Assessment of MR blood-oxygen-level-dependent (BOLD) cerebrovascular reactivity under general anesthesia in children with moyamoya.

Background: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve.

PANDAS/PANS in childhood: Controversies and evidence.

Since first defined in 1998, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) and its later, broader iteration, paediatric acute-onset neuropsychiatric syndrome (PANS), have garnered significant attention and controversy. The role of streptococcal infection in children with explosive onset obsessive-compulsive disorder and new onset tics, the natural history of this entity, and the role of symptomatic and disease-modifying therapies, including antibiotics, immunotherapy, and psychoactive drugs, are all issues that have yet to be definitively addressed. While definitive proof of the autoimmune hypothesis of PANDAS is lacking, given the heightened attention to this entity and apparent rise in use of this diagnostic category, addressing questions around diagnosis, treatment, and etiology is imperative.

Measurement of cerebrovascular reactivity in pediatric patients with cerebral vasculopathy using blood oxygen level-dependent MRI.

Background And Purpose: Cerebrovascular reactivity (CVR) is an indicator of cerebral hemodynamics. In adults with cerebrovascular disease, impaired CVR has been shown to be associated with an increased risk of stroke. In children, however, CVR studies are not common.

Pediatric constraint-induced movement therapy is associated with increased contralateral cortical activity on functional magnetic resonance imaging.

The mechanism behind constraint-induced movement therapy (constraint therapy) success is unknown. Study objectives were to evaluate cortical change after modified constraint therapy and explore a novel approach to quantify developmental disregard. Five participants underwent modified constraint therapy.

Cerebral arteriopathy in children with neurofibromatosis type 1.

Objective: Cerebrovascular abnormalities are serious but underrecognized complications of neurofibromatosis type 1 (NF1). The aim of this study was to investigate the prevalence, clinical presentation, imaging findings, and prognosis of cerebral arteriopathies in childhood NF1.

Methods: Patients followed at the NF1 clinic at the Hospital for Sick Children, Toronto, Ontario, Canada, between 1990 and 2007 were studied.

Spatiotemporal patterns of oscillatory brain activity during auditory word recognition in children: a synthetic aperture magnetometry study.

Objective: We studied the task-induced spatiotemporal evolution and characteristics of cortical neural oscillations in children during an auditory word recognition task.

Methods: We presented abstract nouns binaurally and recorded the MEG response in eight healthy right-handed children (6-12 years). We calculated the event-related changes in cortical oscillations using a beamformer spatial filter analysis technique (SAM), then transformed each subject's statistical maps into standard space and used these to make group statistical inferences.

Cortical reorganization after modified constraint-induced movement therapy in pediatric hemiplegic cerebral palsy.

Constraint-induced movement therapy improves motor function in the affected hand of children with hemiplegic cerebral palsy and results in cortical changes in adults with stroke. This study measured clinical improvement and cortical reorganization in a child with hemiplegia who underwent modified constraint-induced movement therapy for 3 weeks. Clinical, functional magnetic resonance imaging and magnetoencephalography measurements were done at baseline, after therapy, and 6 months after therapy.

Contrast sensitivity is reduced in children with infantile spasms.

Purpose: To investigate whether visual deficits in children with infantile spasm (IS) are the result of seizure activity or of treatment with the anticonvulsant drug vigabatrin (VGB).

Methods: Vision function was determined in three experiments by determining peak contrast sensitivity (CS) and grating acuity (GA) with the sweep visual evoked potential. Cross-sectional study A: 34 children, including 11 patients with childhood epilepsy with exposure to VGB for at least 6 months, 10 with childhood epilepsy exposed to antiepileptic drugs other than VGB, and 13 normally developing children.

Intrahemispheric reorganization of language in children with medically intractable epilepsy of the left hemisphere.

We investigated language representation in nine children (six male, three female; 5.6-17.7 years of age) who underwent surgical treatment of medically intractable epilepsy of the left hemisphere.

Placebo-controlled study examining effects of selegiline in children with attention-deficit/hyperactivity disorder.

There is evidence suggesting a role for dopamine in attention-deficit/hyperactivity disorder (ADHD). Pharmacological treatments that act on the dopamine system have been successful in reducing ADHD symptoms. However, unlike traditional stimulants (i.

Reduced visual function associated with infantile spasms in children on vigabatrin therapy.

Purpose: To use visual evoked potential (VEP) testing to determine whether visual deficits are present in children with a history of vigabatrin use.

Methods: Contrast sensitivity and visual acuity were assessed by visual evoked potential testing and compared between 28 children (mean age, 4.90 +/- 4.

Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children.

Objective: To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin.

Design: An interventional case series report.

Participants: One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto.

Functional MRI study of verbal fluency in a patient with subcortical laminar heterotopia.

Rationale: Double cortex syndrome is a malformation in which there is a band of subcortical heterotopic grey matter separated from the cortex by white matter. The functional activity of the heterotopic neurons is unclear.

Patient: A 13-year-old female was evaluated for seizures.

Effect of intracranial bleeds on the health and quality of life of boys with hemophilia.

Objectives: To compare the health, physical function, and quality of life (QoL) of boys with hemophilia with and without a history of intracranial hemorrhage (ICH).

Study Design: Of 172 patients with hemophilia A or B, 18 (10%) had at least one episode of ICH. For outcome assessments, 16 of 18 (89%) boys with ICH and 32 controls, matched (1:2) for age and severity of hemophilia, were available.

Changes in the electroretinogram resulting from discontinuation of vigabatrin in children.

Electroretinograms (ERGs) have been recorded longitudinally in children before and during treatment with the antiepileptic drug vigabatrin for the past 3.5 years. Vigabatrin induced changes in ERG responses occur in children; the most dramatic changes occur in the oscillatory potentials.

Longitudinal changes in photopic OPs occurring with vigabatrin treatment.

Purpose: Vigabatrin (gamma-vinyl-GABA) is an antiepileptic drug successful in the management of infantile spasms. Photopic ERGs were tested in children followed longitudinally before and during vigabatrin treatment.

Methods: Subjects were 26 infants (age range 1.

The significance of ear plugging in localization-related epilepsy.

Purpose: The localizing value of ear plugging in the treatment of auditory onset partial seizures, to our knowledge, has not been previously described. We propose that ear plugging is a clinical response to a sensory seizure manifested as an auditory hallucination and a tool for identifying the seizure focus in the auditory cortex on the superior temporal gyrus.

Methods: We report on three children who had prior epilepsy surgery for recurrent symptomatic localization-related epilepsy and who, subsequent to their surgery, displayed stereotyped unilateral or bilateral ear plugging at the onset of partial seizures.

Unusual presentation of Lafora's disease.

Lafora's disease is a progressive myoclonus epilepsy with onset in adolescence and a gradual decline in cognitive functions and increase in seizure intractability. We present the case of a 16-year-old with precipitous dementia within 6 months of onset. Peripheral biopsies and EPM2A mutation analysis were negative.

The Hospital for Sick Children, Toronto, Longitudinal ERG study of children on vigabatrin.

The purpose of this longitudinal study was to identify changes in ERG responses associated with vigabatrin treatment. We accomplished this by recording longitudinally ERGs in children before and during vigabatrin treatment and comparing results between children on vigabatrin monotherapy and those taking additional anticonvulsive medications. Thirty-three children on vigabatrin therapy were tested; the duration between visits was approximately 6 months.