1/53. Folinic acid-responsive neonatal seizures.We report three cases of folinic acid-responsive intractable neonatal seizures. All patients were born at term following normal gestation and delivery. In the first infant, seizures began on the 5th day of life and were unresponsive to phenobarbital, pyridoxine, and valproate, but stopped within 24 hours of initiation of folinic acid treatment at the age of 6 months. Her sibling had died at age 6 months with intractable seizures. In the second infant, seizures began in the 2nd hour of life. These were initially controlled with phenobarbital; however, at 3 months of age she developed status epilepticus refractory to anticonvulsants, steroids, and pyridoxine and she required repeated induction of pentobarbital coma. seizures stopped within 24 hours of starting folinic acid. seizures and encephalopathy were noted in the third infant on the 2nd day of life. These were controlled with phenobarbital, but at 8 weeks of age seizures recurred and were difficult to control despite the addition of phenytoin. Immediately after folinic acid was initiated the seizures stopped. Breakthrough seizures in all patients have responded to increases in folinic acid; two of the three remain on standard anticonvulsants. All patients have global developmental delay. Cranial magnetic resonance imaging in the second patient shows diffuse atrophy, and in the third patient shows increased signal on T2 images in the white matter of the frontal and parietal lobes. Analysis of cerebrospinal fluid from these patients using high-performance liquid chromatography with electrochemical detection has consistently revealed an as-yet unidentified compound, which can be used as a marker for this condition. We suggest that cerebrospinal fluid be analyzed for the presence of this compound and a trial of folinic acid be considered in neonates with unexplained early onset intractable seizures.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
2/53. Regional cerebral blood flow after status epilepticus.Two patients with status epilepticus due to specific conditions were examined using MRI and stable Xe/CT CBF. [Case 1] A 30-year-old woman developed a grand mal seizure during delivery. She was comatose, and MRI revealed abnormal high intensity areas bilateral basal ganglia, compatible with eclampsia. Regional CBF was decreased in bilateral occipital lobes and right basal ganglia. Six days after onset. Regional gray matter flow was increased, especially in the thalami and basal ganglia. [Case 2] The patient is a 31-year-old male diagnosed with temporal lobe epilepsy since 10 years. At the onset, he had a prolonged right hemiconvulsion followed by generalized tonic-clonic convulsion. MRI 13 days after onset showed left hemispheric edematous swelling of gray matter. Stable Xe/CT 3 weeks after onset demonstrated increased cortical CBF corresponding to edematous area. The results suggested that regional CBF decreased immediately after status epilepticus and then increased for 1-3 weeks in the interictal period. We speculate that the energy debt incurred during prolonged seizure causes relative ischemic condition in the neurons, with the increase in CBF resulting from accelerated energy production for a long period.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
3/53. A case of post-anoxic encephalopathy with initial massive myoclonic status followed by alternating Jacksonian seizures.To contrast stimulus-sensitive generalized myoclonus with ensuing multifocal localized myoclonus in a patient with post-anoxic coma, we stressed the clinical as well as electroencephalographical differences between his initial generalized and subsequent focal myoclonus. While generalized myoclonus was presumably of extracortical origin and responsive to valproic acid, alternating Jacksonian seizures were definitely cortical and suppressed with phenytoin. These two different types of myoclonus should not be confused in post-anoxic coma.- - - - - - - - - - ranking = 2keywords = coma (Clic here for more details about this article) |
4/53. Late onset heterozygous ornithine transcarbamylase deficiency mimicking complex partial status epilepticus.A 57 year old woman with post-traumatic complex partial seizures was admitted because of recurrent episodes of altered mental state over the preceding 4 years, each lasting up to 5 days. There was a history of dietary protein intolerance since childhood and two of her daughters had died in the neonatal period from unexplained encephalopathies. In hospital she developed fluctuating confusion, amnesia, and sudden episodes of unresponsiveness. An EEG was consistent with complex partial status epilepticus but there was no response to benzodiazepines. Nasogastric feeding and sodium valproate were given and shortly afterwards she lapsed into a deep coma. blood ammonia and urinary orotate were raised, and genetic testing confirmed that she was a carrier of a mutation in exon 3 of the ornithine transcarbamylase gene (C to T at position 92). Treatment with protein restriction, carnitine, and sodium phenylbutyrate led to a full recovery over a period of 3 months. To our knowledge this is the oldest age of onset yet described in a manifesting carrier. She is the fifth patient with heterozygous ornithine transcarbamylase deficiency reported to have had a severe reaction to sodium valproate. Hyperammonaemic encephalopathy should be considered in patients of any age who experience fluctuating confusion.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
