Convulsive Episodes Research Articles

Ictal sinus pause and myoclonic seizure in a child.

Ictal tachycardia and bradycardia are common arrhythmias; however, ictal sinus pause and asystole are rare. Ictal arrhythmia is mostly reported in adults with temporal lobe epilepsy. Recently, ictal arrhythmia was recognized as a major warning sign of sudden unexpected death in epilepsy. We present an interesting case of a child with ictal sinus pause and asystole. A 27-month-old girl was hospitalized due to 5 episodes of convulsions during the past 2 days. Results of routine electroencephalography (EEG) were normal, but she experienced brief generalized tonic seizure for 3 days. During video-monitored EEG and echocardiography (ECG), she showed multiple myoclonic seizures simultaneously or independently, as well as frequent sinus pauses. After treatment with valproic acid, myoclonus and generalized tonic seizures were well controlled and only 2 sinus pauses were seen on 24-hour Holter ECG monitoring. Sinus dysfunction should be recognized on EEG, and it can sometimes be treated successfully with only antiepileptic medication.

Seizures Are Not a Prevalent Feature of Wyburn-Mason Syndrome

Wyburn-Mason syndrome (WMS) is a widely recognized neurocutaneous syndrome in which the unilateral anterior venous malformation in its complete form is associated with eye, brain, and skin involvement. The term WMS also includes the incomplete form in which the patients show unilateral anterior venous malformation as a single anomaly not associated with other districts involvement. Neurologic complications are almost constantly reported in this syndrome. The aim of this report is to describe the neurologic course of two patients affected by the complete form of this very rare syndrome offering particular remarks whether convulsive episodes or epilepsy are leading features of this disorder. Based on the experience registered in the long follow-up in the present patients and in those reported in the literature, seizures and convulsive episodes are uncommon in WMS and, when present, mainly occur in consequence of acute or chronic disruptive events of the arteriovenous malformations.

Ictal dancing associated with hippocampal sclerosis

Ictal dancing is a very rare seizure semiology. Five cases have been previously reported in the literature [1–4]. Even though one case report investigated with stereo-encephalography (SEEG) showing intracranial ictal EEG change arising from right prefrontal region, particularly right dorsolateral prefrontal area in patients with ictal singing associated with dancing, underlying mechanisms and the neural basis of ictal dancing remain unknown [4]. This report presents one more case of ictal dancing in a temporal lobe epilepsy (TLE) patient with pathologically-confirmed hippocampal sclerosis (HS). A 27-year-old right-handed woman suffered from medically intractable TLE. Her seizure began at the age of 10 and consisted of (1) isolated aura; (2) aura with automotor seizure; and (3) secondarily generalized tonic clonic seizure (GTC). Aura is described as palpitation and fear. Despite treatment with several antiepileptic drugs (AEDs), her seizure persisted on a weekly basis. She also had one episode of simple febrile convulsion at the age of 3. She was admitted to the epilepsy monitoring unit in our center for pre-surgical evaluation. Four habitual seizures were recorded. Three of them were aura with automotor seizure. The other was initially similar but was followed by a GTC. All four seizures consistently started with autonomic (palpitation) and affective (fear) aura then followed by oral and bimanual automotor seizure. During two seizures, different patterns of associated complex motor behavior including acting out a dance move was observed

