1/863. Serial electroencephalographic findings in patients with MELAS.To clarify the electroencephalographic characteristics of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), the medical records and electroencephalograms of six patients with MELAS and two of their relatives with MELA (mitochondrial myopathy, encephalopathy, and lactic acidosis, without strokelike episodes) were retrospectively reviewed. All have a point mutation in the mitochondrial dna at nucleotide position 3243. The electroencephalograms (n = 79) were divided into four groups according to the time relation to the strokelike episode: (1) before the first strokelike episode, (2) within 5 days after the strokelike episode (acute stage), (3) between 6 days and 1 month after the strokelike episode (subacute stage), and (4) more than 1 month after the strokelike episode (chronic stage). In the acute stage, 10 of the 11 electroencephalograms (9 strokelike episodes in four patients) revealed focal high-voltage delta waves with polyspikes (FHDPS), which were recognized as ictal electroencephalogram. Ictal events during FHDPS included focal clonic or myoclonic seizure and migrainous headache. In the subacute and chronic stages, focal spikes or sharp waves and 14- and 6-Hz positive bursts were frequently recorded. The authors' results suggest that FHDPSs present a reliable and accurate indicator of a strokelike episode in patients with MELAS.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
2/863. seizures induced by frustration and despair due to unresolved moral and political issues: a rare case of reflex epilepsy.We present a case of reflex-induced simple partial seizures, triggered by feelings of frustration, anger and despair. Such emotions were provoked by pondering over complex national and international, political and moral issues. The present case may suggest that activation of right temporal networks may mediate negative and adverse emotions induced by preoccupation with agitating, controversial issues.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
3/863. Kenny-Caffey syndrome: an Arab variant?We describe 2 unrelated Bedouin girls who met the criteria for the diagnosis of Kenny-Caffey syndrome. The girls had some unusual features--microcephaly and psychomotor retardation--that distinguish the Kenny-Caffey syndrome profile in Arab children from the classical Kenny-Caffey syndrome phenotype characterized by macrocephaly and normal intelligence. The 2 girls did not harbor the 22q11 microdeletion (the hallmark of the DiGeorge cluster of diseases) that we previously reported in another Bedouin family with the Kenny-Caffey syndrome (Sabry et al. J Med Genet 1998: 35(1): 31-36). This indicates considerable genetic heterogeneity for this syndrome. We also review previously reported 44 Arab/Bedouin patients with the same profile of hypoparathyroidism, short stature, seizures, mental retardation and microcephaly. Our results suggest that these patients represent an Arab variant of Kenny-Caffey syndrome with characteristic microcephaly and psychomotor retardation. We suggest that all patients with Kenny-Caffey syndrome should be investigated for the 22q11 microdeletion. Other possible genetic causes for the Kenny-Caffey syndrome or its Arab variant include chromosome 10p abnormalities.- - - - - - - - - - ranking = 21.118321756765keywords = chromosome (Clic here for more details about this article) |
4/863. Anatomical and embryological considerations in the repair of a large vertex cephalocele. Case report.The case of a neonate with a large vertex cephalocele is presented. The anatomical features of this anomaly were evaluated by means of magnetic resonance imaging and magnetic resonance angiography. Fusion of the thalami, dysgenesis of the corpus callosum, and failure of adequate formation of the interhemispheric fissure were characteristics of the major cerebral anomalies associated with the cephalocele. The absence of a falx in the midline, a split configuration of the superior sagittal sinus, and a dysgenetic tentorium with a concomitant abnormal venous drainage pattern were found in association with a large dorsal cyst. Repair of the anomaly was undertaken on the 3rd postnatal day. A cerebrospinal fluid shunt was required to treat hydrocephalus on Day 30. The child is well at age 3 years, but with significant developmental delay. The pathogenesis of this vertex cephalocele relates to semilobar holoprosencephaly and dorsal cyst formation. In addition, a disturbance in the separation of the diencephalic portion of the neural tube from the surface ectoderm or skin during the final phases of neurulation had occurred to help create the large cephalocele. Detailed preoperative imaging studies and awareness of the embryology and anatomy of this lesion facilitated the repair of the cephalocele. The prognosis of the child is determined not only by the presence of hydrocephalus, but also by the number of associated major cerebral anomalies. Options for treatment are discussed.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
