Both patients had early infantile seizures characterized by fragmentary myoclonic jerks associated with burst-suppression pattern on electroencephalography. [ncbi.nlm.nih.gov]

jerks, partial fits and periodic paroxysmal EEG abnormalities. [semanticscholar.org]

Throughout his infancy, he had small myoclonic jerks and is severely impaired. Two hundred and fourteen infants with epileptic seizures were tested. The researchers looked at 109 genes known or suspected to cause seizures. [seizuresinfant.com]

Early infantile epileptic encephalopathy, ferro-cerebro-cutaneous syndrome 26993267,24259288,24706016 PLCB1 Epilepsy with migrating focal seizures in infancy (EMFSI); epileptic encephalopathy; AR inheritance, deletions reported 22690784,26818157 PNKP Ataxia [tests.labmed.washington.edu]

[…] apraxia; MCSZ – Microcephaly seizures and developmental delay; GEFSP – Generalized epilepsy with febrile seizures plus; CIAT – Cognitive impairment with or without cerebellar ataxia; SFN - Small fiber neuropathy; CIP – Congenital insensitivity to pain [centogene.com]

AR 34 23 PNPO Pyridoxamine 5'-phosphate oxidase deficiency AR 15 31 POLG POLG-related ataxia neuropathy spectrum disorders, Sensory ataxia, dysarthria, and ophthalmoparesis, Alpers syndrome, Progressive external ophthalmoplegia with mitochondrial DNA [blueprintgenetics.com]

Ataxia developed in 3, and 3 had profound retardation with hypotonia or dyskinetic movements. [ 1 ] COMMENT. [pediatricneurologybriefs.com]

4 Klinik für Kinder- und Jugendmedizin, Universitätsmedizin Göttingen, Deutschland Background/Purpose: Leukoencephalopathy with VWM is a neurodegenerative disorder mostly beginning during the first years of life with abnormalities of muscle tone and ataxia [thieme-connect.com]

Tonic seizure started clinically with a scream (vertical arrow) and episodic nystagmus (oblique arrows shows EM artefacts). [slideshare.net]

ataxia; SFN - Small fiber neuropathy; CIP – Congenital insensitivity to pain; PEPD – Paroxysmal extreme pain disorder; EIG – Epilepsy idiopathic generalized; MRT – Mental retardation autosomal recessive; FIME – Familial infantile myoclonic epilepsy; [centogene.com]

At five months, inter-ictal EEG revealed hypsarrhythmia, but his attacks were still only myoclonuses. ACTH treatment was effective and the myoclonus frequency markedly decreased. [ncbi.nlm.nih.gov]

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 and Demographics Early myoclonic encephalopathy (EME) is a rare disease with [wikidoc.org]

 EEG - suppression–burst activity, which evolves into hypsarrhythmia,accentuated during sleep,persists for prolonged periods.  Inborn errors of metabolism mc causes.  50% die within weeks or months. 6. [slideshare.net]

Their persistence in our patients represents an anatomic condition for cortical disconnection providing a pathophysiologic basis to burst-suppression phenomena. [ncbi.nlm.nih.gov]

Later; a hypsarhythmic pattern may appear; but it is replaced again by burst suppression. [brainscape.com]

The EEG pattern often shows a suppression-burst pattern with high-voltage bursts of slow waves mixed with multifocal spikes alternating with isoelectric suppression phases ({2:Molinari et al., 2005}). [diseaseinfosearch.org]

West Syndrome (WS) aka as infantile spasms with hypsarrhythmia, EEG abnormality with asynchronous very high amplitude, irregular, continuous multifocal spike an slow wave discharges. [ijponline.biomedcentral.com]

multifocal spike- and sharp-wave discharges, repetitive paroxysmal activity, unusually low-voltage fast activity, and findings consistent with complex partial seizures [7]. [graphyonline.com]

West Syndrome (WS) aka as infantile spasms with hypsarrhythmia, EEG abnormality with asynchronous very high amplitude, irregular, continuous multifocal spike an slow wave discharges. [ijponline.biomedcentral.com]

[…] or slow waves mainly in the rolandic regions (but may also occur elsewhere) Early myoclonic encephalopathy - Ictal EEG The myoclonia do not have an ictal EEG expression and may follow bursts Early infantile epileptic encephalopathy with suppression burst [brainscape.com]

 Inter-Ictal EEG  Initial -20% show normal BG with photoparoxysmal discharges of spikes/ polyspikes-slow waves.  Within 1 year, EEG - abnormal slow BG with frequent asymmetrical paroxysms of polyspikes or spikes-slow waves.  Ictal EEG  Varies according [slideshare.net]

Mutations in STXBP1 occur in patients with Ohtahara and West syndromes and are also present in 10% of patients with undefined early-onset epileptic encephalopathies without the burst-suppression EEG, typical of Ohtahara syndrome. [pediatricneurologybriefs.com]

This is an example of a burst suppression EEG. Each of the letters correspond to a burst of high voltage across the brain and a myoclonic jerk. [seizuresinfant.com]