1/8. L-2-Hydroxyglutaric aciduria presenting as status epilepticus.L-2-Hydroxyglutaric aciduria (L-2-HGA) is a rare organic aciduria with a slowly progressive course regarding CNS involvement. We present a 13.5-year-old female patient who presented at the Emergency Department with a generalized status epilepticus, which promptly responded to intravenous phenytoin. CT and MRI demonstrated subcortical white matter alterations. The neurological examination revealed mild mental retardation, macrocephaly and ataxic gait with cerebellar signs. Repeated urinary organic acid analysis demonstrated increased excretion of 2-hydroxyglutaric acid which was of the L-configuration. The constellation of macrocephaly in a patient with mental retardation, cerebellar tract involvement and subcortical white matter signal alterations on MRI should alert the physician to the possibility of L-2-HGA. Although rare, epileptic seizures or even status epilepticus can be among the presenting symptoms in organic acidurias with a slow course, such as L-2-HGA.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/8. status epilepticus after a massive intravenous N-acetylcysteine overdose leading to intracranial hypertension and death.Cases of N-acetylcysteine overdose have been reported before. In some cases, these overdoses have led to death if an anaphylactoid reaction was present. A healthy 30-month-old girl allegedly ingested acetaminophen at 418 mg/kg. Because the emergency physician feared the time of ingestion might not be accurate, he decided to start the 20.5-hour intravenous N-acetylcysteine protocol 8 hours after ingestion. He mistakenly prescribed the maximum milliliter-per-kilogram volume of the dextrose 5% diluent for the milliliter-per-kilogram volume of N-acetylcysteine 20% to be administered. Five hours after the error was detected (19.5 hours postingestion), the patient started developing myoclonus on the left side of her body, with left eye deviation. This condition persisted intermittently for 3 hours despite treatment with diazepam, lorazepam, and phenytoin. A first computed tomographic scan result was normal. A few hours later, she sustained shorter recurrences of the myoclonus. At 30 hours after ingestion, she started to have irregular breathing and became unresponsive to pain. A repeated computed tomographic scan showed diffuse cerebral edema. A postmortem examination showed the presence of acute anoxic encephalopathy with marked cerebral edema and the beginning of uncal herniation that confirmed the clinical diagnosis of intracranial hypertension and brain death. A cumulative intravenous dose of 2,450 mg/kg of N -acetylcysteine was associated with status epilepticus, intracranial hypertension, and death in a child.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/8. Prolonged coma from refractory status epilepticus.OBJECTIVE: status epilepticus is a life-threatening medical condition. In its most severe form, refractory status epilepticus (RSE) seizures may not respond to first and second-line anti-epileptic drugs. RSE is associated with a high mortality and significant medical complications in survivors with prolonged hospitalizations. methods: We describe the clinical course of RSE in the setting of new onset lupus in a 31-year-old male who required prolonged barbiturate coma. RESULTS: Seizure stopped on day 64 of treatment. Prior to the resolution of seizures, discussion around withdrawal of care took place between the physicians and patient's family. Medical care was continued because of the patient's age, normal serial MRI studies, and the patient's reversible medical condition. CONCLUSION: Few evidence-based data exist to guide management of RSE. Our case emphasizes the need for continuous aggressive therapy when neuroimaging remains normal.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/8. Management of seizures.Seizure patients represent a population that is frequently seen in medical practice. critical care physicians will encounter chronically epileptic patients as well as patients with isolated seizures that develop in the course of treatment of surgical and other medical disorders. In such circumstances, knowledge of seizure type and common antiepileptic medications facilitates appropriate medical management. More emergently, SE may constitute a life-threatening indication for admission to an intensive care unit. Rapid treatment of SE is necessary to prevent multiple medical complications leading to morbidity and mortality.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/8. The use of felbamate in patients with periodic lateralized epileptiform discharges (PLEDs).This case report refers to a patient with PLEDs whose EEGs did not change while on steady levels of phenytoin and phenobarbital, but showed a complete disappearance of these discharges when felbamate was used. This report may serve to remind EEG'ers and epileptologists that the acute use of this drug may be effective in dealing with PLEDs that are often difficult to eliminate. However, physicians must keep in mind the significant risks of using this medication.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/8. Termination of nutrition and hydration in a child with vegetative state.A child in a vegetative state may present difficult decisions for physicians and families regarding the course of treatment. We report a case of a child who entered a prolonged vegetative state following status epilepticus. The child's parents requested termination of artificial means of nutrition and hydration. That request culminated in a complex legal intervention by multiple state agencies and attracted local media attention. This article presents the details of the case and discusses the medical and legal complexities encountered. The diagnosis and prognosis of the persistent vegetative state in children have recently been defined. decision making in these circumstances should be based on adequate, careful clinical evaluation of the medical facts. Hospital ethics committees can provide an independent forum in which the diverse viewpoints in a case may be examined. decision making should optimally be accomplished between families and caretakers.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/8. Intranasal midazolam in patients with status epilepticus.The patient in status epilepticus presents many challenges to the emergency physician. IV access is frequently difficult to achieve, and prolonged attempts at access can jeopardize the patient and endanger the caregiver. We present two cases in which the administration of intranasal midazolam appeared to successfully terminate status epilepticus. No adverse effects were noted. Studies are needed to clarify the safety, optimal dosing, and clinical utility of this treatment modality.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
8/8. Severe encephalopathy in a child: an uncommon cause.Cat scratch disease (CSD) is usually a self-limited disease. Although extremely uncommon, the involvement of the central nervous system has been previously reported in CSD. The intention of this paper is to make physicians aware that CSD could be complicated by encephalopathy. seizures in some patients could be the only clinical manifestation and are resistant to the common anti-epileptic therapy. The seizures resolve with supportive care.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |