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1,435 Possible Causes for Hypomyelination, Paroxysmal Activity - Spikes Temporal Left, 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] Brain magnetic resonance imaging showed diffusely reduced white matter and brainstem volumes with hypomyelination.[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] . • 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] Hypomimic face Action tremor Agraphesthesia Intellectual disability, severe Bradykinesia Arrhythmia Cerebral cortical atrophy Absent speech Dyskinesia Hypsarrhythmia CNS hypomyelination[mendelian.co]

  • 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]

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

  • Benign Adult Familial Myoclonic Epilepsy

    […] or polyspike and slow-wave complexes.  Variable course and outcome.  One half experience encephalopathic effects and suffer from persistent GTCS, myoclonic-atonic status[slideshare.net] Electrophysiological findings reveal giant SSEP and EEG shows general spikes or polyspikes and slow wave complexes. The gene is mapped at chromosome 8q24.[neuroweb.us] Bursts of generalized polyspikes and waves (multiple spike-and-slow-wave complexes) may also be seen, [30] especially during sleep and in syndromes with older age of onset[reference.medscape.com]

    Missing: Hypomyelination
  • Myoclonic-Astatic Epilepsy

    Ictal EEG Myoclonic and atonic seizures discharges of irregular spike-waves or polyspike-waves at 2.5 to 3 Hz or more.[epilepsy.com] At age four, a video-EEG-recording showed frequent head nodding (atonic seizures) and myoclonic astatic seizures associated with diffuse spikes or polyspikes and waves.[ncbi.nlm.nih.gov] Atonia is usually concurrent with the slow wave of a single- or polyspike-wave complex.[medlink.com]

    Missing: Hypomyelination
  • Idiopathic Generalized Epilepsy

    […] and waves with frontocentral predominance Interictal EEG : 4–6 Hz bilateral polyspike and slow wave Responds well to antiseizure drug therapy; seizures become less frequent[amboss.com] EEG findings About half of patients had an abnormal EEG viz. spikes, polyspikes and slow waves.[annalsofian.org] Generalized spike-and-wave or polyspike-and-wave is seen in the interictal EEG. Half of the patients have these abnormalities only in sleep. Caution.[medlink.com]

    Missing: Hypomyelination
  • Juvenile Absence Epilepsy

    The EEG has generalized spike or polyspike slow-wave complexes. JAE is a lifelong disorder, although seizures can be controlled in 70% to 80% of patients.[pennsw.com.au] […] and waves with frontocentral predominance Interictal EEG : 4–6 Hz bilateral polyspike and slow wave Responds well to antiseizure drug therapy; seizures become less frequent[amboss.com] […] variations between the spike/multispike-slow-wave relationships in generalized discharges of spike-polyspike-slow waves. (3) Predominantly brief discharges (shorter than[medlink.com]

    Missing: Hypomyelination
  • 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] .  EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum[slideshare.net]

  • Lennox-Gastaut Syndrome

    […] that are more generalized and more frequent, consisting of polyspikes and slow waves REM sleep – Decreased spike waves Ictal EEG may demonstrate the following characteristics[emedicine.medscape.com] Atonic, massive myoclonic, or myoclonic-atonic seizure – Slow spike waves, polyspike waves, or rapid diffuse rhythms Absence status epilepticus – Continuous spike wave discharges[emedicine.medscape.com] Interictal EEG may demonstrate the following characteristics: A slow background that can be constant or transient Awake – Diffuse slow spike wave Non-REM sleep – Discharges[emedicine.medscape.com]

    Missing: Hypomyelination

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