Nonconvulsive generalized status epilepticus

Guberman, A.; Cantu-Reyna, G.; Stuss, Donald T.; Broughton, Roger

doi:10.1212/wnl.36.10.1284

Cited by 135 publications

(70 citation statements)

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“…In all relevant reports of TAS, myoclonic jerking of the eyelids and perioral muscles is considered a common ictal manifestation (1)(2)(3)(4)(5)(6)(7)(9)(10)(11)(12)(13)(14)(15)(16). In our series, eyelid or perioral myoclonia occurred exclusively in TAS of patients with EMA or PMA, and they are probably pathognomonic for these syndromes.…”

Section: Discussionmentioning

confidence: 48%

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Typical Absence Status in Adults: Diagnostic and Syndromic Considerations

et al. 1998

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Summary: Purpose: To study the electroclinical features of typical absence status (TAS) in adults with syndromes of idiopathic generalized epilepsies (IGEs).Methods: Twenty-one patients with one or more spells of TAS were identified among 136 consecutive adult patients with IGEs. All patients with TAS had comprehensive electroclinical investigations and EEG or video-EEG recorded absences.Results: TAS occurred in 24.4% of 86 patients who had IGEs with typical absences alone or in combination with other seizures presisting in adult life. The prevalence of TAS appeared to be syndrome related, ranging from as high as 57.1% in perioral myoclonia with absences and 46.2% in "phantom" absences with GTCS to as low as 6.7% in juvenile myoclonic epilepsy. A varying degree of impairment of cognition was the cardinal clinical symptom shared in all TAS, but corresponding syndromes of IGE were often betrayed by other symptoms such as eyelid or perioral myoclonia. In phantom absences with GTCS, TAS was more numerous (p S 0.05) and more frequently the first overt seizure type (p = 0.006) than in any other IGE. Only in the syndrome of eyelid myoclonia with absences, TAS was always situation related, mainly as a result of antiepileptic drug discontinuation.Conclusions: The clinical EEG semiology and prevalence of TAS appear to be syndrome related with the highest prevalence in the syndromes of perioral myoclonia with absences and phantom absences with GTCS (p = 0.0024). Key Words:Typical absence status-Nonconvulsive status epilepticusIdiopathic generalized epilepsies-Epileptic syndromesAdults.Absence status is the commone3t form of nonconvulsive status epilepticus, ranging from 53 to 94% in different studies (1-5). It is manifested with prolonged confusional states of varying severity and EEG continuous or repetitive generalized discharges of spikes, multiple lowing. classification of absence status:4. Absence status epilepticus occumng in other epileptic syndromes: progressive myoclonic epilepsies, electrical status epilepticus during slow sleep, Landau-Kleffner syndrome, metabolic or degenerative generalized epilepsies, electrographic gtatus. spikes, and slow waves. Shorvon (6,7) proposed the folTypical (idiopathic) absence status epilepticus occurring in the syndromes of idiopathic generalized epilepsies (IGEs). The EEG discharges are of >2.5 Hz. Atypical (cryptogenic and symptomatic) absence status epilepticus, mainly found in epilepsies, such as the Lennon-Gastaut syndrome. The EEG discharges are slow and <2.5 Hz. De novo absence status epilepticus of late onset, arising de novo in adults without a history of previous epileptic seizures, mainly as the result of drug discontinuation.Although it is well documented in the literature (1-16), absence status is frequently overlooked. It is erroneously considered as complex partial status or other conditions unrelated to epileptic seizures, such as dementia, psychogenic or other behavior disorder (1,10, 12).Furthermore, absence status is usually studied in a unitary fashion without ...

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Section: Discussionmentioning

confidence: 48%

“…Although it is well documented in the literature (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16), absence status is frequently overlooked. It is erroneously considered as complex partial status or other conditions unrelated to epileptic seizures, such as dementia, psychogenic or other behavior disorder (1,10,12).…”

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confidence: 99%

Typical Absence Status in Adults: Diagnostic and Syndromic Considerations

et al. 1998

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“…In absence status, the manifestations may range from minimal behavioral changes to prolonged confusional states (5,10,18). Neuropsychological testing performed ictally in a few cases showed that failure of sustained attention, lack of initiative, paucity of speech, defect in planning, perseveration, and memory disturbances are predominant (10,(18)(19)(20). These cases may be difficult to distinguish from partial complex nonconvulsive status epilepticus of frontal origin (19,21).…”

Section: Discussionmentioning

confidence: 99%

Distinct Behavioral and EEG Topographic Correlates of Loss of Consciousness in Absences

Vuilleumier¹,

Assal²,

Blanke³

et al. 2000

Epilepsia

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Summary:Purpose: To describe the behavioral and EEG topographic correlates of absences with 3-Hz generalized spikewaves and partitioned impairment of consciousness.Methods: Two adult women had so-called "phantom" absences, characterized by brief and mild impairments of consciousness that were previously inconspicuous to both patient and physician. Neuropsychological examination was performed under video-EEG monitoring during absence status. EEG topographic mapping of spike-wave discharges was obtained in the two cases.Results: Only mild attentional and executive disturbances were observed during absence status despite prolonged discharges. Spike-wave bursts were associated with selective i npairment in the initiation of response and self-generated action, whereas short-term storage of external information during discharges was fully preserved. This is consistent with a predominant involvement of frontomesial cortex demonstrated by topographic mapping of spike-wave discharges in the two cases. By contrast, in two other patients with typical absences and a complete lack of retention for information given during the discharges, topographic mapping found a more lateral frontal involvement by spike-wave activity.Conclusions: Different types of absence seizures may impair distinct components of conscious behavior. A predominant involvement of frontomesial thalamocortical circuitry may underlie an "inconspicuous" disorder of consciousness as seen in phantom absences with selective loss of initiation and goaloriented behavior, whereas involvement of more lateral frontal areas in typical absences may additionally disrupt working memory processes. Key Words: Absence seizureAbsence status-Consciousness-Cingulate cortex-Frontal lobe.Absence seizures are defined by impairment of consciousness concomitant with 3-Hz generalized spikewave on EEG (1,2). But absences do not constitute a single disorder, and consciousness is not subserved by a unitary process. Absences occur in several idiopathic epileptic syndromes during childhood or adolescence (1,2) and may occasionally persist into adulthood (3).Absence status also may occur in adults because of drug exposure or withdrawal, or as de novo status (3-5). One form recently emphasized under the term of "phantom absences" is characterized by "brief and mild impairments of consciousness that are inconspicuous to both the patient and physician and may exist for many years before diagnosis" (6,7). It remains unclear whether these cases correspond to a distinct entity (5,7) or a seizure type along a broader continuum of idiopathic epilepsies (8,s). Variable impairment of consciousness during absences (1 0,ll) or subtle cognitive changes related to interictal spikes known as "transient cognitive impairment" (1 2) have been long noted. A5 consciousness cannot be regarded as a unitary phenomenon (13,14), we studied ictal behavior in two cases with so-called phantom absences to learn whether a distinct deficit in the mechanisms of consciousness rather than a different "degree" of deficit m...

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“…Although most patients have known epilepsy, about 20% have no previous history [11]. Reported precipitants include metabolic upset (hyponatraemia [4] and hypoglycaemia [12]), infection [13], cerebrovascular disease [14], and drugs such as carbamazepine [15], ceftazidime [16], chloroquine [17], ifosfamide [18] and, especially, psychotropic medication [19]. Withdrawal or dose reduction of drugs such as benzodiazepines [13,20], psychotropic agents [20] and anticonvulsants [4,13] have also been implicated.…”

Section: Discussionmentioning

confidence: 99%