European Journal of NeurologyVolume 15, Issue 4 p. 317-319 Improving the diagnostic yield in Panayiotopoulos syndrome A. Covanis, A. Covanis Neurology Department, The Children Hospital "Agia Sophia", Athens, GreeceSearch for more papers by this authorC. P. Panayiotopoulos, C. P. Panayiotopoulos Department of Clinical Neurophysiology and Epilepsies, St Thomas' Hospital, London, UKSearch for more papers by this author A. Covanis, A. Covanis Neurology Department, The Children Hospital "Agia Sophia", Athens, GreeceSearch for more papers by this authorC. P. Panayiotopoulos, C. P. Panayiotopoulos Department of Clinical Neurophysiology and Epilepsies, St Thomas' Hospital, London, UKSearch for more papers by this author First published: 18 March 2008 https://doi.org/10.1111/j.1468-1331.2008.02079.xCitations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume15, Issue4April 2008Pages 317-319 RelatedInformation

Die kongenitale Hemiplegie ist eine typische Form der infantilen Zerebralparese. Ihre Häufigkeit wird auf 0,41–0,79/1000 lebend Geborener geschätzt. In der vorliegenden Studie wurden 223 Patienten (122 Jungen, 101 Mädchen) mit kongenitaler Hemiplegie im Alter von 3 Monaten bis 12 Jahren analysiert. Von den Patienten litten 31% an einer leichten, 48% an einer mittelschweren und 21% an einer schweren Hemiplegie. Bei mehr als der Hälfte der Patienten war die obere Extremität betroffen, bei einem Drittel die untere und bei 20% die obere und untere. Die Mehrheit der Patienten (75,8%) zeigte EEG-Veränderungen und 38,6% dieser Patienten entwickelten eine Epilepsie. Der häufigste Anfallstyp waren komplexe Partialanfälle (33%). Der Schweregrad der motorischen Behinderung, der Grad der EEG-Veränderungen und die Häufigkeit einer Epilepsie zeigten eine statistisch signifikante Korrelation. Das Ausmaß von in der Bildgebung nachgewiesenen Hirnsubstanzverlusten stand wiederum in Beziehung zu den 3 vorgenannten klinischen Variablen, insbesondere bei der großen Gruppe von Kindern mit Rinden-Mark-Defekten (84,2%). Von den Sehstörungen war der Strabismus mit 17% am häufigsten; Hördefizite wurden nur bei 8% der Kinder registriert. 38,3% der Patienten zeigte eine normale Intelligenz. Den niedrigsten Intelligenzquotienten wiesen Kinder mit schwerer kongenitaler Hemiparese auf.

Congenital hemiplegia, defined as unilateral motor disability, is the hemiplegic type of cerebral palsy. The prevalence of congenital hemiplegia is estimated to be about 0.41-0.79/1000 live births. We examined 223 children (122 boys and 101 girls) suffering from congenital hemiplegia at the age of 3 months to 12 years. Mild hemiplegia was found in 31%, a moderate form in 48%, and a severe form in 21%. The upper limb was affected in more than half of the patients, only the lower extremity in one third, and both upper and lower limbs in 20%. Electroencephalographic abnormalities were found in 75.8% of the patients. The most frequent type of epilepsy was complex partial seizures (33%). Severity of the motor handicap, grade of EEG abnormalities, and the prevalence of epilepsy showed a significant correlation. The magnitude of the lesions in neuroimaging directly correlated with these three clinical variables, particularly in children with cortical and subcortical defects (84.2%). Strabismus was the most common visual impairment (17%), while hearing impairment was found in 8% of the patients. Of them, 38.3% showed no cognitive deficits, while those with severe congenital hemiplegia were found to have a lower intelligence quotient.

Photosensitivity is an abnormal visual sensitivity of the brain in reaction to flickering light sources or patterns and is expressed in the electroencephalogram as generalized spike-and-wave discharge and in more susceptible individuals as clinical seizures. The most common types of seizures are generalized tonic-clonic, followed by myoclonic and absence. The photosensitive epilepsies are classified as pure photosensitive, where seizures occur only with the flickering light source/pattern or during intermittent photic stimulation (IPS) in the laboratory, and epilepsy with photosensitivity, where spontaneous seizures also occur. Positive response to IPS in idiopathic epilepsy syndromes, which are included in the International Classification or are in development, is reported to range from 7.5% in juvenile absence epilepsy to 100% in pure photosensitive epilepsy.The treatment of photosensitivity and pure photosensitive epilepsy with rare seizures includes general and specific protective measures. For most patients, however, combination treatment with antiepileptic drugs is necessary. Valproic acid monotherapy has a success rate of 73-86%. Levetiracetam appears to be a new alternative therapeutic option. Clobazam, lamotrigine, ethosuximide, and topiramate also have been recommended as second-choice therapies.

