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Cardioembolic stroke: clinical features, specific cardiac disorders and prognosis.

Curr Cardiol Rev. 2010 Aug;6(3):150-61

Authors: Arboix A, Alió J

This article provides the reader with an overview and up-date of clinical features, specific cardiac disorders and prognosis of cardioembolic stroke. Cardioembolic stroke accounts for 14-30% of ischemic strokes and, in general, is a severe condition; patients with cardioembolic infarction are prone to early and long-term stroke recurrence, although recurrences may be preventable by appropriate treatment during the acute phase and strict control at follow-up. Certain clinical features are suggestive of cardioembolic infarction, including sudden onset to maximal deficit, decreased level of consciousness at onset, Wernicke's aphasia or global aphasia without hemiparesis, a Valsalva manoeuvre at the time of stroke onset, and co-occurrence of cerebral and systemic emboli. Lacunar clinical presentations, a lacunar infarct and especially multiple lacunar infarcts, make cardioembolic origin unlikely. The more common high risk cardioembolic conditions are atrial fibrillation, recent myocardial infarction, mechanical prosthetic valve, dilated myocardiopathy, and mitral rheumatic stenosis. Transthoracic and transesophageal echocardiogram can disclose structural heart diseases. Paroxysmal atrial dysrhyhtmia can be detected by Holter monitoring. In-hospital mortality in cardioembolic stroke (27.3%, in our series) is the highest as compared with other subtypes of cerebral infarction. In our experience, in-hospital mortality in patients with early embolic recurrence (within the first 7 days) was 77%. Patients with alcohol abuse, hypertension, valvular heart disease, nausea and vomiting, and previous cerebral infarction are at increased risk of early recurrent systemic embolization. Secondary prevention with anticoagulants should be started immediately if possible in patients at high risk for recurrent cardioembolic stroke in which contraindications, such as falls, poor compliance, uncontrolled epilepsy or gastrointestinal bleeding are absent.

PMID: 21804774 [PubMed - in process]

Usefulness of mild therapeutic hypothermia for hospitalized comatose patients having out-of-hospital cardiac arrest.

Am J Cardiol. 2011 Jul 15;108(2):173-8

Authors: Laish-Farkash A, Matetzky S, Oieru D, Sandach A, Levi N, Or J, Rieck J, Barsheshet A, Hod H

Abstract
Mild therapeutic hypothermia has proved beneficial after out-of-hospital cardiac arrest in the adult population, when the initial rhythm is ventricular fibrillation (VF). In this study, data from 110 consecutive patients with out-of-hospital cardiac arrest due to VF (n = 86) or to non-VF rhythm (n = 24), admitted to an intensive cardiac care unit with restoration of spontaneous circulation and who remained unconscious on admission, were analyzed. Patients were cooled using an external cooling system. Of the patients with VF, 66% had favorable outcomes (Glasgow-Pittsburgh Cerebral Performance Category 1 or 2), and 30% died. Of the patients with non-VF, 8% had favorable outcomes (p <0.001 vs VF), and 63% died (p = 0.004 vs VF). In patients with VF, those with poor outcomes were older than those with favorable outcomes (odds ratio [OR] 1.61, 95% confidence interval [CI] 1.03 to 2.7, p = 0.001) and had previous ejection fractions <35% (OR 7.72, 95% CI 1.8 to 33, p = 0.002). Outcomes were also worse when patients presented to the emergency room with seizures (OR 20.96, 95% CI 2.48 to 177.42, p = 0.003) or hemodynamic instability (OR 14.4, 95% CI 3.47 to 60, p <0.0001). In the non-VF group, the 2 patients with good outcomes were younger than those with unfavorable outcomes (39 ± 16 vs 65 ± 12 years, respectively, p = 0.04), with good left ventricular function on presentation (100% vs 4.5%, p = 0.0001) and with short asystole and/or short time from collapse to restoration of spontaneous circulation. In conclusion, mild therapeutic hypothermia in the adult population is more effective in patients with VF compared to those with non-VF. Good prognostic factors for patients with non-VF could be young age, good left ventricular function, and short anoxic time.

