The significance of myoclonic status epilepticus in postanoxic coma

We report 11 adults who exhibited myoclonic status epilepticus (MSE) after cardiac arrest. Based on pathologic, electroencephalographic, and clinical evidence, we conclude that our patients died from the initial anoxic-ischemic insult rather than as a result of MSE. We suggest that the seizures in these nonsurvivors were self-limited events arising from lethal damage to neurons. Thus, in patients with bilaterally synchronous facial myoclonus, bilateral loss of pupillary or oculovestibular reflexes, and suppression and burst-suppression on EEG, it is not warranted to use anesthetic barbiturates to treat MSE.     

Pregabalin-induced generalized myoclonic status epilepticus in patients with chronic pain

Pregabalin is often used for the treatment of chronic pain syndromes. We here describe two patients with chronic pain and pregabalin-induced myoclonic status epilepticus. Patients treated with pregabalin who experience sudden behavioral changes or mycloni should be investigated for this possible side effect, and pregabalin should be reduced or discontinued if myocloni or status epilepticus occurs.     

Failure of high-dose intravenous magnesium sulfate to control myoclonic status epilepticus

An unsuccessful attempt to terminate myoclonic status epilepticus with elevation of serum magnesium levels is described. During 3 days, serum magnesium was increased from 1.5 mEq/L to 14.2 mEq/L by continuous i.v. infusion of 3-6 g/h of magnesium sulfate. Other anticonvulsants were maintained at nearly constant levels. Cerebrospinal fluid magnesium was 3.5 mEq/L during the infusion. Despite magnesium-related neuromuscular blockade and accompanying cessation of visible myoclonus, the electroencephalogram revealed ongoing blunted sharp-wave activity at the baseline frequency. Transient complications of the infusion included prolongation of the PR interval on the electrocardiogram, hypomotility of the gastrointestinal tract, and peripheral muscle flaccidity, all of which resolved within 24 h of return to normal serum magnesium levels. These results suggest that the therapeutic role of magnesium in myoclonic status epilepticus is limited.     

Mixed myoclonic‐absence status epilepticus in juvenile myoclonic epilepsy

Myoclonic status epilepticus or mixed absence‐myoclonic status is uncommon in juvenile myoclonic epilepsy (JME), often precipitated by sleep deprivation, withdrawal of medication, or inadequate antiepileptic drugs (Thomas et al., 2006 ; Crespel et al., 2013 ). Such episodes respond well to benzodiazepines or valproate (Crespel et al., 2013 ). We present the video‐EEG of a 24‐year‐old woman with JME and bipolar disorder. She had a confusional state five days after withdrawal of clonazepam (14 mg/d) and introduction of oxazepam (200 mg/d), followed by catatonic stupor with subtle myoclonus of the face and the arms. The EEG showed absence status (figures 1, 2), which stopped after IV injection of clonazepam (1 mg) (figure 3). Consciousness returned to normal [Published with video sequence and figures 1 ].     

Non-convulsive status epilepticus: a rare presentation of juvenile myoclonic epilepsy

We present a case of a boy with juvenile myoclonic epilepsy (JME) who presented with features of non-convulsive status epilepticus (NCSE). This case highlights the fact that NCSE, even though not a common occurrence in JME, should be kept in mind when a patient with previous history of seizures presents with subtle changes in sensorium with no obvious cause.     

Gaucher disease: successful treatment of myoclonic status epilepticus with levetiracetam

We present the first reported case of a rapid clinical and electroencephalographic response to intravenous levetiracetam infusion of myoclonic status epilepticus in a patient with progressive myoclonus epilepsy due to Gaucher disease. Under continuous video-EEG monitoring, the clinical myoclonic status and the electrographic ictal discharges resolved within 10 minutes after the infusion was initiated. The patient tolerated the treatment well without any reported side effects. This case suggests that levetiracetam may be a safe, effective, and well tolerated intravenous drug in patients with metabolic myoclonic status epilepticus such as Gaucher disease.     

Levetiracetam-induced myoclonic status epilepticus in myoclonic-astatic epilepsy: a case report.

We report on a 3-year-old boy with myoclonic-astatic epilepsy who developed myoclonic status epilepticus with continuous twitching of the face and unresponsiveness under monotherapy with levetiracetam. Recently, a nonconvulsive status epilepticus in an adult epilepsy patient has also been described. Our observation points to the possibility of a causal relationship between the induction of myoclonic status by levetiracetam in certain patients with Doose's syndrome. However, a spontaneous evolution cannot be excluded. Levetiracetam is a well-known drug for the control of myoclonic seizures. A controlled study would provide a better understanding of any possible aggravating role in certain forms of myoclonic-astatic epilepsy.     

The ketogenic diet in two paediatric patients with refractory myoclonic status epilepticus

Aim. We describe two patients with refractory myoclonic status epilepticus treated with the ketogenic diet. Methods. Between May 1, 2014 and January 1, 2015, two patients who met the diagnostic criteria for refractory myoclonic status epilepticus, seen at our department, were placed on the ketogenic diet and followed for a minimum of six months. Results. One patient with myoclonic epilepsy of unknown aetiology had a 75–90% seizure reduction, and the other with progressive encephalopathy associated with myoclonic epilepsy had a 50% seizure reduction. Both patients retained good tolerability for the diet. At the last control, one patient had isolated myoclonias and EEG showed occasional generalized spike‐and‐polyspike waves; the patient is now successfully attending kindergarten. The quality of life of the second patient improved significantly. In both cases, the number of antiepileptic drugs was reduced. Conclusion. The ketogenic diet is an effective and well‐tolerated treatment option for patients with refractory myoclonic status epilepticus and should be considered earlier in the course of treatment.     