Blindness in Tuberous Sclerosis: A Case Report

Tuberous sclerosis (TS) is inherited as an autosomal dominant trait with variable penetrance characterised by glial cell tumor which arises from the cerebral and the retina. Blindness in association with Tuberous sclerosis (TS) is rare. When visual loss occurs it may be associated with hamartomas from retinal or optic nerve involvement or from intracranial (brain) tumours that affect either the part of the brain that processes visual information or from optic nerve damage following raised intracranial pressure. Very few cases of TS with blindness have been reported globally. Deterioration in academic performance might be the first pointer to the visual impairment. We report a case of a 13 year old girl who presented with increasing number of facial rash over an 11 years period, recurrent headache and deteriorating academic performance of 1 year and loss of vision of 6 months with a recent episode of convulsion. Similar skin rashes without other associated symptoms were noticed on the mother and one of the younger siblings. She was a Tanner stage one in development. She had facial angio fibromas, shagreen patches over the left hypochondria, back regions and face. Ophthalmic evaluation showed a visual acuity of being able to count fingers at not more than one meter from the face and only perception of light in the right and left eye respectively, both eyes had brisk pupillary activities, good mydriasis and clear media. The retinal and optic nerve head appeared normal in the right eye whereas in the left eye was a huge tuberous hamartoma of the optic disc and macular as well as generalised vascular occlusion and subretinal fluid. The Computerized tomography (CT) scan showed an Intraventricular tumour, with calcification within the tumours and subependymal. There was associated obstructive hydrocephalus. Patient was managed by a multi disciplinary team of ophthalmologists, neurosurgeons and radiologists, coordinated by a paediatrician. Conclusion: The diagnosis of tuberous sclerosis complex (TSC) was based on the lesions found on clinical examination, imaging, and pathologic studies. The blindness was multi-factorial in cause including intracranial, retinal and optic nerve tumours. Comprehensive medical history, detailed physical examinations and neuroimaging study are essential in making a diagnosis of TSC. Our patient was mis-diagnosed at various health facilities for many years. This delay in making appropriate diagnosis and instituting treatment could have contributed to the eventual outcome. Keywords: Tuberous Sclerosis, Blindness, Deteriorating Academic Performance

Neonatal outcome after fetal anemia managed by intrauterine transfusion.

In-utero transfusion is now well under control and improves the survival of foetuses monitored for fetal anemia with a survival rate of more than 80%. The aim was to evaluate short-term neonatal outcome after fetal severe anemia managed by intrauterine transfusions. We did a retrospective study of all neonates born after management of severe fetal anemia (n = 93) between January 1999 and January 2013 in our regional center. The two main causes of anemia were maternal red blood cell alloimmunization (N = 81, 87%) and Parvovirus B19 infection (N = 10, 10.8%). In the alloimmunization group, phototherapy was implemented in 85.2% of cases with a maximum level of bilirubin of 114.4 ± 60.7 (mg/dl). Transfusion and exchange transfusion were, respectively, required in 51.9% and in 34.6% of cases. One neonate presented a convulsive episode, and we observed three neonatal deaths. In the parvovirus group, none of the child had anemia at birth and no management was necessary. Contemporary management of Rhesus disease is associated with encouraging neonatal outcomes. In case of Parvovirus infection, no specific management is necessary at. But, in all cases of fetal anemia, children should be followed up with particular attention to neurologic development. • In-utero transfusion is now well under control and improves the survival of fetuses monitored for fetal anemia. • Limited studies are available on the effect of IUT on postnatal outcome in infants with a history of fetal anemia. What is New: • Contemporary management of severe Rhesus disease is associated with encouraging neonatal outcomes. • The majority of infants can be managed with phototherapy and a limited number of top-up transfusions and exchange transfusions. In case of Parvovirus infection, the short-term neonatal outcome is excellent.

Central Pontine Myelinolysis: A Case Study

Abstract Central Pontine Myelinolysis (CPM) commonly presents as a complication of treatment in patients with profound life threatening hyponatraemia. It occurs when the sodium level is corrected too rapidly. Hyponatraemia should never be corrected at a rate greater than 8-10mmol/L of sodium per day. Rapid correction causes extracellular tonicity and will continue to drive water out of the brain’s cells leading to cellular dysfunction. Frequent clinical signs include dysphagia, dysarthria, diplopia and acute para/quadraparesis. Patients can also experience locked in syndrome, where cognitive function is intact but all muscles are paralysed with the exception of eye blinking. CPM gets its name as it occurs when cell dysfunction causes destruction of the myelin sheath of nerve cells in the brain stem, more specifically the pons. It is associated with poor prognosis and prevention is of primary importance. Freddy is a 35 year old freelance graphic designer, fitness instructor and ultra-marathon runner. In October 2011 he competed in the Sahara Marathon in Morocco, a 6 day 255km ultra-marathon. At the end of the third day Freddy was found collapsed and vomiting. He was confused and was suffering severe leg cramps. The next morning Freddy suffered a single convulsive episode and was subsequently transferred to a hospital in Egypt.

