Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this report 52 patients meeting the criteria of centrencephalic myoclonic-astatic petit mal (10) at the beginning of petit mal are included. The results of clinical and encephalographic follow-up examinations are as follows: 1) The type reported here apparently has a petit mal course with peculiar characteristics, it therefore must be separated from Lennox syndrome: centrencephalic myoclonicastatic petit mal, pyknolepsia, bilateral myoclonus (impulsive petit mal). It should file under generalized primary petit mal epilepsy. 2) It is primarily defined by its EEG marker: "centrencephalic" EEG pattern (irregular and/or regular spike-wave groups, photosensibility and abnormal theta- and/or delta-rhythm). Rarely (33%) minor cerebral organic lesions as additional pathogenetic factors are uncovered by clinical and electroencephalographic examinations. 3) The clinical picture is characterized, aside from myoclonic and/or astatic seizures, by frequent absences (80%), rare tonic seizures (6%), petit mal status (25%) and mostly generalized grand mal seizures (62%). 4) There are changes of the course of the disease to Lennox syndrome (N = 6) in in children suffering from marked cerebral organic lesions at the onset of petit mal and in development of severe epilepsy. 5) Least favorite markers with respect to prognosis are concomitant grand mal seizures (p = 0,05), petit mal status (p = 0.008), additional 2/sec spike wave-pattern (spike wave Variant) in the EEG (p = 0.002) and previous seizures with focal signs. Favourite outcome of epilepsy are frequently connected to missing cerebral organic lesions (p = 0.05).
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PMID:[Centrencephalic myoclonic-astatic petit mal. Clinical and electroencephalographic long-term follow-up study in 52 patients (author's transl)]. 9 76

Sodium valproate (Epilim) has been used in the management of 100 patients with previously uncontrolled epilepsy for periods up to 2 years. If all manifestations of epilepsy are considered together, 75% to 100% control of seizures was achieved in 43% of patients, 25% to 74% control in 26%, and no improvement occurred in 31% of patients. Control of 75% to 100% was achieved in 57% of patients with a spike and wave electroencephalogram (EEG) disturbance but only in 35% of those with focal abnormalities, excessive slow activity, or normal records. When the various manifestations of epilepsy were considered individually, the greatest improvement was found among the patients with the minor forms of generalized epilepsy (petit mal absences, myoclonus and atonic attacks) in whom 75% to 100% control was obtained in 67%, compared with 43% of those with major generalized seizures (grand mal) and 30% of those with temporal lobe attacks and other forms of focal epilepsy. Gastrointestinal disturbances and drowsiness were noted as side effects in the early stages of treatment, but the majority of patients tolerated the drug well and many commented on increased mental alertness while taking it. Two patients were over-stimulated and some noticed tremor or twitching as side effects. Some minor abnormalities in blood coagulation studies were noted, but these were transient and did not appear to be of clinical significance. Regular blood counts and biochemical studies have not shown any significant changes. Sodium valproate appears to be a safe and useful anticonvulsant with the advantage that it usually makes patients brighter rather than drowsier. Abnormalities of platelet function have been described in some overseas reports, so that any unexplained bruising or bleeding in a patient taking valproate is an indication for a platelet count and coagulation studies.
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PMID:The anticonvulsant action of sodium valproate (Epilim) in 100 patients with various forms of epilepsy. 40 31

The therapeutic efficacy of orally administered clonazepam has been evaluated in 32 epileptic patients, with substantial improvement in 22. The drug is active in all types of seizures, particularly in myoclonus, petit mal absences, and partial complex epilepsy; it seems the drug of choice in generalized infantile organic epilepsy, although the treatment of these patients is still unsatisfactory. In some patients, the drug seemed less effective after months of therapy. Drowsiness is the main side effect.
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PMID:Treatment of epileptic seizures with clonazepam. A reappraisal. 80 77

Petit mal absences in childhood are often followed by grand mal seizures in adolescence and a coexistence of both types of seizures is not uncommon at all. It is noteworthy, however, that such a coexistence does not imply immediate transition from one type of seizure into the other one. Hence, a grand mal attack may be prepared by all sorts of focal seizures, psychomotor automatisms or bilateral-synchronous myoclonus but immediately preceding petit mal (with generalized spike-wave discharges) is probably extremely rare. The presented case is characterized by unusually late onset of petit mal and grand mal seizures at age 32 and, following a mild head injury, a few episodes of petit mal status (ictal stupor) at age 52. At that time, a series of petit mal absences and eventually a smooth transition from petit mal to grand mal could be recorded. Uncommon frequency characteristics (especially some interspersed spiking at a rate of 9-15/sec) were found at the onset of the ictal spike-wave activity during the petit mal absences. This is regarded as the expression of failing inhibitory mechanisms which generally prevent the immediate development of a grand mal out of a petit mal absence.
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PMID:Immediate transition from a petit mal absence into a grand mal seizure. Case report. 81 90

