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Tatsuya Fujii - One of the best experts on this subject based on the ideXlab platform.

  • outcome of ketogenic Diets in glut1 deficiency syndrome in japan a nationwide survey
    Brain & Development, 2016
    Co-Authors: Tatsuya Fujii, Satoru Takahashi, Keiko Yanagihara, Kuriko Shimono, Jun Natsume, Hirokazu Oguni
    Abstract:

    Abstract Objectives To evaluate the outcome of ketogenic Diets (KDs) in patients with glucose transport type 1 deficiency syndrome (GLUT1DS) in Japan. Methods A nationwide survey for GLUT1DS was conducted by sending questionnaires to board-certified pediatric neurologists nationwide to obtain clinical and laboratory data. Results Among 39 patients whose diagnosis was confirmed molecularly or by the 3- O -methylglucose uptake assay, 31 were treated with KDs for longer than 1 month. Seventeen patients (55%) were on the Modified Atkins Diet, 11 (35%) were on the classic KD, and 3 were on the medium-chain triglyceride (MCT) Diet. The median values and ranges of serum β-hydroxybutyrate levels in patients on the Modified Atkins Diet, classic KD and MCT Diet were 2.5 mM (0.75–4.1), 1.7 mM (0.23–3.5) and 2.6 mM (1.5–3.0), respectively. The KDs were effective on seizures (80%), aggravation after fasting (80%) and ataxia (79%). Thus, ataxia was as responsive as seizures. Two patients on the classic KD with a ketogenic ratio as low as 1:1 showed improvement in neurological symptoms. The development or intelligence quotient measured using the same psychological scales before and after the KDs in 9 patients did not show a significant improvement; the median quotients before and after the Diets were 40 (12–91) and 46 (12–67). Conclusion The KDs were most effective on seizures, transient aggravation after fasting and ataxia. The efficacy on intellectual development was equivocal. The Modified Atkins Diet was more commonly used for GLUT1DS in this study, and its ketogenicity was equivalent to the classic KD.

  • Efficacy and tolerability of Modified Atkins Diet in Japanese children with medication-resistant epilepsy
    Brain & development, 2011
    Co-Authors: Tomohiro Kumada, Tomoko Miyajima, Keiko Saito, Hideki Shimomura, Nozomi Oda, Tatsuya Fujii
    Abstract:

    Ten Japanese patients aged 1.5–17 years with medication-resistant epilepsy were placed on the Modified Atkins Diet (MAD) for 3 weeks during admission to our hospital. Dietary carbohydrate was restricted to 10 g per day. We studied the efficacy of the Diet regarding the seizure frequency and tolerability of the Diet at the end of the 3 weeks on the Diet. Those who decided to continue the MAD at the time of discharge were followed up in the out-patient clinic to observe the effect of the Diet on the seizure frequency. Three of the 10 patients could not continue the Diet during the 3-week admission; one had rotavirus enterocolitis and the other 2 disliked the Diet. Among the remaining 7 patients who could continue the Diet for 3 weeks, 3 achieved the seizure reduction; 2 became seizure-free and 1 showed about 75% reduction in the seizure frequency within 10 days on the Diet. All of these 3 patients continued the Diet after the 3-week admission. The other 4 patients did not show a reduction of the seizure frequency by the end of the 3 weeks on the Diet. Two of them discontinued the Diet on discharge. The remaining 2 still continued the Diet at home and one became seizure-free 3 months after the start of the Diet. In total, 4 of 10 patients achieved >75% reduction in the seizure frequency, although relapse occurred in 2 of the patients, at 5 months and 2 years after seizure reduction, respectively. The MAD was effective and well-tolerated in children with medication-resistant epilepsy in Japan.

  • Modified Atkins Diet and Low Glycemic Index Treatment for Medication-Resistant Epilepsy: Current Trends in Ketogenic Diet
    Journal of Neurology and Neurophysiology, 2011
    Co-Authors: Tomohiro Kumada, Tomoko Miyajima, Ikuko Hiejima, Fumihito Nozaki, Anri Hayashi, Tatsuya Fujii
    Abstract:

    Compared to the classical ketogenic Diet (KD), the Modified Atkins Diet (MAD) and the low glycemic index treatment (LGIT) are more liberal and less restrictive Diet therapies for the treatment of medication-resistant epilepsy. The MAD was first reported by Kossoff et al. in 2003, and gained global popularity with sufficient evidence in its efficacy reported recently in a controlled study. The LGIT was first reported by Pfeifer et al. in 2005, and its use has also become widespread. We reviewed the efficacy of both Diet therapies in the treatment of medication-resistant epilepsy based on the evidence from previous literatures and our own clinical experience. From our experience, the LGIT was more liberal, tolerable, and Dietetically balanced than the MAD. To find out which of these Diets is most appropriate in different epilepsies and different patients, future controlled comparative studies on the efficacy, tolerability, and Dietetic advantages between the MAD and the LGIT are necessary.

