Epilepsia refleja por agua caliente: a propósito de un caso en un lactante / Hot-water epilepsy: case report in an infant

La epilepsia refleja por agua caliente es un tipo de convulsión poco frecuente cuya fisiopatología se desconoce. Estas crisis reflejas suelen iniciarse pocos segundos tras el contacto con el agua y, comúnmente, a temperaturas de 37-48 ºC. Los automatismos y las crisis parciales complejas, con o sin generalización secundaria, son el modo de manifestación principal.La exploración neurológica y el electroencefalograma intercrítico no suelen presentar alteraciones, lo que puede condicionar un retraso en el diagnóstico, por lo que es fundamental la sospecha clínica.El tratamiento antiepiléptico se inicia cuando se asocia a otro tipo de epilepsia o cuando ciertas medidas sencillas, como el descenso de la temperatura del agua en el baño, no controlan las crisis. Es posible la desaparición espontánea. Cuando es necesaria la terapéutica farmacológica, existe, normalmente, buena respuesta.Se presenta el caso de un lactante con diagnóstico de epilepsia refleja por agua caliente.

Hot-water epilepsy is a rare type of seizure whose pathophysiology is unknown. These reflex seizures usually begin a few seconds after contact with water, commonly at temperatures between 37-48 ºC. Automations and complex partial crises, with or without secondary generalization, are the main manifestation mode of this type of reflex epilepsies.Neurological examination and intercritical electroencephalography are usually normal, which may condition a delay in diagnosis, and the clinical suspicion is fundamental.Antiepileptic treatment is initiated when associated with another type of epilepsy or when certain simple measures, such as lowering the water temperature in the bath, do not control crises. Spontaneous disappearance is possible; when pharmacological therapy is necessary, there is usually a good response.We present the case of an infant diagnosed with hot-water epilepsy.

Clinical features of mah-jong reflex epilepsy / 中南大学学报(医学版)

To analyze clinical features of mah-jong reflex epilepsy, and to explore the underlying mechanisms.
 Methods: The clinical data of 15 patient (a study group), who visited in the outpatient department in the Third Xiangya Hospital, Central South University from 2016 to 2018, were collected and analyzed. The clinical data included disease history, 24 h- electroencephalography (EEG) and magnetic resonance imaging (MRI) scan. Next, previous 84 case (a literature group) reports published in the past 20 years were searched and compared.
 Results: Both groups of mah-jong reflex epilepsy were predominant in middle-aged men, with onset ages of (44.53±10.58) and (41.48±17.85) years old, respectively. In the study group, time interval usually lasted (4.00±2.45) h from starting play mah-jong to seizure attack, 73.3% patients complained mental fatigue before seizure attack, and 93.3% of the patients were described to have general tonic-clonic seizure. Few positive result was discovered in auxiliary examination, and the percentage was 9.3% in EEG, while no positive outcome was seen in head MRI. There were no significant differences in gender, age of onset, duration of playing mah-jong before attack, type of attack, and changes of head MRI between the 2 groups (all P>0.05).
 Conclusion: Mental fatigue after long-time mah-jong playing and cognitive dysfunction might be the potential triggers in mah-jong reflex epilepsy.

Central Regulation of Micturition and Its Association With Epilepsy / 대한배뇨장애요실금학회지

Micturition is a complex process involving the bladder, spinal cord, and the brain. Highly sophisticated central neural program controls bladder function by utilizing multiple brain regions, including pons and suprapontine structures. Periaqueductal grey, insula, anterior cingulate cortex, and medial prefrontal cortex are components of suprapontine micturition centers. Under pathologic conditions such as epilepsy, urinary dysfunction is a frequent symptom and it seems to be associated with increased suprapontine cortical activity. Interestingly, micturition can also trigger seizures known as reflex epilepsy. During voiding behavior, frontotemporal cortical activation has been reported and it may induce reflex seizures. As current researches are only limited to present clinical cases, more rigorous investigations are needed to elucidate biological mechanisms of micturition to advance our knowledge on the process of micturition in physiology and pathology.

