Attitudes toward driver's licenses for people with epilepsy: 2012 versus 2014.

BACKGROUND: We compared knowledge about and attitudes toward epilepsy and the issuing of driver's licenses to people with epilepsy among non-medical students before and after media controversies. METHODS: The survey was performed in 2012 and 2014 using a structured questionnaire. Participants were non-medical students who attended a lecture on neurological diseases in children. The proportion of positive answers to each question in 2012 was compared with that in 2014. In addition, questions regarding attitudes toward driver's licenses were compared according to knowledge about car accidents linked to people with epilepsy. RESULTS: More participants were familiar with epilepsy and had a favorable attitude toward epilepsy in 2014 than in 2012. In contrast, the proportion of participants who knew of car accidents linked to people with epilepsy was reduced in 2014 compared with 2012. The proportion of participants who did not think that severe punishment should be given to people with epilepsy if they caused a car accident decreased in 2014 among those without knowledge of car accidents. CONCLUSIONS: Familiarity with and attitudes toward epilepsy were improved in 2014, whereas the decrease in proportion of positive answers on punishment among participants unfamiliar with car accidents suggests a latent worsening of public attitudes.

Worsening of attitudes toward epilepsy following less influential media coverage of epilepsy-related car accidents: An infodemiological approach.

To evaluate changes in the attitudes of nonmedical university students toward epilepsy in 2015, the present study compared the results of questionnaire surveys from four different time periods: before media coverage of epilepsy-related car accidents (2008-2010), during a period of abundant media coverage (2011-2012), after media coverage (2013-2014), and after novel media coverage (2015). The nonmedical students that completed the questionnaire were divided into four groups: 2008-2010, 2011-2012, 2013-2014, and 2015. The rates of students that had read or heard about epilepsy decreased significantly in 2015 compared with those in 2013-2014. Attitudes toward epilepsy had also worsened in 2015. The rates of students that would not oppose their children playing with or attending school alongside children with epilepsy and those who thought that people with epilepsy should be hired in the same way as other people had decreased significantly in 2015 compared with those in 2011-2012 and 2013-2014. Analyses of information-seeking behavior on the Internet showed that the increase in Google search volume and Wikipedia page views was much less in 2015 than in 2011 and 2012. These findings suggest that familiarity with epilepsy had worsened even after media coverage of novel epilepsy-related car accidents. This suggests that media coverage in 2015 was less influential than that in 2011 and 2012.

Clinically silent seizures in a neonate with tuberous sclerosis.

Although seizures during infancy in patients with tuberous sclerosis complex are common, seizures in neonates are infrequent. Here, we report the clinical course and electroencephalography (EEG) findings of a neonate with tuberous sclerosis complex associated with clinically silent seizures. The patient was a girl in whom cardiac tumors were detected on fetal ultrasonography. Brain magnetic resonance imaging during the neonatal period showed subependymal and cortical tubers. Routine EEG indicated unexpected ictal changes with no noticeable clinical symptoms. Ictal EEG was associated with a subtle increase in heart rate and a brief increase in chin electromyogram. These changes were difficult to identify clinically. The patient later developed focal seizures and epileptic spasms and had severe psychomotor delay. The present case suggests the occurrence of clinically silent seizures before the appearance of epileptic spasms in infants with tuberous sclerosis, and that EEG is an option for neonates with a prenatal diagnosis.

Sustained improvement of attitudes about epilepsy following a reduction in media coverage of car accidents involving persons with epilepsy.

To evaluate changes in the attitudes of nonmedical students about epilepsy, the present study compared the results of a questionnaire that was completed in three different time periods: before media coverage of car accidents associated with epilepsy, during a period of abundant media coverage about epilepsy-related accidents, and after media coverage of epilepsy-related accidents. The nonmedical students who completed the questionnaire were divided into three groups: Years 08-10 (preaccident era), Years 11-12 (media coverage era), and Years 13-14 (postmedia coverage era). The rates of students who had read or heard about epilepsy and of students who did not think that epilepsy was a mental disorder increased annually throughout the study period. There was an improvement in attitudes about epilepsy after the media coverage era, and this change was not altered even after a decrease in the media coverage of epilepsy-related car accidents. Additionally, the rate of positive answers did not differ between Years 11-12 and Years 13-14. These findings demonstrate that the familiarity with and improved attitudes about epilepsy were sustained even after the media coverage of car accidents involving persons with epilepsy had decreased.