5/863. Continuous EEG monitoring in the intensive care unit: early findings and clinical efficacy.The assessment of the neurocritical care patient involves serial assessment of neurologic status using bedside clinical examination and a variety of periodic neurophysiologic testing. Continuous electroencephalographic (CEEG) monitoring in the intensive care unit offers a unique means to track neurologic function directly and regionally. CEEG is becoming more widespread with a growing but small body of literature. The purpose of this paper is to outline the current experience with intensive care unit CEEG monitoring. The basic methods and caveats are discussed. We review the underlying rationale for using CEEG which is that secondary neurologic injury commonly occurs in the intensive care unit and at times is hard to detect. CEEG has a proven role in detecting secondary injuries, namely seizures and brain ischemia. The basic tenets of establishing clinical effectiveness for CEEG in the ICU are discussed while acknowledging a need for further study of clinical effectiveness. We review our initial clinical experience of CEEG in 300 patients and outline the clinical efficacy in terms of cost reduction and improvement in outcome (P < 0.01) using CEEG. Finally, several controversial aspects of CEEG are enumerated, and the need for additional study to answer these pressing questions is presented.- - - - - - - - - - ranking = 6keywords = ring (Clic here for more details about this article) |
6/863. A family with an atonic variant of paroxysmal kinesigenic choreoathetosis and hypercalcitoninemia.We report a family with an incompletely atonic variant of paroxysmal kinesigenic choreoathetosis (PKC). Three members of the family experienced attacks of muscle weakness which resembled the choreoathetotic attacks that occur in PKC in terms of their kinesigenicity and duration, clarity of consciousness during the attacks, good therapeutic response to low doses of phenytoin, and familial transmission, but differed from choreoathetotic attacks in PKC in that they were atonic. All three affected individuals were hypercalcitoninemic.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
7/863. Seizure-alert dogs--fact or fiction?Anecdotal evidence suggests that some dogs may be able to sense the onset of seizures and other medical conditions in humans, although this has never been explored scientifically. There is, however, evidence that dogs can be specially trained to recognize specific changes preceding a seizure and give an overt signal enabling the dog to warn his/her owner. The introduction of the use of dogs to detect and accurately predict the onset of a seizure, giving sufficient time for a person to take control of the situation will have a dramatic impact on quality of life. Support dogs, a registered charity which trains dogs to assist disabled people, has successfully trained several 'Seizure-alert dogs'. As training progressed, the dogs were able to provide overt signals to their owners within time periods varying from 15 to 45 minutes prior to a seizure occurring. Each dog had an accurate prediction time and, in each case, the owner's seizure frequency was reduced.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
8/863. Behenoyl cytarabine-associated reversible encephalopathy in a patient with acute myelogenous leukemia.We report a case of reversible encephalopathy syndrome in a 16-year-old girl with acute myelogenous leukemia (AML), who is undergoing during consolidation chemotherapy composed of BH-AC (N4-behenoyl-1-beta-D-arabinofuranosyl cytosine) and idarubicin. On the 6th day of chemotherapy, she was in a drowsy state following generalized tonic clonic seizure lasting 20 minutes. MR images revealed extensive cortical and subcortical white matter brain edema. Alertness returned over the 24 hr following by the discontinuation of BH-AC and intravenous administration of diphenylhydantoin, although she complained of intermittent headaches and visual disturbance. She gradually recovered from these symptoms during subsequent 7 days. Previously noted abnormal signal intensities have nearly disappreared on follow-up MRI obtained on the 22nd day after the first seizure. She was discharged without any neurologic sequela. This case suggests that BH-AC, a derivative of cytosine arabinoside (1-beta-D-arabinofuranosylcytosine) could be a cause of reversible encephalopathy syndrome.- - - - - - - - - - ranking = 2keywords = ring (Clic here for more details about this article) |
9/863. imipenem-induced seizure: a case of inappropriate, excessive, and prolonged surgical prophylaxis.imipenem-cilastatin is a broad spectrum antibiotic that is generally used for antibiotic-resistant hospital-acquired infections. imipenem has been reported to cause CNS toxicity including seizures in 1.5-10% of patients. The authors present a case in which imipenem, inappropriately utilized for surgical prophylaxis in excessive doses (1 gram every six hours) and for a prolonged period of time (24 days), induced a tonic-clonic generalized seizure in a patient with no history of seizure activity. The identification of this ADR lead to a number of clinical and administrative actions within our institution. Drug-induced seizures have occurred and will continue to occur as healthcare professionals exceed recommended dosing guidelines to treat critically ill patients. As pharmacists, it is extremely important to use therapeutic drug monitoring and pharmaceutical care principles to assist physicians in individualizing drug regimens for our patients to prevent these ADRs from occurring, thereby significantly effecting patients' outcomes.- - - - - - - - - - ranking = 2keywords = ring (Clic here for more details about this article) |
10/863. Bilateral femoral neck fractures secondary to hypocalcemic seizures in a skeletally immature patient.We report the case of an 11-year-old, skeletally immature boy who sustained bilateral femoral neck fractures during seizure activity. These fractures were due to hypocalcemia. Although this condition is a rare complication of seizures, its early recognition and treatment are essential to minimize complications associated with the delayed treatment of intracapsular hip fractures.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
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