PURPOSE:To determine the long-term outcome of children with difficult-to-control seizures who remained on the ketogenic diet for <1 year. METHODS:Between 1994 and 1996, 150 children with epilepsy, refractory to at least two medications, initiated the ketogenic diet according to the Hopkins protocol. Three to six years after diet initiation, all the families were contacted by telephone or questionnaire to assess their child's current seizure status, medications, and therapies. RESULTS:Sixty-seven children discontinued the diet within 1 year of initiation. Follow-up data were available for 54 of these children. Ten subsequently had surgery, and three underwent VNS implantation. These operated-on children were significantly more likely to be >50% controlled at follow-up than were those managed with medications alone (p < 0.05). A statistically significant difference in long-term outcome was noted between those who responded while on the diet, even if they discontinued it before 1 year, and those who did not (p < 0.05), but no statistical correlation was found between length of time that they had remained on the diet and long-term prognosis. CONCLUSIONS:Almost half of the children who discontinued the diet during the first year had a decrease in seizures when assessed 3-6 years later. Twenty-two percent of these had become seizure free without surgery. We were unable to ascertain whether this may have been due to new medications. Those who saw some improvement while on the diet were more likely to have a favorable long-term outcome. Resective surgery, in children who were candidates, or vagal nerve stimulation (VNS) implantation, was more likely to result in significant seizure improvement than was management with medications alone. Whether or not the diet was effective, most families did not regret trying it and would recommend it to others.

Although the success rates and complications of various treatment options for children with intractable epilepsy have been described, the actual expectations of parents for these treatments are less clear. Since 1998, parents at our institution have written their goals in a letter before starting their children on the ketogenic diet. One hundred consecutive letters were evaluated. The most common first goal was seizure improvement, second was anticonvulsant reduction, and third was cognitive improvement. Ninety percent requested improvement in cognition or alertness. These expectations were either met or exceeded at 6 months in 52–60% of children. Achieving or surpassing parental expectations for cognitive improvement correlated with longer diet duration (P=0.04), but meeting goals for seizure or anticonvulsant reduction did not. Cognitive improvement (P<0.001) and >90% seizure reduction (P=0.04) at 6 months positively correlated with longer eventual diet duration. Expectations for cognitive improvement need to be discussed prior to beginning the ketogenic diet.

Purpose: Although the ketogenic diet has been in use for > 80 years, little agreement exists as to which patients are most likely to have dramatic, sudden, and complete seizure control.Methods: A case-control study was performed of children with intractable epilepsy started on the ketogenic diet at our institution since June 2001. Patients with a dramatic response were defined as those becoming seizure free within 2 weeks of diet onset. These children were compared with all other patients treated with the diet over the same time period in terms of patient demographics, epilepsy characteristics, and diet parameters.Results: Eighteen early, dramatic responders over a 3-year period were identified and compared with 89 patients who were not similarly improved. The absence of complex partial seizures as the predominant seizure type (0 vs. 23%; p = 0.02) correlated with this dramatic success. The presence of infantile spasms (39% vs. 20%; p = 0.09) approached significance, but all other variables did not.Conclusions: An early, dramatic response to the ketogenic diet is more likely in patients with predominant seizure types other than complex partial. It may also be more likely to occur in children who have infantile spasms. In all other patient demographics and diet parameters, an equal likelihood of similar success was found.

The ketogenic diet has traditionally been considered an anticonvulsant therapy of last resort, despite excellent efficacy and limited side effects. We hypothesized that the ketogenic diet would have similar results in patients with new-onset epilepsy. A retrospective study was conducted of patients started on the ketogenic diet since 1994. Thirteen of 460 (2.8%) patients were started on the ketogenic diet as early (zero or one prior anticonvulsant) therapy for seizures. Of those remaining on the diet, 60% (6 of 10) had a > 90% seizure reduction at 6 months and 100% (6 of 6) had a > 90% reduction at 12 months. Patients with infantile spasms were as likely to achieve > 50% seizure reduction at 6 months as patients with other seizure types (75% vs 60%; P = .6). The ketogenic diet can be a valuable therapy before epilepsy becomes intractable. In the 13 patients reported, efficacy without side effects was achieved similarly to that with patients with intractable epilepsy.

