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Journal articles on the topic 'Hypsarrhytmia'

Author: Grafiati

Published: 3 June 2025

Last updated: 31 July 2025

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1

Baram, Tallie Z., Wendy C. Mitchell, Anne Tournay, O. Carter Snead, Rebecca A. Hanson, and E. J. Horton. "High-dose Corticotropin (ACTH) Versus Prednisone for Infantile Spasms: A Prospective, Randomized, Blinded Study." Pediatrics 97, no. 3 (1996): 375–79. http://dx.doi.org/10.1542/peds.97.3.375.

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Objective. To compare the efficacy of corticotropin (ACTH) (150 U/m2/day) and prednisone (2 mg/kg/day), given for 2 weeks, in suppressing clinical spasms and hypsarrhythmic electroencephalogram (EEG) in infantile spasms (IS). ACTH and prednisone are standard treatments for IS. ACTH at high doses causes severe dose- and duration-dependent side effects, but may be superior to prednisone, based on retrospective or uncontrolled studies. Blinded prospective studies have shown equal efficacy of prednisone and low-dose ACTH, and low versus high-dose ACTH. Design. A prospective, randomized, single-bli

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2

Millichap, J. Gordon. "Asymmetric Hypsarrhythmia." Pediatric Neurology Briefs 9, no. 1 (1995): 2. http://dx.doi.org/10.15844/pedneurbriefs-9-1-3.

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3

Millichap, John J., and J. Gordon Millichap. "Hypsarhythmia or Hypsarrhythmia?" Pediatric Neurology Briefs 29, no. 8 (2015): 64. http://dx.doi.org/10.15844/pedneurbriefs-29-8-7.

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4

Millichap, J. Gordon. "Hypsarrhythmia Variant Patterns." Pediatric Neurology Briefs 11, no. 2 (1997): 10. http://dx.doi.org/10.15844/pedneurbriefs-11-2-2.

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5

Sheetal, S., Nikhil Gladson, Rakesh John, and Yamuna P. Kurup. "West Syndrome in an Infant with Complete Corpus Callosal Agenesis." Archives of Medicine and Health Sciences 12, no. 1 (2023): 120–22. http://dx.doi.org/10.4103/amhs.amhs_105_23.

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West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pat

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6

Sousa, Gean, Claudio Queiroz, Patrícia Sousa, et al. "Identification of Hypsarrhythmia in Children with Microcephaly Infected by Zika Virus." Entropy 21, no. 3 (2019): 232. http://dx.doi.org/10.3390/e21030232.

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Hypsarrhythmia is an electroencephalographic pattern specific to some epileptic syndromes that affect children under one year of age. The identification of this pattern, in some cases, causes disagreements between experts, which is worrisome since an inaccurate diagnosis can bring complications to the infant. Despite the difficulties in visually identifying hypsarrhythmia, options of computerized assistance are scarce. Aiming to collaborate with the recognition of this electropathological pattern, we propose in this paper a mathematical index that can help electroencephalography experts to ide

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7

Chandra, Mahua, Narayan Saha, SK Azimul Hoque, Provat Kumar Sarkar, Shameem Ara Begum, and Banita Mistry. "Clinical and Laboratory Profiles of Children with West Syndrome: Experience of 50 Cases in a Tertiary Hospital in Bangladesh." Bangladesh Journal of Child Health 44, no. 3 (2021): 139–42. http://dx.doi.org/10.3329/bjch.v44i3.52704.

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Background: West Syndrome (WS) consists of epileptic spasms, hypsarrhythmia on EEG and psychomotor delay or regression in children. Objective: To observe the clinical and laboratory profiles of children with west syndrome. Materials & Methods: This was a cross sectional study and done from July 2017 to June 2018 in Pediatric Neurology OPD, NINS among total 50 cases of WS (age of > 2 month to 2 years). WS was diagnosed by direct observation or video recording of spasm, along with history of developmental delay or regression and EEG change. Collected data regarding demography,

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8

Hughes, J. R., and L. G. Tomasi. "The Diminishing Incidence of Hypsarrhythmia." Clinical Electroencephalography 16, no. 4 (1985): 178–82. http://dx.doi.org/10.1177/155005948501600403.