Expressão gênica em larga escala em modelos genéticos de epilepsia / Large-scale gene expression in genetic models of epilepsy

Wistar audiogenic rat (WAR) é um modelo genético de epilepsia de crises audiogênicas desencadeadas após alta intensidade de estimulação sonora. Outro modelo genético recentemente identificado é o da epilepsia generalizada com crises de ausência (GEAS). O objetivo do presente estudo foi caracterizar o perfil de expressão gênica destas duas cepas através de uma análise em larga escala. Para os estudos de expressão foi utilizada inicialmente a tecnologia de microarranjos seguida da validação dos resultados por técnica quantitativa de PCR em tempo real. Os resultados foram analisados em ambiente R, utilizando os pacotes AFFY e RankProd do bioconductor, utilizando o algoritmo MAS 5 os array foram normalizados e calculou-se a intensidade do sinal e a detecção (presença ou ausência de expressão). Após a detecção, os transcritos que estavam ausentes foram removidos. Para a análise estatística foi utilizado o teste RankProd, que é biologicamente projetado para testar e detectar genes diferencialmente expressos em experimentos de microarranjos. Foi utilizado um valor de p ? 0,01 e pfp ? 0,05, a fim de considerar os transcritos diferencialmente expressos. No geral, nossos resultados mostram uma assinatura molecular similar nos dois modelos de ratos genéticos analisados. Houve uma sobreposição na lista de genes diferencialmente expressos encontrados em ambos os modelos, quando comparado com controles. Além disso, descobrimos que duas importantes vias moleculares para epileptogênese: neurotransmissão GABAérgica e potencialização de longo prazo pós-sináptica NMDA-dependente, foram encontrados em ambos os modelos, quando combinamos os dados dos animais WAR e GEAS. No entanto, algumas diferenças nas vias de sinalização expressas nos dois modelos também foram identificadas. Portando os resultados mostram claramente a natureza heterogênea e complexa dos mecanismos moleculares envolvidos na epileptogênese.

Wistar audiogenic rat (WAR) is a genetic epilepsy model susceptible to audiogenic seizures, after high-intensity sound stimulation. Another genetic model we have recently identified is the generalized epilepsy with absence seizures (GEAS) rat. The aim of the present study was to characterize and compare the genetic profile of these two strains using gene expression analysis. Experiments were performed initially using microarray technology followed by quantitative real-time PCR. Results were analyzed in R environment using the Affy and RankProd packages from Bioconductor, using the algorithm MAS 5 we normalized the arrays and calculated the signal intensity and the detection (presence or absence of expression), after the detection, transcripts which were absent in all samples were removed. For statistical analysis we used the Rank Product test, which is biologically motivated and designed to test and detect differentially expressed genes in replicated microarray experiments. This is a simple non-parametric statistical method based on ranks of fold changes. We used a p-value ? 0.01 and a pfp ? 0.05 in order to consider a given transcript to be differentially expressed Overall, the results show a different molecular signature in the two genetic rat models analyzed, since different enriched gene ontology categories were found. However, there was some overlap in the list of genes differentially expressed found in both models when comparing to controls. In addition, we found that two important molecular pathways for epileptogenesis: GABAergic neurotransmission and: Neurophysiological process NMDA-dependent postsynaptic long-term potentiation in CA1 hippocampal neurons, were found to be present in both models when combining data from WAR and GEAS animals.

Eating epilepsy in Oman. A case series and report on the efficacy of temporal lobectomy

Eating epilepsy [EE], where seizures are triggered by eating, is rare and has not been reported in the Gulf region. In EE, the ictal semiology includes partial or generalised seizures. Focal brain changes on imaging, if present, are often confined to the temporal lobe or perisylvian region. Therapeutic options, especially in those patients who are refractory to pharmacotherapy, have not been well-established. We report a series of five patients with EE from Oman, a country located in the eastern part of the Arabian Gulf region, and highlight the usefulness of temporal lobectomy in one patient who had medically-intractable EE. Surgical intervention could be considered as a potential therapeutic option in carefully selected patients with medically-intractable seizures

Anticonvulsant and antiarrhythmic effects of nifedipine in rats prone to audiogenic seizures