Fulminant encephalopathy with marked brain edema and bilateral thalamic lesions.

We encountered two children with acute encephalopathy associated with unique clinical manifestations. Both the patients had status epilepticus at onset and neuroimaging studies revealed marked brain edema and bilateral thalamic lesions. Although they were treated with steroids and immunoglobulin, their outcomes were very poor. A thermolabile variant of carnitine palmitoyltransferase II and an elevated interleukin-6 level in cerebrospinal fluid were observed in one patient each. The constellation of clinical and neuroimaging findings in our patients is apparently not consistent with any established subtype of acute encephalopathy/encephalitis.

[Acute respiratory failures caused by post-tracheotomy tracheomalacia].

A 77-year-old man underwent thoracic surgery. He had a history of two previous operations: parapharyngeal tumor removal with temporal tracheotomy 14 years ago and, two years later, a sinus surgery when, according to our anesthesia registry, intubation was extremely difficult due to stricture of the trachea underneath the tracheotomy scar. Pathology was not fully elucidated. Preoperative examinations including chest x-ray, spirogram and CT were not remarkable. The scar above the suprasternal notch was visibly sunken and retracted with respiration. Stridor was auscultated but breathing was not labored. The patient was anesthetized with propofol and intubation was smooth. During surgery anesthesia was maintained with sevoflurane, remifentanil and rocuronium. However, extubation was followed by desperate gasping and severe respiratory distress. The tracheotomy scar caved in and the airway collapsed. Continuous airway pressure via a facemask restored airway patency and improved breathing. After overnight respiratory support with non-invasive positive pressure ventilation (NPPV), patient was weaned from ventilator. Airway collapse and the two episodes of respiratory failures while under general anesthesia were attributed to post-tracheotomy tracheomalacia.

Extubation and removal of supraglottic airway devices in pediatric anesthesia.

In pediatric anesthesia, respiratory adverse events often occur during emergence from anesthesia and at the time of endotracheal tube or supraglottic device removal. The removal of airway devices and extubation are conducted either while patients are deeply anesthetized or when patients awaken from anesthesia and have regained consciousness. The airways of children are easily irritated by external stimuli and are structurally prone to collapse, and the timing of both methods of airway device removal is similarly associated with various airway complications, including upper airway obstruction, coughing, or serious adverse events such as laryngospasm and desaturation. In current pediatric anesthesia practice, the choice of the timing and method of extubation is made by anesthesiologists. To achieve a smooth and safe recovery from anesthesia, understanding the unique characteristics of pediatric airways and the factors likely to contribute to an increased risk of perioperative complications remains essential. These factors include patient age, comorbidities, and physical conditions. The level of anesthesia and readiness for removal of airway devices should be evaluated carefully for each patient, and quick identification of airway problems and intervention is required if patients fail to maintain the airway and sufficient ventilation after removal of airway devices.

[Comparison of the Parker flex-tip tube with the standard tube for Airtraq intubation].

BACKGROUND: During tracheal intubation with the Airtraq laryngoscope (AL), the tracheal tube tip sometimes impinges on the glottis or other laryngeal structures. We tested a hypothesis that Parker flex-tip tube (PT) makes AL assisted tracheal intubation easier and faster than a conventional tube (Mallinckrod Hi-Lo tube : HT). METHODS: Study 1. Fifty patients were assigned to group PT (n = 25) or group HT (n = 25). After induction of general anesthesia, intubation was performed with AL. We recorded tube passage time, the number of tube impingements, circulatory changes and incidences of postoperative complications. Study 2. Twenty patients were divided into two groups and intubated either by HT or PT. We assessed the tip direction and contact points of each tube. RESULTS: Study 1. The use of PT significantly reduced the number of tube impingements compared to HT. Tube passage time in group PT was shorter than that in group HT, but the difference was not statistically significant. Study 2. The tube tip contact with the vocal cords was more frequent with HT. CONCLUSIONS: PT significantly reduced the number of impingements. The shape of tube tip and the smaller angle of PT when fitted in AL contributed to the relative ease of tube passage.

Sonoclot® predicts operation time and blood loss after cardiopulmonary bypass in children.