PMID: 21545984 [PubMed - indexed for MEDLINE]

A epilepsia é um distúrbio neurológico crônico, com repercussões psicossociais, físicas, educacionais e neuropsicológicas. OBJETIVOS: Verificar a percepção de professores do primeiro grau de Florianópolis/SC acerca da criança com epilepsia, e investigar sobre o nível de conhecimento dos mesmos sobre a epilepsia. MÉTODO: Utilizou-se "Questionário de percepção estigma para professores do primeiro grau" (Fernandes e Souza, 2004) em 37 professores do ensino fundamental de 4 escolas (3 particulares e 1 pública) de Florianópolis/SC. RESULTADOS E CONCLUSÃO: Verificou-se que a média de idade das participantes foi de 37 anos e 8 meses, tendo na sua maioria 3º grau completo. Os professores da escola pública apresentaram maior nível de instrução em relação às demais (X²=8.852, p=0.034). A maioria indicou conhecimento regular ou insuficiente sobre epilepsia. Acerca da autoavaliação sobre o nível de conhecimento de professoras, proteger a cabeça é importante (X²=9.931, p=0.033) e permanecer junto a criança. A maioria acredita que o aluno deve frequentar escola comum (n=35). Os professores ficaram divididos em relação a prática de atividades físicas. A maioria refere que a epilepsia é causada por fatores genéticos. A promoção de informação é válida, buscando inserção social e compreensão da condição da criança com epilepsia na escola.The epilepsy is a chronic neurological disturb, with psicossocial, physical, educational and neuropsychological repercussions. OBJECTIVES: To verify the perception of professors of the basic education Florianópolis/SC concerning the child with epilepsy, and to investigate the level of knowledge of the teachers about the epilepsy. METHOD: The "Questionnaire of perception stigma for professors of the first degree" (Fernandes and Souza,2004) was used in 37 professors of the basic education of 4 schools (3 private and 1 public) of Florianópolis/SC. RESULTS AND CONCLUSION: Was verified that the average of age of the participants was of 37 years and 8 months, having in its majority complete graduation on university. The teachers of the public school had greater level of instruction in relation to others (X ²=8.852, p=0.034). The majority indicated regular or insufficient knowledge (n=24) on epilepsy. Concerning the auto-evaluation on the level of knowledge of teachers, to protect the head is important (X²=9.931, p=0.033) and to remain next to child. The majority believes that the child must frequent school common (n=35). The professors had been divided in relation the practical of physical activities. The majority relates that the epilepsy is caused by genetic factors. The information promotion is valid, searching social insertion and understanding of the condition of the child with epilepsy in the school.

INTRODUÇÃO: Há um interesse crescente nas relações entre sono e epilepsia incentivado pela compreensão de que existem interações potencialmente relevantes nas duas direções. Embora o papel do sono na hipersincronização e a crescente preocupação na geração de crises sejam bem conhecidos, o grau no qual o sono pode facilitar ou induzir a um fenômeno epileptogênico, nas epilepsias lesionais, permanece indefinido. As epilepsias lesionais parecem apresentar um mecanismo particular de epileptogenicidade e o esclarecimento do papel da macro e microarquitetura do sono pode auxiliar na antecipação e monitorização de fenômenos epilépticos relacionados ao sono, conforme a etiologia da epilepsia. OBJETIVO: revisar e discutir as relações entre sono e epilepsia na infância e adolescência relacionando as alterações estruturais do sono à etiologia da epilepsia. MÉTODOS: revi-são bibliográfica utilizando o banco de dados Medline, abrangendo os estudos publicados nos últimos quinze anos, com as palavras-chave (unitermos) sono e epilepsia. CONCLUSÕES: epilepsia refratária durante a infância parece influenciar a organização do sono principalmente naqueles pacientes com etiologia lesional. A definição do tipo de epilepsia pode ser importante na antecipação dos distúrbios de sono nesta população.ABSTRACT INTRODUCTION: There has been a growing interest in the relations between sleep and epilepsy, kindled by the realization that there are many potentially relevant two-way interactions. Even though the hyper-synchronizing role of sleep and its attending increase in the probability of seizure generation are well known, the degree to which sleep may facilitate or induce epileptogenic phenomena in lesional epilepsies remains unclear. The lesional epilepsies seems to have intrinsic epileptogenic properties and the knowledge about sleep macro and microarchiteture could help clinician to anticipate and monitor sleep-related epileptic phenomena according to the etiology of the epilepsy OBJECTIVE: Discuss the relationship between sleep and epilepsy in childhood and adolescence. METHODS: Literature review in journals indexed through Medline, from the last 15 years. CONCLUSION: Refractory epilepsy during childhood influences sleep organization mainly in patients with lesional etiology. The definition of the type of epilepsy is important to anticipate sleep disorders in this population.

