Assessing the feasibility of using the ketogenic diet in autism spectrum disorder.

BACKGROUND: Evidence demonstrating efficacy of dietary interventions for autism spectrum disorder (ASD) remains inconsistent. Recent research on the ketogenic diet (KD) for the treatment of ASD has suggested a benefit. Children with ASD often demonstrate ritualised food-specific behaviours, taste and texture aversions, and an increased prevalence of food restrictions and allergies. There is a need to investigate how these features contribute to initiation and adherence of the KD. Two surveys were administered to assess the feasibility of utilising the KD for ASD. METHODS: First, paper surveys were given to caregivers of children presenting to outpatient neurology clinics. Next, experienced clinicians were recruited and surveyed online using Qualtrics. Chi-squared analysis was used to compare ASD and non-ASD caregiver responses. Descriptive metrics were used to present clinician responses. Responses to each question were evaluated individually. RESULTS: One hundred and fourteen surveys were collected from caregivers. There were no significant differences in (1) stated feasibility of adopting a new diet, (2) a carbohydrate restricted diet, (3) diet restrictions, (4) documented allergies or (5) personal/cultural restrictions between groups with and without ASD. Seventy clinician responses were collected. The majority (67.4%) indicated that feasibility for a child with ASD to adopt a KD for any reason depends on ASD severity. Some respondents 73% rated adherence to the KD as more difficult compared to age-matched controls, whereas 26% considered it similar. Multiple familial and child characteristics were rated as increasing the difficulty of successful KD. CONCLUSIONS: The results of the present study suggest that it is feasible for children with ASD to adopt a KD, and success is highly individualised to child and family.

Seizure frequency in children with epilepsy: factors influencing accuracy and parental awareness.

RATIONALE: The objective of this study was to ascertain the accuracy of clinical reports to determine the seizure frequency in children diagnosed with epilepsy. METHODS: We reviewed the clinical record of 78 children (January-May of 2006) admitted to the EEG-video monitoring with epilepsy diagnosis. Clinical reports of parents and the files of EEG-video monitoring were reviewed to determine parents' awareness for seizures. RESULTS: During video-EEG monitoring, 1244 were recorded on 78 children. Seizures were confirmed in 1095 of which 472 were correctly reported (38%) by parents whereas 623 remained under-reported (50%). Parents' report thus had a sensitivity of 43%, positive predictive value of 76% to identify seizures. Based on the EEG-video monitoring, seizures were reported accurately in 22 (28%) and under-reported in 38 (49%) children. In the under-reported group, none of the seizures were recognized in 10 (13%), only a portion identified in 28 children. The parents' report describing seizure frequency has limited value for young children (p=0.01) and children with absence seizures (p=0.03). However, clinical reports were accurate for the children with developmental delay (p<0.06) or not being on any anticonvulsant drug (AED) therapy (p=0.02). CONCLUSION: Our results indicate that a significant number of seizures remain under-reported by parents of children with epilepsy. The current study underscores that the seizure frequency should be interpreted with caution for young children and children with absence seizures. Video-EEG recording has a complimentary role to the clinical observation for the accurate assessment of seizure frequency in children.

Subclinical seizures in children diagnosed with localization-related epilepsy: clinical and EEG characteristics.

OBJECTIVE: Subclinical seizures (SCSs) are characterized by paroxysmal rhythmic epileptiform discharges that evolve in time and space in the absence of objective clinical manifestation or report of a seizure. The aim of this study was to evaluate the frequency and characteristics of SCSs in children with localization-related epilepsy (LRE). METHODS: The results of video/EEG monitoring were reviewed to identify patients with SCS. We identified 187 children diagnosed with LRE, in 32 of whom SCSs were reported in the EEG recording. RESULTS: SCSs were reported only in the children who had received a diagnosis of either symptomatic or cryptogenic LRE. All children had a history of clinical seizure(s). The ictal onset of SCSs was most frequent from the temporal and frontal lobes. SCSs were lateralized to the left hemispheres in 19, right hemisphere in 8, and both hemispheres independently in 5 children. SCSs were more often reported in young children, and associated with a history of developmental delay, infantile spasms, and frequent seizures. EEG abnormalities included background slowing and lack of normal sleep architecture in addition to the epileptiform activity. Seizure freedom was reported less often in children with SCSs. Six patients seizure free at the time of the admission were found to have SCSs. CONCLUSION: Subclinical seizures are not uncommon in children with LRE, in particular, with younger age, developmental disability, and medically refractory clinical course. Video/EEG monitoring will be informative in selected children with LRE to assess the seizure frequency more accurately.