Background: As the circulating blood volume is relatively small in pediatric patients, blood components are quickly lost when bleeding, which make it more difficult to stop the bleeding. Particularly in pediatric cardiac surgery, loss of clotting factors associated with cardiopulmonary bypass (CPB) would likely to be prominent. As a result, bleeding is further promoted and the operation time is extended. In order to search for the relation between clotting factors and the amount of bleeding, we used a viscoelastic point of care test. Objectives: We used Sonoclot® as viscoelastic point of care test to evaluate coagulation function before CPB and before weaning from CPB in pediatric cardiac surgery. Design: Retrospective. Setting: Single-institutional. Participants: We included 55 pediatric patients (median age 13 months [IQR 5-32]) who underwent cardiac surgery under CPB from December 2015 to November 2016. Interventions: None. Measurements and main results: Sonoclot® analysis was performed after induction of anesthesia (pre-data, or baseline data) and before any heparinized saline was given, and right after modified ultrafiltration after weaning from CPB (post-data). Post-data was compared with post-CPB operation time and post-CPB blood loss by multiple regression analysis. Furthermore, effects of fresh frozen plasma (FFP) added on CPB on coagulation function and post-platelet function (describe as PFSC) was assessed. Activated coagulation time (describe as ACTSC) and clot rate (describe as CRSC) showed no significant change between baseline data and post-data. Post-PFSC was worsened by prolonged CPB time (p < 0.05) and correlated to bleeding amount and operation time after CPB (p < 0.05). Total amount of platelet concentrate (PC) transfused was higher in patients with smaller PFSC (p < 0.05). Total amount of FFP and PC transfused correlated with bleeding amount and operation time after CPB (p < 0.05). In the subgroup analysis, PFSC declined in the FFP-included group, whereas there was no significant difference in coagulation function. Addition of FFP to CPB did not significantly affect CR, whereas PFSC deteriorated as CPB time was prolonged (CPB time = 1/(0.0021∗PFSC + 0.0055)). Conclusion: Sonoclot® is useful to evaluate coagulation function in pediatric patients who undergo CPB. Preventive administration of FFP or PC in CPB circuit could reduce bleeding after CPB.

Extent of Leptomeningeal Capillary Malformation is Associated With Severity of Epilepsy in Sturge-Weber Syndrome.

BACKGROUND: Individuals with Sturge-Weber syndrome (SWS) often expereince intractable epilepsy and cognitive decline. We hypothesized that the extent of the leptomeningeal capillary malformation (LCM) may correlate with the severity of neurological impairment due to SWS. We tested the hypothesis in a cross-sectional study of seizure severity and electroencephalographic (EEG) findings and a retrospective cohort study for surgical indications related to the extent of the LCM. METHODS: We enrolled 112 patients and classified them according to LCM distribution: (1) bilateral, (2) hemispheric, (3) multilobar, and (4) single lobe. Age at seizure onset, seizure semiology and frequency, and EEG findings were compared. Surgical indications were evaluated for each group by Fisher exact test, and predictors for surgery were evaluated by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-Neurological Score (SWS-NS). RESULTS: The bilateral and hemispheric groups had early seizure onset (4.0 months old and 3.0 months old), frequent seizures (88.9% and 80.6% had more than one per month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). The groups' EEG findings did not differ substantially. Surgical indications were present in 77.8% of the bilateral, 88.1% of the hemispheric, and 46.8% of the multilobar groups. Seizure more than once per month was a predictor of surgical treatment. Seizure subscore improved postoperatively in the hemispheric and multilobar groups. Even after surgical treatment, the bilateral and hemispheric groups exhibited higher SWS-NSs than members of the other groups. CONCLUSION: Our study demonstrated a strong association between extensive LCM and epilepsy severity. Surgical intervention improved seizure outcome in patients with SWS with large LCMs.

[Four cases of latent aortic aneurysm--preoperative screening of aortic pathology is necessary for elderly patients].

We report four cases of latent aortic aneurysm revealed by contrast computed tomography (CT) before operations. The incidence of aortic diseases has not been publicized in Japan. However, the report of autopsies demonstrated that the elderly has a high incidence of aortic aneurysm and aortic dissection. Preoperative screening of aortic disease is necessary for elderly patients in order to perform appropriate perioperative managements. At present, contrast CT is the most reliable method for rapid detection of the complicated aortic disease.

