Atypical Seizures Research Articles

Dravet syndrome an unusual cause of atypical febrile seizure: a case report

Dravet syndrome is a rare refractory epileptic encephalopathy with an underlying genetic basis. It presents with recurrent febrile seizures in infancy. Though development is normal in infancy, neurodevelopmental delay and neurologic disability begin after the onset of seizure at the age of around 2-4 years. The outcome can be improved by early diagnosis (genetic testing for SCN1A gene) and timely treatment. In this case report we are presenting a 3-year-old female child with recurrent refractory epilepsy associated with fever and was diagnosed with Dravet syndrome.

Assessment of the diagnostic utility of the electroencephalogram in pediatric emergencies

IntroductionWe analyze the diagnostic utility of urgent EEG (electroencephalogram) performed in children under 16 years of age in our center. Material and methodsDescriptive, retrospective, observational study of consecutive patients from 0 to 16 years of age, who underwent an urgent EEG for any reason, from January to December 2022. ResultsOf the 388 patients, 70 were children: 37 (52.85%) women, and 33 (47.14%) men. Average age: 6.27 ± 4.809. Of the 70 patients, 6 (8.57%) had previous epilepsy. Reasons for consultation: 17 febrile seizures, 10 first focal seizures, 10 first TCG seizures, 6 paroxysmal episodes, 6 absences, 3 myoclonus of extremities, 3 syncope, 2 SE, 2 visual alterations, 2 low level of consciousness, 2 cyanosis, 2 suspected meningitis or encephalitis, 1 choking, 1 atypical headache, 1 chorea, 1 presyncope, 1 language delay. Of the 70 patients, 47 had a normal EEG (67.14%). Of the 47 patients with a normal EEG, 10 were diagnosed with epilepsy, and 3 of them began receiving antiepileptic treatment upon discharge. None of the patients with suspected syncope or paroxysmal disorder (17 patients, 24.28%) had EEG abnormalities. Of the 17 patients with atypical febrile seizures, 3 had EEG abnormalities. ConclusionsA third of the EEG records performed in the Emergency Department showed alterations, probably due to the time taken. Almost half of the patients with suspected epilepsy or EE showed EEG abnormalities, which confirmed the diagnosis in these cases and encouraged the clinician to start drug treatment. No case with a high suspicion of epilepsy was dismissed due to the normality of the EEG recording in our series. No patient diagnosed with syncope or paroxysmal disorder had EEG abnormalities. Nearly a quarter of patients with atypical febrile seizures showed EEG abnormalities. We barely register cases of status epilepticus, probably due to the degree of complexity of our center.

Developing an alternative care pathway for emergency ambulance responses for adults with epilepsy: A Discrete Choice Experiment to understand which configuration service users prefer. Part of the COLLABORATE project

IntroductionTo identify service users’ preferences for an alternative care pathway for adults with epilepsy presenting to the ambulance service. MethodsExtensive formative work (qualitative, survey and knowledge exchange) informed the design of a stated preference discrete choice experiment (DCE). This hypothetical survey was hosted online and consisted of 12 binary choices of alternative care pathways described in terms of: the paramedic's access to medical records/ ‘care plan’, what happens next (described in terms of conveyance), time, availability of epilepsy specialists today, general practitioner (GP) notification and future contact with epilepsy specialists. DCE scenarios were described as: (i) typical seizure at home. (ii) typical seizure in public, (iii) atypical seizure. Respondents were recruited by a regional English ambulance service and by national public adverts. Participants were randomised to complete 2 of the 3 DCEs. ResultsPeople with epilepsy (PWE; n = 427) and friends/family (n = 167) who completed the survey were representative of the target population. PWE preferred paramedics to have access to medical records, non-conveyance, to avoid lengthy episodes of care, availability of epilepsy specialists today, GP notification, and contact with epilepsy specialists within 2–3 weeks. Significant others (close family members or friends) preferred PWE experiencing an atypical seizure to be conveyed to an Urgent Treatment Centre and preferred shorter times. Optimal configuration of services from service users’ perspective far out ranked current practice (rank 230/288 possible configurations). DiscussionPreferences differ to current practice but have minimal variation by seizure type or stakeholder. Further work on feasibility of these pathways in England, and potentially beyond, is required.