To the Editor: I was surprised that recently published practice parameters did not contain conflict of interest disclosure statements.1,2 Authors of American Academy of Neurology (AAN) practice parameters should disclose their potential conflicts of interests. This is particularly relevant for practice parameters that review pharmaceutical agents or procedures in which the authors have a potential financial conflict of interest. The disclosures should be broad and include all types of relationships with sponsoring companies including equity holdings, contracted research, and various consulting relationships. Given the stature of the practice parameters in influencing practice across multiple specialties, consideration should be given for a non-zero limit required for disclosure. In addition, donation of remuneration from a potentially conflicting sponsor to a not-for-profit organization (e.g., charitable organization, university discretionary account, or equivalent) should not preclude the need to disclose. Disclosure statements should also be considered for authors whose salaries are directly linked to the volume of procedures or devices that they are reviewing. Although not well studied, one would speculate that under such circumstances, “self-serving” bias might influence how an author seeks, interprets, and reports available evidence, as likely occurs with gifts.3 Consideration should also be given to the AAN itself to disclose its potential financial conflicts of interest when the sponsors of reviewed drugs or procedures have donated substantial amounts to the organization, even if only as unrestricted educational grants. The AAN has already made the list of not-for-profit organizations that have substantial ties to industry4 and, I suspect, wants to avoid the negative publicity that could result from an inquisitive investigative journalist.5 As the public demands more accountability of the scientific enterprise, and financial conflicts of interest receive continued attention at the national level, including the recently issued guidance statement by the Department of Health and Human Services,6 it will be important for neurologists to take a proactive position, affirming to the public and patients that they are our primary interests and no others. Disclosure statements for issued practice guidelines should be one component of this affirmation. The effects of corporate sponsorship and potential conflicts of interest on the conduct and reporting of randomized controlled trials have received considerable attention.7,8 Similar scrutiny for the development and reporting of clinical practice guidelines has only just begun,9 but will likely increase (and should increase) given their potential to shape clinical practice, particularly those in training. Potential conflicts of interest are so pervasive that when disclosure statements are not present, one could not be faulted for assuming that someone has something to hide. On April 26th, 2004, near the time of the release of the practice parameters addressing the new antiepileptic drugs (AEDs), I received an electronic message from the AAN, which included the following: “These new evidence-based guidelines are the most comprehensive, unbiased review of the newest epilepsy drugs.” Even though I thought the guidelines were excellent, the lasting impact of the guidelines for me was “Methinks he doth protest too much,” and a wish that disclosure statements were present both for the authors and for the AAN.

Aims To evaluate the safety and efficacy of intravitreal injection of liposomes encapsulating infliximab in experimental autoimmune uveoretinitis (EAU) rats. Methods Liposomes containing infliximab were prepared and characterised for mean particle size, entrapment efficiency, polydispersity index (PDI) and zeta potential. In vitro release profile and the stability of infliximab-lip were evaluated. EAU rats were intravitreally injected with saline, infliximab, infliximab-lip or unloaded liposomes. Clinical signs and ocular histological sections were graded. Infliximab concentrations were determined with competitive ELISA. Safety of the intravitreal injections was evaluated by electroretinography (ERG) and histopathological examination. Retinal biodistribution and clearance of rhodamine-conjugated liposomes containing infliximab were evaluated with a laser scanning confocal microscope. Results The mean particle size of infliximab liposomes was 351.3±58 nm and entrapment efficiency was 90.65%±2.68%. PDI and zeta potential of infliximab liposomes were 0.386 and −20.8±9.78 mV, respectively. Stability test data showed that the infliximab-lip was stable for 60 days at room temperature. In EAU rats, intravitreal injection of infliximab and infliximab-lip greatly reduced intraocular inflammation determined by clinical scores and histopathological analyses (n=4). The mean concentrations of infliximab decreased quickly in infliximab injection group and were lower than those in infliximab-lip injection group (n=4 eyes, p<0.05 after 3 days post injection). No retinal toxic effects were detected after intravitreal injection of infliximab-lip in ERG (n=4 rats, p>0.05) and histopathological sections compared with normal rats. Confocal microscopy showed that fluorescent liposomes were observed in almost every layer of the retina and remained detectable for >30 days after injection. Conclusions Intravitreal injection of liposomal infliximab can prolong the persistence of the drug in vitreous body and demonstrated a satisfactory safety and significant therapeutic potentials in EAU. The use of biodegradable particles for therapeutic antibody delivery may provide a promising approach for the treatment of ocular diseases.

Efficacy and tolerability of the new antiepileptic drugs : commentary on the recently published practice parameters.

