Cases reported "Status Epilepticus"

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1/12. Exceptionally long absence status: multifactorial etiology, drug interactions and complications.

    To our knowledge, petit mal status lasting longer than 2-3 days has been documented only once [1]. We report a 66 year-old man with well-documented, idiopathic generalized epilepsy who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status. Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.
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2/12. Do not overlook acute isoniazid poisoning in children with status epilepticus.

    A previously healthy 2-year-old girl was admitted with generalized convulsive status epilepticus. She was in a stupor and could respond only to painful stimuli. She also had severe metabolic acidosis. Although initial liver function tests were normal, they were found to be moderately high on the fifth day of admission; however, they dropped to their normal ranges on the twelfth day of admission. Initially, the patient was diagnosed as having idiopathic status epilepticus, and classic anticonvulsant agents, including diazepam, phenytoin, and then phenobarbital, were given. However, her seizures did not subside, and diazepam infusion was initiated. After initiation of diazepam infusion, the seizures were completely controlled. On the fourth day of admission, her parents said that she had accidentally received 20 tablets (a total dose of 2000 mg) of isoniazid just before admission to our hospital. Later, we injected 200 mg of pyridoxine intravenously. During follow-up, her general condition improved, and anticonvulsant agents were discontinued because an electroencephalogram was found to be norma. She was discharged from the hospital on the twelfth day of admission. At the fourth month of follow-up, she was seizure free. Because of this case, we would like to re-emphasize that acute isoniazid poisoning should also be considered in a child with unexplained status epilepticus.
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3/12. Confirmation of nonconvulsive limbic status epilepticus with the sodium amytal test.

    Is it a seizure? This question can be difficult for a clinician to answer, and it may be more critical if the possible seizure lasts >30 min. Long-duration questionable seizure activity changes the question to, "Is it status epilepticus?" status epilepticus (SE) can be divided into convulsive and nonconvulsive types. Convulsive SE is the most easily recognized, whereas nonconvulsive SE is more clinically variable and controversial. The term nonconvulsive SE is more often applied to patients who are severely obtunded or comatose with minimal or no motor movements, or in a stupor of altered consciousness reflecting generalized ictal activity. Nonconvulsive SE also can be caused by focal seizure activity, sometimes restricted to deep small volumes of brain in which scalp EEG may not be diagnostic. We present the case of a patient who had dominant limbic hippocampal SE, but in whom the diagnosis could not be confirmed until a modified novel use of the sodium amytal test was performed.
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4/12. Prolonged postictal stupor: nonconvulsive status epilepticus, medication effect, or postictal state?

    We describe a patient who entered a stuporous state after receiving benzodiazepine treatment for generalized tonic-clonic status epilepticus. A diagnosis of generalized NCSE with tonic seizures was made on the basis of the clinical picture and response to barbiturate anaesthetic, although the EEG pattern was not typical of the changes previously described in tonic seizures-tonic status epilepticus. This report discusses the differential diagnosis of postictal stupor, nonconvulsive status epilepticus with tonic seizures and sedation caused by the emergency treatment of status epilepticus, and summarizes the literature on tonic seizures and tonic status epilepticus.
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5/12. Recurrent absence status epilepticus (spike-and-wave stupor) associated with lamotrigine therapy.

    We report on the aggravation of absence seizures by lamotrigine leading to absence status epilepticus in a child. The patient is a 10-year-old girl with a history of absence seizures, learning disabilities, and hereditary neuropathy with liability to pressure palsies. Lamotrigine (up to 12 mg/kg/day) was prescribed after a brief course of valproate was associated with restlessness. Long-acting methylphenidate was also administered. The initial response to lamotrigine appeared to be excellent. The first episode of absence status epilepticus occurred during a febrile illness while lamotrigine was being gradually discontinued. Following this event, lamotrigine dose was increased to 10 mg/kg/day and methylphenidate was continued. Six weeks later, a second absence status epilepticus episode ensued without fever. Sulthiame and clonazepam were substituted for lamotrigine, whereas methylphenidate therapy was continued. A psychiatrist prescribed risperidone 1 month later owing to obsessive-compulsive behavior. Nine months later, she remained free of absence seizures. Whereas the first absence status epilepticus event could have been triggered by fever, the second episode occurred while the daily lamotrigine dose was being increased. Moreover, the patient is seizure free following lamotrigine discontinuation. Hence, it is quite possible that lamotrigine caused seizure aggravation and absence status epilepticus in this child.
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6/12. A case of epilepsy characterized by spike-wave stupor followed for long period.

