José L. Fernández-Torre

Nonconvulsive status epilepticus in adults: Electroclinical differences between proper and comatose forms☆

OBJECTIVE Nonconvulsive status epilepticus (NCSE) represents an important percentage of status epilepticus in adults, but detailed studies of both NCSE proper and comatose NCSE are lacking. We retrospectively analyzed a prospectively collected series of 50 adult patients with a diagnosis of NCSE whose electroencephalograms (EEGs) have been interpreted for a period of 10 years by the same investigator. METHODS Two groups, NCSE proper and comatose NCSE were considered. All clinical, EEGs, neuroimaging data, antiepileptic treatment and outcome were analyzed. RESULTS Thirty-two patients (64%) had NCSE proper and 18 patients (36%) comatose NCSE. The mean age was 56 years (range 19-89 years). Fourteen (44%) were diagnosed with absence status epilepticus (ASE), one had simple partial status epilepticus (SPSE) and 17 (53%) had complex partial status epilepticus (CPSE). The mean episode duration (33.2±13.9 versus 60.6±34.0), mean number of antiepileptic drugs (AEDs) (1.46±0.5 versus 2.77±1.39) and neuroimaging anomalies (50% versus 16%) was significantly greater in the partial/focal NCSE proper subgroup than in the ASE subgroup. The mean age (56.0±19.9 versus 69.4±12.1), number of elderly individuals (46% versus 77%), mean duration of the episode (49.1±30.4 versus 153.3±142.6), mortality rate (6% versus 61%) and admission at ICU (18% versus 83%) was significantly higher in the comatose NCSE group than in the NCSE proper group (p<.05). Conversely, a previous history of chronic epilepsy was significantly more frequent (62% versus 5.6%) in the NCSE proper group. The mean duration of comatose NCSE was significantly greater in the surviving subgroup (102.5±29.1 versus 233.1±65.3; p<.05). CONCLUSIONS Our study demonstrates that there are sufficient differences regarding age of onset, history of previous epilepsy, episode duration, mortality rate and clinical presentation between NCSE proper and comatose NCSE to recommend adoption in clinical practice. These results should be taken into account when developing future classifications and therapeutic trials on NCSE. SIGNIFICANCE A distinction between NCSE proper (ambulatory forms of NCSE) and comatose NCSE is useful in the clinical practice and, therefore, it should taken in account in the design of future investigations on this heterogeneous epileptic condition.

Cephalosporin-induced Nonconvulsive Status Epilepticus: Clinical and Electroencephalographic Features

Nonconvulsive status epilepticus (NCSE) is an epileptic condition lasting ≥30 min, clinically manifested by an altered mental state and associated with continuous or recurrent epileptiform activity on electroencephalogram (EEG). All of these symptoms exist without overt tonic, clonic, or tonic–clonic movements. In general, an immediate or short-term clinical improvement will be achieved with antiepileptic treatment. Although diverse pathologic processes such as cancer, ring chromosome 20, neurosyphilis, subarachnoid hemorrhage, or Creutzfeldt– Jakob disease have been related to this epileptic entity, the diagnosis of drug-induced NCSE has notably increased on recent years.

Utility of bilateral Bispectral index (BIS) monitoring in a comatose patient with focal nonconvulsive status epilepticus.

We report the case of a patient with an extensive right cerebral hematoma complicated by focal nonconvulsive status epilepticus (NCSE) in whom the use of the new bilateral BIS-Vista™ monitor was helpful in managing profound sedation and antiepileptic treatment in the absence of continuous EEG monitoring (CEEG). The analysis of color density spectral array (CDSA) showed stereotyped changes indicative of recurrent focal nonconvulsive seizures (NCSz) and NCSE. We noted a close correlation between NCSz and BIS value changes. EEGs during working hours always confirmed the persistence of focal NCSE. After several days of sedation, CDSA disclosed a gradual resolution of NCSE that was also confirmed by electroencephalography. The patient died of cardiorespiratory complications a few days later.