Calcium ion participates in the regulation of neural transmission and the presynaptic release of neurotransmitters. It is also involved in epileptic events, cardiac arrhythmias and abnormal conduction of stimuli. The purpose of the present study was to evaluate the effects of nifedipine, a calcium channel blocker, on epileptic seizures and on reperfusion arrhythmias in rats prone to audiogenic epileptic seizures (Wistar audiogenic rats, WAR) and in normal Wistar rats (N = 6/group). The seizure severity index was applied after an intraperitoneal injection of 20 or 40 mg/kg nifedipine (N20 and N40 groups, respectively). The Langendorff technique was used to analyze cardiac function, as well as the incidence and severity of the reperfusion arrhythmias after ligature and release of the left coronary artery in rats treated or not with nifedipine. We found that nifedipine treatment decreased seizure severity (0.94 ± 0.02 for WAR; 0.70 ± 0.10 for WAR + N20; 0.47 ± 0.08 for WAR + N40) and increased the latent period (13 ± 2 s for WAR; 35 ± 10 s for WAR + N20; 48 ± 7 s for WAR + N40) for the development of seizures in WAR. Furthermore, the incidence and severity of the reperfusion arrhythmias were lower in WAR and normal Wistar rats injected with nifedipine. In WAR, these effects were mediated, at least in part, by a decrease in heart rate. Thus, our results indicate that nifedipine may be considered to be a potential adjuvant drug for epilepsy treatment, especially in those cases associated with cardiac rhythm abnormalities.

Epilepsia de água quente numa mulher grávida: relato de caso / Hot water epilepsy in a pregnant woman: case report

A epilepsia de água quente é uma forma singular de epilepsia reflexa desencadeada por se banhar e jorrar água quente na cabeça. Embora seja mais frequente em crianças e predomine no sexo masculino, o presente artigo discute o caso de uma mulher grávida de 28 anos de idade que apresentava convulsões reflexas ao jorrar água na cabeça ao tomar banho. O tratamento homeopático constitucional teve sucesso terapêutico.

Hot water epilepsy is a unique form of reflex epilepsy triggered by bathing and pouring hot water over the head. Although it is mostly seen in infants and children, with predominance in males, the present article discusses the case of a 28-year-old pregnant woman with reflex seizures triggered by pouring hot water over the head while having a bath during pregnancy and successfully treated with constitutional homeopathic medication.

Reflex epilepsy

Seizures triggered by sensory stimulation are termed [reflex epileptic seizures]. Since the nerve structures responsible for seizures are situated between the sensory afferent pathway and the efferent tract responsible for epilepsy, thus acting as a reflex centre. The concept of reflex epilepsy was widespread in the 19[th] century, when it was considered that among the major causes of epilepsy was chronic sensory stimulation e.g. teething, intestinal worms, refractive error, or chronic constipation

Effect of eyes closure, eyes closed and photic stimulation on the epileptiform discharges in children with epilepsies / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the effect of eyes closure and closed on the epileptiform discharges, and the relationship between eyes closure or closed sensitivity and photosensitivity.</p><p><b>METHODS</b>During video-EEG monitoring, open-close eyes tests and intermittent photic stimulation (IPS) under different eyes conditions were carried out in epilepsy patients. The eyes conditions were divided into eyes opened, eyes closure (the period immediately after closing of the eyes and only lasts for less than 3 s), and eyes closed (the period lasting for more than 3 s and persisting as long as the eyes remain closed). From 135 patients, 13 cases whose EEG showed eyes closure or closed sensitivity had been selected out for our study. The clinical, eyes closure or closed sensitivity and the relationship between eyes closure or closed sensitivity and photosensitivity were analyzed.</p><p><b>RESULTS</b>Of the 13 patients, 8 were female, and 5 were male. The age ranged from 6 years to 17 years (median 11 years). The age at onset of epilepsy ranged from 1.5 years to 14.5 years (median 9 years). Epileptiform discharges could be provoked by eyes closure in 10 of 13 patients, associated with photosensitivity in 8 patients. Of the 8 patients, 6 cases showed photosensitivity under eyes closure and only 4 cases showed photosensitivity under eyes closed. Eyes closed sensitivity had been seen in 3 patients, with photosensitivity in 2 patients. Evoked EEG patterns and discharges regions by eyes closure and eyes closed conditions had not obvious differential and might be strongly related to the types of epilepsies. The intensity and frequencies of photosensitivity under different eyes conditions were different. After valproate acid treatment, the eyes closure or closed sensitivity and photosensitivity improved in 4 cases, with improvement of photosensitivity prior to eyes conditions sensitivity.</p><p><b>CONCLUSIONS</b>Eyes closure and eyes closed could induce EEG paroxysms, which involved in some types of epileptic syndromes and appeared common in female patients. Eyes closure or closed sensitivity might coexist or be disassociated with photosensitivity. Testing eyes closure, eyes closed and IPS during EEG monitoring would contribute to finding the EEG discharges, further help to diagnose epilepsies and epileptic syndromes and guide treatment.</p>