    A 34-year-old housewife with nonconvulsive status epilepticus was followed up for 20 years since the initial fit. She maintained some contact with reality during the stupor and recalled the episode to some extent, while the EEG showed continuous spike-wave complexes. During the clinical course, the main type of seizures was spike-wave stupor, of which the maximum frequency was several times a week in the hospital and the duration was many minutes to several hours, and also secondarily generalized convulsive seizures occurred approximately once a month. The lasting control of spike-wave stupor was not achieved in spite of the various medications for the long follow-up period. The ictal EEGs of spike-wave stupor always showed the frontal origin. The meaning of the term "nonconvulsive status epilepticus" in this case was briefly discussed.
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7/12. Fatal seizures after flumazenil administration in a patient with mixed overdose.

    OBJECTIVE: To report a fatal case of refractory status epilepticus precipitated by flumazenil use in a mixed benzodiazepine-tricyclic antidepressant overdose. CASE SUMMARY: A 39-year-old woman was brought to the emergency room (ER) in a stupor from a suspected suicidal overdose of an unknown mixture of drugs. Past medical history included seizures and psychiatric disorders managed with benzodiazepine and tricyclic antidepressants. Initial ER electrocardiogram showed a QRS interval of 136 milliseconds. The patient developed refractory seizures after being given flumazenil. lorazepam, phenytoin, and phenobarbital were administered; however, seizures persisted for 4 hours, resulting in rhabdomyolysis, acute renal failure, severe brain damage, and death. DISCUSSION: flumazenil should be used with caution in patients with chronic benzodiazepine use, prior seizure history, or when a mixed overdose is suspected. flumazenil may unmask tricyclic antidepressant-induced seizures by antagonizing the antiepileptic effect of concomitantly ingested benzodiazepine. In this patient seizures occurred within two minutes of flumazenil administration. As benzodiazepine-induced central nervous system depression is rarely life-threatening, the use of flumazenil must be balanced against potential risk. CONCLUSIONS: Seizure risk factors should be assessed in all patients in whom flumazenil use is considered. If risk factors are present, the benefit of flumazenil use is outweighed by the potential risk. If flumazenil is used, resulting seizures may require larger doses of benzodiazepine.
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8/12. Temporary abolition of seizure activity by flumazenil in a case of valproate-induced non-convulsive status epilepticus.

    We report on a 33-year-old female suffering from frequent complex-partial seizures who developed a non-convulsive status epilepticus after one week of antiepileptic therapy with valproate (VPA) which had been added to a basic medication with barbexaclone (BBC) in rapidly increasing dosage. The electroencephalogram (EEG) showed continuous rhythmic generalized sharp and slow wave activity with a frontal maximum. Intravenous administration of 3.0 mg of the benzodiazepine (BZ) receptor antagonist flumazenil under monitoring with video-EEG led to an immediate and marked electroclinical improvement, whereas 6.0 mg of the BZ receptor agonist midazolam was followed by a deterioration both clinically and in the EEG. We discuss the concept of VPA-encephalopathy and the possible mechanisms of the action of flumazenil on VPA-induced as well as on other toxic and metabolic encephalopathies. flumazenil might antagonize increased benzodiazepine receptor activity with agonistic and even convulsive properties in these encephalopathic syndromes. Further investigations are needed concerning the relation of drug-induced or metabolic encephalopathies and central benzodiazepine receptor activity. We recommend a therapeutic trial with flumazenil, if stupor or decreased seizure control develop in patients treated with valproate.
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9/12. "Obtundation status (Dravet)" caused by complex partial status epilepticus in a patient with severe myoclonic epilepsy in infancy.

    PURPOSE: We report a 1-year 7-month-old boy with severe myoclonic epilepsy in infancy (SME) who exhibited complex partial status epilepticus (CPSE), which was confirmed by ictal video-EEG analysis. This boy first had a hemiconvulsion in a hot bath at age 3 months. Thereafter, he exhibited both partial and generalized seizures that were extremely intractable. At age 9 months, he had a prolonged episode of impaired consciousness that fit the "obtundation status in SME" defined by Dravet et al. methods AND RESULTS: Ictal EEG revealed persisting irregular spike-and-wave complexes over the left hemisphere, predominantly in the occipitotemporal area, and confirmed CPSE. The EEG abnormalities with decreased level of the consciousness continued approximately 6 h after onset of the symptoms even with AED administration. CONCLUSIONS: Because SME features both generalized and focal seizures, both types of nonconvulsive status may be seen in SME. Although Dravet et al. already reported long-lasting atypical absences in patients with SME as "obtundation status," we demonstrated CPSE in an infant with SME who exhibited a prolonged stuporous state.
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10/12. Nonconvulsive status epilepticus in thrombotic thrombocytopenic purpura.

    Fluctuating neurologic symptoms, including confusion, stupor, and convulsions, are common signs in thrombotic thrombocytopenic purpura (TTP). We describe a patient with TTP who had a clinical seizure, intermittent stupor, and a declining course despite aggressive treatment. An EEG showed continuous rhythmic epileptiform discharges, and she improved with anticonvulsants. Fluctuating stupor in TTP has generally been attributed to microvascular occlusive disease, but nonconvulsive status epilepticus is a treatable condition that can cause similar symptoms.
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