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Early Myoclonic Encephalopathy
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]
As infants develop, the EEG may evolve to a hypsarrhythmia pattern, which is the term for an EEG with multifocal high amplitude spikes with a very abnormal background.[epilepsy.com]
At three months of age the baby developed spasms with no hypsarrhythmia. Throughout his infancy, he had small myoclonic jerks and is severely impaired.[seizuresinfant.com]
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West Syndrome
West syn·drome ( west ), an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.[medical-dictionary.thefreedictionary.com]
Example of hypsarrhythmia CAUTION Hypsarrhythmia may not be present early after the onset of epileptic spasms. The EEG may need to be repeated.[epilepsydiagnosis.org]
Similarly, some children with infantile spasms never exhibit hypsarrhythmia.[infantilespasmsproject.org]
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Lennox-Gastaut Syndrome
There is also a tendency for psychosis to develop with time. 20% of all patients with Lennox-Gastaut syndrome have prior infantile spasms with hypsarrhythmia. [ 3 ] Although[patient.info]
This condition often evolves to West syndrome with infantile spasms and a chaotic EEG pattern (hypsarrhythmia) at 4-8 months of age.[childneurologyfoundation.org]
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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]
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Myoclonic Epilepsy of Infancy
In the vast majority, this pattern is hypsarrhythmia- a very chaotic looking EEG with high amplitude brain waves.[medhelp.org]
Other terms currently in usage are "infantile spasms," "hypsarrhythmia" and "massive myoclonic seizures." 2. Myoclonic epilepsy of older children.[pediatrics.aappublications.org]
Infants have epileptic (infantile) spasms, developmental regression around 4-6 months of age, and the EEG shows hypsarrhythmia pattern.[epilepsyfoundationmn.org]
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Neurofibromatosis Type 1
The EEG findings can vary from normal to focal or multifocal spike waves, spike and slow spike wave complexes at 2 Hz consistent with Lennox-Gastaut syndrome , and even a hypsarrhythmia[epilepsy.com]
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D-Glyceric Aciduria
EEG revealed multifocal generalized epileptic activity in a hypsarrhythmia pattern. Organic acid analysis (GC-MS) in urine revealed increased glyceric acid excretion.[wwe.geoscience.net]
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6-Pyruvoyl-Tetrahydropterin Synthase Deficiency
0002015 Excessive salivation Mouth watering Oversalivation Watery mouth [ more ] 0003781 Falls 0002527 Global developmental delay 0001263 Hyperkinesis Muscle spasms 0002487 Hypsarrhythmia[rarediseases.info.nih.gov]
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10q22.3q23.3 Microdeletion Syndrome
Prominent nose 10% Abnormality of the dentition 10% Hypospadias 10% Multifocal seizures 10% Hyperventilation 10% Poor eye contact 10% Full cheeks 10% Inability to walk 10% Hypsarrhythmia[mendelian.co]
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Miller Dieker Syndrome
Approximately 80 % have infantile spasms; however, the EEG does not show typical hypsarrhythmia in some patients [ 4 ].[jaclinicalreports.springeropen.com]
Electroencephalography indicated frequent multifocal independent spikes, sharp waves, and early features of evolving hypsarrhythmia.[ssajm.org]
She had an epileptic encephalopathy with infantile spasms (West syndrome) with a typical hypsarrhythmia pattern on her electroencephalogram (EEG).[jaclinicalreports.springeropen.com]