Malformations of cortical development (MCD) are critical brain development disorders associated with varied abnormalities in both anatomic structures and neural functioning. It is also a very common etiology to the epilepsy, in which the alteration on excitability of cortical neurons is hypothesized as one of important causes to the epileptic seizures. Due to the key role in regulating neuron firing properties, the plasticity of axon initial segment (AIS) was investigated in present study to further determine the relation between MCD and epilepsy. Our results showed a prolonged decrease in the length of AIS occurred in MCD animal models. Besides, the AIS was also found greatly shortened in MCD models during the acute, but not chronic phase of status epileptics compared with intact controls. Our findings of identification of AIS plasticity in MCD animal models and its hypersensitivity to status epilepsy are significant in furthering our understanding of the pathophysiological mechanisms involved in this disorder.
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http://dx.doi.org/10.1007/s11064-016-1936-7 | DOI Listing |
Front Neurosci
January 2025
Department of Neurology, Rosamund Stone Zander Translational Neuroscience Center, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
Malformations of cortical development encompass a broad range of disorders associated with abnormalities in corticogenesis. Widespread abnormalities in neuronal formation or migration can lead to small head size or microcephaly with disorganized placement of cell types. Specific, localized malformations are termed focal cortical dysplasias (FCD).
View Article and Find Full Text PDFSAGE Open Med Case Rep
January 2025
College of Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
A 15-year-old girl presented with new onset tonic-clonic seizures, encephalopathy, abdominal pain, and hypertension with a history of weight loss and emesis. Brain magnetic resonance imaging scans showed diffuse, bilateral cortical and subcortical gray and white matter signal abnormalities. Electroencephalography showed background slowing and disorganization.
View Article and Find Full Text PDFRinsho Shinkeigaku
January 2025
Department of Internal Medicine IV, Division of Neurology, Osaka Medical and Pharmaceutical University Faculty of Medicine.
In an 81-year-old man, brain diffusion-weighted MRI revealed punctate high-intensity lesions in the bilateral frontal cortex. Three months later, these lesions had extended into the cerebral cortices. Six months after the original MRI, the patient developed cognitive decline.
View Article and Find Full Text PDFPsychiatr Clin North Am
March 2025
Department of Neurology, Johns Hopkins University School of Medicine, Kennedy Krieger Institute, Baltimore, MD, USA.
The pathophysiology of tic disorders involves an alteration in the transmission of messages through the cortico-basal ganglia-thalamo-cortical circuit. A major requirement for the passage of a message through this circuit is an intact chemically mediated synaptic neurotransmitter system (ie, neurotransmitters and second messengers). This article reviews the scientific evidence supporting the involvement of a variety of neurotransmitters (ie, dopamine, glutamate, gamma-aminobutyric acid, serotonin, acetylcholine, and the opioid system).
View Article and Find Full Text PDFClin Pediatr (Phila)
February 2025
Department of Pediatric Surgery, St. John's Medical College Hospital, Bangalore, Karnataka, India.
Renal histologic changes in congenital ureteropelvic junction obstruction (UPJO), although well documented, are sparsely studied in children. This study aims to establish a histological grading depending on the glomerular and tubulo-interstitial changes in hydronephrotic kidneys and determine correlation with age at surgery and impact on function post-pyeloplasty. A renal cortical wedge biopsy was obtained after pyeloplasty and histological changes were graded from 1 to 4.
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