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38 Possible Causes for Generalized Polyspikes, Hypomyelination, Polyspike-and-Slow-Waves

  • West Syndrome

    All patients had striking hypomyelination of cerebrum, reduced volume of white matter and cortical atrophy on MRI.[ncbi.nlm.nih.gov] Interictal The background EEG is often highly disorganized with high voltage irregular slow waves intermixed with multifocal spikes and polyspikes, this is termed 'hypsarrhythmia[epilepsydiagnosis.org] The generalized tonic seizures began with bi-frontal polyspikes on EEG. Our diagnosis was lissencephaly presenting with West syndrome and generalized tonic seizures.[ncbi.nlm.nih.gov]

  • Generalized Clonic or Tonic-Clonic Seizures

    Neonatal onset Apraxia Postural instability Cerebral hypomyelination Olivopontocerebellar atrophy Pruritus Muscle fibrillation Headache Amaurosis fugax Involuntary movements[mendelian.co] […] spike-and-wave, or generalized polyspike-and-wave complexes.[jstage.jst.go.jp] . • Ictal EEG consists of “Fast activity” (10 Hz or more) and slow waves, with an occasional spike-and-wave pattern. • The EEG interictal expression is of “spike-and-wave[medlink.com]

  • Juvenile Myoclonic Epilepsy

    Gruss and Rafal Ploski, Developmental epileptic encephalopathy with hypomyelination and brain atrophy associated with PTPN23 variants affecting the assembly of UsnRNPs, European[doi.org] Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes.[ncbi.nlm.nih.gov] slow wave discharges.[en.wikipedia.org]

  • Epilepsy

    […] in ACTL6B, coding for a subunit of the neuron-specific chromatin remodeling complex nBAF, cause early onset severe developmental and epileptic encephalopathy with brain hypomyelination[doi.org] Generalized polyspike-slow wave in EEG was common to see in myoclonic seizure and can help to make the classification.[ncbi.nlm.nih.gov] Gruss and Rafal Ploski, Developmental epileptic encephalopathy with hypomyelination and brain atrophy associated with PTPN23 variants affecting the assembly of UsnRNPs, European[doi.org]

  • Myoclonic Jerking

    FOLR1 Cerebral folate deficiency FOXRED1 Leigh syndrome, Mitochondrial complex I deficiency GALC Krabbe disease GFAP Alexander disease GJC2 Leukodystrophy, hypomyelinating[genda.com.ar] “Electrographically they were time locked with generalized polyspikes on the EEG record,” wrote the authors.[neurologyadvisor.com] Ictal EEG : The myoclonic component is associated with a generalized spike or polyspike. The atonic component is associated with the aftergoing high voltage slow wave.[medlink.com]

  • Familial Progressive Myoclonic Epilepsy

    FOLR1 Cerebral folate deficiency FOXRED1 Leigh syndrome, Mitochondrial complex I deficiency GALC Krabbe disease GFAP Alexander disease GJC2 Leukodystrophy, hypomyelinating[genda.com.ar] EEG showed multiple spike and polyspike-slow wave generalised discharges enhanced by photic stimulation.[jmg.bmj.com] Lafora Body Disease  The characteristics of LBD include:  generalized tonic–clonic seizures (GTCS),  resting and action myoclonus,  ataxia,  dementia,  polyspike and[slideshare.net]

  • Progressive Myoclonic Epilepsy Type 8

    GJC2 Leukodystrophy hypomyelinating type 3 AIMP1 Leukodystrophy hypomyelinating type 4 HSPD1 Leukodystrophy hypomyelinating type 5 FAM126A Leukodystrophy hypomyelinating[centogene.com] Waking electroencephalogram in a 24-year-old woman showing generalized polyspike-and-wave discharges on a slow background ( left panel ) with occasional focal occipital discharges[neupsykey.com] […] fast spikes, polyspikes and polyspike‐and‐wave discharges Slow background activity, spike‐ and polyspike‐and‐wave discharges and multifocal spikes activated by non‐REM sleep[onlinelibrary.wiley.com]

  • Menkes Disease

    Early cerebellar atrophy and hypomyelination were evident on magnetic resonance imaging at 5 weeks of age.[ncbi.nlm.nih.gov] Tracings were considered abnormal if any of the following findings were present: background slowing or disorganization, spike waves or polyspikes, or diffuse spike-and-slow-wave[doi.org] EEG showed gross generalized polyspike waves. Opthalmoscopic examination showed pale optic disc.[casesjournal.biomedcentral.com]

  • Progressive Myoclonic Epilepsy Type 3

    GJC2 Leukodystrophy hypomyelinating type 3 AIMP1 Leukodystrophy hypomyelinating type 4 HSPD1 Leukodystrophy hypomyelinating type 5 FAM126A Leukodystrophy hypomyelinating[centogene.com] The EEG is characterized by background slowing and generalized polyspikes, which may be more prominent in the posterior head regions. (11,16).[path.upmc.edu] The EEG is characterized by slowing of background activity and the presence of generalized spike-wave and polyspike-wave complexes and bursts of slow waves. 5 High-voltage[clinicalgate.com]

  • Familial Infantile Myoclonic Epilepsy

    PubMed PubMedCentral Google Scholar Saitsu H, Tohyama J, Kumada T et al (2010) Dominant-negative mutations in alpha-II spectrin cause West syndrome with severe cerebral hypomyelination[link.springer.com] […] background Irregular generalized spike or polyspike and slow wave complexes Photosensitive, decreased during sleep Prognosis: Slow progression, may live to 60s Lafora Disease[emilytam.com] Ictal EEG Generalized polyspike or spike and slow-wave discharges.[epilepsy.com]

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