Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We analyzed electroclinical seizures observed by long video split-screen recording in 21 patients with Lennox-Gastaut syndrome (LGS). All patients had atypical absence seizures, 18 (81%) had tonic seizures, and 4 (21%) had myoclonic-atonic seizures. Tonic seizures were axial with flexion or extension of the head or trunk, or global with generalized tonic spasm mimicking infantile spasm, or involved the eyeballs only (either brief, with upward deviation of eyeballs or long, with oscillatory nystagmus). EEG showed either a bilateral 10-13-Hz rhythm or generalized synchronous spike wave at 3 Hz. Myoclonic-atonic seizures involving limbs, trunk, or neck were either brief or massive; the discharges were 2-3.5-Hz spike wave. Atypical absence seizures evolved gradually, terminated abruptly, and manifested alone or with subtle motor activity or oral automatism. EEG discharges were variable and of different types: (a) Diffuse irregular spike wave at 2-2.5 Hz with or without fragmentation (consciousness was regained during fragmentation or when spike wave discharges were < 2 Hz), (b) irregular diffuse fast activity at 10-13 Hz, or (c) a combination of fast spike wave or sharp waves of increasing amplitude followed by synchronous spike wave discharges at 3 Hz.
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PMID:Electroclinical seizures in Lennox-Gastaut syndrome. 842 43

Epileptic spasms represent a subcategory of motor seizures that have been extensively documented and recently re-classified by the International League Against Epilepsy as either generalized, focal or of unknown onset. Atypical characteristics continue to be reported in case studies, emphasizing the divergent morphological traits and putting into question the underlying aetiopathophysiology. Here, we report the findings of an infant with a triad of clinical manifestations during a single ictal event, comprising a cluster of epileptic spasms, vertical binocular nystagmus, and a focal tonic seizure. A video recording is presented that enabled clinical data to be correlated with EEG modifications. To date, a focal lesion has not been identified on brain imaging. The co-occurrence of these ictal paroxysms provides insight into the anatomical localization of seizure onset and complex epileptic networks involved, and challenges the pathophysiological hypothesis for epileptic spasms, implicating cortical-subcortical dysfunction and the implication of structures deep within the sulcus. Furthermore, the focal components both clinically and electrographically implicate involvement of the frontal eye field in the generation of vertical ictal nystagmus. [Published with video sequence on www.epilepticdisorders.com].
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PMID:A triad of infantile spasms, nystagmus and a focal tonic seizure. 3006 26