[Awake intubation using lightwand technique under conscious sedation with remifentanil].

We experienced two cases of difficult airway due to deformities in the oral cavities. The first patient was a 62-year-old woman with a large benign soft palate tumor and the second was a 64-year-old woman with macroglossia secondary to acromegaly. Both patients were evaluated difficult to ventilate via face mask and presenting serious risks for tracheal intubation under general anesthesia. The tracheal intubation was planned using the lightwand (Trachilight) under conscious sedation with continuous administration of remifentanil. Remifentanil (0.1-0.25 microg x kg(-1) x min(-1)) maintained the patients' spontaneous ventilation and increased their tolerance to the pain and discomfort caused by insertion of the lightwand. In both patients, remifentanil mildly suppressed the coughing reflex as well as the autonomic responses to stimuli to the airway. Tracheal intubation was managed successfully in both cases and the operations were completed under general anesthesia. Although the patients were aware of being intubated, they could not recall the procedures postoperatively. The awake intubation technique using the lightwand under conscious sedation with remifentanil can be safely applied to a patient with difficult airway.

[Case of postoperative pulmonary embolism after tonsillectomy in a healthy young woman].

An 18-year-old healthy woman with no previous history of coagulation disorders underwent general anesthesia for tonsillectomy. The procedure was uneventful. After the surgery, she was ordered to rest on bed overnight. The next day, the patient experienced transient syncope followed by hypotension (< 60 mmHg) and severe dyspnea. Epinephrine 2 mg administration restored the blood pressure promptly, yet dyspnea persisted. The lung-perfusion scintigraphy showed upper-lobe perfusion defect in the left lung and she was diagnosed as having pulmonary embolism. She received low-molecular-weight heparin and warfarin therapy and recovered fully. The postoperative laboratory analysis did not show thrombophilic disorders or prothrombotic state. The pulmonary embolism was speculated to have occurred due to deep vein thrombosis which developed after postoperative immobilization. The prophylactic maneuvers such as elastic stockings were not applied to the patient preoperatively, who had been considered unlikely to develop deep vein thrombosis. Although deep vein thrombosis in children and adolescence are rare, postoperative children should be monitored carefully for thrombotic complications. Postoperative bed rest should be minimized in terms of prevention of thrombosis.

Focal seizures and epileptic spasms in a child with Down syndrome from a family with a PRRT2 mutation.

We describe a girl with Down syndrome who experienced focal seizures and epileptic spasms during infancy. The patient was diagnosed as having trisomy 21 during the neonatal period. She had focal seizures at five months of age, which were controlled with phenobarbital. However, epileptic spasms appeared at seven months of age in association with hypsarrhythmia. Upon treatment with adrenocorticotropic hormone, her epileptic spasms disappeared. Her younger brother also had focal seizures at five months of age. His development and interictal electroencephalogram were normal. The patient's father had had infantile epilepsy and paroxysmal kinesigenic dyskinesia. We performed a mutation analysis of the PRRT2 gene and found a c.841T>C mutation in the present patient, her father, and in her younger brother. We hypothesized that the focal seizures in our patient were caused by the PRRT2 mutation, whereas the epileptic spasms were attributable to trisomy 21.

Efficacy and tolerability of high-dose phenobarbital in children with focal seizures.

OBJECTIVE: We retrospectively reviewed the outcomes of children with focal epilepsy treated with oral high-dose phenobarbital. METHODS: We reviewed data on children (aged<15 years) with focal seizures treated with high-dose phenobarbital (>5 mg/kg/day to maintain a target serum level >40 µg/mL) for at least 6 months. Seizure frequency was evaluated after phenobarbital titration, and 1 and 2 years after high-dose phenobarbital treatment commenced. Treatment was judged effective when seizure frequencies fell by ⩾75%. RESULTS: Seven boys and eight girls were treated. The median age at commencement of high-dose phenobarbital therapy was 30 months. The maximal serum phenobarbital level ranged from 36.5 to 62.9 µg/mL. High-dose PB was effective in seven. In two patients, treatment was transiently effective, but seizure frequency later returned to the baseline. High-dose PB was ineffective in six. No significant association between effectiveness and any clinical variable was evident. Drowsiness was recorded in nine patients, but no patient developed a behavioral problem or hypersensitivity. CONCLUSION: Oral high-dose phenobarbital was effective in 7 of 15 patients with focal epilepsy and well tolerated. High-dose PB may be useful when surgical treatment is difficult.