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9

Donat, J., W. Lo, and F. Wright. "Asymmetrical hypsarrhythmia and infantile spasms." Electroencephalography and Clinical Neurophysiology 87, no. 2 (1993): S40—S41. http://dx.doi.org/10.1016/0013-4694(93)91013-q.

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10

Caraballo, Roberto Horacio, Natalio Fejerman, Bernardo Dalla Bernardina, et al. "Epileptic spasms in clusters without hypsarrhythmia in infancy." Epileptic Disorders 5, no. 2 (2003): 109–13. http://dx.doi.org/10.1684/j.1950-6945.2003.tb00571.x.

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Spasms are defined as epileptic seizures characterized by brief axial contraction, in flexion, extension or mixed, symmetric or asymmetric, lasting from a fraction of a second to 1‐2s, and are associated with a slow‐wave transient or sharp and slow‐wave complex, followed or not by voltage attenuation. Epileptic spasms usually appear in clusters and are age‐dependent. This type of epileptic spasms associated with the particular EEG pattern, hypsarrhythmia, constitutes the basis for the diagnosis of West syndrome. The question is, how to nosologically define those patients who clearly present ep

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11

Gorchkhanova, Zareta K., Elena D. Belousova, Ekaterina A. Nikolaeva, and Aleksandra M. Pivovarova. "Epilepsy and EEG features in Angelman syndrome." L.O. Badalyan Neurological Journal 4, no. 4 (2023): 234–46. http://dx.doi.org/10.46563/2686-8997-2023-4-4-234-246.

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Аngelman syndrome (AS) is an orphan disease caused by loss of function of the maternal allele of the UBE3A gene on chromosome 15 (region 15q11.2-q13), characterized by severe mental and speech retardation, movement disorders, unique behavior in the form of frequent laughter, and epileptic seizures with characteristic anomalies in EEG. Epilepsy is often drug-resistant, generalized seizure types dominate, among which myoclonic seizures and atypical absences are the most common. Infantile spasms are rare in AS children, but the EEG pattern, especially in infancy, in the form of diffuse slow-wave

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12

Khreisat, Wael. "Clinical Profile of Infants with Hypsarrhythmia." Acta Informatica Medica 19, no. 3 (2011): 149. http://dx.doi.org/10.5455/aim.2011.19.149-152.

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13

Sebastian, Fortini, Carolina Vilte, Ricardo Cersósimo, and Roberto Caraballo. "Epileptic spasms in clusters without hypsarrhythmia." Journal of Pediatric Epilepsy 03, no. 03 (2015): 181–88. http://dx.doi.org/10.3233/pep-14089.

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14

Millichap, J. Gordon. "Infantile Spasms, Hypsarrhythmia, and Adrenoleukodystrophy (ALD)." Pediatric Neurology Briefs 2, no. 8 (1988): 60. http://dx.doi.org/10.15844/pedneurbriefs-2-8-7.

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15

Keith, Ronald Mac. "INFANTILE SPASMS AND HYPSARRHYTHMIA OR HYPSARHYTHMIA." Developmental Medicine & Child Neurology 7, no. 2 (2008): 204. http://dx.doi.org/10.1111/j.1469-8749.1965.tb10916.x.

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16

Aysina, Victoriya A., Lyudmila M. Kuzenkova, and Olga V. Kozhevnikova. "Current data on early diagnosis of West syndrome. Electroencephalographic evolution of hypsarrhythmia." L.O. Badalyan Neurological Journal 1, no. 3 (2020): 169–76. http://dx.doi.org/10.17816/2686-8997-2020-1-3-169-176.

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The review of current ideas about the early diagnosis of West syndrome is presented in terms of electroencephalography data based on a characterization of the period of prehypsarrhythmia preceding the appearance of the pattern of hypsarrhythmia. There are considered examples of our observations. Also, our data from a study of a group of children with hypsarrhythmia are described. A lot of studies of clinical manifestations and neurophysiological aspects of West syndrome have been conducted after the onset of clinical manifestations in the form of spasms, and variants of the transformation of W

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17

Sehgal, Rachna, Sheffali Gulati, Savita Sapra, Manjari Tripathi, Ravinder Mohan Pandey, and Madhulika Kabra. "Prognostic Utility of Clinical Epilepsy Severity Score Versus Pretreatment Hypsarrhythmia Scoring in Children With West Syndrome." Clinical EEG and Neuroscience 48, no. 4 (2016): 280–87. http://dx.doi.org/10.1177/1550059416662425.

