Purpose: Hypsarrhythmia is an electroencephalographic pattern associated with epileptic spasms and West syndrome. West syndrome is a devastating epileptic encephalopathy, originating in infancy. Hypsarrhythmia has been deemed to be the interictal brain activity, while the electrodecremental event associated with the spasms is denoted as the ictal event. Though characterized as chaotic, asynchronous and disorganized based on visual inspection of the EEG, little is known of the dynamics of hypsarrhythmia and how it impacts the developmental arrest of these infants.
Methods: As an exploratory and feasibility study, we explored the dynamics of both hypsarrhythmia and electrodecremental events with EEG phase synchronization methods, and in a convenience sample of three outpatients with epileptic spasms. As ictal events are associated with prolonged phase synchronization, we hypothesized that if hypsarrhythmia was indeed the interictal brain activity that it would have lower phase synchronization than the electrodecremental event (ictal phase).
Results: We calculated both the phase synchronization index and the temporal variability of the index in three patients with infantile spasms. Two patients had hypsarrhythmia and electrodecremental events and one had hemi-hypsarrhythmia. We found that the hypsarrhythmia pattern was a more synchronized state than the electrodecremental event.
Conclusions: We have observed that the hypsarrhythmia pattern may represent a more synchronized state than the electrodecremental event in infants with epileptic spasms. However, larger studies are needed to replicate and validate these findings. Additionally, further inquiry is required to determine the impact that increased synchronization may have on developmental outcomes in infants with epileptic spasms.
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http://dx.doi.org/10.1016/j.seizure.2018.03.026 | DOI Listing |
Brain Dev
December 2024
Division of Neurology, Saitama Children's Medical Center, 1-2 Chuo-ku Saitama-shi, Saitama 330-8777, Japan.
Purpose: This study aimed to elucidate the distribution of intracranial gamma-aminobutyric acid (GABA) receptors in patients with infantile epileptic spasms syndrome (IESS) of normal brain MRI findings using I-iomazenil single-photon emission computed tomography (IMZ-SPECT).
Methods: This retrospective study compared IMZ-SPECT images from 20 patients with IESS of unknown etiology with normal brain MRI (unknown IESS group) and 23 patients with developmentally normal epilepsy of the same age (developmentally normal group). A three-dimensional stereotactic region of interest (ROI) template was used to divide the brain into 24 segments (left and right callosomarginal, precentral, central, parietal, angular, temporal, posterior cerebral, pericallosal, lenticular nucleus, thalamus, hippocampus, and cerebellum), and the mean accumulation of I-iomazenil in each ROI was calculated.
Ann Med
December 2025
Research Group of Humanities and Qualitative Research in Health Science of Universidad Rey Juan Carlos (Hum&QRinHS), Department of Physical Therapy, Occupational Therapy, Physical Medicine and Rehabilitation, Universidad Rey Juan Carlos, Alcorcón, Spain.
Purpose: This study describes the experience of parents of children with developmental and epileptic encephalopathies (DEE) and how the disease impacts their daily lives.
Materials And Methods: A descriptive qualitative study was conducted using purposeful sampling. Twenty-one parents of children with DEEs caused by SCN1A, KCNQ2, CDKL5, PCDH19, and GNAO1 variants were included.
Epilepsia
December 2024
Department of Neurosurgery, Nagoya University School of Medicine, Nagoya, Japan.
Objective: At our institute, most pediatric patients undergo epilepsy surgery following a thorough presurgical evaluation without intracranial electroencephalography (EEG). We conducted an initial validation of our noninvasive presurgical strategy by assessing the seizure and developmental outcomes of 135 children.
Methods: All 135 pediatric patients were <15 years old, had undergone curative surgery, and were followed for at least 2 years postoperatively.
Diseases
December 2024
Department of Pediatrics, Dokkyo Medical University, Tochigi 321-0293, Japan.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by mutations in the TSC1 and TSC2 genes, leading to the dysregulation of the mammalian target of rapamycin (mTOR) pathway. This dysregulation results in the development of benign tumors across multiple organ systems and poses significant neurodevelopmental challenges. The clinical manifestations of TSC vary widely and include subependymal giant cell astrocytomas (SEGAs), renal angiomyolipomas (AMLs), facial angiofibromas (FAs), and neuropsychiatric conditions such as autism spectrum disorder (ASD).
View Article and Find Full Text PDFBMJ Case Rep
December 2024
Internal Medicine, Hirslanden Klinik Sankt Anna, Luzern, Switzerland
A patient with reactivated varicella zoster virus (VZV) manifesting in the left-sided dermatome L3 and S2-S4 developed tonic spasms which morphed into myoclonic jerks, paresis, rigidity and hypoesthesia of the left leg. Later, stimuli-sensitive myoclonus progressed to affect the upper body and was accompanied by fever surges with high-frequency myoclonus, hypertensive derailment, dysphagia and other features of the brainstem with autonomic dysfunction. Cerebrospinal fluid tested positive for VZV, MRI showed no signs of myelitis and EEG was negative for epilepsy.
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