Acta Neurologica ScandinavicaVolume 108, Issue s180 p. 3-4 Epilepsy and comorbidity: a global approach to patient management E. Ben-Menachem, E. Ben-Menachem Department of Clinical Neuroscience, Neurology Division, Sahlgrenska University Hospital, Gothenburg, SwedenSearch for more papers by this authorA. Covanis, A. Covanis The Children's Hospital ’Aghia Sophia’, Department of Neurology/Neurophysiology, Athens, GreeceSearch for more papers by this author E. Ben-Menachem, E. Ben-Menachem Department of Clinical Neuroscience, Neurology Division, Sahlgrenska University Hospital, Gothenburg, SwedenSearch for more papers by this authorA. Covanis, A. Covanis The Children's Hospital ’Aghia Sophia’, Department of Neurology/Neurophysiology, Athens, GreeceSearch for more papers by this author First published: 26 September 2003 https://doi.org/10.1034/j.1600-0404.108.s180.1.xCitations: 1 Elinor Ben-Menachem, Department of Clinical Neuroscience, Neurology Division, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden Tel.: + 46 31 3422483 e-mail: elinor.ben-menachem@neuro.gu.se Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume108, Issues180October 2003Pages 3-4 RelatedInformation

Centrotemporal spikes are the EEG marker of Rolandic epilepsy, while ictus emeticus is one of the main seizure manifestations of Panayiotopoulos syndrome. Ictus emeticus has not been reported in Rolandic epilepsy. Out of a population of 1340 children with focal afebrile seizures we studied 24 children who had emetic manifestations in at least one seizure and centrotemporal spikes in at least one EEG. They were of normal neurological status and had a follow-up of at least two years after the last seizure. All children had sleep EEG following sleep deprivation. Two groups of patients were identified. Group A (12 patients) with EEG centrotemporal spikes only and group B (12 patients) with centrotemporal spikes and spikes in other locations. In 21 patients, ictal emetic manifestations culminated in vomiting and in three only nausea or retching occurred. The commonest presentation was ictus emeticus at onset followed by deviation of the eyes or staring, loss of contact and floppiness. In 79%, seizures occurred during sleep. Autonomic status epilepticus occurred in 37.5%. The mean age at onset was 5.3 years. Overall analysis of the clinical and EEG data points out that the vast majority of these patients primarily suffer from Panayiotopoulos syndrome. Twenty patients (83%) had ictal semiology typical of Panayiotopoulos syndrome, but five also had concurrent Rolandic symptoms and four later developed pure Rolandic seizures. The other four patients (17%) had typical Rolandic seizures with concurrent ictus emeticus. These findings suggest a link between Rolandic epilepsy and Panayiotopoulos syndrome, the two most important phenotypes of the benign childhood seizure susceptibility syndrome.

Summary: Purpose: To determine prevalence, clinical, EEG features, and prognosis of Panayiotopoulos syndrome and to examine the proposition that clinical manifestations are more important than EEG findings.Methods: We analyzed retrospectively the clinical and EEG records of 1,340 children with one or more focal seizures seen in the last 18 years, supplemented with a prospective study from 1998. Panayiotopoulos syndrome was defined by clinical criteria, mainly ictal emesis, irrespective of EEG findings.Results: We analyzed 43 of 90 patients with Panayiotopoulos syndrome who were seizure free >2 years. Girls predominated. Mean age at first seizure was 5 years. Seizures consisted mainly of autonomic manifestations; ictal emesis was often the first symptom, culminating in vomiting in 86%. Of nonautonomic manifestations, lateral eye deviation was the most common; visual symptoms were exceptional. Impairment of consciousness ensued in all seizures, half of which ended with hemi or generalized convulsions. Nearly 46.5% of cases had at least one seizure >30 min, constituting autonomic status epilepticus. Seizures during sleep (84%) were more common than those in wakefulness. EEG showed occipital spikes in 29 patients. Of the other 14 cases, five had extraoccipital abnormalities or brief generalized discharges, and nine had normal awake and sleep EEG. Prognosis was excellent. All 43 children have been free of seizures for ≥2 years, 53% having a single seizure, and 47%, an average two to three seizures.Conclusions: Panayiotopoulos syndrome is common and needs wider recognition. EEG shows occipital or extraoccipital abnormalities, is normal in one third of patients, and does not determine clinical manifestations or prognosis, which is excellent despite the high prevalence of lengthy seizures.

Purpose: A recent genome-wide scan revealed a major susceptibility locus for idiopathic generalized epilepsies (IGEs) in the chromosomal region 8p12 in 32 IGE families without members with juvenile myoclonic epilepsy (JME). This study explored the presence of an IGE locus in the chromosomal region 8p12.Methods: Our study included 176 multiplex families of pro-bands with common IGE syndromes. Parametric and nonparametric multipoint linkage analyses were carried out between the IGE trait and six microsatellite polymorphisms encompassing the putative susceptibility locus. To explore the associated phenotype-genotype relation, two distinct subgroups of families were selected by the presence (n = 64) or absence (n = 112) of a family member with JME. To adjust the phenotypic spectrum toward adolescent-onset IGEs, a third subgroup of 28 families without JME was chosen through an IGE proband with seizure onset at age 10-20 years.Results: Parametric and nonparametric multipoint linkage analyses provided no evidence for linkage between IGE and markers encompassing the putative IGE locus in the chromosomal region 8p12. Furthermore, we found no hint of linkage along the candidate region in any of the three family subgroups.Conclusions: We failed to provide evidence for a major IGE locus in the chromosomal region 8p12. On the contrary, these parametric linkage results provide strong evidence against linkage across the candidate region under a broad range of genetic models. If there is a susceptibility locus for IGE in the chromosomal region 8p12, then the size of the effect or the proportion of linked families is too small to detect linkage in the investigated family sample.