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10 Possible Causes for 3-4 Hz Spikes, Generalized Spike-and-Slow-Waves, Mild Cerebellar Hypoplasia

  • Benign Adult Familial Myoclonic Epilepsy

    , high-amplitude spike and slow-wave complexes 3.5 Hz, typically 4 s duration.  Absence seizures in JAE are more sporadic.  EEG is slightly faster with generalized spike[slideshare.net] hypoplasia-hydranencephaly syndrome Multiple acyl-CoA dehydrogenase deficiency Multiple acyl-CoA dehydrogenase deficiency, mild type Multiple acyl-CoA dehydrogenase deficiency[orpha.net] Childhood absence epilepsy with tonic-clonic seizures and electroencephalogram 3-4-Hz spike and multispike-slow wave complexes: linkage to chromosome 8q24.[reference.medscape.com]

  • West Syndrome

    Lennox-Gastaut Syndrome: LGS is characterized by multiple types of seizures and an abnormal EEG with generalized slow spikewave discharges.[cdkl5.com] Magnetic resonance imaging of the brain revealed mild cerebral and cerebellar atrophy, high-signal-intensity areas in the white matter, and hypoplasia of the corpus callosum[ncbi.nlm.nih.gov] Interictal EEG displays diffuse and asynchronous high voltage spikes and slow waves suggesting the hypsarrhythmic pattern. B.[bmcneurol.biomedcentral.com]

  • Unverricht-Lundborg Syndrome

    Slow ( 2.5 Hz) electroencephalographic spike and wave discharges in atypical absence epilepsy (ie, Lennox-Gastaut syndrome). Spike, generalized.[emedicine.medscape.com] hypoplasia-hydranencephaly syndrome Multiple acyl-CoA dehydrogenase deficiency Multiple acyl-CoA dehydrogenase deficiency, mild type Multiple acyl-CoA dehydrogenase deficiency[orpha.net] .  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]

  • Neuronal Ceroid Lipofuscinosis

    EEG revealed frequent generalized spikes, polyspikes, and waves, prominent on awakening and during photic stimulation without significant change throughout the clinical course[ncbi.nlm.nih.gov] cerebellar ataxia and white matter edema Leukonychia totalis Leukotriene C4 synthase deficiency Levine-Critchley syndrome Levy-Hollister syndrome Lewandowsky-Lutz syndrome[csbg.cnb.csic.es] Photic stimulation at 1 to 3 Hz elicited discrete spike and wave discharges in the electroencephalogram, which were diminished at a higher frequency of stimulation.[ncbi.nlm.nih.gov]

  • Angelman Syndrome

    EEG demonstrates high-amplitude 2-3Hz delta activity with spike and slow-wave discharges and sleep-activated generalized epileptiform discharges.[ncbi.nlm.nih.gov] Cerebellar hypoplasia Seizures Pathophysiology [ edit ] Adenylosuccinate lyase deficiency is responsible for a range of symptoms that involve psychomotor retardation, often[en.wikipedia.org] High amplitude rhythmic 4-6 Hz activity, prominent in the occipital regions, with spikes, which can be facilitated by eye closure, is often seen in children under the age[ncbi.nlm.nih.gov]

  • Adult-Onset Autosomal Recessive Cerebellar Ataxia

    There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com] […] ataxia and mental retardation known as the disequilibrium syndrome. 36, 45 Imaging shows significant cerebellar hypoplasia and mild cerebral cortex thickening and gyral simplification[ajnr.org] There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com]

  • Autosomal Recessive Spinocerebellar Ataxia 8

    There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com] cerebellar hypoplasia or vermian aplasia • Joubert syndrome – absence of cerebellar vermis and “molar tooth sign” at cerebellar & mid brain junction • Fragile X associated[slideshare.net] There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com]

  • Adenylosuccinate Lyase Deficiency

    Electroencephalogram (EEG) of case 1 had revealed high-amplitude (200-300 μV), generalized 2-4 Hz spike-wave complexes with slow background rhythm of delta range [Figure 1[pediatricneurosciences.com] Cerebellar hypoplasia Seizures Pathophysiology [ edit ] Adenylosuccinate lyase deficiency is responsible for a range of symptoms that involve psychomotor retardation, often[en.wikipedia.org] In childhood, the EEG showed high voltage 4-6 Hz activity and in adulthood it showed rhythmic triphasic 2-3 Hz activity of high amplitude (200-500 mV), mixed with spikes or[pediatricneurosciences.com]

  • Pachygyria-Mental Retardation-Seizures

    […] hemispheres being pronounced at the posterior region, generalized spike-slow-waves, and synchronized spike and sharp wave discharges at the posterior of the right hemisphere[dusunenadamdergisi.org] Autosomal recessive LIS with cerebellar hypoplasia consists of mild frontal predominant LIS, plus severe hippocampal and cerebellar hypoplasia and dysplasia.[karger.com] In Patients 1, 3, and 4, seizures characterized by generalized rhythmic bilat- eral synchronous spike and polyspike/wave discharges at a frequency of 4 to 12 cycles/sec were[docslide.com.br]

  • Autosomal Recessive Spinocerebellar Ataxia 3

    There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com] cerebellar hypoplasia or vermian aplasia • Joubert syndrome – absence of cerebellar vermis and “molar tooth sign” at cerebellar & mid brain junction • Fragile X associated[slideshare.net] […] of cerebellum and corpus callosum [ 63 , 64 ] AOA3 (1 family) PIK3R5 615217 Oculomotor apraxia, sensorimotor involvement, teenage onset, cerebellar atrophy [ 65 ] SCAR13[cerebellumandataxias.biomedcentral.com]

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