Purpose: The Atkins diet may induce ketosis as does the ketogenic diet, without restrictions on calories, fluids, protein, or need for an inpatient fast and admission. Our objective was to evaluate the efficacy and tolerability of a modified Atkins diet for intractable childhood epilepsy.Methods: Twenty children were treated prospectively in a hospital-based ambulatory clinic from September 2003 to May 2005. Children aged 3 - 18 years, with at least three seizures per week, who had been treated with at least two anticonvulsants, were enrolled and received the diet over a 6-month period. Carbohydrates were initially limited to 10 g/day, and fats were encouraged. Parents measured urinary ketones semiweekly and recorded seizures daily. All children received vitamin and calcium supplementation.Results: In all children, at least moderate urinary ketosis developed within 4 days ( mean, 1.9). Sixteen ( 80%) completed the 6-month study; 14 chose to remain on the diet afterward. At 6 months, 13 ( 65%) had > 50% improvement, and seven ( 35%) had > 90% improvement ( four were seizure free). Mean seizure frequency after 6 months was 40 per week ( p = 0.005). Over a 6-month period, mean serum blood urea nitrogen increased from 12 to 17 mg/dl ( p = 0.01); creatinine was unchanged. Cholesterol increased from 192 to 221 mg/dl, ( p = 0.06). Weight did not change significantly ( 34.0-33.7 kg); only six children lost weight. A stable body mass index over time correlated with > 90% improvement ( p = 0.004).Conclusions: A modified Atkins diet is an effective and well-tolerated therapy for intractable pediatric epilepsy.

The ideal timing of anticonvulsant reduction for a child started on the ketogenic diet is unclear. The records of 81 children started on the ketogenic diet consecutively over a 2-year period were reviewed for a 6-month period after diet initiation. During their first 6 months on the ketogenic diet, medications were tapered in 53 of 81 (65%) patients, with 30 of 53 (57%) considered “early” (tapered during diet initiation or within the first month afterward). No differences were seen between the early and late groups with respect to percentage with >90% seizure reduction at 3 months (47% vs 48%), diet duration (1.1 vs 0.9 years), percentage remaining on the ketogenic diet to date (73% vs 65%), or improved alertness (63% vs 57%). Nine of fifty-three (17%) had transient seizure increases during medication tapering, with no correlation to the timing in which this occurred; however, five were taking benzodiazepines or barbiturates. All had >50% seizure reduction at 3 months despite the transient worsening. Early reduction of anticonvulsants in children initiated on the ketogenic diet appears to be safe and well tolerated. However, it offers no specific advantage compared with a later taper.

Seven primitive reflexes used by physical and occupational therapists in evaluating children with cerebral palsy were each graded on a 0 to 4+ scale to constitute a Primitive Reflex Profile. The reflexes studied were the asymmetrical tonic neck reflex, the symmetrical tonic neck reflex, the tonic labyrinthine reflex, the positive support reflex, the derotational righting reflex, the Moro reflex, and the Galant reflex. The Primitive Reflex Profile was studied in 53 cerebral palsied patients to assess both the feasibility of its administration and its usefulness in discriminating functional levels of ambulation. The Primitive Reflex Profile was administered by at least two members of a team consisting of four pediatric developmentalists and two physical therapists. In using this instrument, the extreme functional groups were clearly defined and showed the expected overlap with the intermediate classification.

Exploration of the history, terminology, and classification of cerebral palsy reveals it as a complex entity. Criteria exist that may make early diagnosis and appropriate intervention possible. Dealing with patients who have cerebral palsy requires recognition of the associated problems and deficits. Comprehensive management usually requires a multidisciplinary setting, which is used to outline the contributions of many therapeutic modalities--developmental pediatrics, occupational and physical therapy, hearing and speech, psychology, social services, special education, ophthalamology, neurology, orthopedics and neurosurgery. The poor prognosis for full function within the community is recognized, as are areas of research that require investigation.