Efficacy and safety of fosphenytoin for benign convulsions with mild gastroenteritis.

OBJECTIVE: To clarify the efficacy and safety of fosphenytoin for seizures in children with benign convulsions and mild gastroenteritis. METHODS: Using the mailing list of the Annual Zao Conference on Pediatric Neurology, we recruited patients who met the following criteria: (1) clinical diagnosis of benign convulsions with mild gastroenteritis and (2) treatment with intravenous fosphenytoin. Benign convulsions with mild gastroenteritis were defined as a condition of (a) seizures associated with gastroenteritis without electrolyte imbalance, hypoglycemia, or dehydration in patients (b) between 6 months and 3 years of age with (c) no preexisting neurological disorders, (d) no impaired consciousness, and (e) a body temperature less than 38.0 °C before and after the seizures. The efficacy of fosphenytoin was categorized as effective when cessation of seizures was achieved. RESULTS: Data from 16 child patients were obtained (median age, 20 months). Seizures were completely controlled after the initial dose of fosphenytoin in 14 of 16 patients. The median loading dose of fosphenytoin was 22.5 mg/kg. In 10 patients, fosphenytoin was administered after other antiepileptic drugs such as diazepam and midazolam were used. Adverse effects of fosphenytoin, excessive sedation, or intravenous fluid incompatibility were not observed in any patients. CONCLUSION: Fosphenytoin is effective and well tolerated among children with benign convulsions with mild gastroenteritis.

Anti-aquaporin 4 antibody-positive acute disseminated encephalomyelitis.

OBJECTIVE: To describe the clinical and neuroimaging features of a young female patient with acute disseminated encephalomyelitis associated with anti-aquaporin-4 antibodies. METHODS: The patient had mild encephalopathy 14 days after influenza vaccination. Cerebrospinal fluid analysis revealed an increased cell count and a marked increase in myelin basic protein. Magnetic resonance imaging (MRI) demonstrated multiple lesions in the juxtacortical white matter. The patient was diagnosed with acute disseminated encephalomyelitis and treated with methylprednisolone pulse therapy. She recovered in 1 month. However, right retrobulbar optic neuritis appeared 2 months after discharge, and serum anti-aquaporin 4 antibodies were measured with a cell-based assay. RESULTS: Anti-aquaporin 4 antibodies were present in the patient's serum. She was treated with a prolonged course of oral prednisolone. The patient was negative for serum anti-aquaporin 4 antibodies 8 months after the second clinical event, and prednisolone was discontinued 13 months after the second clinical event. Serum anti-aquaporin 4 antibodies remained negative 4 months after the discontinuation of prednisolone. There was no evidence of relapse at 9 months after discontinuation of steroids. CONCLUSIONS: This case will expand the spectrum of anti-aquaporin-4 antibody-related central nervous system disorders. The measurement of anti-aquaporin 4 antibody may be considered in patients with a clinical diagnosis of acute disseminated encephalomyelitis and a second clinical event within a short interval.

Amplitude-integrated EEG revealed nonconvulsive status epilepticus in children with non-accidental head injury.

OBJECTIVE: We describe the clinical course and amplitude-integrated EEG findings in three children with non-accidental head injury and discuss on the importance of continuous aEEG monitoring in infants. METHODS: NCSE was defined as a continuous 30-min seizure or briefer seizures occurring consecutively comprising at least 30 min of any 1-h period. Non-accidental head injury was diagnosed on the basis of neuroimaging findings such as subdural hemorrhage. Antiepileptic treatment was performed with continuous amplitude-integrated EEG monitoring. RESULTS: The age of the patients ranged from 48 days to nine months. All of them had loss of consciousness and seizures on presentation. Nonconvulsive status epilepticus without clinical symptoms were recognized in all patients. Vigorous antiepileptic treatment against nonconvulsive status epilepticus was made in two patients, whereas nonconvulsive status epilepticus disappeared within one hour without additional treatment in one. CONCLUSIONS: Our experience indicates that nonconvulsive status epilepticus were not uncommon in children with non-accidental head injury. Continuous amplitude-integrated EEG monitoring will be one of the useful methods in encephalopathic children in order to estimate seizure burden objectively and to treat seizures appropriately.