INTRODUÇÃO: O citoesqueleto é uma complexa rede de proteínas que determina a forma da célula. Ele é fundamental para que ocorra a movimentação celular; proporciona o suporte estrutural e mobilidade de organelas intracelulares e a estrutura para movimentação e separação de cromossomos durante a divisão celular. Os componentes principais do citoesqueleto são os microfilamentos, os filamentos intermediários e os microtúbulos. Os microtúbulos são formados por dímeros de α e β tubulina que se associam à proteínas específicas, as proteínas asssociadas aos microtúbulos (MAPs). A associação diferencial entre estas proteínas possibilita ampla variedade na modulação de função dos componentes do citoesqueleto no meio celular. As MAPs expressas no sistema nervoso central (SNC), MAP2 e tau, possuem diferentes isoformas geradas por processamento alternativo. O objetivo da presente revisão é de descrever e discutir as principais funções das proteínas do citoesqueleto em condições normais e patológicas, com destaque na fisiopatologia das epilepsias. RESULTADOS: As MAPs possuem funções essenciais nas células neuronais, agem principalmente na formação estrutural destas células, garantindo sua morfologia e regulando funções específicas. Alterações nos níveis de expressão de proteínas estruturais estão envolvidas em diversas patologias do SNC como a esquizofrenia, a epilepsia do lobo temporal, as displasias corticais e as desordens do desenvolvimento. Estudos com modelos animais de epilepsia e tecido humano proveniente de pacientes epilépticos têm mostrado que as crises epilépticas podem modificar a expressão das proteínas do citoesqueleto. CONCLUSÕES: Apesar do significativo conhecimento existente sobre o citoesqueleto e proteínas associadas aos microtúbulos, não se sabe exatamente os mecanismos responsáveis pelas modificações estruturais encontradas em algumas patologias. Além do papel bem estabelecido do citoesqueleto como componente estrutural e citoarquitetônico, sua participação como facilitador do tráfico intracelular de neurotransmissores e outras macromoléculas é função ainda a ser melhor explorada e compreendida.INTRODUCTION: The cytoskeleton is a complex network of protein fibers that determines the shape of cells. It is essential for the movements of cells and provides the structural support and movements of organelles within the cells, α and β the framework for moving and separating chromosomes during the cell division. The main components of the cytoskeleton are microfilaments, intermediate filaments and microtubules. Microtubules are assembled dimers of a and b tubulin that bind to specific proteins, the microtubules associated proteins (MAPs). Differential association between these proteins enables a wide variety in functional modulation of the cytoskeleton components in the cellular environment. The MAPs expressed in the central nervous system (CNS), MAP2 and tau, have different isoforms generated by alternative splicing. The aim of the present short review is to describe and discuss the key functions of cytoskeleton proteins in physiological and pathological states, mainly in the epileptic condition. RESULTS: The MAPs have critical roles in neurons, they act mainly on the structural formation of these cells, ensuring their morphology and regulating specific functions. Changes in the expression levels of structural proteins are involved in various CNS pathologies such as schizophrenia, temporal lobe epilepsy, cortical dysplasia and developmental disorders. Studies with animal models of epilepsy and human tissue from epileptic patients have shown that seizures can change the expression of cytoskeletal proteins. CONCLUSIONS: Despite the significant amount of knowledge on cytoskeleton and microtubules associated proteins, the precise mechanisms responsible for structural changes found in some pathological conditions are still not known. Besides the well-established role of the cytoskeleton as a structural and cytoarchitectural component, its participation in the facilitation of intracellular trafficking of neurotransmitters and other macromolecules is a function to be further explored and understood.

