Subsequently, non-epileptic erratic myoclonus, various partial seizures and flexor spasms were observed. [ncbi.nlm.nih.gov]

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]