Guillain–Barre´ syndrome with bilateral facial palsy with incidental arachnoid cyst

This case report describes the findings of a 10-year-old female child admitted with fever and headache for 3 days along with several episodes of vomiting and generalized seizures for 1 day. She regained consciousness 50 minutes after admission with initial treatment, but developed muscle weakness, followed by tetraplegia with areflexia, bilateral facial palsy and respiratory insufficiency. The nerve conduction study (NCV) had evidence of demyelinating neuropathy and the cerebrospinal fluid (CSF) showed albumin-cytological dissociation. These neurologic findings were consistent with the diagnosis of Guillain-Barré syndrome. The patient was treated with immunoglobulin and other supportive cares. Mechanical ventilation was started on day 3 of hospital admission in face of respiratory insuficiency. Recovery, however, was relatively quick and extubation was possible on 6th day. Work up for CNS infection was non-contributory. MRI of brain done during the course, revealed an archnoid cyst in right temporal region which might have contributed to the episode of unconsciousneess, vomiting and convulsion. After two weeks of hospitalization the patient was discharged from the hospital on wheel chair with satisfectory improvement of powers of facial and limb muscles. When the patient was seen at the outpatient department four weeks after hospital discharge, she was able to walk with help with normal facial nerve function on both sides. G B syndrome presenting like meningitis; bilateral facial palsy; and co-presence of an archnoid cyst makes this case worth sharing.DOI: http://dx.doi.org/10.3126/ajms.v6i5.11627Asian Journal of Medical Sciences Vol.6(5) 2015 71-74

Intracranial Mesenchymal Chondrosarcoma: A Case Report

Primary intracranial mesenchymal chondrosarcoma is an extremely rare tumor. We present a case of primary intracranial mesenchymal chondrosarcoma in a young female who presented with headache, loss of vision, and one episode of convulsions associated with nausea and vomiting. MRI brain revealed a large, well-marginated, lobulated, partially calcified, intensely enhancing lesion with a broad dural base in bilateral frontal regions with marked mass effect suggestive of meningioma. Bilateral frontal craniotomy with gross tumor excision was performed. Histopathology revealed mesenchymal chondrosarcoma. Adjuvant radiotherapy was delivered and patient is currently on follow-up. We emphasize that intracranial mesenchymal chondrosarcoma is a rare neoplasm and should be considered in the differential diagnoses of intracranial mass along with meningioma. Treatment of choice is surgery followed by adjuvant radiotherapy. We review the relevant literature and discuss the characteristics of this rare tumor.

Novel halogenated derivates of JWH-018: Behavioral and binding studies in mice

JWH-018 is a synthetic CB1 and CB2 agonist illegally marketed as products named “Spice” or “herbal blend” for its psychoactive effects which are much higher than those produced by cannabis. In the last year, the European Monitoring Centre for Drugs and Drug Addiction reported to the Italian National Early Warning System the seizure of plant material containing new halogenated derivatives of JWH-018 (JWH-018 Cl and JWH-018 Br). The present study aimed to investigate the in vitro and in vivo activity of these two new synthetic cannabinoids in mice. In vitro competition binding experiments performed on mouse and human CB1 receptors revealed a high affinity and potency of the halogenated compounds. Synthetic cannabinoids (0.01–6 mg/kg i.p.) impaired motor activity and induced catalepsy in mice and their effects were more severe with respect to those evoked by Δ9-THC. Moreover, they increased the mechanical and thermal pain threshold and induced a marked hypothermia. It is interesting to note that whereas high doses of JWH-018 cause seizures, myoclonia and hyperreflexia, the halogenated compounds, in particular JWH-018Br, were less effective. Behavioral and neurological changes were prevented by the selective CB1 receptor antagonist AM 251. These data demonstrate for the first time that JWH-018 Cl and JWH-018 Br act similarly to JWH-018 while inducing less convulsive episodes and myoclonias. These data support the hypothesis that the halogenated compounds may have been introduced onto market to produce similar intoxicating effects as JWH-018 while causing less side effects.