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This cross-sectional study assessed the impact of clinical epilepsy severity and pretreatment hypsarrhythmia severity on epilepsy and cognitive outcomes in treated children with West syndrome. Thirty-three children, aged 1 to 5 years, with infantile spasms were enrolled if pretreatment EEG records were available, after completion of ≥1 year of onset of spasms. Neurodevelopment was assessed by Development Profile 3 and Gross Motor Function Classification System. Epilepsy severity in the past 1 year was determined by the Early Childhood Epilepsy Severity Score (E-Chess). Kramer Global Score of h

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18

Millichap, J. Gordon. "ACTH Therapy in Epileptic Spasms without Hypsarrhythmia." Pediatric Neurology Briefs 19, no. 5 (2005): 40. http://dx.doi.org/10.15844/pedneurbriefs-19-5-12.

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19

Wiedemann, Anna, Birgit Stark, and Gudrun Gröppel. "The significance of focal pattern in hypsarrhythmia." Brain Disorders 17 (March 2025): 100188. https://doi.org/10.1016/j.dscb.2025.100188.

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20

Nenadovic, Vera, Robyn Whitney, Jason Boulet, and Miguel A. Cortez. "Hypsarrhythmia in epileptic spasms: Synchrony in chaos." Seizure 58 (May 2018): 55–61. http://dx.doi.org/10.1016/j.seizure.2018.03.026.

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21

Yanai, Shinji, Taketsugu Minami, Tomoya Yamamoto, et al. "Magnetoencephalographic analysis of hypsarrhythmia in West Syndrome." Journal of Epilepsy 10, no. 3 (1997): 131–38. http://dx.doi.org/10.1016/s0896-6974(97)90005-0.

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22

Kishi, Takamasa, Yoshihisa Nejihashi, Michi Kajiyama, and Kazuhiro Ueda. "Successful zonisamide treatment for infants with hypsarrhythmia." Pediatric Neurology 23, no. 3 (2000): 274–77. http://dx.doi.org/10.1016/s0887-8994(00)00183-1.

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23

Van Putten, M. J. A. M., and C. J. Stam. "Is the EEG really "chaotic" in hypsarrhythmia." IEEE Engineering in Medicine and Biology Magazine 20, no. 5 (2001): 72–79. http://dx.doi.org/10.1109/51.956822.

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24

Drury, Ivo, Ahmad Beydoun, Elizabeth A. Garofalo, and Thomas R. Henry. "Asymmetric Hypsarrhythmia: Clinical Electroencephalographic and Radiological Findings." Epilepsia 36, no. 1 (1995): 41–47. http://dx.doi.org/10.1111/j.1528-1157.1995.tb01663.x.

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25

Yang, Ming‐Tao, Wang‐Tso Lee, Steven Shinnforng Peng, et al. "The roles of electroencephalography and neuroimaging in children with holoprosencephaly." Epileptic Disorders 6, no. 3 (2004): 173–80. http://dx.doi.org/10.1684/j.1950-6945.2004.tb00066.x.

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ABSTRACT We analyze the respective roles of neuro‐imaging and EEG in the assessment of 11 children with holoprosencephaly and epilepsy. Seizures were present in seven patients (64%) ; six were treated with antiepileptic drugs ; five had intractable epilepsy. Two of the patients with intractable epilepsy became seizure‐free under polytherapy. Fourteen EEG recordings were performed in eight patients. The abnormal EEG findings included slow waves, focal epileptiform discharges, slow spike‐and‐wave complexes, hypsarrhythmia, frontal fast activity, fronto‐occipital gradients of amplitudes (posterio

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26

Falsaperla, Raffaele, Robinson Gutierrez, Gabriela Valenzuela, et al. "Single-Epileptic Spasms with or without Hypsarrhythmia: A Study of 16 Patients." Journal of Pediatric Epilepsy 06, no. 03 (2017): 149–55. http://dx.doi.org/10.1055/s-0037-1603521.