Long-term outcome of symptomatic infantile spasms established by video-electroencephalography (EEG) monitoring.

In this study, we examine the long-term clinical outcome of children with symptomatic infantile spasm. The children between 2 and 18 years of age diagnosed with symptomatic infantile spasms were reviewed. Sixty-eight children (age range, 2-13 years; mean, 4.5 years) met the inclusion criteria. Children who underwent epilepsy surgery were excluded. Age of onset for infantile spasms ranged from 1 to 24 months (mean, 7.1 months). Developmental delay was noted in all; there was seizure freedom in 14 children (20.5%). Infantile spasms were reported as the only seizure type in 10 (14.5%) children older than age 2 years. During the follow-up; symptomatic generalized epilepsy was diagnosed in 23 children (34%) and focal epilepsy in 21 (31%). The long-term outcome of these children remains unchanged in the majority of the children with symptomatic infantile spasms. We could not establish any risk factor that might be related to favorable or adverse outcome.

The frequency of non-epileptic spells in children: results of video-EEG monitoring in a tertiary care center.

RATIONALE: the diagnosis of non-epileptic spells (NES) in children can be challenging, even for experienced clinicians. Our objective was to describe the characteristics of such events. METHODS: this was a retrospective study conducted from January 2004 to December 2006. Inclusion criteria were age >1 month and <18 years and the diagnosis of NES established by video-EEG monitoring. RESULTS: among 746 monitored children (1203 recorded video-EEG sessions), 109 (14.6%) had NES. The mean age of patients with NES was 6.6 years (range 0.1-18). Seventy patients were diagnosed with NES alone; the remaining 39 with both NES and epilepsy. Developmental delay was more frequent among patients with a co-morbid diagnosis of epilepsy (p<0.001). Similar clinical events were reported in both of these groups, save for crying spells/irritability which was more common in children with epilepsy. Frequent manifestations of NES included staring spells in preschool children, crying/irritability, tremor and eye deviation in young children and preschoolers, and limb shaking in adolescents. All of the patients with epilepsy and 19 (27%) of those without epilepsy were receiving antiepileptic drugs. CONCLUSION: our data highlights the importance of accurate diagnosis of NES toward the appropriate treatment of affected children.

Diagnostic utility of an epilepsy monitoring unit.

Guidelines recommend that patients with persist- ent seizures not responsive to standard antiepileptic drugs after 1 year be referred to specialized epilepsy centers for evaluation, which may include inpatient video-electroencephalographic monitoring. This 1-year retrospective, limited-focus review of 213 admissions and 3-year postdischarge review of epilepsy surgery referrals sought to determine if admission to an inpatient adult epilepsy monitoring unit resulted in a definitive diagnosis, a change in diagnosis or treatment, and whether referral patterns were consistent with guidelines. The median duration of time since symptom onset prior to admission was 15 years. At discharge, 87.8% of admissions received a definitive diagnosis. Epilepsy was diagnosed in 73.3% and excluded in 21.6%. Admissions without epilepsy had been treated with antiepileptic drugs for a median of 9 years. Admission resulted in change in treatment in 79%. This review documents the diagnostic utility of an epilepsy monitoring unit while highlighting that time since symptom onset to admission exceeded established guidelines.

Recommendations for the care of women with epilepsy.

The clinical care of women with epilepsy entails special considerations over the life span. Endogenous depression is more prevalent in persons with epilepsy than in the general population and may be unrecognized. Seizure frequency may be influenced by hormonal fluctuations, as reflected by catamenial patterns in up to 25% of women and by changes at menopause. Fertility is lower in women with epilepsy. These women should be evaluated for anovulatory cycles and particularly for polycystic ovary syndrome, with its attendant health risks. It is important to provide folate supplementation during the childbearing years and to evaluate bone health throughout life, providing calcium and vitamin D supplementation when indicated. Particular consideration is indicated before conception and during pregnancy to minimize both potential teratogenicity secondary to antiepileptic drugs (AEDs) and the risks that seizures pose to fetus and mother. At delivery, vitamin K is indicated. Some infants may need to be monitored for AED withdrawal, while others may require a perinatal team if malformations are identified in utero. Breast-feeding is possible, with sedation rarely being a problem. Recognition, evaluation, and management of these issues will minimize the negative impact of epilepsy and improve lifelong quality of life.