A twist in the diagnosis … and the uterus! A case report of third trimester uterine torsion

We report the case of a 26-year-old lady gravida 5, with a previous Caesarean section who presented with 2 episodes of convulsions, shock and evidence of intrauterine foetal death. She was known to have epilepsy since childhood. Anticipating a scar dehiscence or rupture uterus she was posted for emergency laparotomy under spinal anaesthesia. Intra-operatively, 180° uterine torsion was evident. The uterus was untwisted and a lower segment caesarean section was performed to deliver the dead foetus. As the uterus remained flabby and did not retracted after correction of torsion, subtotal hysterectomy was done. She recovered well after surgery. The present case highlights uterine torsions, a rare but potentially dangerous and unexpected obstetric complication and reiterates the need for a timely intervention for better maternal and foetal outcome.

Posterior Reversible Encephalopathy Syndrome During Induction Treatment of Philadelphia Positive Acute Lymphoblastic Leukemia in an Adult Patient: First Case Report and Literature Review

Posterior reversible encephalopathy syndrome (PRES) is a reversible clinical and radiological entity. The circumstances of occurrence are numerous and the pathophysiology is unclear. The main theory is related to the onset of hypertension leading to the deterioration of the cerebral autoregulation. This is an acute or subacute syndrome, often preceded by convulsive episodes. Symptoms are headaches, impaired consciousness, and vomiting or visual disturbances. The brain Magnetic Resonance Imaging (MRI) is the gold standard and highlights evidences of hyperintensities on T2 (bilateral, symmetrical, reversible, and predominantly posterior). Several cases have been reported as a complication during the treatment of childhood acute lymphoblastic leukemia (ALL), but few cases have been published in adult patients and especially in Philadelphia positive ALL. We report the first adult case of Philadelphia positive ALL with development of PRES after induction therapy.

Impact of cyclic nucleotides on the antiseizure activity of acute and chronic diazepam administration in isoniazid-induced seizures and pentylenetetrazole kindling in rats

BackgroundIt is estimated that ~10% of the population suffers a single convulsive episode during their lifetime. Epilepsy is the second most common chronic neurological disorder after stroke, affecting ~0.5% of the population in developed countries and 1.5-2% in developing countries. Diazepam (DZ) is among the benzodiazepines used most widely in status epilepticus as well as resistant and refractory seizures. However, tolerance to its antiseizure activity is among the obstacles facing it use on a wider basis. Exploration of the role of cyclic nucleotides in seizures might enable finding ways to combat tolerance as well as discover new treatment modalities.Materials and methodsSeizure severity, electroencephalography, and levels of γ-aminobutyric acid (GABA), glutamate, cyclic adenosine monophosphate (cAMP), and cyclic guanosine monophosphate (cGMP) in brain homogenate were assessed upon single administration of isoniazid and repeated pentylenetetrazole injections, whether untreated or treated with DZ, either acutely or on a chronic basis, respectively.ResultsA single DZ injection, 10 min after single isoniazid, could significantly improve seizure severity, associated with increased power of the fast β wave, implying ameliorated cognitive functions, together with an equal GABA/glutamate ratio versus significant reduction in the cAMP/cGMP ratio. Repeated DZ injections, 10 min after each pentylenetetrazole injection, on alternate days for 1 month and every 2 days for another month produced a significant improvement in seizure severity, accompanied by reduced power of the fast β and the slow δ waves, associated with an increased GABA/glutamate ratio and an unchanged cAMP/cGMP ratio.ConclusionThe current study assumes that a certain interplay exists between GABA/glutamate on the one hand and cAMP/cGMP on the other so that DZ could exert an anticonvulsant effect on an acute basis despite an unchanged GABA/glutamate ratio as well as upon chronic administration in the presence of an unchanged cAMP/cGMP ratio. In addition, the unchanged cAMP/cGMP ratio associated with chronic DZ administration suggests that cyclic nucleotides might lead to loss of some of the effects of DZ, as shown by reduced β wave power, indicating a reduction in cognitive abilities, concentration, and learning abilities, in contrast to higher β power after single use.