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Objective We evaluated the electroclinical features, etiology, treatment, and outcome of 16 patients with single-epileptic spasms (ESs) with or without hypsarrhythmia (WoH). Methods Nine boys and seven girls had single-ESs. ESs were considered as single epileptic spasm variants when no other spasm occurred for 1 minute before and after each spasm. Age at the onset of ESs was between 2 and 84 months, with a mean age of 11 months. Results We recognized a group of 15 patients with single-ESs as the main type of seizure; 6 patients with WoH and 9 patients with hypsarrhythmia, respectively. Nine of

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27

Sankhyan, Naveen, Ananthanarayanan Kasinathan, Sumeet Dhawan, and Renu Suthar. "Pseudo hypsarrhythmia: An early marker of angelman syndrome." Annals of Indian Academy of Neurology 22, no. 3 (2019): 359. http://dx.doi.org/10.4103/aian.aian_413_18.

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28

Darteyre, Stephane, Laure Mazzola, Philippe Convers, Marine Lebrun, and Dorothée Ville. "Angelman syndrome and pseudo-hypsarrhythmia: a diagnostic pitfall." Epileptic Disorders 13, no. 3 (2011): 331–35. http://dx.doi.org/10.1684/epd.2011.0446.

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29

Donat, Jane F., and Warren D. Lo. "Asymmetric Hypsarrhythmia and Infantile Spasms in West Syndrome." Journal of Child Neurology 9, no. 3 (1994): 290–96. http://dx.doi.org/10.1177/088307389400900314.

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30

Weng, Julius K., Regina Ahn, and Shaun A. Hussain. "Hypsarrhythmia is associated with widespread, asymmetric cerebral hypermetabolism." Seizure 71 (October 2019): 29–34. http://dx.doi.org/10.1016/j.seizure.2019.06.004.

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31

Messer, Ricka, and Kelly G. Knupp. "Infantile Spasms: Opportunities to Improve Care." Seminars in Neurology 40, no. 02 (2020): 236–45. http://dx.doi.org/10.1055/s-0040-1705121.

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AbstractInfantile spasm (IS) is a distinct epilepsy syndrome characterized by epileptic spasms (the clinical seizure type) and hypsarrhythmia (the electrographic abnormality). IS is frequently accompanied by impaired neurodevelopment and is often associated with structural, genetic, or metabolic etiologies. Prompt treatment of this severe epileptic encephalopathy improves long-term outcomes but remains elusive in many situations. Despite common misconceptions, even patients with identified etiologies or preexisting developmental delay benefit from proven standard therapies, including adrenocor

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32

Chandra, Mahua, Narayan Saha, Provat Kumar Sarkar, et al. "Response and Outcome of Moderate Dose Adrenocorticotropic Hormone in the Treatment of West Syndrome." Journal of National Institute of Neurosciences Bangladesh 7, no. 2 (2022): 108–12. http://dx.doi.org/10.3329/jninb.v7i2.58096.

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Background: West Syndrome (WS) consist of a triad of epileptic spasms, hypsarrhythmia on EEG and arrest or regression of psychomotor development. Although ACTH has been found to be effective in the treatment of WS, questions remain regarding the optimum dosage, type of ACTH, duration of therapy, and its comparative efficacy with other treatment options. Objective: To assess the response and outcome of treatment with moderate dose (100 IU/m2) ACTH in children of west syndrome. Methodology: This was a prospective observational study and done over 1-year period (July 2017 to June 2018

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33

Haginoya, Kazuhiroa, Kimiya Kon, Soichiro Tanaka, et al. "The origin of hypsarrhythmia and tonic spasms in West syndrome: evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia." Brain and Development 21, no. 2 (1999): 129–31. http://dx.doi.org/10.1016/s0387-7604(98)00082-5.

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34

Prygunova, T. M. "West syndrome: long-term outcomes depending on etiology and treatment (literature review)." Russian Journal of Child Neurology 13, no. 4 (2019): 53–63. http://dx.doi.org/10.17650/2073-8803-2018-13-4-53-63.

