Hypsarrhythmia: Causes & Reasons

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Possible Causes for Hypsarrhythmia

Developmental and Epileptic Encephalopathy Type 2

[…] at around age 3-6 months, and further from hypsarrhythmia to diffuse slow spike-waves at around age 1. [ncbi.nlm.nih.gov]

The seizures evolved to brief tonic spasms, and hypsarrhythmia on EEG was noticed at age of 5 months, indicating the transition to West syndrome. [ncbi.nlm.nih.gov]

 EEG - suppression–burst activity, which evolves into hypsarrhythmia,accentuated during sleep,persists for prolonged periods.  Inborn errors of metabolism mc causes.  [slideshare.net]

Developmental and Epileptic Encephalopathy Type 17

Over her lifetime, neither hypsarrhythmia nor diffuse slow spike-wave pattern were seen. [ncbi.nlm.nih.gov]

[…] at around age 3-6 months, and further from hypsarrhythmia to diffuse slow spike-waves at around age 1. [ncbi.nlm.nih.gov]

In 7 of the 9 patients with OS, EEG findings transitioned from suppression-burst pattern to hypsarrhythmia. [ncbi.nlm.nih.gov]

Kohlschütter-Tönz Syndrome

Hypsarrhythmia phenotype Nervous System Diseases Finding 50 0.100 0 CUI: C0553721 Disease: Diminished sweating Diminished sweating phenotype Finding 44 0.100 0 CUI: C0007758 [disgenet.org]

The signs and symptoms of Kohlschütter-Tönz Syndrome may include: Ataxia Cerebellar hypoplasia Cerebral atrophy Epileptic encephalopathy Hypoplasia of dental enamel Hypsarrhythmia [dovemed.com]

[…] cerebellum [ more ] 0001321 Cerebral atrophy Degeneration of cerebrum 0002059 Epileptic encephalopathy 0200134 Hypoplasia of dental enamel Underdeveloped teeth enamel 0006297 Hypsarrhythmia [rarediseases.info.nih.gov]

Congenital Disorder of Glycosylation Type 1I

[…] recessive inheritance 0000007 Coloboma Notched pupil 0000589 Global developmental delay 0001263 Hepatomegaly Enlarged liver 0002240 Hyperreflexia Increased reflexes 0001347 Hypsarrhythmia [rarediseases.info.nih.gov]

Developmental and Epileptic Encephalopathy Type 4

Patients 1, 2, 3, and 5 had hypsarrhythmia and/or suppression-burst pattern on EEG, and responded poorly to multiple anti-epileptic drugs. [journals.lww.com]

Other signs and symptoms may include intellectual disability, reduced muscle tone (hypotonia ), hypsarrhythmia (an irregular pattern seen on EEG ), dyskinesia (involuntary [rarediseases.info.nih.gov]

The EEG will show an irregular pattern of suppression-bursts, progressing to hypsarrhythmia. Burst suppression is present in EEG tests during both wakefulness and sleep. [news-medical.net]

Developmental and Epileptic Encephalopathy Type 26

EIEE26 patients manifest multiple types of seizures, delayed psychomotor development, poor or absent speech, hypotonia, hypsarrhythmia. [uniprot.org]

 EEG - suppression–burst activity, which evolves into hypsarrhythmia,accentuated during sleep,persists for prolonged periods.  Inborn errors of metabolism mc causes.  [slideshare.net]

[pubmed:6254446] Genetic study of infantile spasms with hypsarrhythmia. [pubmed:870317] Expansion of the ARX spectrum. [bio2rdf.org]

Developmental and Epileptic Encephalopathy Type 12

When he was 9 months old, epilepic spasms were observed with hypsarrhythmia on EEG. He also showed severe developmental delay. [unboundmedicine.com]

[…] of infancy is associated with an expanded spectrum of clinical features (including severe gut dysmotility and a movement disorder) and electrographic features including hypsarrhythmia [kclpure.kcl.ac.uk]

Two of these patients had modified hypsarrhythmia, as described above. [academic.oup.com]

Tuberous Sclerosis

During this therapy, both ES and hypsarrhythmia remained completely resolved. [ncbi.nlm.nih.gov]

Hypsarrhythmia is seen on EEG. [medbullets.com]

They can begin in very early childhood as infantile spasms, sometimes with hypsarrhythmia. [autism-help.org]

Congenital Disorder of Glycosylation Type 1D

[…] fingers Microcephaly Muscular hypotonia of the trunk Abnormality of the nervous system Cerebellar atrophy Cerebral atrophy Global developmental delay Hyperreflexia Hypertonia Hypsarrhythmia [ncbi.nlm.nih.gov]