In 13 healthy adult cats chronically implanted with parasagittal electrodes applied to the dural surface, curarization was performed and baseline recordings of the visual evoked response (VER), auditory evoked response (AER), and brainstem auditory evoked response (BAER) were made. Following the procedure of Prince and Farrell (1969), the animals were then given intramuscular doses of 300,000 to 500,000 U/kg of penicillin with the subsequent development of diffuse, bilaterally symmetrical, photosensitive spike-and-wave discharges in the EEG from 1 to 1 1/2 hr later and concomitant facial myoclonus, arrest of movement, and "absence-like" staring in non-curarized animals. The VER, AER, and BAER were monitored at 15-min intervals for several hours during which time the VER consistently decreased in amplitude up to the time at which the first spike-and wave bursts could be elicited by photic stimulation, approximately 1 hr after injection, after which all early components (0-200 msec) of the VER were progressively increased from 150 to 300% until spontaneous spike-and-wave bursts were consistently recorded (1 1/2-2 hr). Coincident with this change, a marked increase in late components (200-500 msec) was also observed. Both th early diminution and later augmentation of the VER were equally observable in visual and nonvisual cortex. Changes in the AER were also recorded with the development of this model, and were similar to those of the VER but of a lesser degree. The amplitudes of waves I through V of the BAER were found to increase from 28 to 88% maximal at 1 1/2 hr following penicillin injection. These data and the similarity of this model to human petit mal epilepsy argue against increased inhibitory impulses to the visual system during the ictal discharge being responsible for the subjective loss of visual information during petit mal absence. If the amplitude of the evoked response is directly related to the functional integrity of a sensory system, this suggests that the impairment of sensory input, or absence, during spike-and-wave paroxysm is due to interference with sensory processing rostral to the brainstem ascending auditory pathway, and probably does not occur in primary sensory cortex but rather in cortical or subcortical association tracts.
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PMID:Visual and auditory evoked responses during penicillin-induced generalized spike-and-wave activity in cats. 82 23

Effects of acute and chronic paleocerebellar stimulation were evaluated in four experimental models of epilepsy in 24 adult cats chronically implanted with bilaterally symmetric parasagittal electrocorticographic electrodes and anterior lobe cerebellar stimulation electrodes. Pentylenetetrazol was given intraveneously in 50-mg increments or 4% enflurane was inspired until grand mal seizures occurred spontaneously or were triggered by photic or auditory stimuli. Alpha-chloralose, 50 mg/kg, was injected intraperitoneally to produce a model of stimulus-sensitive myoclonus and sodium penicillin G, 350,000 units/kg, was injected intramuscularly to produce a model of petit mal epilepsy. One- to 250-Hz electrical stimulation of paleocerebellar cortical surfaces was performed with constant-voltage or constant-current stimulators at threshold and suprathreshold intensities with average intensities of 8 V and 2.5 mA, respectively. Acute or chronic, threshold or suprathreshold paleocerebellar stimulation did not predictably alter the electrographic or clinical manifestations in any of these four models.
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PMID:Effects of acute and chronic paleocerebellar stimulation on experimental models of epilepsy in the cat: studies with enflurane, pentylenetetrazol, penicillin, and chloralose. 114 12

Eighteen women and five men had typical absences. These included 10% of a consecutive hospital series of 200 adult patients with epileptic disorders. The absences began between the ages of seven and 46 years and varied in type and severity. Twenty patients also had generalised tonic-clonic seizures, ranging in frequency from one in a lifetime to one per month. Myoclonic jerks of the limbs occurred in 11 patients but were not associated with the absence attacks. Eyelid myoclonus consistently occurred with absence attacks in four patients and perioral myoclonus in two patients. Absence status occurred in five patients. Absence seizures were frequently unrecognised or misdiagnosed as complex partial seizures. Satisfactory control was achieved with sodium valproate. Electroencephalography, particularly video-electroencephalography, was invaluable in the diagnosis, but focal abnormalities in seven patients might have been erroneously interpreted as indicating partial seizures. This series showed that clinical and EEG manifestations are often syndrome-related and that there are more epileptic syndromes with typical absences than those presently recognised.
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PMID:Typical absence seizures in adults: clinical, EEG, video-EEG findings and diagnostic/syndromic considerations. 146 93