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West syndrome is a severe form of childhood epilepsy associated with drug-resistant seizures and intellectual disability. The disease is characterized by specific seizures called infantile spasms, hypsarrhythmia on the electroencephalogram, and delayed psychomotor development. The identification of the disease etiology may affect the prognosis and the choice of therapy. The following prognostic factors have been described so far: etiology, abnormal neurological signs, presence of other epileptic seizures before and after epileptic spasms, response to therapy regardless of the drug type. Diffic

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35

Shrestha, Bikash, Kamal Kiran, and Ranjit Ghulliani. "West Syndrome: How Early is Early for its Presentation?" Journal of Nepal Paediatric Society 33, no. 3 (2013): 239–42. http://dx.doi.org/10.3126/jnps.v33i3.8639.

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West syndrome is the triad of mental retardation, infantile spasms and characteristic hypsarrhythmia on electroencephalograph. West syndrome usually presents in mid-infancy period. We present this as a rare presentation of West syndrome in the neonatal period. DOI: http://dx.doi.org/10.3126/jnps.v33i3.8639 J. Nepal Paediatr. Soc. 2013;33(3):239-242

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36

VÖLZKE, E., H. DOOSE, and E. STEPHAN. "The Treatment of Infantile Spasms and Hypsarrhythmia with Mogadon." Epilepsia 8, no. 1 (2007): 64–70. http://dx.doi.org/10.1111/j.1528-1157.1967.tb03821.x.

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37

Kim, Min-Jee, Mi-Sun Yum, Hye-Ryun Yeh, and Tae-Sung Ko. "Fast oscillation dynamics during hypsarrhythmia as a localization biomarker." Journal of Neurophysiology 119, no. 2 (2018): 679–87. http://dx.doi.org/10.1152/jn.00662.2017.

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Hypsarrhythmia in West syndrome, although hard to define, is characterized by chaotic and disorganized electrical activity of the brain and is often regarded as a generalized EEG pattern without any localization value. Using event-related spectral perturbation (ERSP), we tried to determine the brain dynamics during hypsarrhythmia. Routine 1-h scalp EEGs were retrieved from 31 patients with infantile spasms and 20 age-matched controls. Using the EEGLAB toolbox of MATLAB 2015b, the ERSPs of fast oscillations (FOs; 20–100 Hz) of selected channels were analyzed and compared among groups according

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38

Hrachovy, Richard A., and James D. Frost. "Infantile Epileptic Encephalopathy with Hypsarrhythmia (Infantile Spasms/West Syndrome)." Journal of Clinical Neurophysiology 20, no. 6 (2003): 408–25. http://dx.doi.org/10.1097/00004691-200311000-00004.

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39

Caraballo, Roberto Horacio, Victor Ruggieri, Gabriel Gonzalez, et al. "Infantile spams without hypsarrhythmia: A study of 16 cases." Seizure 20, no. 3 (2011): 197–202. http://dx.doi.org/10.1016/j.seizure.2010.11.018.

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40

Parmeggiani, Antonia, Perrine Plouin, and Olivier Dulac. "Quantification of diffuse and focal delta activity in hypsarrhythmia." Brain and Development 12, no. 3 (1990): 310–15. http://dx.doi.org/10.1016/s0387-7604(12)80312-3.

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41

Philippi, Heike, Gabriele Wohlrab, Uli Bettendorf, et al. "Electroencephalographic evolution of hypsarrhythmia: Toward an early treatment option." Epilepsia 49, no. 11 (2008): 1859–64. http://dx.doi.org/10.1111/j.1528-1167.2008.01715.x.

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42

Oguni, Hirokazu, Makoto Funatsuka, Kaori Sasaki, et al. "Effect of ACTH Therapy for Epileptic Spasms without Hypsarrhythmia." Epilepsia 46, no. 5 (2005): 709–15. http://dx.doi.org/10.1111/j.1528-1167.2005.37504.x.

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43

Marino, S1 Falsaperla R1 Marino SD1 Smilari P2 Greco F. 2. Vitaliti G. 1. Caccamo M2 Barbagallo M3 &. *Pavone P1 2. "WEST VARIANT IN MILLER-DIEKER SYNDROME." GLOBAL JOURNAL OF ENGINEERING SCIENCE AND RESEARCHES 5, no. 9 (2018): 1–5. https://doi.org/10.5281/zenodo.1420146.

