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]

Kohlschütter-Tönz Syndrome

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]

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]

West Syndrome

We report on a 3-year-old girl with West syndrome and with focal hypsarrhythmia. [ncbi.nlm.nih.gov]

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

OBJECTIVE: Fast oscillations (FOs) were first explored from scalp electroencephalographic (EEG) data from hypsarrhythmia in West syndrome (infantile spasms) to investigate [ncbi.nlm.nih.gov]

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 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]

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]

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]

Developmental and Epileptic Encephalopathy Type 3

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]

All three cases with NKH survived with increasing microcephaly, muscle tonicity; all developed infantile spasm with hypsarrhythmia on EEGs. [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]

Nonketotic Hyperglycinemia

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

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

At three months of age he developed flexor spasms and hypsarrhythmia. [ncbi.nlm.nih.gov]

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]

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]

Benign Familial Infantile Epilepsy Type 4

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

mental retardation Hypsarrhythmia chaotic background with high amplitude asynchronous slow waves and spikes not always present (e.g. [emilytam.com]

Patients may progress to West syndrome, which is characterized by tonic spasms with clustering, arrest of psychomotor development, and hypsarrhythmia on EEG. [genecards.org]

Progressive Myoclonic Epilepsy Type 7

After several months, and always within the first year of life, the seizures may change to infantile spasms and the EEG shows a pattern called hypsarrhythmia. [epilepsy.org.uk]

[…] burst suppression and evolves to intermittent high amplitude spike and wave complexes West Syndrome Clinical features: Infantile spasms Arrest of psychomotor development Hypsarrhythmia [neurodiffdx.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]

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]

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]

While EIEE is diagnosed by a suppression-burst pattern, West syndrome is typically accompanied by hypsarrhythmia, and when Lennox-Gastaut develops, a slow spike-wave pattern [news-medical.net]

Vitiligo

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

The diagnosis is made if the infant or child has at least two of the three symptoms of hypsarrhythmia, mental retardation, or infantile seizures. [medicinenet.com]

The EEG shows a very disorganised pattern, called hypsarrhythmia. [epilepsy.org.uk]

Uncinate Seizure

Hormones, Huntingtons Chorea (Disease), Hydantoins, Hydrocephalus, Hyperekplexia (Pathological Startle Response), Hyperglycemia, Hyperventilation, Hyponatremia, Hypoxia, Hypsarrhythmia [springerpub.com]

Most sz disappear by 5 yo, some develop adult seizures (Lennox-Gaustalt) Hypsarrhythmia: continous large bilateral slow waves w/ high voltage 0-3 ACTH (Prednisone, Vigabatrin [learnneurosurgery.com]

Spasms, Infantile An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. [ncbi.nlm.nih.gov]

Granulomatosis with Polyangiitis

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]

Benign Adult Familial Myoclonic Epilepsy

[…] pursuit Spastic tetraplegia Hyporeflexia Agraphesthesia Infantile spasms Resting tremor Slurred speech Drooling Apraxia Parkinsonism Postural instability Cerebellar atrophy Hypsarrhythmia [mendelian.co]

Infants have epileptic (infantile) spasms, developmental regression around 4-6 months of age, and the EEG shows hypsarrhythmia pattern. [epilepsyfoundationmn.org]

Stroke

In the presence of epileptic spasms or generalized seizure types, the background may show widespread slowing or hypsarrhythmia. [epilepsydiagnosis.org]

Hypsarrhythmia can be seen in those with epileptic spasms. Activation EEG abnormality is enhanced by sleep deprivation and in sleep. [epilepsydiagnosis.org]

Aicardi's Syndrome

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

Commonly described electroencephalogram (EEG) abnormalities include asymmetric hypsarrhythmia and bilateral independent bursts of high amplitude sharp and slow waves admixed [raredisorders.imedpub.com]

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]

Myoclonic-Astatic Epilepsy

Infantile spasms (West syndrome) West syndrome usually occurs in the first year of life and consists of the triad of infantile spasms, developmental deterioration, and a hypsarrhythmia [emedicine.medscape.com]

The origin of hypsarrhythmia and tonic spasms in West syndrome: evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia. [malattierare.regione.veneto.it]

West Syndrome Edit This is a triad of developmental delay, seizures termed infantile spasms, and EEG demonstrating a pattern termed hypsarrhythmia. [house.wikia.com]

Epilepsy with Generalized Tonic-Clonic Seizures

Although epileptic spasms have a semiology that is similar to GTS, spasms are more rapid in onset (lasting for 1 to 2 seconds), occur in clusters, and have hypsarrhythmia [neupsykey.com]

Infantile spasms have a hypsarrhythmia pattern on EEG which has an asymmetric disorganized mixture of spikes and slow waves. 9. [hawaii.edu]

EEG shows hypsarrhythmia with bilateral spikes, most prominent over the left parietal region. MRI shows a left parietal dysplasia. [onlinelibrary.wiley.com]

Haddad Syndrome

The electrodecremental response and hypsarrhythmia each reflect diffuse or multifocal/system dysfunction. [onlinelibrary.wiley.com]

West Syndrome West syndrome (WS) is an epileptic encephalopathy characterized by infantile spasms and hypsarrhythmia, and often by psychomotor regression including autistic [onlinelibrary.wiley.com]

It is not yet clear whether different mechanisms are responsible for hypsarrhythmia and infantile spasms (IS) and whether the psychomotor regression is a direct consequence [onlinelibrary.wiley.com]

Disorder of Manganese Transport

Via whole-exome sequencing, we studied a child that presented with cranial asymmetry, severe infantile spasms with hypsarrhythmia, and dysproportionate dwarfism. [uniprot.org]

Monosomy 1p36 Syndrome

0000238 Hypertelorism Wide-set eyes Widely spaced eyes [ more ] 0000316 Hypoplasia of the corpus callosum Underdevelopment of part of brain called corpus callosum 0002079 Hypsarrhythmia [rarediseases.info.nih.gov]

Almost 20% of all persons with the disorder have infantile spasms associated with hypsarrhythmia on EEG. [ncbi.nlm.nih.gov]

[…] the heart (auscultation, electrocardiogram, echocardiography) in infancy Waking/sleeping video-EEG-polygraphic studies (mainly in infancy) to detect infantile spasms with hypsarrhythmia [ncbi.nlm.nih.gov]

Ganglioglioma

Interictal electroencephalography (EEG) showed diffuse bursts of slightly irregular high-voltage spikes and slow waves without hypsarrhythmia. [ncbi.nlm.nih.gov]

Infantile spasms developed at age 6 months, an electroencephalogram revealing simultaneous hypsarrhythmia. [nature.com]