INTRODUCTION: Disturbances in intrauterine environment can have harmful effects on the fetus and pathological consequences persisting throughout adolescence and adulthood. Protein restriction during the prenatal period has a significant impact on growth and development of the central nervous system. Food restriction increases the risk of neurological disorders such as epilepsy. OBJECTIVE: To relate the programming model by malnutrition and its implications in experimental epilepsy. Material and methods: There has been research papers published in the databases Medline, PubMed, CAPES journals, ScienceDirect and Scielo. The keywords selected for the study included epilepsy, Status Epilepticus, pilocarpine, malnutrition, programming. RESULTS AND DISCUSSION: Several studies in animal models or humans highlights the possible adverse effects of malnutrition at the onset of epileptic seizures. The vulnerability immunological, biochemical and electrolyte abnormalities and hypoglycemia may be the factors responsible for the intensification of the epileptogenic process in malnourished individuals. CONCLUSION: Malnutrition negatively changes the epileptogenic circuitry.INTRODUÇÃO: Perturbações ao ambiente intrauterino podem ter efeitos prejudiciais sobre o feto e consequências patológicas persistentes ao longo da adolescência e da idade adulta. Restrição protéica durante o período pré-natal tem repercussões significativas sobre a ontogenia e o desenvolvimento do sistema nervoso central. Restrição alimentar nessa fase da vida aumenta o risco de distúrbios neurológicos como a epilepsia. OBJETIVO: Relacionar o modelo de programação pela desnutrição e suas implicações na epilepsia experimental. MATERIAL E MÉTODOS: Procedeu-se a pesquisa em artigos científicos publicados nos Bancos de Dados Medline, PubMed, Periódicos CAPES, ScienceDirect e Scielo. As palavras-chave selecionadas para a pesquisa incluíram epilepsia, status epilepticus, pilocarpina, desnutrição, programming. RESULTADOS E DISCUSSÃO: Diversos estudos realizados em modelos animais ou humanos destacam os possíveis efeitos adversos da desnutrição no início das crises epilépticas. A vulnerabilidade imunológica, alterações bioquímicas como anormalidades eletrolíticas e hipoglicemia podem ser os fatores responsáveis pela intensificação dos processos epileptogênicos em indivíduos desnutridos. Conclusão: A desnutrição altera negativamente a circuitaria epileptogênica.

A Epi-Brasil realiza várias atividades para fortalecer o movimento nacional de epilepsia. Uma delas é o Encontro Nacional de Associações e Grupos de Pacientes com Epilepsia, mostrado neste artigo. O evento foi realizado nos dias 18 e 19 de março de 2011 na cidade de Ipatinga, MG, contando com a participação de mais de 250 pessoas e 10 entidades. Como acontece todos os anos, o evento teve dois momentos principais: 1. Palestrantes debateram temas atuais sobre a epilepsia e 2. As associações puderam expor suas atividades, conquistas e dificuldades, reforçando a reflexão e a troca de experiências. Além disso, a Assembléia Geral da Epi-Brasil teve as seguintes resoluções: 1) tema escolhido para o próximo ano: "Queremos Políticas Públicas para a Epilepsia". 2) O X Encontro Nacional das Associações e Grupos de Pacientes em Epilepsia: realizado nos dias 16 e 17 de março de 2012, em Goiânia, sob a organização da ASPEG. 3) Fortalecer sempre o trabalho e a participação das associações na Federação, através inclusive da melhor troca de informações entre as mesmas. 4) Continuar com a cadeira no Conselho Nacional de Saúde e no Fórum Nacional de Patologias Crônicas. 5) Permanecer na luta para aprovar, com o Ministério da Saúde, a Minuta que institui um programa de epilepsia na atenção básica. Com isso, o evento foi encerrado na certeza de que estamos no caminho certo.Epi-Brazil performed some activities as a part of national movement of epilepsy in our country. The IX National Meeting of Associations and Support Group of People with Epilepsy was held in March 18-19th, 2011, in Ipatinga, MG, with the participation of over 250 people and 10 entities. The event was performed in two principal moments: 1. A board of specialists on epilepsy discussed important themes about epilepsy and 2. The epilepsy' associations presented their activities, difficulties and successes. The major resolutions of the General Assembly were: 1) "We want public policy for Epilepsy" was chosen as the central theme of the 2011 campaign; 2) X National Meeting of Associations and Support Group of People with Epilepsy will take place in March 16-17th of 2012, in Goiania-GO. 3) Reinforcement of involvement of associations in the Epi-Brazil, especially through better exchange of information. 4) Continuity in the Conselho Nacional da Saúde and Fórum Nacional de Patologias Crônicas. 5) Staying in the discussion with the Ministry of Health to pass a Law that establishes a National Program for Epilepsy in primary care.