Clinico-epidemiological profile and complications of hand foot mouth disease among cases attending tertiary care centre in Northern Kerala, India

Background: Hand-Foot-Mouth Disease (HFMD), has suddenly become more prevalent than ever in Kerala and is reported in almost all districts. Earlier studies showed EnteroVirus-A71 to be the causative agent, but frequent outbreaks are also caused by coxsackievirus A6 and A16. In 2022 there was a surge in the HFMD cases being reported to the institution with few cases requiring hospital admission due to complications. Methods: Study was a hospital based descriptive study in Government Medical College, Kannur, Kerala, India among Paediatric cases of HFMD, presented to the institution from April to July, 2022. Data was collected by direct interview using a semi structured questionnaire. It was coded and entered in MS Excel and analysed using SPSS 21. Results: Of the total 50 cases studied, 60% of the cases were in the age group of 1-3 years. Majority (58%) were females, with maximum cases being reported in the month of May. 32% had contact history with 18% being family contacts. Fever (72%), lesions on the hand (86%), foot (92%) and oral cavity (68%) were the most common clinical presentation. Complications developed in 10% of the cases, requiring hospital admission. Most common complication being neurological complication- acute cerebellar ataxia and atypical febrile seizure. A major factor noted among the cases that developed complications was that all were having chronic malnutrition. Conclusions: Even though majority of the cases had uncomplicated course, 10% cases showed complications. Therefore, awareness should be made regarding chances of complications and its risk factors. Assessment of nutritional status should be done in all cases.

Neurologic Manifestations of Influenza in Children.

We assessed the neurologic manifestation of influenza among hospitalized children with influenza (neuro-flu), comparing their demographic and clinical characteristics to hospitalized children without neurologic manifestation (classic-flu). A retrospective, cohort study. All children with laboratory confirmed influenza (PCR), admitted to the Soroka University Medical Center (SUMC) between 2016 and 2019 were included. Overall, 951 patients were identified: 201 with neuro-flu, and 750 with classic-flu. Seizures (n = 125) were the most common neurological manifestation of neuro-flu (seizure-flu): 73 simple febrile seizures, 45 atypical febrile seizures, and 7 afebrile seizures. Neurological comorbidities rates were significantly higher in neuro-flu versus classic-flu (13.0% vs. 6.0%), while respiratory and cardiac comorbidities were less common in neuro-flu (4.5% and 0.5% vs. 8.0% and 4.5%, respectively). Neuro-flu (compared with classic-flu) was associated with leukocytosis (21.0% vs. 13.0%, P < 0.001) and lower C-reactive protein (CRP) levels (2.4 ± 4.1 vs. 3.3 ± 5.4, P = 0.03). Notably, patients with classic flu had a more prominent respiratory disease, as they had more chest radiographs (CXR) performed (60.5% vs. 45.0%, P < 0.001), higher rates of pneumonia (27.0% vs. 12.0%, P < 0.001), and antibiotic (antibacterial) treatment (60.0% vs. 42.0%, P < 0.001). Influenza can appear as a neurologic disease, manifested mainly with febrile seizures. Children with neuro-flu have more neurologic comorbidities, suggesting that neuro-flu is mainly driven by host-factors, rather than by pathogen-factors. The relatively lower rates of pneumonia in neuro-flu suggests that these patients are admitted in the early stage of the influenza infection, which triggers the neurologic response.

Anatomical features decide the atypical seizure manifestation of parahypothalamic hamartomas.

