Non-surgical endodontic management of immature permanent mandibular first molar: a 3 year follow-up.

BACKGROUND: Deep carious lesions in immature permanent molars with pulp necrosis pose a serious challenge to contemporary paedodontic practice. A further complex clinical scenario is an immature permanent molar with varying root formation in both roots. This case report demonstrates the successful management of an immature permanent mandibular first molar. CASE REPORT: An 8-year-old boy was referred for endodontic management of 36 by a general practitioner. After clinical and radiographic evaluation, a diagnosis of recurrent chronic periapical abscess with abrupt root closure in the mesial root and cessation of root closure in the distal root was made. Apexification was undertaken in three appointments. In the first appointment, under local analgesia and isolation, the mesial canals were prepared to size #F3 with rotary Protaper Universal and the distal canal was left unprepared. Both canals were irrigated with 5% sodium hypochlorite and calcium hydroxide intracanal medicament was placed. One week later, the mesial canals were obturated and a 4 mm white MTA apical plug was placed in the distal canal. Partial extrusion of the MTA was seen periapically. After 24 h, the hard-setting of the MTA plug was verified, remainder of the distal canal was obturated and core filling was placed followed by a preformed metal crown as an intermediate restoration. FOLLOW-UP: The patient was asymptomatic and showed resolution of the lesion, normal thickness of the PDL space with continuity of the lamina dura after 12 months. Extruded MTA was partially resorbed. Three years follow-up showed complete resorption of the extruded MTA and an intact lamina dura.

Early thalamic lesions in patients with sleep-potentiated epileptiform activity.

OBJECTIVE: To compare the prevalence and type of early developmental lesions in patients with a clinical presentation consistent with electrical status epilepticus in sleep either with or without prominent sleep-potentiated epileptiform activity (PSPEA). METHODS: We performed a case-control study and enrolled patients with 1) clinical features consistent with electrical status epilepticus in sleep, 2) ≥1 brain MRI scan, and 3) ≥1 overnight EEG recording. We quantified epileptiform activity using spike percentage, the percentage of 1-second bins in the EEG tracing containing at least 1 spike. PSPEA was present when spike percentage during non-REM sleep was ≥50% than spike percentage during wakefulness. RESULTS: One hundred patients with PSPEA (cases) and 47 patients without PSPEA (controls) met the inclusion criteria during a 14-year period. Both groups were comparable in terms of clinical and epidemiologic features. Early developmental lesions were more frequent in cases (48% vs 19.2%, p = 0.002). Thalamic lesions were more frequent in cases (14% vs 2.1%, p = 0.037). The main types of early developmental lesions found in cases were vascular lesions (14%), periventricular leukomalacia (9%), and malformation of cortical development (5%). Vascular lesions were the only type of early developmental lesions that were more frequent in cases (14% vs 0%, p = 0.005). CONCLUSIONS: Patients with PSPEA have a higher frequency of early developmental lesions and thalamic lesions than a comparable population of patients without PSPEA. Vascular lesions were the type of early developmental lesions most related to PSPEA.

Rufinamide for the treatment of epileptic spasms.

OBJECTIVE: The purpose of this study was to determine the safety and efficacy of rufinamide for treatment of epileptic spasms. METHODS: We retrospectively reviewed patients treated with rufinamide for epileptic spasms from January 2009 to March 2010. Age, presence of hypsarrhythmia, change in seizure frequency following rufinamide initiation, and side effects were assessed. Patients who had a ≥ 50% reduction in spasm frequency were considered responders. RESULTS: Of all 107 children treated with rufinamide during the study period, 38 (36%) had epileptic spasms. Median patient age was 7 years (range: 17 months to 23). One patient had hypsarrhythmia at the time of treatment with rufinamide, and 9 other patients had a history of hypsarrhythmia. Median starting dose of rufinamide was 9 mg/kg/day (range: 2-18) and median final treatment dose was 39 mg/kg/day (range: 8-92). All patients were receiving concurrent antiepileptic drug therapy, with the median number of antiepileptic drugs being 3 (range: 2-6). Median duration of follow-up since starting rufinamide was 171 days (range: 10-408). Responder rate was 53%. Median reduction in spasm frequency was 50% (interquartile range=-56 to 85%, P<0.05). Two patients (5%) achieved a >99% reduction in spasms. Rufinamide was discontinued in 7 of 38 patients (18%) because of lack of efficacy, worsening seizures, or other side effects. Minor side effects were reported in 14 of 38 patients (37%). CONCLUSIONS: Rufinamide appears to be a well-tolerated and efficacious adjunctive therapeutic option for children with epileptic spasms. A prospective study is warranted to validate our observations.

Circadian patterns of pediatric seizures.

