Changes in pediatric referrals after the 2009 ketogenic diet consensus recommendations.

BACKGROUND: In 2009, the International Ketogenic Diet Study Group published recommendations for children receiving ketogenic diet (KD) therapy for epilepsy. The document included a table listing epilepsy syndromes and conditions in which the KD has been particularly beneficial, hoping that physicians would refer children for the KD sooner. PURPOSE: To measure the impact of these 2009 recommendations on referral practice, we compared children initiated on the KD at Johns Hopkins Hospital (JHH) 10 years before and after the recommendations. RESULTS: Overall, children referred to the KD who met indications increased from the pre- to post-recommendation group, 44 % (112/256) to 69 % (175/255) (p < 0.001), with JHH neurologists specifically referring more frequently (10/112, 9 % to 58/175, 33 %) (p < 0.01). Referrals increased for Glut-1 deficiency (0 % to 2.4 %, p = 0.015), Dravet syndrome (0 % to 6.7 %, p < 0.01), Rett syndrome (0.4 % to 3 %, p = 0.018), and formula-fed only status (16 % to 31 %, p < 0.01). The chances of > 50 % seizure reduction for all children referred improved slightly between decades (56 % to 61 %, p = 0.30). CONCLUSIONS: Following the 2009 recommendations, our study shows there was an increase in referrals for children with indications at our center. Referrals from neurologists at our own institution increased the most. Ketogenic diet efficacy improved slightly over time but did not reach significance.

A Comparison of Patients' and Neurologists' Assessments of their Teleneurology Encounter: A Cross-Sectional Analysis.

Background and Objectives: To better understand patients' and neurologists' assessments of their experiences regarding effectiveness of teleneurology encounters. Methods: Following an audio-video telehealth visit, neurologists asked patients to participate in a survey-based research study about the encounter, and then, the neurologists also recorded their own evaluations. Data were analyzed using standard quantitative and qualitative techniques for dichotomous and ordered-category survey responses in this cross-sectional analysis. Results: The study included unique encounters between 187 patients and 11 general neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of the patients (66.8%, 125/187) were female. One third (33.2%; 62) were patients new to the NYU Langone Health neurology practices. The most common patient chief complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Most patients (94.7%, 177/187) reported that the teleneurology encounter satisfied their needs. Patients and their neurologists agreed that the experience was effective in 91% (162/178) of encounters, regardless of whether the visit was for a new or established patient visit. Discussion: More than 90% of new and established patients and their neurologists agreed that teleneurology encounters were effective despite some limitations of the examination, the occasional need for patient assistance, and technical difficulties. Our results provide further evidence to justify and to expand the clinical use of teleneurology.

Neurologists' Evaluations of Experience and Effectiveness of Teleneurology Encounters.

Background and Objectives: To better understand neurologists' assessments of the experiences and effectiveness of teleneurology encounters. Methods: After completing an audio-video telehealth visit with verbally consenting patients, neurologists recorded their evaluations of the encounter. Data were analyzed using standard quantitative and qualitative techniques. Results: The study included unique encounters between 187 patients and 11 neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of patients (66.8%, 125/187) were female. One third of patients (33.2%; 62) were new patients. The most common patient complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Neurologists reported that they completed a virtual examination that provided enough information for medical decision-making in 94.9% of encounters (169/178, 9 missing responses). Fourteen of 25 examination elements important for medical decision-making could be performed sufficiently during virtual encounters. Examination assistance was needed for 16.4% (30/183) of patients, who were, on average, 17.3 years older than those who did not require assistance (62.9 years vs. 45.6 years, p = 0.0002). In 19.1% (34/178) of encounters, neurologists learned clinically relevant information from seeing patients in their homes. Neurologists' assessments of the effectiveness of encounters were not related to the presence (97.2%, 35/36 effective) or absence (95%, 134/141 effective) of technical difficulties (p = 0.5729) in 177 encounters (10 missing responses). Discussion: Neurologists reported that nearly 95% of teleneurology encounters were effective despite limitations of the virtual examination, occasional need for patient assistance, and technical difficulties.

Trajectories of Neurologic Recovery 12 Months After Hospitalization for COVID-19: A Prospective Longitudinal Study.