BackgroundThe intrahypothalamic phenotype of hypothalamic hamartomas (HH) is associated with epilepsy, and the parahypothalamic phenotype usually leads to central precocious puberty but not neurological comorbidities or seizures. No study has confirmed the pathological role of parahypothalamic hamartomas in epileptogenesis, and the underlying mechanism is yet to be elucidated.ObjectiveWe aimed to investigate whether parahypothalamic hamartomas are intrinsically epileptogenic and elucidate the underlying pathway of epileptogenesis.MethodsWe reviewed 92 patients with HH-related epilepsy, categorized them by the classification system of Delalande and Fohlen, and further classified Type I (corresponding to parahypothalamic HH) into the following three groups based on the relationship between the lesion and mammillary bodies (MB): entirely invaded (Group 1), partially connected (Group 2), and not connected at all (Group 3). We examined different anatomical features with their relationship to clinical manifestations. Stereoelectroencephalography (SEEG) was implanted in both HH and extra-HH cortices in different groups to identify the epileptogenic zone. Corticocortical evoked potentials (CCEPs) were also used to determine the pathological correlation among different regions to determine the related epileptogenic network.ResultsA total of 13 patients presented with parahypothalamic HH and 10 (76.9%) presented with non-GS only, with late-onset age and normal cognitive development, which is different from classical clinical features. SEEG showed that HH is intrinsically epileptogenic in MB-involved parahypothalamic groups. No statistical difference was found in onset age (p = 0.213), and lesions horizontally oriented from the tuber cinereum without connection to MB were not involved in seizure genesis. CCEP indicated a pathological connection among HH, middle cingulate cortex, and insular cortex.ConclusionThe parahypothalamic HH can also cause epilepsy and is different from classic HH-related seizures, by non-GS only with the late-onset age and normal cognitive development. MB is proven to be related to non-GS by the mamillo-cingulate-cortex pathway.

Revocable attention deficit hyperactivity disorder- An eye-opener

To highlight the clinical and electroencephalographic features, treatment strategies and outcome in atypical variant of benign childhood epilepsy with centrotemporal spikes (BECTS). Subject underwent video electroencephalograph (EEG), high-resolution magnetic resonance imaging (MRI), neuropsychological evaluation and language assessment and response to medication was followed up. In addition to sodium valproate, treatment with intravenous methylprednisolone (given as monthly bolus doses) was given as the seizures remained refractory to anti-seizure medications (ASM) alone. Earlier onset, increased frequency and duration of focal seizures compared to prototype Rolandic epilepsy are cardinal features suggestive of atypical variant of BECTS. Head drop and truncal sway due to axial or axiorhizomelic atonia occurring several times per day or week was the key manifestation. When the atypical seizures commenced, one or more of the following problems can occur: hyperactivity, attention deficit, and mild cognitive or language dysfunction. Child became seizure free on a combination of intravenous methylprednisolone, with sodium valproate and his hyperactivity reversed. BECTS in children with an early age of onset, frequent and prolonged seizures and scholastic decline pari passu with seizure onset are more likely to evolve into atonic-BECTS. Carbamazepine, oxcarbazepine and some benzodiazepines may worsen these seizures. They can become seizure free with immunomodulatory therapy, namely methylprednisolone (given as monthly bolus doses) or intravenous immunoglobulin (IVIG), and have complete resolution of the transient motor and cognitive impairment. Atonic-BECTS needs to be differentiated from Lennox–Gastaut syndrome since it is potentially treatable and children recover with no sequel.

Preterm Infant of Diabetic Mother with a Retrorectal Mass: A Troubling Tail Tale.

Preterm Infant of Diabetic Mother with a Retrorectal Mass: A Troubling Tail Tale.

An unexpected diagnosis in early pediatric age group: Fahr syndrome manifested with epileptic seizure

Intracerebral calcifications are one of the essential parameters in the diagnosis of some neurological diseases. Fahr group is a rare pathologic entity presenting with basal ganglion calcifications. Symmetrical basal ganglion calcifications and an etiologic reason (metabolic, hormonal, or infectious) constitute the main picture of Fahr syndrome. This syndrome usually begins at the third and fourth decade and is often with accompanying neuropsychiatric symptoms. We presented the case of a 7 years old child who was admitted to the hospital with an atypical tonic-clonic epileptic seizure. Bilateral basal ganglion calcifications were detected at the level of globus pallidus. Clinical and laboratory findings were compatible with secondary hyperparathyroidism.