Temporal changes in brain MRI findings in Rasmussen syndrome.

Epileptic Disord. 2011 Sep 15;

Authors: Yamazaki E, Takahashi Y, Akasaka N, Fujiwara T, Inoue Y

Abstract
Introduction. MRI data is essential for early diagnosis and evaluation of surgical indication in patients with Rasmussen syndrome (RS). In the present study, we examined the status and evolutionary changes in MRI lesions to identify the MRI characteristics of RS. Methods. MRI of 15 RS patients was examined regarding frequency and distribution of atrophic lesions on T1-weighted images and high intensity lesions on FLAIR or T2-weighted images. Results. In 13 patients, atrophic lesions were observed predominantly in the frontal lobes with various extent of involvement. High intensity lesions were also observed in 13 patients. High intensity lesions were significantly more prevalent in the cortex of patients with later onset and were present in the insula in 37.5% of epilepsia partialis continua (EPC) type patients and in 57.1% of non-EPC type. Early MRI showed various combinations of atrophic lesions or high intensity lesions in seven of nine patients who underwent MRI examinations within one year of their first seizure. Serial MRI revealed high intensity lesions with characteristic features of regression (20.0% of patients), fluctuation (regression followed by reappearance; 33.3%) and expansion (46.7%). Appearance and reappearance of high intensity lesions in the cortex and/or subcortical white matter were associated with aggravation of seizures. Bilateral high intensity lesions were observed in three patients with unilateral epileptogenic foci, who were successfully treated by surgical intervention. Conclusion. Dynamic evolutionary changes in lesions (regression, fluctuation and expansion of high intensity lesions), as observed on MRI, may be a diagnostic feature of Rasmussen syndrome.

PMID: 21926050 [PubMed - as supplied by publisher]

Transient parietal hand syndrome after cortical resection.

Epileptic Disord. 2011 Sep 15;

Authors: Oster JM, Apetauerova D, Thomas C, Cosgrove GR

Abstract
This case study with video recording describes a brief neurological examination of a rare post-surgical finding in a patient with intractable seizures who had a right parietal topectomy in order to cure focal and disabling epilepsy. Contemporaneously during the resection and while awake intraoperatively, the patient developed features characterised by involuntary, purposeless, and almost ballistic movements of the contralateral left upper extremity. These involved the shoulder and, more distally, the arm with less involvement of the hand itself and some clonic movement at the elbow, persisting for approximately 24 hours after surgery. Although identified in only one case in our series, we have named the resulting clinical phenomenology "parietal hand syndrome". A Medline search does not reveal any other such case in the English literature with the clinical elements and actual video documentation of neurological examination noted in our case report in the immediate post-operative setting. In this regard, this is a unique clinical report. [Published with video sequences].

PMID: 21926049 [PubMed - as supplied by publisher]

Side effects of phenobarbital in epilepsy: a systematic review.