BACKGROUND AND OBJECTIVE: Little is known about trajectories of recovery 12 months after hospitalization for severe COVID-19. METHODS: We conducted a prospective, longitudinal cohort study of patients with and without neurologic complications during index hospitalization for COVID-19 from March 10, 2020, to May 20, 2020. Phone follow-up batteries were performed at 6 and 12 months after COVID-19 onset. The primary 12-month outcome was the modified Rankin Scale (mRS) score comparing patients with or without neurologic complications using multivariable ordinal analysis. Secondary outcomes included activities of daily living (Barthel Index), telephone Montreal Cognitive Assessment (t-MoCA), and Quality of Life in Neurologic Disorders (Neuro-QoL) batteries for anxiety, depression, fatigue, and sleep. Changes in outcome scores from 6 to 12 months were compared using nonparametric paired-samples sign test. RESULTS: Twelve-month follow-up was completed in 242 patients (median age 65 years, 64% male, 34% intubated during hospitalization) and 174 completed both 6- and 12-month follow-up. At 12 months, 197/227 (87%) had ≥1 abnormal metric: mRS >0 (75%), Barthel Index <100 (64%), t-MoCA ≤18 (50%), high anxiety (7%), depression (4%), fatigue (9%), or poor sleep (10%). Twelve-month mRS scores did not differ significantly among those with (n = 113) or without (n = 129) neurologic complications during hospitalization after adjusting for age, sex, race, pre-COVID-19 mRS, and intubation status (adjusted OR 1.4, 95% CI 0.8-2.5), although those with neurologic complications had higher fatigue scores (T score 47 vs 44; p = 0.037). Significant improvements in outcome trajectories from 6 to 12 months were observed in t-MoCA scores (56% improved, median difference 1 point; p = 0.002) and Neuro-QoL anxiety scores (45% improved; p = 0.003). Nonsignificant improvements occurred in fatigue, sleep, and depression scores in 48%, 48%, and 38% of patients, respectively. Barthel Index and mRS scores remained unchanged between 6 and 12 months in >50% of patients. DISCUSSION: At 12 months after hospitalization for severe COVID-19, 87% of patients had ongoing abnormalities in functional, cognitive, or Neuro-QoL metrics and abnormal cognition persisted in 50% of patients without a history of dementia/cognitive abnormality. Only fatigue severity differed significantly between patients with or without neurologic complications during index hospitalization. However, significant improvements in cognitive (t-MoCA) and anxiety (Neuro-QoL) scores occurred in 56% and 45% of patients, respectively, between 6 and 12 months. These results may not be generalizable to those with mild or moderate COVID-19.

A prospective study of long-term outcomes among hospitalized COVID-19 patients with and without neurological complications.

BACKGROUND: Little is known regarding long-term outcomes of patients hospitalized with COVID-19. METHODS: We conducted a prospective study of 6-month outcomes of hospitalized COVID-19 patients. Patients with new neurological complications during hospitalization who survived were propensity score-matched to COVID-19 survivors without neurological complications hospitalized during the same period. The primary 6-month outcome was multivariable ordinal analysis of the modified Rankin Scale(mRS) comparing patients with or without neurological complications. Secondary outcomes included: activities of daily living (ADLs;Barthel Index), telephone Montreal Cognitive Assessment and Neuro-QoL batteries for anxiety, depression, fatigue and sleep. RESULTS: Of 606 COVID-19 patients with neurological complications, 395 survived hospitalization and were matched to 395 controls; N = 196 neurological patients and N = 186 controls completed follow-up. Overall, 346/382 (91%) patients had at least one abnormal outcome: 56% had limited ADLs, 50% impaired cognition, 47% could not return to work and 62% scored worse than average on ≥1 Neuro-QoL scale (worse anxiety 46%, sleep 38%, fatigue 36%, and depression 25%). In multivariable analysis, patients with neurological complications had worse 6-month mRS (median 4 vs. 3 among controls, adjusted OR 1.98, 95%CI 1.23-3.48, P = 0.02), worse ADLs (aOR 0.38, 95%CI 0.29-0.74, P = 0.01) and were less likely to return to work than controls (41% versus 64%, P = 0.04). Cognitive and Neuro-QOL metrics were similar between groups. CONCLUSIONS: Abnormalities in functional outcomes, ADLs, anxiety, depression and sleep occurred in over 90% of patients 6-months after hospitalization for COVID-19. In multivariable analysis, patients with neurological complications during index hospitalization had significantly worse 6-month functional outcomes than those without.

Seizure mimics in children: An age-based approach.

BACKGROUND: In order to distinguish seizure mimics from seizures in children it is important to clarify the event duration, frequency, semiology, and any precipitating factors. METHODOLOGY: This review provides a succinct and up-to-date overview aimed at general and subspecialty non-neurologist pediatric and adolescent health care providers to help guide a diagnostic approach when a child presents with paroxysmal events of unclear etiology. RESULTS: In many cases, seizure mimics are consistently triggered by an event, location, or emotion, and often resolve with distraction or tactile stimulation. Suspicion should be raised for seizures when events occur out of deep sleep, there is a loss of consciousness with the event, movements are not suppressible, and there is a period of fatigue afterword (minutes to hours). Further, a past medical history of developmental delay (e.g. autism), developmental regression, or neurologic injury increases patients' risk for seizures. CONCLUSIONS: Common seizure mimics are highlighted in the age group in which they are most prevalent.

Potential Role of Febrile Seizures and Other Risk Factors Associated With Sudden Deaths in Children.