5/53. diffusion-weighted magnetic resonance imaging in nonconvulsive status epilepticus.BACKGROUND: In human and experimental models, diffusion-weighted magnetic resonance imaging (DWI) findings in status epilepticus (SE) have been reported to show that apparent diffusion coefficients are reduced during the initial phase and normalized or increased in the later phase of prolonged SE. This effect is caused by cytotoxic edema induced by excitotoxicity. In humans, only focal DWI abnormalities have been reported in partial SE. OBJECTIVES: To report and discuss the DWI findings suggesting diffuse neuronal injury in a patient with nonconvulsive SE. DESIGN AND methods: A 56-year-old man was admitted because of changing levels of consciousness over 3 days. On admission he was comatose. He had nystagmoid eye movement, forced eye blinking, and oroalimentary automatism. The results of a search for possible infectious and metabolic etiologies were negative, and electroencephalographic findings showed continuous, semirhythmic, bifrontal sharp waves of 2 Hz. phenytoin and midazolam hydrochloride were infused to alleviate the seizure activities. He underwent DWI initially (3 days after the onset of seizure) and at the 5-month follow-up. SETTING: The neurology department of a tertiary referral center. RESULTS: During SE, DWI findings showed marked, diffuse gyriform cortical hyperintensity throughout the brain. The apparent diffusion coefficient decreased in the corresponding areas, especially in the occipital lobes. Findings from T2-weighted magnetic resonance imaging showed the intense cortical hyperintensity with gyral swelling and no involvement of brainstem, basal ganglia, thalamus, and white matter. The follow-up DWI findings showed marked atrophy and hypointensity in the corresponding regions. The apparent diffusion coefficient increased in the corresponding regions. CONCLUSIONS: diffusion-weighted imaging in our patient indicated that the magnetic resonance imaging abnormalities of the affected cortex were due to cytotoxic edema caused by neuronal excitotoxicity during prolonged SE. diffusion-weighted imaging can be used in the localization of seizure focus for predicting the prognosis of the affected tissue and for researching the basic pathophysiology of SE.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
6/53. Periodic lateralized epileptiform discharges after complex partial status epilepticus associated with increased focal cerebral blood flow.Periodic lateralized epileptiform discharges (PLEDs) are typically associated with encephalitis, cerebral abscess, cerebral infarct, and status epilepticus. There is considerable debate as to whether this pattern is ictal or interictal when seen in association with status epilepticus. We present a patient with complex partial status epilepticus who developed PLEDs and remained comatose despite optimal drug therapy. technetium 99m single-photon emission computed tomography (SPECT) showed hyperperfusion that resolved with further aggressive antiepileptic drug therapy, indicating that this pattern may indeed be ictal. Further studies are needed to define the significance of PLEDs in patients with status epilepticus. The role of SPECT in differentiating PLEDs as an interictal or ictal pattern also requires further study.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
7/53. The use of topiramate in refractory status epilepticus.In cases of refractory status epilepticus (RSE) unresponsive to sequential trials of multiple agents, a suspension of topiramate administered via nasogastric tube was effective in aborting RSE, including one patient in a prolonged pentobarbital coma. Effective dosages ranged from 300 to 1,600 mg/d. Except for lethargy, no adverse events were reported.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
8/53. status epilepticus as a manifestation of hepatic encephalopathy.OBJECTIVES: seizures have been described as a rare manifestation of hepatic encephalopathy. MATERIAL AND methods: We present a 54-year-old female, with 6-year history of decompensated, hepatitis b liver cirrhosis, admitted with generalized seizures. She reported a history of recurrent episodes of hepatic encephalopathy, spontaneous bacterial peritonitis, tense ascites and variceal hemorrhage. neurologic examination revealed a comatose patient, without papilledema. Laboratory examinations were suitable with cirrhosis and mild renal failure. blood gas examination revealed severe metabolic acidosis and hypoxemia. plasma NH3 levels upon admission were twice normal. brain computed tomography and magnetic resonance imaging were normal. Electroencephalogram showed diffuse sharp waves, consistent with hepatic encephalopathy, grades III-IV. RESULTS: status epilepticus was refractory to continuous antiepileptic treatment. However, it was resolved after 24-h therapy with lactulose. blood NH3 levels were simultaneously normalized with clinical improvement. CONCLUSIONS: We consider the status epilepticus of our patient to be a rare manifestation of hepatic encephalopathy.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
9/53. Successful neurosurgical treatment of childhood complex partial status epilepticus with focal resection.The treatment of complex partial status epilepticus continues to be controversial, especially with regard to the intensity of the treatment. Medical therapy and drug-induced coma are sometimes required. Rarely this may not be effective. A healthy 4-year old girl was first seen in complex partial status epilepticus. She had a 1-year history of cryptogenic partial-onset seizures. Detailed magnetic resonance imaging (MRI) studies were normal. Her course was refractory to multiple medical therapies and multiple subpial transection (MST). An urgent epilepsy surgery evaluation resulted in a focal cortical resection being performed over the right mesial parietal region with resultant seizure freedom and no significant neurologic deficit 2 years later. This patient illustrates the need to consider occult focal cortical dysplasia as a cause of nonconvulsive status epilepticus (NCSE) in children, and if it is not responsive to medical management, the utility of performing an urgent epilepsy surgery evaluation.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
10/53. Use of an anesthesia cerebral monitor bispectral index to assess burst-suppression in pentobarbital coma.A seven-year-old child with generalized status epilepticus who was placed in a barbiturate coma was monitored with the bispectral index monitor in addition to the standard full channel electroencephalogram. This child had a low bispectral index number and high suppression ratio on the bispectral index monitor when the desired level of pentobarbital coma was induced. There was excellent correlation of the bispectral index monitor to the suppression ratio. The burst rate also correlated well to the bispectral index number and to the suppression ratio. Therefore the bispectral index monitor could allow the patient in barbiturate coma to leave the intensive care unit for diagnostic or therapeutic procedures and may one day replace the full-channel electroencephalogram in the management of patients in barbiturate coma.- - - - - - - - - - ranking = 8keywords = coma (Clic here for more details about this article) |
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