Epileptic Disord. 2011 Sep 15;

Authors: Zhang LL, Zeng LN, Li YP

Abstract
Aim. In recent years, phenobarbital, as an antiepileptic drug, has become less popular based on adverse events, especially cognitive and behavioural side effects. Despite the development of better tolerated new generation AEDs, phenobarbital is still widely used particularly in developing countries because of its low cost. The purpose of this review was to: (i) investigate whether phenobarbital can be safely used as an antiepileptic drug and (ii) determine the questions which need to be addressed in order to comprehensively and adequately evaluate the safety of phenobarbital for the treatment of epilepsy. Methods. The literature was searched using the Cochrane Central Register of randomised controlled trials (1800-2009), Medline (1966-2009), Embase (1966-2009) and three Chinese databases. Results. Twenty studies were finally included in this systematic review. The determination of adverse effects of combined antiepileptic drugs (AEDs) from different studies was complicated by numerous factors including study design, different descriptions of adverse events and a lack of standardised data collection. These factors may also have been responsible for the heterogeneity present in the meta-analysis. The data did not demonstrate any evidence of association between phenobarbital and a higher risk of adverse events. However, phenobarbital appeared to be associated with a higher rate of adverse drug reaction related withdrawal (ADR-related withdraw), compared to carbamazepine, valproic acid and phenytoin. This may have been due to a concern for possible adverse effects of phenobarbital. Conclusions. Phenobarbital was associated with a higher rate of drug withdrawal although there was no evidence to suggest that phenobarbital caused more adverse events compared to carbamazepine, valproic acid or phenytoin. However, in the case of pregnant women, it is important for clinicians to evaluate the benefits and risks of phenobarbital administration before making a final recommendation. Furthermore, unified scales for the assessment of cognitive function should be applied for future studies particularly in children.

PMID: 21926048 [PubMed - as supplied by publisher]

Definition of the neurological phenotype associated with dup (X)(p11.22-p11.23).

Epileptic Disord. 2011 Sep 15;

Authors: Broli M, Bisulli F, Mastrangelo M, Fontana E, Fiocchi I, Zucca C, Bonaglia MC, Buono S, Musumeci SA, Romano C, Reitano S, Savio M, Vitello GA, Bernardi B, Cevolani D, Agati R, Poda R, Gallassi R, Giorda R, Zuffardi O, Bernardina BD, Seri M, Tinuper P

Abstract
The aim of this study was to describe in detail the neurological features of nine patients carrying the recently reported microduplication at Xp11.22-11.23. Clinical and neurological examination, brain magnetic resonance imaging (except for two patients), electroencephalography and a neuropsychological assessment specific for language disturbances were performed in nine patients with microduplication at Xp11.22-11.23, disclosed by comparative genomic hybridisation array. Six patients were familial cases belonging to three unrelated pedigrees and three were sporadic cases. The patients had the following characteristics: mild dysmorphic facial features (except for two patients), mental retardation with moderate to severe global language deterioration, electroencephalographic epileptiform discharges during wakefulness and especially during sleep or electrical status epilepticus during slow sleep in younger cases, and negative brain magnetic resonance imaging. The main clinical features of this new microduplication syndrome were mild facial dysmorphisms, from increased electroencephalogram abnormalities during sleep to electrical status epilepticus during slow sleep, and mental retardation mainly involving language function in the absence of detectable brain lesions. In the absence of detectable brain lesions, speech delay may be associated with electrical status epilepticus during slow sleep or, alternatively, related to abnormal brain expression of a dosage-sensitive gene contained within the duplication region.

PMID: 21926047 [PubMed - as supplied by publisher]

A new form of alpha-dystroglycanopathy associated with severe drug-resistant epilepsy and unusual EEG features.

Epileptic Disord. 2011 Sep 13;

Authors: Di Rosa G, Messina S, D'Amico A, Bertini E, Pustorino G, Spanò M, Tortorella G

Abstract
We describe two unrelated girls with congenital muscular dystrophy associated with alpha-dystroglycan deficit with no identified genetic defect, both presenting severe drug-resistant epilepsy with predominant myoclonic seizures and an unusual similar EEG pattern. Severe epilepsy has been unusually described in patients with congenital muscular dystrophies, mainly associated with Walker-Warburg, Fukuyama and muscle-eye-brain diseases. [Published with video sequences].