Juvenile myoclonic epilepsy (JME), a common form of idiopathic generalized epilepsy, has a distinct clinical and electroencephalographic profile. Often JME is not recognized, with serious consequences on the sufferers. We examined factors contributing to the missed diagnosis even in an epilepsy clinic. Of 70 JME patients, 66 (91.4%) were not diagnosed on referral and 22 (33%) were not initially recognized in the epilepsy clinic. The correct diagnosis was established after a mean of 8.3 +/- 5.5 years from disease onset and an interval of 17.7 +/- 10.4 months from first evaluation in the epilepsy clinic. Myoclonic jerks, the hallmark of the disease, were not usually reported by patients. Similarly, relevant questioning may not be included in the history. Absence seizures antedating jerks by many years, myoclonic jerks reported as unilateral, generalized tonic-clonic seizures occurring during sleep and focal EEG abnormalities are other factors contributing to not recognizing JME. Our study reemphasizes the need to have not only a correct seizure diagnosis but also a correct epilepsy-disease diagnosis.
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PMID:Juvenile myoclonic epilepsy: factors of error involved in the diagnosis and treatment. 191 75

This is a comparative video-electroencephalographic (EEG) study of typical absence seizures in 4 epileptic syndromes. In 20 patients, 224 absences were recorded and analysed. Significant clinical and EEG differences were found in the seizure patterns of childhood absence epilepsy (CAE), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy with absences (JMEA) and myoclonic absence epilepsy (MAE). Clinically, CAE demonstrated more severe impairment of consciousness than JAE while, in JMEA, ictal manifestations were frequently mild and difficult to detect. In the latter, the adolescent patient usually continued his activity, was able to perform even mathematical calculations and often his speech was not disturbed. In children with JMEA, impairment of consciousness was more apparent and sometimes severe. Automatisms occurred in all 4 epileptic syndromes and were proportional to the severity of the demonstrated impairment of consciousness, being rare in JMEA but frequent in CAE and JAE. Expressive speech and overbreathing usually persisted for 1-2 s after the onset of the EEG ictal discharge in CAE. It was less disturbed in JAE where in some absence seizures, interrupted speech and overbreathing were restored during the ictus. A characteristic clinical manifestation of CAE was opening of the eyes in all absence seizures within 1.8 +/- 0.6 s (max. 2.5 s) from the onset of the EEG paroxysms. This early eye-opening behaviour was not observed in JMEA. In MAE, rhythmical myoclonic jerks at 3 Hz make the diagnosis unmistakable. Myoclonic jerks were extremely rare in the absences of JMEA, although all patients had independent myoclonic jerks on awakening. The ictal EEG discharge was longer in JAE (mean 16.3 +/- 7.1 s) than in CAE (12.4 +/- 2.1 s) or JMEA (6.6 +/- 4.2 s). The opening phase of the EEG paroxysms did not show significant differences in CAE, JAE and JMEA but significant changes were found in their initial and terminal ictal phases. In JMEA, the spike-multiple spike-slow wave complexes were not rhythmic and frequently demonstrated variable spike-slow wave relationships. Ictal discharge fragmentations and spike-wave discharges looking like compressed capital Ws were often seen and are characteristic of JMEA. seen and are characteristic of JMEA. Absence seizures demonstrated a more severe impairment of expressive rather than receptive speech, irrespective of differences between syndromes. Evoked as well as spontaneous automatisms occurred in the same patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Differentiation of typical absence seizures in epileptic syndromes. A video EEG study of 224 seizures in 20 patients. 250 85

Positron emission tomography (PET) with fluorine-18-labeled fluorodeoxyglucose (18FDG) has demonstrated the epileptogenic lesion in partial epilepsy to be hypometabolic interictally . This finding is useful for localizing the area of resection when surgical therapy is contemplated. 18FDG scans during partial seizures show increased metabolism in areas of ictal onset and spread and in other regions of decreased metabolism that could reflect postictal effects. In the generalized epilepsies, petit mal absences and generalized convulsions induced by electroconvulsive shock therapy (ECT) are associated with global hypermetabolism, while global hypometabolism is seen in the postictal period following ECT. More information about the factors that influence the interictal hypometabolic zone in partial epilepsy should improve the diagnostic value of this finding for presurgical localization and perhaps also for the evaluation of other therapeutic regimens. New techniques for more dynamic PET studies with improved resolution, combined with computerized electroencephalographic analysis, should allow more accurate interpretation of ictal, as well as interictal, phenomena. Application of PET technology to other paroxysmal disorders may provide a basis for new diagnostic classifications that have therapeutic and prognostic value and may allow clearer differentiation among epileptic phenomena, myoclonus, and movement disorders. More clinical and animal research is needed, however, before we can delineate fundamental mechanisms of human epilepsy from PET data. To this end, it is now possible to use combined multidisciplinary parallel approaches in patients and animals to define specific aspects of epileptic disorders clinically, to intensively investigate them with experimental models in the animal laboratory, and to verify the relevance of these experimental results by returning to clinical studies.
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PMID:The use of positron emission tomographic scanning in epilepsy. 643 Feb 15


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