Self abortion of attacks in patients with Hot Water Epilepsy.

A cross sectional hospital based study was undertaken to find out the various clinical aspects and management of Hot Water Epilepsy (HWE) in children. Of the 71 cases analysed, 67.6% had onset of seizures in the first decade of life. Seizures occurred frequently towards the end of head bath (71.8%). In 14.1% cases, seizures were precipitated with cold-water head bath also. Complex partial seizures (60.6%) and generalized atonic seizures (21.1%) were common. Spontaneous non-reflex epilepsy was seen in 47.9% cases. Self-induction and self-abortion of seizures were seen in 16.9% and 12.7% patients respectively. Family history was available in 32.4% of cases. Majority had good response to continuous prophylactic treatment with antiepileptic drugs. We conclude that high incidence of spontaneous seizures and generalized atonic seizures seem to be peculiar to our geographical area. "Self abortion of attacks"may be of immense help in controlling the attacks.

Kindling & mossy fibre sprouting in the rat hippocampus following hot water induced hyperthermic seizures.

BACKGROUND & OBJECTIVES: Hot water epilepsy (HWE) is well recognized reflex epilepsy with possible genetic susceptibility. Rat model and human experimentation had proven that HWE is a type of hyperthermic seizure with possible kindling on repeated stimulation in animals. The present study was undertaken to investigate kindling associated with hyperthermic seizures induced by repeated hot water stimulation in the rat model and to prove hyperthermic kindling. METHODS: Epileptic seizures were induced in 36 male Wistar albino rats by means of hot water sprays at 48 h time intervals. Progression of seizure activity was investigated by studying the behaviour, severity and duration of the seizure. Threshold of rectal temperatures and timed latency for seizure induction were studied. Seizure discharges (EEG) were recorded from ventral hippocampus in six of these rats. Timm's staining was used to study the neuronal sprouting as a consequence of kindling. Studying the seizure threshold, latency, duration of seizure discharge and behavioural seizure following a stimulus-free interval of 30 days tested permanence of kindling. RESULTS: Following 8-12 episodes of hot water stimulations there was progressive epileptic activity manifested in the form of lowering of rectal temperature thresholds from 41.5 to 40.0 degrees C, drop in latency for developing seizures from 185 to 118 sec, increase in duration of hippocampal seizure discharge from 15 to 140 sec, along with progressive increase in complexity of EEG after discharges, increase in behavioural seizure severity from Grade 1 to 5 in all the rats, and neuronal sprouting observed in supragranular molecular layer and in stratum lacunosum. INTERPRETATION & CONCLUSION: Our study covered all aspects of kindling and provided a useful animal model for human hot water epilepsy. Hyperthermic seizures induced by hot water in the rat model kindle as demonstrated by Timm's staining.

Reflex Epilepsy Induced by Playing Oriental Card or Board Games

BACKGROUND AND PURPOSE: There are currently few studies on clinical profiles of reflex epilepsy induced by thinking and spatial tasks. We studied the clinical characteristics of reflex epilepsy induced by playing oriental card and board games. METHODS: This study included 17 patients who presented with seizures that occur predominantly while playing games. We collected clinical data via protocol-based interviews. EEGs and brain MRI were performed. RESULTS: All of the subjects were men, and all of them were older than 30 years at the onset of seizure. Thirteen patients (76%) experienced their seizures while playing the oriental card game "Go-stop" and the remaining four patients (24%) experienced them while playing the oriental board game "Baduk". Generalized tonic-clonic seizures were frequently preceded by prodromal symptoms, but myoclonus was not evident. Most patients had no spontaneous seizures and generalized epileptiform discharges on EEGs, and infrequent seizures that were well controlled. CONCLUSIONS: Our patients exhibited some features that differ from those described previously in the literature, suggesting that the clinical spectrum of reflex epilepsy induced by thinking and spatial tasks is wide.

