Zahava Turner

A case-control evaluation of the ketogenic diet versus ACTH for new-onset infantile spasms

Purpose: ACTH is currently the standard first‐line therapy for new‐onset infantile spasms, but it has significant side effects. We hypothesized the ketogenic diet (KD), previously reported as beneficial for intractable infantile spasms, would have similar efficacy, but better tolerability than ACTH when used first‐line.

Infantile spasms treated with the ketogenic diet: Prospective single-center experience in 104 consecutive infants

Purpose:  In 2002, we reported our preliminary experience using the ketogenic diet (KD) for predominantly intractable infantile spasms (IS) in 23 infants. Since that time, we have increased our use of the KD for this condition including those with new‐onset IS.

A randomized, crossover comparison of daily carbohydrate limits using the modified Atkins diet.

The modified Atkins diet is a dietary therapy for intractable epilepsy that mimics the ketogenic diet, yet does not restrict protein, calories, and fluids. The ideal starting carbohydrate limit is unknown. Twenty children with intractable epilepsy were randomized to either 10 or 20 g of carbohydrates per day for the initial 3 months of the modified Atkins diet, and then crossed over to the opposite amount. A significantly higher likelihood of >50% seizure reduction was noted for children started on 10 g of carbohydrate per day at 3 months: 60% versus 10% (P=0.03). Most parents reported no change in seizure frequency or ketosis between groups, but improved tolerability with 20 g per day. A starting carbohydrate limit of 10 g per day for children starting the modified Atkins diet may be ideal, with a planned increase to a more tolerable 20 g per day after 3 months.

Long-term outcomes of children treated with the ketogenic diet in the past.

Purpose:  The ketogenic diet has well‐established short‐ and long‐term outcomes for children with intractable epilepsy, but only for those actively receiving it. However, no information exists about its long‐term effects years after it has been discontinued.

When do seizures usually improve with the ketogenic diet

Purpose: Parents often expect immediate seizure improvement after starting the ketogenic diet (KD) for their children. The purpose of this study was to determine the typical time to seizure reduction as well as the time after which it was unlikely to be helpful in those children started on the KD.

Do Patients With Absence Epilepsy Respond to Ketogenic Diets

Dietary therapies are established as beneficial for symptomatic generalized epilepsies such as Lennox-Gastaut syndrome; however, the outcome for idiopathic generalized epilepsy has never been specifically reported. The efficacy of the ketogenic and modified Atkins diet for childhood and juvenile absence epilepsy was evaluated from both historical literature review and patients treated at Johns Hopkins Hospital. Upon review of 17 published studies in which absence epilepsy was included as a patient subpopulation, approximately 69% of 133 with clear outcomes patients who received the ketogenic diet had a >50% seizure reduction, and 34% of these patients became seizure free. At Johns Hopkins Hospital, the ketogenic diet (n = 8) and modified Atkins diet (n = 13) led to similar outcomes, with 18 (82%) having a >50% seizure reduction, of which 10 (48%) had a >90% seizure reduction and 4 (19%) were seizure free. Neither age at diet onset, number of anticonvulsants used previously, particular diet used, nor gender correlated with success.

Management and risk factors for dyslipidemia with the ketogenic diet.

A prospective study was performed of all children started on the ketogenic diet at our institution for intractable epilepsy from January 2003 to March 2007 (n = 137), examining for baseline and follow-up total cholesterol and triglyceride levels. Interventions for dyslipidemia were analyzed for their effectiveness. At baseline, 25% of children had hypercholesterolemia (>200 mg/dL), which increased to 60% for those receiving the ketogenic diet. Children receiving a solely formula-based ketogenic diet were less likely to have hypercholesterolemia than those eating solid food after adjusting for age and initial ketogenic ratio (P < .001). Only a slightly higher likelihood of a 20% decrease in cholesterol occurred for those children in whom a dietary intervention was made compared with observation alone (60% vs 41%; P = .11). Hypercholesterolemia occurs in most children receiving a solid food based ketogenic diet but improved in approximately half, even without interventions.

Prospective Study of the Modified Atkins Diet in Combination With a Ketogenic Liquid Supplement During the Initial Month

The modified Atkins diet is a high-fat, low-carbohydrate treatment for intractable childhood epilepsy. As data suggest that a stricter diet onset can be more effective, we added a ketogenic supplement to the modified Atkins diet during its initial month. Thirty children with intractable epilepsy were prospectively started on the modified Atkins diet in combination with a daily 400-calorie KetoCal ® shake. At 1 month, 24 (80%) children had >50% seizure reduction, of which 11 (37%) had >90% seizure reduction. There was no significant loss of efficacy during the second month after KetoCal® was discontinued. The use of this ketogenic supplement increased daily fat intake and thus the ketogenic ratio (1.8:1 versus 1.0:1 in the modified Atkins diet alone, P = .0002), but did not change urinary or serum ketosis. The addition of a ketogenic supplement to the modified Atkins diet during its initial month appears to be beneficial.

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.

Use of dietary therapies amongst patients with GLUT1 deficiency syndrome.

PURPOSE GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. METHODS A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously, and information analyzed in a database. RESULTS Surveys were received from 92 families, of which 90 (98%) had been treated with dietary therapies. Diets used were extremely varied: 59 were treated with the classic ketogenic diet (KD), 29 with the Modified Atkins Diet (MAD), 4 with the Medium-chain Triglyceride (MCT) Diet and 2 with the low glycemic index treatment. The mean diet duration was 5.5 years (range: 1 month-20 years). Of those with seizures, 95% of the children had >50% seizure reduction and 80% had >90% seizure reduction. Children who were seizure-free were currently younger on average (8.2 vs. 11.6 years, p=0.01) and slightly younger at GLUT1DS diagnosis (3.8 vs. 5.3 years, p=0.05). There was an equal percentage of children seizure-free receiving the KD/MCT Diets compared to the MAD/Low Glycemic Index Treatment (74% vs. 63%, p=0.30). The majority (64%) were not receiving anticonvulsants. CONCLUSION This represents the largest series of KD experience in children with GLUT1DS. Nearly all patients surveyed were on dietary therapies for long durations with reported excellent seizure control, often without anticonvulsant drugs. Several different ketogenic diets were utilized with similar efficacy. Early diagnosis and treatment were correlated with success.