[…] more ] 0001508 Food intolerance 0012537 High palate Elevated palate Increased palatal height [ more ] 0000218 Hyperreflexia Increased reflexes 0001347 Hypertonia 0001276 Hypsarrhythmia [rarediseases.info.nih.gov]

Patients with CDG-IIm exhibit early-infantile epileptic encephalopathy, developmental delay, hypotonia, variable ocular anomalies, seizures, hypsarrhythmia, poor feeding, [themedicalbiochemistrypage.org]

Hyperphosphatasia with Mental Retardation Type 5

Disease description An autosomal recessive neurologic disorder characterized by developmental delay, mental retardation, tonic seizures associated with hypsarrhythmia, dysmorphic [uniprot.org]

Methylmalonic Acidemia with Homocystinuria Type cblX

[…] and broad skull 0000248 Failure to thrive Faltering weight Weight faltering [ more ] 0001508 Generalized hypotonia Decreased muscle tone Low muscle tone [ more ] 0001290 Hypsarrhythmia [rarediseases.info.nih.gov]

West Syndrome

[…] of clinical spasms [hypsarrhythmia without infantile spasms (HWIS)] or typical clinical spasms may manifest in absence of hypsarrhythmia [infantile spasms without hypsarrhythmia [ncbi.nlm.nih.gov]

[…] the corpus callosum is important in the generalization of hypsarrhythmia. [ncbi.nlm.nih.gov]

PURPOSE: Hypsarrhythmia, the pathognomonic EEG pattern of West syndrome, is typically characterized by a high amplitude, arrhythmic, and asynchronous pattern. [ncbi.nlm.nih.gov]

Familial Infantile Myoclonic Epilepsy

Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. [childneurologyfoundation.org]

Results: All six affected boys had myoclonic seizures and TCS; two had infantile spasms, but only one had hypsarrhythmia. [neurology.org]

Infantile spasm, IS, X-linked infantile spasm syndrome, X-linked infantile spasms, West's syndrome, Tonic spasms with clustering, arrest of psychomotor development and hypsarrhythmia [rarediseases.info.nih.gov]

Pyridoxine Dependency Syndrome

Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. [childneurologyfoundation.org]

Multiple variations of this pattern are possible, including focal or asymmetric hypsarrhythmia. [patient.info]

By Afsaneh Khetrapal BSc (Hons) West Syndrome is characterized by infantile spasms, developmental reversion, and a specific chaotic brain activity pattern called hypsarrhythmia [news-medical.net]

Developmental and Epileptic Encephalopathy Type 3

Evolve to hypsarrhythmia and then to diffuse slow spike waves Transformation Persists for long period Evolves to West syndrome and then to Lennox-Gastaut syndrome Epidemiology [wikidoc.org]

The course observation clarifies differences between both syndromes; SBs evolve to hypsarrhythmia around 3-4 months of age, and sometimes further to diffuse slow spike-waves [ncbi.nlm.nih.gov]

At five months, inter-ictal EEG revealed hypsarrhythmia, but his attacks were still only myoclonuses. [ncbi.nlm.nih.gov]

Focal Epilepsy - Speech Disorder - Mental Retardation

Variations of the prototypic pattern (modified hypsarrhythmia) include hypsarrhythmia with increased interhemispheric synchronization, asymmetrical hypsarrhythmia, hypsarrhythmia [pediatrics.emory.edu]

 EEG - suppression–burst activity, which evolves into hypsarrhythmia,accentuated during sleep,persists for prolonged periods.  Inborn errors of metabolism mc causes.  [slideshare.net]

Hrachovy et al. described the variations of the pattern: increased interhemispheric synchronization, asymmetrical hypsarrhythmia, hypsarrhythmia with a consistent focus of [euroformhealthcare.biz]

Diomedi-Bernardi-Placidi Syndrome

Results: All six affected boys had myoclonic seizures and TCS; two had infantile spasms, but only one had hypsarrhythmia. [neurology.org]

West: infantile spasms, hypsarrhythmia. Proud: microcephaly, ACC, spasticity, seizures, ataxia, genital anomalies. [pediatrics.aappublications.org]

The EEG showed a hypsarrhythmia pattern. Truncal hypotonia, spasticity, dystonia and/or myoclonus, scoliosis, and dysphagia are also features. [disorders.eyes.arizona.edu]

Nonketotic Hyperglycinemia

The EEG changed to hypsarrhythmia during early or mid-infancy. [indiana.pure.elsevier.com]

This is the first case with Cri du chat syndrome associated with nonketotic hyperglycinemia, infantile spasms, hypsarrhythmia, and heterotopia. [ncbi.nlm.nih.gov]