Clinical Experiences with Vagus Nerve Stimulation for Medically Intractable Epilepsy / 대한간질학회지

PURPOSE: Recently, Vagus nerve stimulation (VNS) has been reported to show promising results as an adjunctive therapy for medically intractable seizures. We report early experiences with VNS for medically intractable epilepsy in young adults and pediatric patients. METHODS: Eleven patients ages ranging from 7 years to 29 years underwent implantation of vagal nerve stimulators (Cyberonics, Houston, TX) from September 1999 to April 2003. We reviewed clinical findings in 11 patients and recorded changes of seizure frequency, quality of life (QOL), and antiepileptic drug (AED). RESULTS: The mean age of seizure onset was 4.5 years old (range:3 months-11 years). The seizure duration before VNS was mean 8.7 years (range:1.5-19 years). Seven patients had symptomatic partial epilepsies, and one had cryptogenic partial epilepsy. Unclassified patients had 2 Lennox-Gastaut syndrome and 1 reflex epilepsy. Two patients received total callosotomy for reducing drop attack, but refractory. One patient, who underwent a temporal lobectomy, failed to obtain desirable results. One implantation was performed with total callosotomy, simultaneously. All of implantations were successful, except for one wound revision due to a subcutaneously protruded anchoring device of electrode. About two weeks after the implantation, programming of the stimuli was started and increased the output current to the levels, at which patients was tolerated. The most common adverse effect was hoarseness or voice alteration (44%). Mean reduction of seizure frequency compared with baseline before VNS was 23.6% after 3 months, 33.5% after 6 months, 41.3% after 1 year, and 46.6% at latest follow-up. Three patients had no response to VNS. One patient was added 1 one new AED after VNS. Two patients were reduced 1 or 2 drugs. But there was no correlation between VNS effect and AED change. Six patients had some improvement of QOL. Mean follow-up period, which was 28 months (range:12-48 months). CONCLUSIONS: We concluded that VNS has a role of adjunctive therapy for medically intractable epilepsy and the further studies should be focused on the prediction of unresponsiveness and the adjustment of VNS parameters for maximal efficacy in patients with various backgrounds.

Bloqueo de las crisis audiogénicas y del choque electroconvulsivo por la 2-fenil-4, 4-bis-(hidroximetil)-2-oxazolina / Block of the audiogenic seizures and of the electroconvulsive shock by 2-phenyl-4, 4-bis-(hydroxymethyl)-2-oxazoline

Algunas aril-oxazolinas han sido descritas como sustancias activas sobre el sistema nervioso central, con efectos y aplicaciones diversas como depresoras, anestésicos, anticonvulsivos, etc. El presente trabajo se trazó como objetivo fundamental estudiar el posible efecto de la 2-fenil-4,4-bis (hidroximetil)-2-oxazolina (OX) obtenida por síntesis química bajo microondas, en 2 modelos experimentales de epilepsia: el choque electroconvulsivo por estimulación repetitiva en ratones y el de inducción de crisis convulsiva por estímulo audiogénico en el gerbo mongol. La dosis de 150 mg/kg de OX redujo el número de pulsos eléctricos necesarios para inducir la crisis tónica producida por el choque eléctrico, así como su duración. Esta misma dosis bloqueó las crisis inducidas por el estímulo audiogénico en el gerbo, y disminuyó significativamente su severidad (grados de crisis) y ocurrencia. Estos resultados permiten afirmar que OX presenta un efecto antiepiléptico relacionado posiblemente con una inhibición de la sinapsis glutamatérgica en el hipocampo

Hot water epilepsy - a report of three cases.

Hot water epilepsy (HWE) is a rare form of reflex epilepsy caused by bathing with hot water. In this paper, we describe three cases with hot water epilepsy.It occurs generally in children with normal psychomotor development and children continue to develop normally after seizure. HWE has usually a favorable prognosis by first avoiding lukewarm water and secondly using either intermittent oral prophylaxis with benzodiazepines or conventional AEDs.