Importance: Sudden unexplained death in childhood (SUDC) is the fifth leading category of death among toddlers but remains underrecognized and inadequately studied. Objective: To assess the potential role of febrile seizures (FS) and other risk factors associated with SUDC and describe the epidemiology, mechanisms, and prevention of SUDC. Design, Setting, and Participants: This case series study reviewed 622 consecutive sudden child death cases aged 1 to 17 years from 2001 to 2017 from 18 countries. Data were collected from family members of children who died suddenly; these families voluntarily registered with the SUDC Foundation. Data analysis was conducted from November 2017 to February 2019. Main Outcome Measures: Certified manner of death characterized as accident, natural, or undetermined. Results: A total of 391 families with decedents aged 1 to 6 years completed a comprehensive interview on medical and social histories, and circumstances of death with forensic evaluations revealing a cause of death (sudden explained death in childhood [SEDC]) or no cause of death (SUDC). Of these children, 231 (59.1%) were male, the mean (SD) age at death was 24.9 (12.8) months, and 104 (26.6%) had a history of FS. Compared with the general population FS prevalence (2%-5%), FS prevalence among SUDC (28.8%; 95% CI, 23.3%-34.2%) and SEDC (22.1%; 95% CI, 14.8%-29.3%) were elevated. The odds of death during sleep was 4.6-fold higher in SUDC than in SEDC cases (odds ratio, 4.61; 95% CI, 1.92-11.09; adjusted P = .008). The siblings of SUDC cases were followed up for 3144 life-years, and none died prematurely from SUDC. Conclusions and Relevance: This analysis of the largest SUDC cohort confirmed an increased FS rate and found significantly increased rates of FS among SEDC. This study suggests that seizures may contribute to some SUDC and SEDC deaths. The risk of sudden death in a sibling was low. To develop and assess preventive strategies, population-based studies are needed to define the epidemiology and spectrum of risk factors and identify biomarkers of patients with FS at high risk of sudden death.

Modified Pediatric ASPECTS Correlates with Infarct Volume in Childhood Arterial Ischemic Stroke.

BACKGROUND AND PURPOSE: Larger infarct volume as a percent of supratentorial brain volume (SBV) predicts poor outcome and hemorrhagic transformation in childhood arterial ischemic stroke (AIS). In perinatal AIS, higher scores on a modified pediatric version of the Alberta Stroke Program Early CT Score using acute MRI (modASPECTS) predict later seizure occurrence. The objectives were to establish the relationship of modASPECTS to infarct volume in perinatal and childhood AIS and to establish the interrater reliability of the score. METHODS: We performed a cross sectional study of 31 neonates and 40 children identified from a tertiary care center stroke registry with supratentorial AIS and acute MRI with diffusion weighted imaging (DWI) and T2 axial sequences. Infarct volume was expressed as a percent of SBV using computer-assisted manual segmentation tracings. ModASPECTS was performed on DWI by three independent raters. The modASPECTS were compared among raters and to infarct volume as a percent of SBV. RESULTS: ModASPECTS correlated well with infarct volume. Spearman rank correlation coefficients (ρ) for the perinatal and childhood groups were 0.76, p < 0.001 and 0.69, p < 0.001, respectively. Excluding one perinatal and two childhood subjects with multifocal punctate ischemia without large or medium sized vessel stroke, ρ for the perinatal and childhood groups were 0.87, p < 0.001 and 0.80, p < 0.001, respectively. The intraclass correlation coefficients for the three raters for the neonates and children were 0.93 [95% confidence interval (CI) 0.89-0.97, p < 0.001] and 0.94 (95% CI 0.91-0.97, p < 0.001), respectively. CONCLUSION: The modified pediatric ASPECTS on acute MRI can be used to estimate infarct volume as a percent of SBV with a high degree of validity and interrater reliability.

Decreased relative efficacy of the ketogenic diet for children with surgically approachable epilepsy.

PURPOSE: In children with intractable, surgically approachable epilepsy, the ketogenic diet is often perceived as less efficacious than surgery. METHODS: A retrospective chart review was performed of 554 children started on the ketogenic diet since 1994. Forty-five children were identified as surgical candidates, with at least 2 focal routine EEGs, ictal video-EEG, and corresponding focal neuroimaging findings, of whom 24 eventually had resective surgery and were followed subsequently. A comparison cohort group was also created of 45 children matched for age and seizure frequency that received the diet but were not surgical candidates (multifocal or generalized seizures). RESULTS: Of the 24 children who received both dietary and surgical therapies, there was a higher likelihood after 6 months of both >90% seizure reduction (71% versus 17%) and seizure freedom (63% versus 0%) following surgery, both p < 0.0001. Similarly, the 45 cohort children who were not surgical candidates were more likely to be seizure-free (29% versus 13%, p = 0.041) and remained on the diet for shorter duration (10 months versus 18 months, p = 0.035) compared to the surgical group. CONCLUSION: Children with surgically approachable epilepsy do respond to the diet, but are more likely to be seizure-free following surgery.