Factors associated with anti-seizure medication utilization for eclamptic seizures: 1995–2015

ObjectiveWe sought to ascertain the drivers of the use of anti-seizure medications (ASMs) other than magnesium sulfate (MgSO4) in seizure management in a cohort of pregnant and postpartum women with eclamptic seizure. MethodsCases of seizure activity attributed to eclampsia from 1995–2015 at 2 large urban academic medical centers were identified and reviewed by a neurologist and an obstetrician. Analyses focused on patterns of ASM utilization among women according to timing, recurrence, posterior reversible encephalopathy syndrome, and specialty consultation with additional sub-analysis focusing on recurrent seizures only. Results93 cases of eclampsia were identified. 100% of subjects received MgSO4. 52% of women received an ASM in addition to MgSO4. Subjects with seizures occurring post-partum, with >1 seizure, or who had a formal neurology consult were more likely to receive an ASM in addition (risk ratio [RR] 3.05 [95% confidence interval [CI] [1.30–7.11], RR 3.01 [95% CI 1.29–7.02], and RR 6.29 [2.37, 16.71] respectively). Postpartum recurrent seizures or those receiving a neurology consult were also more likely to be treated with ASMs compared to recurrent or comanaged seizures occurring before delivery (RR 1.55 [1.02, 2.37] and 1.65 [1.02, 2.69]). ConclusionsIn this retrospective cohort, patients with atypical seizure presentation (e.g., postpartum and/or recurrent) and women who were comanaged with a neurologist were more likely to receive an ASM other than MgSO4.

Long QT syndrome with potassium voltage-gated channel subfamily H member 2 gene mutation mimicking refractory epilepsy: case report

BackgroundEpileptic seizures can be difficult to distinguish from other etiologies that cause cerebral hypoxia, especially cardiac diseases. Long QT syndrome (LQTS), especially LQTS type 2 (LQT2), frequently masquerades as seizures because of the transient cerebral hypoxia caused by ventricular arrhythmia. The high rate of sudden death in LQTS highlights the importance of accurate and early diagnosis; correct diagnosis of LQTS also prevents inappropriate treatment with anti-epileptic drugs (AEDs).Case presentationWe report a case of congenital LQT2 with potassium voltage-gated channel subfamily H member 2 gene (KCNH2) mutation misdiagnosed as refractory epilepsy and treated with various AEDs for 22 years. The possibility of cardiac arrhythmia was suspected after the patient presented to the emergency room and the electrocardiograph (ECG) monitor showed paroxysmal ventricular tachycardia during attacks. Atypical seizure like attacks with prodromal uncomfortable chest sensation and palpitation, triggered by auditory stimulation, and typical ventricular tachycardia monitored by ECG raised suspicion for LQT2, which was confirmed by exome sequencing and epileptic seizure was ruled out by 24-h EEG monitoring. Although the patient rejected implantation of an implantable cardioverter defibrillator, β blocker was given and the syncope only attacked 1–2 per year when there was an incentive during the 5 years follow up.ConclusionsOur case illustrates how long LQTS can masquerade convincingly as epilepsy and can be treated wrongly with AEDs, putting the patient at high risk of sudden cardiac death. Careful ECG evaluation is recommend for both patients with first seizure and those with refractory epilepsy.

Successful management of status epilepticus with yoga prana vidya healing as a complementary therapy: A case study of atypical febrile seizure

: Epilepsy is one of the most common disorders of the nervous system and affects people of all ages, races, and ethnic backgrounds, and seen more commonly in children. Atypical febrile seizure with Status epilepticus is a disorder known for significant morbidity and mortality. Despite newer antiepileptic drugs, 30 % of subjects are refractory to the conventional treatment with a risk of recurrence and neuro-developmental disorder. This paper presents one such case of a child handled successfully by Yoga Prana Vidya (YPV) Energy healing complementarily to conventional therapy. This is a prospective case study of a 2-month-old infant, an emergency case of atypical febrile seizures with status epilepticus, where YPV Healing intervention was done from the beginning while admitting doctors had given a guarded prognosis. This case study reports the normal physical, mental &amp; psychological development of the subject following YPV Healing with 3-year follow up, and the subject was observed to be normal without any recurrence of the disease or neuro-developmental abnormality.: Although the use of Complementary and Alternative Medicine (CAM) has been evaluated globally, there are few studies available on the use of Energy healing in various ailments. YPV Healing has been emerging as an effective modality of treatment for various physical, psychological ailments without any side effects. It can be effectively used from distance even without physically examining the subject. It has been used as a complementary or alternative modality of treatment in some severe life-threatening emergency conditions, and further studies may be conducted with appropriate sample sizes.

