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21 Possible Causes for Central Focal Spikes, Hypomyelination, Polyspike-and-Slow-Waves

  • West Syndrome

    All patients had striking hypomyelination of cerebrum, reduced volume of white matter and cortical atrophy on MRI.[] Interictal The background EEG is often highly disorganized with high voltage irregular slow waves intermixed with multifocal spikes and polyspikes, this is termed 'hypsarrhythmia[] Brain magnetic resonance imaging showed diffusely reduced white matter and brainstem volumes with hypomyelination.[]

  • 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[] In infancy, the EEG background was normal (1), showed diffuse (3) or focal slowing that was temporal (1) and in the left hemisphere (1), and showed focal (central, temporal[] Generalized polyspike-slow wave in EEG was common to see in myoclonic seizure and can help to make the classification.[]

  • Generalized Clonic or Tonic-Clonic Seizures

    Neonatal onset Apraxia Postural instability Cerebral hypomyelination Olivopontocerebellar atrophy Pruritus Muscle fibrillation Headache Amaurosis fugax Involuntary movements[] . • 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[] Hypomimic face Action tremor Agraphesthesia Intellectual disability, severe Bradykinesia Arrhythmia Cerebral cortical atrophy Absent speech Dyskinesia Hypsarrhythmia CNS hypomyelination[]

  • 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[] slow wave discharges.[] The EEG typically shows a characteristic pattern with generalized 4–6 Hz polyspike and slow wave discharges.[]

  • Familial Progressive Myoclonic Epilepsy

    FOLR1 Cerebral folate deficiency FOXRED1 Leigh syndrome, Mitochondrial complex I deficiency GALC Krabbe disease GFAP Alexander disease GJC2 Leukodystrophy, hypomyelinating[] The cortical site of origin of the spike discharges and the site of origin of the associated focal myoclonic seizures do not necessarily coincide. 22 Pathophysiology The term[] EEG showed multiple spike and polyspike-slow wave generalised discharges enhanced by photic stimulation.[]

  • Progressive Myoclonic Epilepsy Type 8

    GJC2 Leukodystrophy hypomyelinating type 3 AIMP1 Leukodystrophy hypomyelinating type 4 HSPD1 Leukodystrophy hypomyelinating type 5 FAM126A Leukodystrophy hypomyelinating[] , multifocal, or bilateral distribution and time locked to sharp-wave or spike-wave discharges on the contralateral central areas. 34, 35 ENM has a wide etiological spectrum[] […] 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[]

  • Benign Familial Infantile Epilepsy Type 4

    […] hyperintensities X 7% CDKL5 Atypical Rett syndrome, normal early interictal EEG XD SLC25A22 Cerebellar hypoplasia, CC malformation AR STXBP1 AD 6% SPTAN1 Cerebral atrophy, hypomyelination[] Benign childhood epilepsy with centrotemporal spikes (BECTS) is a type of focal idiopathic epilepsy more common in childhood.[] […] background Irregular generalized spike or polyspike and slow wave complexes Photosensitive, decreased during sleep Prognosis: Slow progression, may live to 60s Lafora Disease[]

  • Early Infantile Epileptic Encephalopathy Type 2

    A disorder (OMIM:612164) characterised by neonatal or infantile onset of seizures, suppression-burst pattern on EEG, profound mental retardation, and MRI evidence of hypomyelination[] In infancy, the EEG background was normal (1), showed diffuse (3) or focal slowing that was temporal (1) and in the left hemisphere (1), and showed focal (central, temporal[] […] paroxysms of polyspikes or spikes-slow waves.  Ictal EEG  Varies according to seizure type. 16.[]

  • Temporal Lobe Epilepsy

    Jun Tohyama, Noriyuki Akasaka, Hitoshi Osaka, Yoshihiro Maegaki, Mitsuhiro Kato, Naka Saito, Sumimasa Yamashita and Kousaku Ohno, Early onset West syndrome with cerebral hypomyelination[] IQ lower than 70, previous surgery for epilepsy, focal extratemporal spikes or slowing on scalp-recorded EEG, or evidence on MRI of extratemporal lesions capable of producing[] […] complex, polyspikes, runs of rapid spikes, sharp waves, sharp-and-slow-wave complex, slow sharp waves, spikes and spike and slow waves.[]

  • Generalized Epilepsy with Febrile Seizures Plus

    […] gamma 1, 45kDa 17q21.31 Cystic Fibrosis GJC2 gap junction protein, gamma 2, 47kDa 1q42.13 Spastic Paraplegia 44, Autosomal Recessive, Spastic Paraplegia 44, Leukodystrophy, Hypomyelinating[] In infancy, the EEG background was normal (1), showed diffuse (3) or focal slowing that was temporal (1) and in the left hemisphere (1), and showed focal (central, temporal[] EEG recordings showed a variety of changes: generalized spike wave and polyspike wave activity, focal discharges, focal or diffuse slowing or were normal.[]

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