Sleep apnea: manifestation or cause of seizures in children? Case report

Introduction. Sleep apnea (SA) in children is underdiagnosed. Compromising somatic development and altered quality of life are frequently present. Central sleep apnea (CSA) may be the cause of convulsive episodes in sleep or could be their result. Polysomnography is the gold-standard of SA objective assessment. Material and methods. We present a 7-year-old boy hospitalized in September 2013 for choking episodes occurred during sleep. The assessment was made by history, clinical examination, laboratory investigations (functional, imaging, biological) and interdisciplinary checkups. Results. Repeated respiratory tract infection and a history of about 20 days of episodes of choking were established by anamnesis. Were diagnosed: underweight status; chronic rhinitis; tonsillar hypertrophy; obstructive ventilatory dysfunction; mixed sleep apnea with predominant central component; atopic status; convulsions. Imaging explorations revealed normal aspects. Complex hygienic-dietary and medical treatment has led to the disappearance of respiratory disorders during sleep, with normalization of spirometric parameters and nutritional status. Conclusions. Polygraphic sleep study identified sleep apnea, indicating the predominance of the central component and facilitating etiologic diagnosis. Interdisciplinary management led to favorable evolution of the case.

Concurrent multiple brain tuberculomas with miliary tuberculosis - A rare presentation

A 45 year old immunocompetent female presented to us with fever, dry cough, vomitingand headache. There was a single episode of convulsion after hospital admission. ChestXray was normal. After through clinical examination and relevant investigations we came toknow that it was a case of concurrent multiple brain tuberculomas with miliary tuberculosis.Patient was managed with antitubercular drugs and other supportive therapy promptly and sheresponed well. Occurence of tuberculoma in the brain in a patient with miliary tuberculosisis a rare phenomenon.DOI: http://dx.doi.org/10.3126/ajms.v6i2.10279Asian Journal of Medical Sciences Vol.6(2) 2015 118-120

P18.08 * A MULTICENTRIC, OBSERVATIONAL, NON INTERVENTIONAL STUDY OF EFFICACY AND SAFETY OF LACOSAMIDE FOR THE TREATMENT OF EPILEPTIC SEIZURES IN PATIENTS WITH BRAIN TUMORS

INTRODUCTION: Epileptic seizures are common in oncologic patients. Seizures could be the initial symptom of a brain tumor in 20-40% of the cases and 40% of the patients with brain tumors will have a seizure throught the diseqse. Management of these complications is crucial in the treatment of brain tumors. The ideal antiepileptic drug (AED) would be that one which could ensure a high and proved efficacy, wide security, low interactions, easy and quick to titrate and available in i.v. formulation. Until today there is a scarce number of publications on the use of lacosamide (LCM) in patients with btain tumors, although there is some experience in routine clinical practice that has previously shown a very promising profile of efficacy and safety. This study tries to compile some of these experiences in Spain. OBJECTIVES: To evaluate the efficacy of LCM in the management of seizures in patients with brain tumors. Secondary objectives were the evaluation of safety and tolerability of LCM . DESIGN: Multicentric, observational, non-interventional retrospective study. Patients who suffered at least 1 convulsive episode and at least treated with 1 dose of LCM were recruited. RESULTS: 37 patients with brain tumor suffering seizures were recruited in 6 centers all over the country, and 24 of them (64.9%) could be followed-up until the end of the study (after 6 months). Of these 37 patients, 26 (70%) were male, the age was 54.14 ± 14 years. 28 patients had one lesion and 9 had several lesions, 7 of them were metastatic. The average of total number of seizures from the onset of LCM treatment to the last visit performed shows a statistically significant change from the initiation of LCM treatment, showing a reduction of -16.8 ± 41'7 seizures. (p <000.1) The reason for withdrawing from the study was death by cancer in 7 of all the 37 initially included (53.85%), being a lack of LCM efficacy in 2 patients. A total of 12 more patients needed a LCM modification of treatment, 9 due to lack of efficacy, 3 due to adverse events. CONCLUSIONS: In a retrospective, non-interventional study with brain tumor patients, LCM has shown to be effective and safe in the management of seizures in neurooncological patients.