Electrographic and clinical effects of intracarotid sodium amobarbital on bilateral myoclonic status epilepticus.

The effects of rapid intracarotid injection of 20 to 100 mg of sodium amobarbital were studied in three patients with bilateral myoclonic status epilepticus due to Jakob-Creutzfeldt disease, sequelae to anoxic encephalopathy, and hepatic coma, respectively. In each instance, the drug produced prompt abatement of clonic jerks contralaterally and attenuation of electroencephalographic epileptiform discharges ipsilaterally. These results suggest that the cerebral cortex actively participates in the elaboration of certain types of bilateral myoclonus in human beings.     

Nonconvulsion status epilepticus in patients with juvenile myoclonic epilepsy: types and frequencies.

Juvenile myoclonic epilepsy (JME) is an idiopathic, age-related generalized epileptic syndrome, featuring generalizedtonic-clonic and absence seizures as well as myoclonic jerks. Except for some case reports, little is known about type and frequency of nonconvulsive status epilepticus (NCSE) in adult patients with JME. In a retrospective study we therefore reviewed all patients with JME, who had been referred to our hospital between 1994 and 1999 for the occurrence of NCSE. Of the 69 patients with JME seen within these 6 years three women had typical absence status-one of them two times-and one woman had impulsive-petit-mal status. All four patients had a history of myoclonic, absence and generalized tonic-clonic seizures. Because of the extreme rarity of impulsive-petit-mal status the corresponding case history is presented in detail. According to our findings the prevalence of NCSE in JME can be estimated at 5.8%, the incidence at 1.2% per year with a clear preponderance of female gender.     

Life-threatening status epilepticus following gabapentin administration in a patient with benign adult familial myoclonic epilepsy

We report the case of a 57-year-old man who experienced life-threatening myoclonic status after the administration of gabapentin. Based on familial data, the patient was determined to be a member of a previously described family with benign adult familial myoclonic epilepsy (BAFME). The myoclonic status did not respond to benzodiazepines, but resolved after discontinuing the gabapentin. As for other idiopathic generalized epilepsies, gabapentin may precipitate myoclonic status in a benign syndrome, such as BAFME, as is reported herein for the first time. A correct diagnosis and prompt discontinuation of the drug may reverse a potentially severe, life-threatening condition.     

Delirium caused by nonconvulsive status epilepticus

We report about a patient (66 years) who was referred to our psychiatric hospital because of a progressive confusional state with acute onset. The colleagues of the referring psychiatric hospital considered a first manic episode as the cause of the symptoms and under therapy with haloperidol the confusional state had shown a progression.The clinical examination's findings were a mild central facial paresis on the right side and a mild hemiparesis on the right side with elevated reflex levels.The patient was disoriented, he had cognitive and mnestic deficits. His reasoning was slowed, incoherent and perseverating. The patient had a slight euphoria.An EEG recording showed a continuous regional EEG-seizure pattern. In combination with the clinical symptoms we diagnosed a nonconvulsive status epilepticus. Under anticonvulsive treatment with Lorazepam and Valproic acid the status epilepticus sustended but a control EEG recording showed signs of a Valproate-encephalopathy. Under treatment with Topiramate symptoms ameliorated but due to a vascular dementia the patient still showed fluctuating symptoms of cognitive and mnestic disturbances.     

Hemimegalencephaly

Hemimegalencephaly (HME) is an uncommon sporadic nonfamilial congenital dysplastic abnormality of the central nervous system, characterized by enlargment of one cerebral hemisphere, with cranial asymmetry, hemiparesis, epilepsy, and mental retardation. It can occur in isolation or associated with various anomalies, namely skin disorders. The main neuropathologic findings are hemispheric gigantism, macro- and/or micropolygyria, cortical thickening with lack of lamination, blurred boundaries of the gray and white matter, and large ortho- and heterotopic neural cells. The results obtained by morphological investigations carried out on six patients with HME, compared with the findings recorded in similar studies performed on one patient with tuberous sclerosis (TS) and another with pachygyria, allow the authors to (a) confirm the dysplastic nature of HME and its autonomy from TS; (b) demonstrate that ortho- and heterotopic neuronal cells do not differentiate completely during proliferation and migration from the germinal matrix; (c) document, by means of flow cytometric study, a normal euploid DNA content in the enlarged hemisphere, consequently ruling out heteroploidy as a cause of both cell hypertrophy and enlargement of the malformed cerebral hemisphere.     

非惊厥性癫(癎)持续状态

非惊厥性癫(癎)持续状态(NCSE)系临床常见但易忽视的癫(癎)持续状态(SE)发作类型,据估计,占所有癫(癎)持续状态的20%～50%.2004年,英国癫(癎)研究基金会(ERF)将非惊厥性癫(癎)持续状态定义为:由于持续性癫(癎)样脑电活动导致的一系列非惊厥临床征象[1].