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1.
J Pediatr ; 179: 259-262, 2016 12.
Article in English | MEDLINE | ID: mdl-27634625

ABSTRACT

Subacute sclerosing panencephalitis (SSPE) is a fatal complication of measles infection. We present a case of a fully vaccinated 3-year-old boy who was diagnosed with and treated for autoimmune encephalitis before arriving at a diagnosis of SSPE. We discuss the challenges of diagnosing SSPE in developed countries.


Subject(s)
Subacute Sclerosing Panencephalitis/prevention & control , Child, Preschool , Humans , Male , Measles/complications , Measles/prevention & control , Measles Vaccine , Subacute Sclerosing Panencephalitis/virology
2.
Semin Pediatr Neurol ; 23(2): 127-33, 2016 05.
Article in English | MEDLINE | ID: mdl-27544469

ABSTRACT

Several factors, such as epilepsy syndrome, poor compliance, and increased seizure frequency increase the risks of sudden unexpected death in epilepsy (SUDEP). Animal models have revealed that the mechanisms of SUDEP involve initially a primary event, often a seizure of sufficient type and severity, that occurs in a brain, which is vulnerable to SUDEP due to either genetic or antecedent factors. This primary event initiates a cascade of secondary events starting, as some models indicate, with cortical spreading depolarization that propagates to the brainstem where it results in autonomic dysfunction. Intrinsic abnormalities in brainstem serotonin, adenosine, sodium-postassium ATPase, and respiratory-control systems are also important. The tertiary event, which results from the above dysfunction, consists of either lethal central apnea, pulmonary edema, or arrhythmia. Currently, it is necessary to (1) continue researching SUDEP mechanisms, (2) work on reducing SUDEP risk factors, and (3) address the major need to counsel families about SUDEP.


Subject(s)
Death, Sudden , Epilepsy , Animals , Child , Death, Sudden/etiology , Death, Sudden/prevention & control , Epilepsy/complications , Epilepsy/metabolism , Epilepsy/physiopathology , Humans
4.
Neuron ; 78(6): 1063-74, 2013 Jun 19.
Article in English | MEDLINE | ID: mdl-23727119

ABSTRACT

Benzodiazepines (BZs) allosterically modulate γ-aminobutyric acid type-A receptors (GABAARs) to increase inhibitory synaptic strength. Diazepam binding inhibitor (DBI) protein is a BZ site ligand expressed endogenously in the brain, but functional evidence for BZ-mimicking positive modulatory actions has been elusive. We demonstrate an endogenous potentiation of GABAergic synaptic transmission and responses to GABA uncaging in the thalamic reticular nucleus (nRT) that is absent in both nm1054 mice, in which the Dbi gene is deleted, and mice in which BZ binding to α3 subunit-containing GABAARs is disrupted. Viral transduction of DBI into nRT is sufficient to rescue the endogenous potentiation of GABAergic transmission in nm1054 mice. Both mutations enhance thalamocortical spike-and-wave discharges characteristic of absence epilepsy. Together, these results indicate that DBI mediates endogenous nucleus-specific BZ-mimicking ("endozepine") roles to modulate nRT function and suppress thalamocortical oscillations. Enhanced DBI signaling might serve as a therapy for epilepsy and other neurological disorders.


Subject(s)
Diazepam Binding Inhibitor/physiology , Inhibitory Postsynaptic Potentials/genetics , Receptors, GABA-A/metabolism , Thalamus/physiology , Allosteric Regulation/genetics , Amino Acid Substitution/genetics , Animals , Benzodiazepines/metabolism , Diazepam Binding Inhibitor/deficiency , Diazepam Binding Inhibitor/genetics , Female , Male , Mice , Mice, 129 Strain , Mice, Inbred C57BL , Mice, Knockout , Mice, Transgenic , Mutation/genetics , Neural Inhibition/genetics , Receptors, GABA-A/genetics , Receptors, GABA-A/physiology , gamma-Aminobutyric Acid/metabolism
5.
Epilepsy Behav ; 21(1): 65-70, 2011 May.
Article in English | MEDLINE | ID: mdl-21497558

ABSTRACT

The superior longitudinal fasciculus (SLF) II and cingulum are two white matter tracts important for attention and other frontal lobe functions. These functions are often disturbed in children with drug-resistant (DR) partial epilepsy, even when no abnormalities are seen on conventional MRI. We set out to determine whether abnormalities in these structures might be depicted on diffusion tensor imaging (DTI) studies in the absence of abnormalities on conventional MRI. We compared the DTI findings of 12 children with DR partial epilepsy with those of 12 age- and gender-matched controls. We found that the SLF II fractional anisotropy (FA) values of the patients were significantly lower than those of the controls (means: 0.398±0.057 and 0.443±0.059, respectively, P=0.002). Similarly, apparent diffusion coefficient (ADC) and parallel diffusivity values for SLF II were also significantly lower in the patients. There were no differences in the FA and ADC values of the cingulum. Our findings are consistent with abnormal structural connectivity of the frontal lobe in children with DR partial epilepsy and provide a possible explanation for the previously reported functional abnormalities related to the SLF II in these patients.


Subject(s)
Brain Mapping , Epilepsies, Partial/pathology , Frontal Lobe/pathology , Adolescent , Anisotropy , Case-Control Studies , Child , Child, Preschool , Diffusion Magnetic Resonance Imaging/methods , Drug Resistance , Electroencephalography , Epilepsies, Partial/drug therapy , Female , Humans , Male , Video Recording
6.
Pediatr Neurol ; 44(4): 239-53, 2011 Apr.
Article in English | MEDLINE | ID: mdl-21397164

ABSTRACT

The past few years have witnessed increasing interest in devising programs to enhance early childhood development. We review current understandings of brain development, recent advances in this field, and their implications for clinical interventions. An expanding body of basic science laboratory data demonstrates that several interventions, including environmental enrichment, level of parental interaction, erythropoietin, antidepressants, transcranial magnetic stimulation, transcranial direct current stimulation, hypothermia, nutritional supplements, and stem cells, can enhance cerebral plasticity. Emerging clinical data, using functional magnetic resonance imaging and clinical evaluations, also support the hypothesis that clinical interventions can increase the developmental potential of children, rather than merely allowing the child to achieve an already predetermined potential. Such interventions include early developmental enrichment programs, which have improved cognitive function; high-energy and high-protein diets, which have increased brain growth in infants with perinatal brain damage; constraint-induced movement therapy, which has improved motor function in patients with stroke, cerebral palsy, and cerebral hemispherectomy; and transcranial magnetic stimulation, which has improved motor function in stroke patients.


Subject(s)
Brain Injuries , Brain/physiology , Child Development , Early Intervention, Educational , Science/methods , Animals , Brain/cytology , Brain Injuries/pathology , Brain Injuries/physiopathology , Brain Injuries/therapy , Child , Humans , Neurogenesis/physiology , Neuronal Plasticity/physiology , Recovery of Function/physiology
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