Subacute encephalopathy with seizures in alcoholics (SESA syndrome) revisited

PURPOSE The aim of this paper is to describe two additional cases of subacute encephalopathy with seizures in alcoholics (SESA syndrome), and to propose that this entity now should be considered as a subtype of nonconvulsive status epilepticus (NCSE). METHODS We retrospectively analyzed the clinical characteristics, electroencephalography (EEG), neuroimaging data, and prognosis of these two further cases of SESA syndrome. In addition, we compare our findings with the cases previously described in the English literature in order to propose new diagnostic criteria. RESULTS Two adults with history of chronic alcohol abuse were admitted because of confusion and seizures. A routine EEG showed frequent periodic lateralized epileptiform discharges (PLEDs) localized over the right temporal regions. In one case, we captured two complex partial seizures (CPSs) arising from the right hemisphere. Neuroimaging studies revealed subjacent chronic vascular pathology. Following transfer to the intensive care unit (ICU), both improved to antiepileptic treatment and were discharged with full recovery. CONCLUSION On the basis of our findings and a review of the literature, we suggest that SESA syndrome represents a subtype of partial or localization-related NCSE given its particular clinical, electroencephalographic, neuroimaging and prognostic characteristics.

Early-onset absence epilepsy: Clinical and electroencephalographic features in three children

To describe the clinical and electroencephalographic features of three infants diagnosed as having early-onset absence seizures. Two males and one female, aged 21-29 months were seen in our neuropaediatric outpatient clinic because of daily episodes of motor arrest and loss of contact. Neurological examination and mental development was considered normal in all of them. Two out three had first-degree relatives with seizures with onset in the childhood and favourable evolution in the adulthood. A video-electroencephalogram was requested. Ictal EEG revealed a normal background and generalised spike-and-wave complexes at 3-3.5 Hz accompanied by disruption of ongoing activity in keeping with absence seizures. The duration of seizures ranged from 2 to 10s. One child (patient 2) experienced rhythmic myoclonic jerks in upper limbs and head as those described in myoclonic absences. Clinical and electroencephalographic follow-up ranged from 8 months to 4 years. Two children were on treatment with valproate and in the case of the patient 3, the combination of valproate and ethosuximide was necessary. Control of absence seizures was achieved in all our cases. Absence seizures should be considered as a possible cause of transient impairment of consciousness even among infants minor than 3 years of age. A video-electroencephalogram is the method of choice in the diagnostic evaluation and syndromic classification of these paroxysmal events.

Subacute Encephalopathy with Seizures in Alcoholics (SESA Syndrome): Report of an Unusual Case

Subacute encephalopathy with seizures in alcoholics (SESA syndrome) is a rare neurologic disorder complicating the chronic abuse of alcohol. This acute or subacute condition is characterized by transient neurologic deficits, seizures and severe lateralized abnormalities on the electroencephalogram (EEG). Our case demonstrates that in SESA syndrome, convulsive (generalized tonic-clonic) and nonconvulsive (complex partial) seizures may coexist.

Abdominal Epilepsy in an Adolescent with Bilateral Perisylvian Polymicrogyria

Summary: Purpose: We report a patient with recurrent episodes of severe periumbilical pain accompanied by headache, pallor, dizziness, and visual hallucinations who was subsequently diagnosed as having abdominal epilepsy and a bilateral sylvian cortical malformation.

New understanding of nonconvulsive status epilepticus in adults: treatments and challenges.

Nonconvulsive status epilepticus (NCSE) in adults represents a constellation of conditions producing ongoing ictal impairment with myriad clinical presentations. These may vary from minimal obtundation, to psychosis to coma for which electroencephalography is essential to diagnosis. Treatment is highly variable and is tailored to the level of consciousness, probable outcome and underlying etiology. There is a new and emerging understanding of the nature of NCSE, its classification and the challenges in treatment. The aim of this article is to review the classic and most recent literature on NCSE in adults focusing attention on the clinical settings in which NCSE frequently occurs, in order to improve awareness of the issues involved in electroclinical diagnosis, treatment and prognostication.