A Retrospective Study of Polyallergy as a Marker of Nonepileptic Seizures in the Epilepsy Monitoring Unit

Distinguishing epileptic seizures from nonepileptic seizures (NES) can be difficult, given their similar motoric manifestations and a common comorbidity. Although video electroencephalography in an epilepsy monitoring unit ideally remains the gold standard for the identification of NES, a number of "soft signs" have been proposed to indicate a greater likelihood that convulsive episodes are NES rather than epileptic in nature. Trainees at our institution have cited the presence of multiple listed allergies as indicative of a higher likelihood of NES. The goal of this study was to provide a statistical analysis of polyallergy and its association with patients with NES admitted for video electroencephalograph monitoring. Records of all epilepsy monitoring unit patients at St. Joseph's Hospital and Medical Center between 2006 and 2012 were reviewed for age, sex, electroencephalograph diagnosis, antiepileptic drugs previously tried, and allergy number. A total of 1834 patient records were used for analysis. The group classified as nonepileptic had the highest average number of allergies at 1.56, whereas the group classified as epileptic had the lowest average number of allergies at 0.70. A logistic regression model revealed that for every increase in the number of allergies greater than zero, the odds of being nonepileptic increases by 1.38 times. This study supports polyallergy as a predictive sign for NES. Based on a logistic regression model, we claim that each additional allergy is associated with an increase in the likelihood of a nonepileptic diagnosis by 38%.

ABSTRACT 218

Background and aims: Thromboembolic events are frequently discussed in recent years, for congenital factors increase the risk of thrombosis. Protein C and S deficiencies, antithrombin failure, prothrombin 20210A mutation, activated protein C resistance are the considerable causes leading to hereditary thrombophilia. Here, a young infant who developed an unexpected necrotic hematoma was presented. Methods: CASE A 37-day old female patient with afebrile seizure was admitted. She had an uneventful perinatal period with first degree consanguinity with maternal history of two abortions. Her examination revealed normal between brief convulsive episodes. Complete blood count, biochemical tests, lumbar puncture findings and metabolic studies were normal. She was commenced phenobarbital treatment after hospitalization. EEG revealed no pathologic finding. MRI of brain was unremarkable. On follow-up, he had no seizure of 36 hours after phenobarbital treatment. However, she developed necrotic hematoma on the right hand around the catheter entrance hole while she was only taking liquid treatment. The necrotic hematoma in conjunction with maternal history was considered a thromboembolic event. Thus, factor levels, bleeding parameter, APC-Antithrombin III level, cardiolipin and phospholipid antibody levels have been studied. APC resistance was 55.2 sec. (N>120sec.). Low molecular weight heparin was started. Conclusions: Extravasations of emergency drugs such as calcium and dopamine may develop necrosis while maintenance therapy is not expected to. In the presence of such catheter problems, thrombophilia should be considered. Activated protein C resistance is one of the most important disorders in inherited thrombophilia. Anti-thrombotic prophylaxis may be started to the patients.