Atypical neurological manifestations in Wernicke’s encephalopathy due to hyperemesis gravidarum

ABSTRACT Objectives: Hyperemesis gravidarum is known to induce nutritional, water and electrolyte deficiencies which can be fatal if not treated urgently. Thiamine deficiency may lead to a constellation of neurological symptoms that include Wernicke encephalopathy. Moreover, Wernicke encephalopathy is typically manifested as ocular paresis, ataxia and confusion. Methods: Retrospective review of 6 women who developed neurological abnormalities following hyperemesis gravidarum and were treated with varying dosage of parenteral thiamine. Results: Five women developed atypical neurological symptoms, namely, slurred speech, visual loss, seizure and aggressive behaviour while one woman developed typical clinical triad of Wernicke encephalopathy after hyperemesis gravidarum. Magnetic Resonance Imaging (MRI) scans revealed abnormalities suggestive of Wernicke encephalopathy in three women only. All women improved after parenteral thiamine administration during hospital stay and had a complete neurological recovery during 2 months follow up. Discussion: Wernicke encephalopathy may not be necessarily associated with the typical neurological triad and may not have noticeable hyperintensity signal in dorsomedial thalami, mammillary bodies, hippocampus and periaqueductal region during magnetic resonance imaging. Atypical neurological signs and symptoms following hyperemesis gravidarum would invariably respond immediately to appropriate dosage of parenteral thiamine. A lower loading dosage of thiamine (100 mg thrice daily) appeared adequate for management in women with normal MRI scans.

Study of febrile seizures among children admitted in a tertiary care hospital

Background: Febrile seizure is a common problem in children below 6years of age. This study was conducted to evaluate the clinical profile of children presenting with febrile seizures and to identify the risk factors for recurrence of febrile seizures in these children. Materials and Methods: The study was done on all children in the 6 month to 6 year age who were diagnosed as having febrile convulsion admitted in Pediatric ward or intensive care unit of Bangalore Baptist Hospital a tertiary care centre, Bangalore from January 2018 to august 2019. Results: There were112children which constituted 7.8% of total Paediatric admissions. Mean age of presentation was 25.815.19 months with peak in less than 2 years of age. Mean duration of seizure was 1.25 ( 1.23) min. Out of which 64% had a febrile seizure for the first time and 36% had recurrent febrile seizures and 68 (60.7%) were malesand 44 (39.2%) females. Simple febrile seizure and complex febrile seizure were observed in 80 (71.4%) and 22 (19.6%) of patients respectively. The majority of children (71.8%) had a generalized tonic-clonic seizure followed by tonic seizuresaround 17.3% had positive family history. Upper respiratory tract infections were the commonest cause of fever in these children. Low temperature at the onset of Febrile Seizure (p=0.001), short duration of fever before the onset of Febrile Seizure (0.026) and atypical Febrile Seizure (p=0.022) were the risk factors for a recurrent febrile seizure. Conclusions: Febrile Seizure is a common pediatric problem seen in males, observed predominantly in children below the age of two years and simple febrile seizure was the commonest variety. Almost onethird of children with Febrile Seizure are at risk for recurrence at a later date. The risk factors for these recurrences are modest rise in body temperature at the onset of febrile seizure, the onset of seizure within 12 hours of fever and atypical presentation. Keywords: Febrile seizure, FS.

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra.

Purpose:To describe the seizure pattern, treatment strategies and outcome in a series of children with Rolandic seizures or childhood epilepsy with centrotemporal spikes.Materials and Methods:Patients were defined as Rolandic epilepsy if on electroencephalographic studies high voltage spike and waves were seen in centrotemporal areas, could be followed by slow waves, often activated on sleep and could shift from one side to other or be secondarily generalized. Typical (TRS) or benign were those with normal intellect. Atypical rolandic seizures (ARS) were those associated with neuroregression of language and cognitive milestones. Patients were treated with antiepileptic drugs if more than one episode occurred or the first episode was generalized status epilepticus.Results:Thirty-three patients were included over the period of eight years (2012-2020). There was male preponderance (21 males versus 12 females). Four patients (12.12%) later evolved into Landau Kleffner syndrome (ARS group). The mean age of onset of epilepsy in the TRS group (29 patients) was 7.2 (+/-2.2) with the youngest patient being 4 years and the eldest being 12 years. In the ARS group the mean age of onset was 5 yrs. (+/-1.41). In the TRS group, 23 (79.31%) patients were managed on monotherapy AED. Seventeen patients (58.62%) responded (remission) to carbamazepine monotherapy alone. Six patients (20.68%) could afford oxcarbazepine monotherapy and went in remission with this therapy. In the ARS group all patients required three drugs (valproate, clobazam and levetiracetam). By the end of the study period, 23/33 (75.75%) patients remained seizure free.Conclusions:Most patients with rolandic seizures have excellent prognosis being seizure free around puberty. The neurological outcome in most patients was normal.