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<strong>Introduction. </strong>Miller-Dieker syndrome (MDs) is a severe malformative condition characterized by a smooth surface known also as lissencephaly . The syndrome is linked to a contiguous gene deletion of short arm of chromosome 17 (17p13.3) causing cerebral dysregulation.  Affected children present with facial dysmorphisms, severe intellectual disability and drug resistant seizures. Infantile spasms are the most frequently reported types of seizures with the EEG recording presenting with the typical aspect of hypsarrhythmia<strong>. </strong> <strong>Case Report. </strong>Recen

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44

Mohan, Vishnu. "West Syndrome: A Case Study Through Audiological Perspective." International Journal of Health Sciences and Research 12, no. 7 (2022): 115–17. http://dx.doi.org/10.52403/ijhsr.20220716.

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West Syndrome is a disorder with combination of symptoms such as infantile spasm along with its typical age of onset and specific kind of electroencephalogram pattern termed as hypsarrhythmia Here in this article I report a case of West syndrome with left hemi paresis, diagnosed in a 2.5 year old baby boy Key words: West Syndrome, Hearing Evaluation, Auditory brainstem response .

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45

Murugan, Akila, P. Umapathy, and Sabitha Panchagiri. "West Syndrome – Infantile Spasms: A Rare Paediatric Case Report." Journal of Drug Delivery and Therapeutics 13, no. 4 (2023): 6–8. http://dx.doi.org/10.22270/jddt.v13i4.6009.

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West syndrome (WS) is a rare, severe form of epilepsy with onset in infancy and early childhood. It involves clustered epileptic spasm episodes, the aberrant interictal electroencephalogram pattern known as hypsarrhythmia, and neuropsychomotor delay. In this view, we present a case of a 6-year-old female child a known case of west syndrome, global developmental delay, hypothyroidism, and post-operative laparoscopic gastrotomy performed under general anaesthesia on March 31/2022, in view of recurrent aspiration and came with the chief complaints of seizure 6-7 episodes per day, decreased urine

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46

Paprocka, J., B. Rzepka-Migut, N. Rzepka, A. Jezela-Stanek, and E. Morava. "Infantile Alexander disease with late onset infantile spasms and hypsarrhythmia." Balkan Journal of Medical Genetics 22, no. 2 (2019): 77–82. http://dx.doi.org/10.2478/bjmg-2019-0017.

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AbstractAlexander disease (AxD) is a rare autosomal dominant leukodystrophy with three clinical subtypes: infantile, juvenile and adult. Forms differ by age of symptoms occurrence and the clinical presentation. Although recent data suggest considering only two subtypes: type I (infantile onset with lesions extending to the cerebral hemispheres); type II (adult onset with primary involvement of subtentorial structures). Dominant mutations in the glial fibrillary acidic protein (GFAP) gene in AxD cause dysfunction of astrocytes (a type III intermediate filament). The authors discuss the clinical

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47

Millichap, J. Gordon, and John J. Millichap. "Oral Prednisolone vs. IM Corticotropin and Hypsarrhythmia* in West Syndrome." Pediatric Neurology Briefs 28, no. 8 (2014): 59. http://dx.doi.org/10.15844/pedneurbriefs-28-8-3.

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48

Shevell, Michael I., Patricia Colangelo, Eileen Treacy, Robert C. Polomeno, and B. Rosenblatt. "Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome)." Pediatric Neurology 15, no. 4 (1996): 337–39. http://dx.doi.org/10.1016/s0887-8994(96)00161-0.

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49

Bazigou-Fotopulu, Eleni, A. Gouliamos, C. Skiadas, and V. Kiriakidou. "Computed tomography in children with an electroencephalographic pattern of hypsarrhythmia." Electroencephalography and Clinical Neurophysiology 61, no. 3 (1985): S182—S183. http://dx.doi.org/10.1016/0013-4694(85)90701-1.

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50

Salonen, R., M. Somer, M. Haltia, M. Lorentz, and R. Norio. "Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome)." Clinical Genetics 39, no. 4 (2008): 287–93. http://dx.doi.org/10.1111/j.1399-0004.1991.tb03027.x.

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