Long-term fish oil supplementation attenuates seizure activity in the amygdala induced by 3-mercaptopropionic acid in adult male rats

Several studies have provided evidence of significant effects of omega-3 fatty acids on brain functionality, including seizures and disorders such as epilepsy. Fish oil (FO) is a marine product rich in unsaturated omega-3 fatty acids. Considering that the amygdala is one of the brain structures most sensitive to seizure generation, we aimed to evaluate the effect of long-term chronic FO supplementation (from embryonic conception to adulthood) on the severity of seizures and amygdaloid electroencephalographic activity (EEG) in a 3-mercaptopropionic acid (3-MPA)-induced seizure model using adult rats. Female Wistar rats were fed a commercial diet supplemented daily with FO (300mg/kg) from puberty through mating, gestation, delivery, and weaning of the pups. Only the male pups were then fed daily with a commercial diet supplemented with the same treatment as the dam up to the age of 150days postpartum, when they were bilaterally implanted in the amygdala to record behavior and EEG activity before, during, and after seizures induced by administering 3-MPA. Results were compared with those obtained from rats supplemented with palm oil (PO) and rats treated with a vehicle (CTRL). The male rats treated with FO showed longer latency to seizure onset, fewer convulsive episodes, and attenuated severity compared those in the PO and CTRL groups according to the Racine scale. Moreover, long-term FO supplementation was associated with a reduction of the absolute power (AP) of the fast frequencies (12–25Hz) in the amygdala during the seizure periods. These findings support the idea that chronic supplementation with omega-3 of marine origin may have antiseizure properties as other studies have suggested.

Actual insights into the clinical management of febrile seizures

Febrile seizures (FS) are a benign epileptic manifestation of infancy occurring between 3months and 5years of age and affecting an estimated 2-5% of children. They have usually no important negative effects on motor and cognitive development. Simple FS (generalized seizures, lasting less than 10min and single episodes during the same febrile event) have a benign prognosis in almost all cases and do not require an extensive diagnostic workup. In complex FS (focal semiology and lasting more than 10min, more than one episode during the same febrile event), a more detailed clinical, electroencephalographic, laboratory, and neuroimaging evaluation is necessary because of a higher percentage of underlying detectable causes and a mildly higher risk for later development of epilepsy. Febrile status epilepticus is the most severe type of complex FS even if its morbidity and mortality is extremely low. Simple FS plus (more than one convulsive episode in 24h) have the same benign prognosis of simple FS. Neither intermittent nor continuous prophylaxis is actually recommended both in simple and complex FS because its side effects outweigh its possible benefits. This review summarizes recent developments into the clinical management of FS including a suggested algorithm for simple and complex FS, the concept of simple FS plus, the controversies about the relationships between FS and hippocampal sclerosis, the relationships between FS and complex syndrome such as Dravet syndrome, genetic epilepsy with FS plus or febrile infection-related epilepsy syndrome, and the results of recent epidemiologic studies on febrile status epilepticus.

Fronto-orbital advancement and cranial remodeling for Craniosynostosis in Apert syndrome - A Case Report

Apert syndrome is a form of acrocephalosyndactyly a congenital disorder characterized by malformations of the skull face hands and feet. We present here a case of 6 month old male child with history of feeding difficulties and nasal regurgitation of feeds recurrent episodes of cough since birth. There was abnormal shape of the head and webbing of fingers and toes. There was one episode of convulsion at the age of 5 montha. There was vomiting since 2 days. Examination revealed brachycephaly with prominent eyes downward slanting flat forehead proptosis epicanthic folds hypertelorism low set ears short and depressed nasal bridge mid facial hypoplasia cleft palate and sSyndactyly. Both upper and lower limbs exhibited mild developmental delay for the age. The condition was diagnosed as Apert syndrome. Patient underwent ldquoFronto-orbital advancement and cranial remodeling. At sixth month follow-up the child was conscious and alert Anthropometric examination revealed weight- 12 kg Height -78 cms Head circumference- 46 cms. And developmental assessment showed the child walking with support pincer grasp was present and the child was playing a simple ball game. At age of one year the physical and mental developments were almost normal.