Self induced photosensitive epilepsy.

The authors report a case of a 12-year-old girl who had rare self induced photosensitive epilepsy. She used to move her right hand over the right eye while simultaneously rubbing the forehead since the age of 8. During these episodes she was lost in herself. Lately these episodes were followed by brief spell of unconsciousness. The EEG examination, in its third attempt, revealed bilateral multiple symmetric spikes on photic stimulation. She admitted that she often induced the episode herself and derived pleasure out of it. She responded well to Sodium valproate.

Reflex Epilepsy Induced by Tooth Brushing: A Case Report / 대한간질학회지

Reflex epilepsy describes seizures which are precipitated by clearly recognized stimuli. Reflex epileptic seizures triggered by tooth brushing are rare. We report a case with reflex epilepsy occurring exclusively during tooth brushing. He began having seizures at age 28. Neurological examinations and brain magnetic resonance images were found normal. On long term video-EEG monitoring, he presented two episodes of simple partial seizure induced by brushing molar and premolar teeth, which was right facial clonic movement persisting 3-5 seconds. He was unresponsive during the event, but had no postictal confusion. Ictal EEG showed 6 Hz rhythmic theta activity in the left frontocentral area. Interictal EEG was normal. The findings of EEG suggest that this reflex epilepsy might have epileptic focus in the left frontocentral area. Reasonable precaution such as gargling or less vigorous brushing of his teeth with fingers effectively prevented seizure recurrence.

Reflex Epilepsy Provoked by Mental Activities Mainly Associated with Use of the Hands / 대한간질학회지

There have been very few reports on reflex seizures induced by writing. Complex precipitating behaviors such as calculation, writing, copying of complicated figure, and spatial construction have been recognized as triggering factor of epileptic seizures. We report a case of a patient with a very unusual form of complex reflex epilepsy in which seizures are usually induced by writing or spatial construction. Neurological examination was normal and brain magnetic resonance imaging showed mild brain atrophy without focal lesion. During long-term video-EEG monitoring, she was instructed to write a letter with the right hand. About 20 minutes after writing, she experienced similar aura and motion arrest and then showed an elevation of bilateral upper extremities like myoclonic jerk followed by generalized tonic clonic seizure. On ictal EEG, spike and wave complexes appeared from both centroparietal region followed by 10 Hz rhythmic activities for 20 seconds. Interictal EEG was normal. Ictal-interictal subtraction image of brain SPECT (99mTc-HMPAO) showed hyperperfusion in the middle frontal gyrus and precentral gyrus of left frontal lobe and superior and inferior frontal gyrus of right frontal lobe. We suggest that this reflex epilepsy might be originated from both central areas with maximum on the left side. Oxcarbazepine was effective in controlling this reflex epileptic seizure.

Do endogenous opioids and nitric oxide participate in the anticonvulsant action of dipyrone?

It was previously reported that systemic administration of dipyrone inhibited the tonic component of generalized tonic-clonic seizures in both the electroshock and the audiogenic seizure models. The aim of the present study was to investigate the mechanisms involved in the anticonvulsant action of dipyrone by assessing the role of nitric oxide and opioids in the electroshock (female 60- to 90-day-old Wistar rats, N = 5-11) and audiogenic seizure (female 60- to 90-day-old Wistar audiogenic rats, N = 5-11) models of epilepsy. Naloxone (5 mg/kg, sc) significantly reversed the anticonvulsant effect of dipyrone in rats submitted to the induction of audiogenic seizures (ANOVA/Bonferroni's test), suggesting the involvement of opioid peptides in this action. In the electroshock model no reversal of the anticonvulsant effect of dipyrone by naloxone (5 mg/kg, sc) was demonstrable. The acute (120 mg/kg, ip) and chronic (25 mg/kg, ip, twice a day/4 days) administration of L-NOARG did not reverse the anticonvulsant action of dipyrone in the audiogenic seizure model, suggesting that the nitric oxide pathway does not participate in such effect. Indomethacin (10, 20 and 30 mg/kg, ip) used for comparison had no anticonvulsant effect in the audiogenic seizure model. In conclusion, opioid peptides but not nitric oxide seem to be involved in the anticonvulsant action of dipyrone in audiogenic seizures