Seizure Exacerbation and Hormonal Cycles.

1. Diana Bordalo, MD* 2. Raquel Oliveira, MD† 3. Paula Fonseca, MD*,‡ 4. Teresa Temudo, PhD§ 1. *Department of Pediatrics, 2. ‡Adolescent Medicine Unit, Unidade de Vila Nova de Famalicao, Vila Nova de Famalicao, Portugal; 3. †Department of Pediatrics, Unidade Local de Saude do Alto Minho, Viana do Castelo, Portugal; 4. §Neuropediatric Unit, Department of Pediatrics–Centro Materno-Infantil do Norte, Centro Hospitalar do Porto, E.P.E. Porto, Portugal A 16-year-old girl presents to the pediatric emergency department after an episode of loss of consciousness, preceded by dizziness and followed by a generalized tonic-clonic seizure that lasted ∼10 minutes, with spontaneous recovery. She reports 2 previous episodes of loss of consciousness, one not witnessed, the other interpreted as a vasovagal syncope. Family history is positive for epilepsy in a great-grandmother. She has been otherwise previously healthy. Upon physical assessment, she is hemodynamically stable, nonfebrile, and has a normal neurological examination. Initial laboratory findings are normal, including urine toxicology screening, serum electrolytes, and inflammatory markers. An electrocardiography and an electroencephalography (EEG) performed 24 hours after the seizure are normal. Continuous ambulatory electrocardiography monitoring is normal. Due to the atypical seizure presentation with unwitnessed episodes, a tilt table test and a brain magnetic resonance imaging are performed, both reported as normal. During the follow-up period, the patient has recurrent seizures, and notes that these episodes follow a monthly pattern, always on the third day of the menstrual cycle. She had her menarche at ∼12 years old with irregular periods for the first 2.5 to 3 years, during which time she was seizure-free. She currently experiences some …

A study of prevalence of abnormal EEG and its association between various clinical presentations of atypical febrile seizures

Background: Quantitative EEG is a rapidly emerging tool in the diagnosis and follow up of various neurological disorders and can act as predictive marker for subsequent epilepsy in children with complex febrile seizure. The present study aimed to estimate the prevalence of abnormal electroencephalogram (EEG) and to find the association between Quantitative EEG (qEEG) and various clinical features of atypical febrile seizures(AFS).Methods: EEG was recorded along with clinical features including the age at onset, duration of episode, number of episodes in a day, type of seizure and the recurrences from the children aged between 6-60 months with atypical febrile seizures. EEG recordings were classified into Normal and abnormal EEG with epileptiform changes by the expert interpretation and the distribution of above said clinical features in the both groups were analyzed. It is also attempted to find the association between qEEG and few of the clinical features.Results: Prevalence of abnormal EEG in atypical febrile seizures was 33.9%. There were no significant differences in the distribution of abnormal EEG and their association with various clinical features of AFS. Significantly increased absolute power of θ and α waves were recorded from the frontal montages in the children with epileptiform changes in the EEG.Conclusions: qEEG changes can be also considered as marker of severity of febrile seizure episodes. Many prospective studies with long-term follow up are required to establish the predictability of future epilepsy by qEEG.