Typical absence status epilepticus as late presentation of idiopathic generalised epilepsy in an elderly patient

Typical absence status epilepticus as late presentation of idiopathic generalised epilepsy in an elderly patient Sir Editor, Absence status epilepticus (ASE) is a type of non-convulsive status epilepticus in which continuous or recurrent generalised epileptiform discharges are associated with a varying grade of consciousness impairment. It usually occurs in patients with previous diagnosis of epilepsy, particularly in patients suffering from certain idiopathic generalised epilepsy (IGE) syndromes such as juvenile absence epilepsy or juvenile myoclonic epilepsy. In these cases, the term typical ASE is usually employed. 1 However, the episodes of ASE occurring de novo in middle-aged or elderly patients are generally considered a situation-related non-con-vulsive status epilepticus most often consequence of a benzodia-zepine withdrawal syndrome. 2,3 We describe the case of an elderly woman who presented with an episode of typical ASE as late complication of an unrecognised picture of IGE. A 68-year-old woman was admitted to our hospital because of a generalised tonic–clonic seizure (GTCS) and agitation. She had antecedents of myocardial infarction and depression on treatment with salicylic acid, lovastatine and melitracen-flupenthixol. On neurologic examination, she was disoriented to time and place, and a marked slowness in mental activity was observed. Laboratory tests and a CT scan of the brain were normal. Twelve hours later, her neurologic state was unchanged and an urgent electroencephalogram (EEG) was requested. The EEG showed frequent and recurrent generalised paroxysms of 4–6 Hz poly-spike-wave and spike-wave complexes intermixed by brief periods (2–4 s) of normal background activity (Fig. 1). The diagnosis of ASE was done. The onset of treatment of valproic acid (1 500 mg/24 h) was accompanied by a complete normal-isation of the patient mental status for the next 24 h. Subsequently , a second EEG revealed occasional generalised paroxysms of spike-wave discharges and the occurrence of a photoparox-ysmal response at rates of 5, 10 and 20 Hz. When the patient was interrogated she referred a past history of GTCSs during childhood. These seizures were never studied and no EEG was performed at that time. Magnetic resonance imaging of the brain disclosed multiple hyperintense white matter lesions of probable ischaemic origin. The patient has remained seizure-free on treatment with valproic acid for the last two years; however, several EEGs have showed occasional and brief generalised fast spike-wave discharges without clinical manifestations. The previous history of GTCSs during infancy, ictal and interictal EEG anomalies and the existence of photosensitivity support strongly the diagnosis …

Epileptic negative myoclonus in a case of atypical benign partial epilepsy of childhood: a detailed video-polygraphic study

PURPOSE To describe the clinical and electroencephalographic features of a child diagnosed as having atypical benign partial epilepsy (ABPE) who suffered from frequent lapses of postural tone in the right lower limb that were considered to represent a focal epileptic negative myoclonus (ENM). MATERIAL AND METHODS Electrophysiological evaluation included four serial waking and two sleep EEGs. Moreover, the investigation included a polygraphic recording with simultaneous video-EEG monitoring performed in rest, during hyperventilation, and while standing up with the aim of capturing patients typical seizures. RESULTS During awake the EEG showed frequent bilateral centrotemporal discharges maximal over the left central area. Moreover, we recorded short generalised paroxysms of spike-and-wave discharges accompanied by a clear transient cognitive impairment that were in keeping with absences. We also captured several episodes of sudden and unexpected loss of postural tone in the right lower limb. These focal inhibitory seizures were associated with brief diffuse but asymmetrical paroxysms of irregular spike-and-wave discharges maximal over the left side. EMG flattening of the right quadriceps muscle was time-locked to the midline of the slow-wave component of the diffuse spike-and-wave discharges. CONCLUSIONS ENM leading to focal lapses of postural tone in lower extremities may be a predominant type of seizures in ABPE. A polygraphic recording with video-EEG monitoring is essential to confirm the diagnosis, and to detect other concomitant seizures, such as atypical absences. In our case, ENM could be more likely due to a transient disruption of cortical function.