningitis (TBM) is the most common form of neurotuberculosis and the fifth most common form of extrapulmonary TB. Early diagnosis and prompt treatment are the cornerstones of effective disease management. The accurate diagnosis of TBM poses a challenge due to an extensive differential diagnosis, low bacterial load and paucity of cerebrospinal fluid (CSF) especially in children.

Approach to a child with acute flaccid paralysis.

Acute flaccid paralysis (AFP) is a clinical syndrome characterized by rapid onset weakness, that many times includes respiratory and bulbar weakness. AFP is a broad clinical entity with an array of diagnostic possibilities. An accurate and early diagnosis of the cause has important bearing on the management and prognosis.

Macrocephaly with diffuse white matter changes simulating a leukodystrophy in Menkes disease.

Menkes disease is a rare inherited disorder of copper metabolism caused by mutations in the ATP7A gene. Its clinical course is characterized by early neurological regression, seizures, hypotonia and kinky friable hair. Neuroimaging typically reveals severe brain atrophy with subdural fluid collections and excessive tortuosity of cerebral arteries.

Use of the modified Atkins diet in infantile spasms refractory to first-line treatment.

This prospective, open label, uncontrolled study was performed to evaluate the efficacy and tolerability of the modified Atkins diet in children with refractory infantile spasms. Fifteen consecutive children aged six months to three years having daily infantile spasms in clusters with electroencephalographic evidence of hypsarrhythmia despite treatment with hormonal treatment (oral corticosteroids/adrenocorticotrophic hormone) and/or vigabatrin, and at least one additional anti-epileptic drug were enrolled. Children with known or suspected inborn errors of metabolism or systemic illnesses were excluded.

Limb-girdle myasthenia gravis in a 10-year-old girl: a case report.

A 10-year-old girl presented with progressive proximal limb muscle weakness without facial, ocular, or bulbar muscle involvement. There was no fatigability or diurnal fluctuation in symptoms. Her weakness worsened with febrile illnesses and recovered with accruing disabilities over a few weeks.

Leukoencephalopathy with brain stem and spinal cord involvement and high lactate: a genetically proven case without elevated white matter lactate.

A 17-year-old Indian boy with gradually progressive ataxia with onset at 12 years of age is described. Magnetic resonance imaging (MRI) of the brain revealed extensive, inhomogeneous signal abnormalities in the cerebral white matter, with involvement of selected tracts in the brain stem and spinal cord. The imaging findings were characteristic of leukoencephalopathy with brain stem and spinal cord involvement and high lactate, a recently described leukodystrophy.

Approach to the child with coma.

Coma and other states of impaired consciousness represent a medical emergency. The potential causes are numerous, and the critical window for diagnosis and effective intervention is often short. The common causes of non-traumatic coma include central nervous system infections, metabolic encephalopathy (hepatic, uremic, diabetic ketoacidosis etc.

Management of raised intracranial pressure.

Appropriate management of raised intracranial pressure begins with stabilization of the patient and simultaneous assessment of the level of sensorium and the cause of raised intracranial pressure. Stabilization is initiated with securing the airway, ventilation and circulatory function. The identification of surgically remediable conditions is a priority.