PMID: 21914590 [PubMed - as supplied by publisher]

Quality of life in patients with partial-onset seizures under adjunctive therapy with zonisamide: results from a prospective non-interventional surveillance study.

Epileptic Disord. 2011 Sep 13;

Authors: Helmstaedter C, Stefan H, Witt JA

Abstract
This prospective, multicenter, non-interventional surveillance study (ZADE study) explored seizure outcome and tolerability of adjunctive treatment with zonisamide (ZNS) in a non-selected sample of patients with partial-onset seizures in everyday clinical practice. Changes in quality of life (QOL) and health status were also recorded. Clinical status was assessed before and 4 months after introduction of ZNS. The herein reported evaluation of QOL and health status was based on a representative subsample of 207 patients. In this subgroup, a reduced QOL had been apparent in 68% of patients at baseline. After introduction of ZNS, all measures improved, with ameliorations in QOL in up to 35% of patients. Major determinants for a better QOL outcome were (1) a better score at baseline, (2) a higher degree of seizure reduction, and (3) a lower number of concomitant AEDs. Tolerability was subjectively rated as good by 89% of patients. With a ZNS dose of 244.8±108 mg/d at study end, seizure frequency had dropped from 8.8±19.2 within 8 weeks before baseline to 3.6±9.1 seizures within the period of 8 weeks before study end. A total of 79% of patients responded to ZNS treatment with a ≥50% reduction in seizure frequency; 34% became seizure free. In conclusion, adjunctive treatment with ZNS seems to be efficacious and well tolerated. QOL improvement was predicted by baseline score, seizure outcome, and overall drug load, and is thus more likely a result of enhanced seizure control, rather than an intrinsic psychotropic effect of zonisamide.

PMID: 21914589 [PubMed - as supplied by publisher]

Lamotrigine is favourable for startle-induced seizures.

Epileptic Disord. 2011 Sep 6;

Authors: Ikeda H, Imai K, Ikeda H, Shigematsu H, Shishido T, Takayama R, Fujiwara T, Takahashi Y, Inoue Y

Abstract
Falling due to startle-induced seizures (SISs) often leads to injury. The triggers of SIS are mostly unexpected auditory stimuli, which are too common to avoid in daily life. As SISs are often refractory to conventional medications, effective therapeutic options have to be established. We report a small series of six patients treated with lamotrigine (LTG) as add-on therapy. Seizure control was improved greatly in three of the six patients, resulting in less restricted daily life, but no effect was observed in two and a skin rash developed in one. Patient 1 was a 19-year-old man. His seizure comprised of a sudden tonic extension of the extremities induced by auditory or visual stimulus. He fell down due to SISs, five to ten times a day, with frequent injuries. After adding LTG to treatment with valproate (VPA) and clobazam (CLB), SISs were reduced to once a month. Patient 2 was a 51-year-old woman. Sudden tonic extension of all limbs induced by unexpected sounds frequently threw her down onto the floor. Addition of LTG to treatment with CLB, zonisamide and phenytoin reduced her SISs from several to less than once a day. Patient 3 was a seven-year-old girl with post-encephalitic epilepsy. After adjunctive treatment of LTG to VPA, the severity of SISs became milder thus avoiding injury, although seizure frequency did not decrease. LTG is potentially effective for the treatment of SISs and may prevent falling. The addition of LTG treatment dramatically improved the lives of the patients presented here and should be considered as an option for startle-induced seizures.

PMID: 21896426 [PubMed - as supplied by publisher]

Non-convulsive status epilepticus of frontal origin as the first manifestation of Hashimoto's encephalopathy.