OBJECTIVE: To evaluate the relationship of sleep/wake and day/night pattern to various seizure subtypes and epilepsy localizations. METHODS: Charts of 380 consecutive pediatric patients with epilepsy undergoing video-EEG (V-EEG) over 2 years were reviewed for seizure semiology, EEG localization, occurrence during the day (6 am-6 pm) or night, during wakefulness and sleep, 3-hour time blocks throughout 24 hours, and various epilepsy localizations, and etiology. RESULTS: A total of 1,008 seizures were analyzed in 225 children (mean age 8.5 ± 5.7 years). Sleep and wakefulness predicted seizure semiology and localization more reliably than daytime and nighttime. Auras, gelastic, dyscognitive, atonic, hypomotor, and myoclonic seizures, and epileptic spasms occurred more often in wakefulness, while tonic, tonic-clonic, automotor, and hypermotor seizures occurred more frequently in sleep (p < 0.05). Clonic, atonic, myoclonic, and hypomotor seizures occurred more frequently during daytime. Hypermotor and automotor seizures occurred more frequently at night (p < 0.05). Generalized seizures (6 am-12 pm), temporal lobe seizures (9 pm-9 am), frontal lobe seizures (12 am-6 am), parietal lobe seizures (6 am-9 am), and occipital lobe seizures (9 am-noon and 3-6 pm) revealed specific circadian patterns (p < 0.05). In addition, generalized and temporal lobe seizures occurred more frequently in wakefulness, while frontal and parietal seizures occurred more frequently in sleep, independent of day or night pattern (p < 0.05). CONCLUSION: Sleep and wakefulness, as well as time of day and night, are important considerations in proper characterization of seizure types and epilepsy localization. These findings may contribute to a better understanding of the mechanisms of nonrandom distribution of seizures, and may provide information for individualized treatment options.

Higher evening antiepileptic drug dose for nocturnal and early-morning seizures.

We describe 17 children with nocturnal or early-morning seizures who were switched to a proportionally higher evening dose of antiepileptic drugs and were retrospectively reviewed for seizure outcome and side effects. Of 10 children with unknown etiology, clinical presentation was consistent with nocturnal frontal lobe epilepsy (NFLE) in 5 and benign epilepsy with centrotemporal spikes (BECTS) in 3. After a mean follow-up of 5.3 months, 15 patients were classified as responders; 11 of these became seizure free (5 NFLE, 1 BECTS, 5 with structural lesions) and 4 (2 BECTS, 2 with structural lesions) experienced 75-90% reductions in seizures. Among two nonresponders, seizures in one had failed to resolve with epilepsy surgery. Nine subjects (53%) received monotherapy after dose modification, and none presented with worsening of seizures. Two complained of transient side effects (fatigue/somnolence). Differential dosing led to seizure freedom in 64.7% (11/17) of patients, and 88.2% (15/17) experienced ≥ 50% reductions in seizures.

Microdeletion/duplication at 15q13.2q13.3 among individuals with features of autism and other neuropsychiatric disorders.

BACKGROUND: Segmental duplications at breakpoints (BP4-BP5) of chromosome 15q13.2q13.3 mediate a recurrent genomic imbalance syndrome associated with mental retardation, epilepsy, and/or electroencephalogram (EEG) abnormalities. PATIENTS: DNA samples from 1445 unrelated patients submitted consecutively for clinical array comparative genomic hybridisation (CGH) testing at Children's Hospital Boston and DNA samples from 1441 individuals with autism from 751 families in the Autism Genetic Resource Exchange (AGRE) repository. RESULTS: We report the clinical features of five patients with a BP4-BP5 deletion, three with a BP4-BP5 duplication, and two with an overlapping but smaller duplication identified by whole genome high resolution oligonucleotide array CGH. These BP4-BP5 deletion cases exhibit minor dysmorphic features, significant expressive language deficits, and a spectrum of neuropsychiatric impairments that include autism spectrum disorder, attention deficit hyperactivity disorder, anxiety disorder, and mood disorder. Cognitive impairment varied from moderate mental retardation to normal IQ with learning disability. BP4-BP5 covers approximately 1.5 Mb (chr15:28.719-30.298 Mb) and includes six reference genes and 1 miRNA gene, while the smaller duplications cover approximately 500 kb (chr15:28.902-29.404 Mb) and contain three reference genes and one miRNA gene. The BP4-BP5 deletion and duplication events span CHRNA7, a candidate gene for seizures. However, none of these individuals reported here have epilepsy, although two have an abnormal EEG. CONCLUSIONS: The phenotype of chromosome 15q13.2q13.3 BP4-BP5 microdeletion/duplication syndrome may include features of autism spectrum disorder, a variety of neuropsychiatric disorders, and cognitive impairment. Recognition of this broader phenotype has implications for clinical diagnostic testing and efforts to understand the underlying aetiology of this syndrome.

Magnetic resonance volumetric analysis of hippocampi in children in the age group of 6-to-12 years: a pilot study.