Assessment of thyroid function among children with epilepsy receiving anticonvulsant monotherapy: a hospital based prospective study

Background: Data on influence of antiepileptic drugs (AED) on thyroid profile in children is limited and is still controversial. This study aimed to investigate the effects of valproate, levetiracetam, phenobarbitone and oxcarbazepine monotherapy on thyroid function in children after one year of therapy.Method: A total of 106 children (39 girls and 67 boys) with new onset and controlled epilepsy treated with valproate (n=52), phenobarbitone (n=12), oxcarbazepine (n=14) and levetiracetam (n=28) were enrolled in the study. Serum thyroxine (T4, T3) and thyroid-stimulating hormone (TSH) level were measured before and at one year of therapy.Results: At baseline average T4, T3 and TSH concentrations were not different between the drug groups. Except levetiracetam all antiepileptics increased TSH after one year of therapy and there was significant difference in TSH increment in valproate treated patients compared to other anticonvulsants. All anti-epileptics except levetiracetam was found to decrease T4 and T3 after one year of therapy but there was no significant difference among the groups, unlike TSH. Sodium valproate was the most frequently used antiepileptic drug. None of children had any symptoms of hypothyroidism, only 3% had signs of hypothyroidism which included goitre on examination. Out of various seizure disorders generalised tonic clonic type was most common (47.5%) followed by atypical febrile seizure (23%).Conclusions: All antiepileptic drugs studied except levetiracetam had varying degrees of deleterious effects on thyroid function.

Telephone-based follow-up of children with epilepsy: Comparison of accuracy between a specialty nurse and a pediatric neurology fellow

BackgroundChildhood epilepsy forms a significant burden on the health-care delivery system. Only a few pediatric neurologists available in most of the developing countries and caregivers face a lot of financial and logistic hardships, apart from a long waiting period for initial and follow up visits. Telemedicine is a proposed effective alternative in overcoming this burden. MethodsTelephonic consultation by a pediatric neurology fellow was compared with that of a specialty nurse; both against face-to-face consultation (gold standard). Care-givers of children 4 months-18 years with epilepsy were telephonically consulted 24−48 hours before their scheduled hospital appointment by one specialty nurse and one pediatric neurology fellow at least 24 h apart in a random sequence. During the hospital visit, another pediatric neurology fellow blinded to the telephonic consultation, documented the same after Face-to-Face interview. ResultsIn 141 children with epilepsy, 504 critical clinical events were identified. Telephonic consultation by pediatric neurology fellow had a sensitivity of 99 %, 97 %, and 100 % and specificity of 100 % each in detecting whether the child had any breakthrough seizure, any adverse event and whether the drug compliance was adequate or poor respectively, as compared to face-to-face consultation. Telephonic consultation by specialty nurse had a sensitivity of 91 %, 84 %, and 98 % and specificity of 97 %, 99 %, and 81 % in detecting whether the child had any breakthrough seizure, adverse event and whether the drug compliance was adequate or poor respectively. But the specialty nurses fared poorly in identifying atypical seizure semiologies like atonic and myoclonic seizures and documenting an exact number of breakthrough seizures, as well as few subjective adverse effects like behavioral abnormality and scholastic worsening, which was performed excellently by the pediatric neurology fellow. ConclusionsTelephonic consultation in childhood epilepsy by pediatric neurology fellow has excellent sensitivity and specificity. A specialty nurse has also acceptable sensitivity and specificity in comparison with a face-to-face consultation.

Genetic epilepsy with febrile seizures plus (GEFS+)

Febrile seizures (FS) occur in about 2–3% of children aged 3 months to 5 years. Atypical febrile seizures are those with a focal component. Each subsequent febrile attack increases the risk of transformation into epilepsy. After the third febrile seizure, the risk of additional episodes of febrile seizures is already approaching 50%, and the risk of formation of epilepsy is 15.8%. Recent studies show the great contribution of genetic causes to the development of genetic epilepsy with febrile seizures plus (GEFS+). GEFS+ includes a combination of some febrile seizures with subsequent afebrile attack, or recurring febrile seizures after 6 years. The genetic causes of GEFS+ are both monogenic (in particular, disorders in the SCN1B, SCN1A, GABRG2, GABRD, SCN9A, STX1B, HCN1 genes, etc.) and copy number variations. Twin methods suggest that different genetic factors play a role in the case of FS, FS+ and FS with subsequent epilepsy. Genetic cause can be found in about 30% of cases, that affects not only the final diagnosis and prognosis for the patient, but also the prevention of disease in the family. In GEFS+ seizures are usually generalized tonic-clonic, less often myoclonic, myoclonic-atonic seizures, absences and status epilepticus, but sometimes they also describe focal seizures. The clinical picture of patients with GEFS+ varies from family febrile seizures (the least severe cases) to Drave-like syndrome (the most severe cases), although all of them have a predominantly normal level of intellect.