Epileptic Disord. 2011 Sep 6;

Authors: Monti G, Pugnaghi M, Ariatti A, Mirandola L, Giovannini G, Scacchetti S, Nichelli P, Meletti S

Abstract
Hashimoto's encephalopathy is an often misdiagnosed, life threatening, condition which improves promptly with steroid therapy. Since clinical manifestations are heterogeneous and non-specific, the diagnosis is often difficult. Several case reports of Hashimoto's encephalopathy presenting with partial or generalised seizures are described, but only a few have focused on status epilepticus as the first clinical manifestation. We report two patients presenting with repetitive and prolonged seizures characterised by progressive reduction in contact and reactivity associated with frontal/diffuse polyspike-and-wave activities. This condition, which can be interpreted as a form of non-convulsive status epilepticus (NCSE) of frontal origin, was refractory to antiepileptic drugs but responded promptly to high doses of intravenous steroid treatment. In cases of unexplained encephalopathy with EEG documentation of NCSE, the early recognition and treatment of Hashimoto's encephalopathy may lead to a favourable prognosis. [Published with video sequences].

PMID: 21896425 [PubMed - as supplied by publisher]

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

Epileptic Disord. 2011 Sep 6;

Authors: Ferrari TP, Hamad AP, Caboclo LO, Centeno RS, Zaninotto AL, Scattolin M, Carrete Junior H, Lancellotti CL, Yacubian EM

Abstract
A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].

PMID: 21896424 [PubMed - as supplied by publisher]

Recurrence of childhood absence epilepsy as pyknolepsy in adolescence.

Epileptic Disord. 2011 Sep 6;

Authors: Yozawitz EG, Scantlebury MH, Robinson J, Jehle R, Moshé SL

Abstract
A developmentally normal adolescent boy with a history of childhood absence epilepsy presented with recurrence of pyknolepsy after a seven-year period of remission. The characteristics of his EEG showed the same 3-Hz generalised spike-wave discharge as in his previous EEG in childhood. To our knowledge, this is the first case report describing recurrence of childhood absence epilepsy as pyknolepsy in an adolescent. [Published with video sequences].

PMID: 21896423 [PubMed - as supplied by publisher]

Bilaterally independent epileptic spasms in a case of Aicardi syndrome.

Epileptic Disord. 2011 Aug 26;

Authors: Kobayashi K, Watanabe K, Yoshinaga H, Ohtsuka Y

Abstract
A girl with Aicardi syndrome was observed to have two distinct types of asymmetric epileptic spasms, as detected by ictal video-EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating spasms. [Published with video sequences].

PMID: 21873145 [PubMed - as supplied by publisher]

Perioral myoclonia with absences and myoclonic status aggravated by oxcarbazepine.

Epileptic Disord. 2011 Aug 26;

Authors: Vrielynck P, Rostomashvili N, Degroote E, Ghariani S, van Rijckevorsel K

Abstract
Perioral myoclonia with absences belongs to the "idiopathic generalised epilepsy syndromes in development", currently not yet cited in the ILAE classification. This epilepsy syndrome is associated with a seizure type that appears to be specific. Here, we report polygraphic recordings of this seizure type in a young boy, previously misdiagnosed with focal epilepsy. EEG and clinical features were useful to differentiate diagnosis of his seizures from other absence or myoclonic seizures. Interestingly, some seizures were associated with neck myoclonia. Home video recording of myoclonic status aggravated by inappropriate treatment is also presented. [Published with video sequences].

PMID: 21873142 [PubMed - as supplied by publisher]

A case imitating Panayiotopoulos syndrome.

Epileptic Disord. 2011 Aug 24;

Authors: Bolukbasi F, Hamamcioglu I, Demirbilek V, Dogangun B, Dervent A

Abstract
Panayiotopoulos syndrome is one of the most common childhood-specific epileptic disorders. It is characterized by autonomic symptoms; the most common being emesis. However, the presence of emetic symptoms may cause misdiagnosis, for example with some organic disorders which have similar autonomic features. On the other hand, since this syndrome has been recently well documented, the tendency for early recognition may lead to overdiagnosis. Here, we present a case which could be mistaken for Panayiotopoulos syndrome based on anamnesis, however, with the aid of ictal video-EEG monitoring, the patient was shown to present with a non-epileptic psychogenic seizure. This report is an example of careful evaluation in order to avoid over- or underdiagnosis of this benign disorder. [Published with video sequences].

PMID: 21865129 [PubMed - as supplied by publisher]