Atrophy of the mesial temporal structures, especially the hippocampus, has been implicated in temporal lobe epilepsy. However, to date, there is very scant data regarding normal volumes of the hippocampus in the pediatric population. This is a pilot study to estimate the normal volumetric data for the Indian pediatric population between 6 and 12 years of age. We have also tried to understand whether age and gender have an effect on the hippocampal volumes in this age group. The study group comprised 20 children, 6-12-years old without history of epilepsy or other neurological deficits. There were nine boys and 11 girls. All scans were performed on a 1.5T GE echo speed scanner. 3D fast SPGR sequence was prescribed in the coronal plane. The images were post-processed on an Advantage Windows 3.1 workstation. Using an automated program, the same observer calculated the hippocampal area, in cubic centimeters, clockwise and anticlockwise. The clockwise/anticlockwise data were subjected to correlation analysis for detecting intra-observer agreement. The mean and SD for left and right hippocampal volumes were estimated. The lower and upper limits for normal hippocampal volumes were determined using 95% (+/- 2SD) limits on either side of the mean. In order to understand the effect of age on various hippocampal volumes we performed regression analysis. Mann-Whitney's test was used to test the significance of differences for gender variations. Correlation analysis established that there was intra-observer agreement. In the Indian pediatric population we have found the mean right hippocampal volume (RHV) to be 2.75 cm(3) and mean left hippocampal volume (LHV) to be 2.49 cm(3). Mean hippocampal volume was found to be 2.67 cm(3) (SD = 0.42). The upper and lower limits for hippocampal volumes were 3.51 cm(3) and 1.83 cm(3), respectively, based on 95% (+/- 2SD) limits on either side of the mean. There was no effect of age or gender on the hippocampal volumes. In the Indian pediatric population we determined hippocampal volumes in a small series of healthy children. We found that hippocampal volumes < or =1.83 cm(3) (< or =2SD) can be considered to be abnormal. These findings can be used as normative data to evaluate cases of hippocampal sclerosis in the Indian population.

Relapse of herpes encephalitis after acyclovir therapy: report of two new cases and review of the literature.

Relapse of herpes simplex virus (HSV) encephalitis following acyclovir therapy has been reported infrequently in children beyond the neonatal period. The pathogenic mechanism of the recurrence is not fully understood. We report two new cases that support a mechanism of latent HSV infection with reactivation of the disease. Our patients were 2 years (#1) and 8 months (#2) old at initial infection. Both presented with fever, lethargy, focal seizures, and focal motor abnormalities. Serum HSV antibodies (Abs) were negative. The patients were treated with acyclovir for 14 and 21 days, respectively. They were readmitted at 1 month, and 4 days after discharge, respectively, with recurrent lethargy, seizures, and choreo-athetoid movements. Serum and CSF HSV Abs were significantly increased. CSF PCR was positive. In patient # 2 acyclovir-sensitive HSV was isolated from a brain biopsy. Both patients were re-treated with acyclovir, but progressed to a neurovegetative state. In our cases, latent HSV infection and reactivation is the most likely explanation for recurrent encephalitis. The immuno-pathogenic mechanisms of the infection recurrence are discussed. Based on the reported cases in the literature, patients younger than 2 years of age and with lower total dose of acyclovir treatments have a higher risk of recurrence.

Absence of hippocampal sclerosis in children with multiple daily seizures since infancy.

We report a series of nine children with multiple daily seizures since infancy who underwent functional hemispherectomy that included en bloc resection of the hippocampus and the temporal neocortex. In all cases, the hippocampi were normal by conventional histology despite the fact that these patients had suffered from recurrent seizures over a long period of time. This observation suggests that extremely frequent seizures in childhood are not invariably associated with the development of hippocampal sclerosis.

Gabapentin treatment in a child with delayed-onset hemichorea/hemiballismus.

A 13-year, 6-month-old female was evaluated for subacute onset of left-sided hemichorea/hemiballismus, with an old, right parietal, cortical, and subcortical stroke as the presumed cause. Treatment with gabapentin was initiated, with good results at 6-month follow-up. Discussion of the differential diagnosis and evaluation of delayed-onset movement disorders in children and the mechanism of action of gabapentin is included.

Seizure onset from periventricular nodular heterotopias: depth-electrode study.

The association between gray matter heterotopias and seizures is well established; whether seizures originate from these lesions is not known. We evaluated three patients with intractable complex partial seizures and periventricular nodular heterotopias (PNHs) with video-EEG monitoring with multiple depth electrodes, including placement in the PNH, to determine whether seizures originate from the PNH. In two of the three patients, all seizures arose from the PNH as low-voltage beta activity. In the third patient, 80% arose from the hippocampi and 20% from the heterotopia. PNHs may serve as an epileptogenic focus in patients with intractable epilepsy.

Migratory leptomeningeal inflammation with relapsing polychondritis.

We report a case of relapsing polychondritis with focal sensorimotor seizures, aseptic meningitis, and migratory leptomeningeal enhancement on contrast MRI. These abnormalities on imaging studies correlated accurately with laterality of the patient's seizures, facilitating early aggressive management of his neurologic symptoms.

Infant botulism: a rare cause of colonic ileus.

We have recently seen two patients with infant botulism, one of whom had radiologic evidence of autonomic and neuromuscular dysfunction. Both infants had been fed small amounts of honey, which is often contaminated with Clostridium botulinum spores, during the Jewish New Year celebration.