[…] on diffuse slow background activity constituted the usual abnormality during wakefulness, but more severe disorganization of the EEG occurred in sleep with emergence of hypsarrhythmia [indiana.pure.elsevier.com]

Autosomal Recessive Primary Microcephaly Type 10

[…] atrophy Patent ductus arteriosus Thin vermilion border Epileptic spasms Cortical gyral simplification Global brain atrophy Generalized seizures Epileptic encephalopathy Hypsarrhythmia [mendelian.co]

Hypoplasia of the corpus callosum Arrhythmia Pneumonia Hypoplasia of the brainstem Apnea Tetraplegia Respiratory insufficiency Focal seizures Generalized myoclonic seizures Hypsarrhythmia [mendelian.co]

Joint Dislocation

Atypical or asymmetric hypsarrhythmia therefore may be associated with poor seizure control ( Kalra et al., 2002 ). [frontiersin.org]

Multiple variations of this pattern are possible, including focal or asymmetric hypsarrhythmia. [patient.info]

Being female, late onset, and early control of the hypsarrhythmia, were factors of a good prognosis. [analesdepediatria.org]

Neonatal Diabetes Mellitus

As a new entity DEND-syndrome (developmental delay-epilepsy-neonatal diabetes) has been described, in which generalized epilepsy is most common and hypsarrhythmia has been [thieme-connect.com]

Infantile spasms with hypsarrhythmia on the electroencephalogram were severe and refractory to steroids. [ncbi.nlm.nih.gov]

The clinical features are extremely similar to the three cases described in the initial paper. 1 Hypsarrhythmia has been described before 1 and there is striking similarity [nature.com]

Aicardi's Syndrome

The combination of infantile spasms and an EEG pattern called ‘hypsarrhythmia’ is known as West syndrome. [epilepsy.org.uk]

An electroencephalogram (EEG) may show the characteristic pattern of hypsarrhythmia - sometimes called West's syndrome. [ 8 ] MRI scan or CT scan of the head will help demonstrate [patient.info]

Background West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram ( EEG ) pattern termed hypsarrhythmia, [emedicine.medscape.com]

Developmental and Epileptic Encephalopathy Type 6

(2) asymmetric hypsarrhythmia, (3) hypsarrhythmia with episodes of voltage attenuation, (4) hypsarrhythmia with a consistent focus of epileptic discharges or focal slowing [intechopen.com]

The EEG often evolves to atypical hypsarrhythmia which is transient or multifocal spike and sharp waves 3-4 months after the onset of the disease. [hindawi.com]

EEG findings may be consistent with hypsarrhythmia or burst-suppression patterns. [ggc.org]

CDG syndrome (Hypsarrhythmia) ALG3-CDG syndrome (Hypsarrhythmia) Abnormal Heart Murmurs (heart murmur) Acute Porphyrias (heart ... ... [familydiagnosis.com]

Source: NHS Choices UK 10 Causes List for Arrhythmias Some of the possible causes of Arrhythmias or similar disorders may include: 11 ALG13-CDG syndrome (Hypsarrhythmia) ALG2 [familydiagnosis.com]

Disorder of Peroxisomal Alpha- Beta- and Omega-Oxidation

Phosphoribosyl pyrophosphate synthetase Prevalence : unknown; Laboratory proﬁle: Increased urinary orotate, hypouricemia; Clinical features: Developmental disabilities, seizures with hypsarrhythmia [extern.synlab.com]

Blitz-Nick-Salaam Attacks

Infantile spasms are seizures characterized by sudden flexion of the arms, forward flexion of the trunk, extension of the legs, and hypsarrhythmia on electroencephalography [msdmanuals.com]

Zurück zum Zitat Fattinger S, Schmitt B, Bölsterli B et al (2013) Impaired slow wave downscaling in infantile spasms with hypsarrhythmia. [springermedizin.de]

Typically, the interictal pattern is hypsarrhythmia (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal spike discharges). [msdmanuals.com]

Developmental and Epileptic Encephalopathy Type 23

Developmental and Epileptic Encephalopathy Type 19

About 75% of all patients with EIEE develop West syndrome, which is characterized by BNS seizures with hypsarrhythmia in the EEG and a developmental arrest. [medizinische-genetik.de]

The EEG often evolves to atypical hypsarrhythmia which is transient or multifocal spike and sharp waves 3-4 months after the onset of the disease. [hindawi.com]

EEG findings may be consistent with hypsarrhythmia or burst-suppression patterns. [ggc.org]

Congenital Disorder of Glycosylation Type 2M

Developmental and Epileptic Encephalopathy Type 15