  • Modified Atkins Diet for the Treatment of Nonconvulsive Status Epilepticus in Children
    Journal of child neurology, 2009
    Co-Authors: Tomohiro Kumada, Tomoko Miyajima, Nobusuke Kimura, Keiko Saito, Hideki Shimomura, Nozomi Oda, Tatsuya Fujii
    Abstract:

    The authors describe the use of a Modified Atkins Diet for the treatment of 2 children with nonconvulsive status epilepticus. Patient 1 was a 4-year-and-11-month-old girl diagnosed with frontal lobe epilepsy. Since the age of 3 years and 10 months, she had daily nonconvulsive status epilepticus resistant to antiepileptic agents. Patient 2 was a 5-year-and-5-month-old girl with subcortical band heterotopia. She had nonconvulsive status epilepticus daily since the age of 5 years. They were treated with the Modified Atkins Diet, in which carbohydrate intake was restricted to 10 g/d without restriction on protein, caloric, or fluid intake. The nonconvulsive status epilepticus disappeared 5 and 10 days after the initiation of the Diet treatment, respectively. They have been on the Diet treatment and free from nonconvulsive status epilepticus for 19 and 4 months, respectively. The Modified Atkins Diet appears to be very effective for the treatment of nonconvulsive status epilepticus.

  • Brief Communication Modified Atkins Diet for the Treatment of Nonconvulsive Status Epilepticus in Children
    2009
    Co-Authors: Tomohiro Kumada, Tomoko Miyajima, Nobusuke Kimura, Keiko Saito, Hideki Shimomura, Nozomi Oda, Tatsuya Fujii
    Abstract:

    The authors describe the use of a Modified Atkins Diet for the treatment of 2 children with nonconvulsive status epilepticus. Patient 1 was a 4-year-and-11-month-old girl diagnosed with frontal lobe epilepsy. Since the age of 3 years and 10 months, she had daily nonconvulsive status epilepticus resistant to antiepileptic agents. Patient 2 was a 5-year-and-5-month-old girl with sub-cortical band heterotopia. She had nonconvulsive status epilepticus daily since the age of 5 years. They were treated with the Modified Atkins Diet, in which carbohydrate intake was restricted to 10 g/d without restriction on protein, caloric, or fluid intake. The nonconvulsive status epilepticus disappeared 5 and 10 days after the initiation of the Diet treatment, respectively. They have been on the Diet treatment and free from nonconvulsive status epilepticus for 19 and 4 months, respectively. The Modified Atkins Diet appears to be very effective for the treatment of nonconvulsive status epilepticus

Oebele F. Brouwer - One of the best experts on this subject based on the ideXlab platform.

  • The efficacy of the Modified Atkins Diet in North Sea Progressive Myoclonus Epilepsy: an observational prospective open-label study.
    Orphanet journal of rare diseases, 2017
    Co-Authors: Martje E. Van Egmond, Amerins Weijenberg, Margreet Van Rijn, Jan Willem J. Elting, Jeannette M. Gelauff, Rodi Zutt, Deborah A Sival, Roald A. Lambrechts, Marina A.j. Tijssen, Oebele F. Brouwer
    Abstract:

    North Sea Progressive Myoclonus Epilepsy is a rare and severe disorder caused by mutations in the GOSR2 gene. It is clinically characterized by progressive myoclonus, seizures, early-onset ataxia and areflexia. As in other progressive myoclonus epilepsies, the efficacy of antiepileptic drugs is disappointingly limited in North Sea Progressive Myoclonus Epilepsy. The ketogenic Diet and the less restrictive Modified Atkins Diet have been proven to be effective in other drug-resistant epilepsy syndromes, including those with myoclonic seizures. Our aim was to evaluate the efficacy of the Modified Atkins Diet in patients with North Sea Progressive Myoclonus Epilepsy. Four North Sea Progressive Myoclonus Epilepsy patients (aged 7–20 years) participated in an observational, prospective, open-label study on the efficacy of the Modified Atkins Diet. Several clinical parameters were assessed at baseline and again after participants had been on the Diet for 3 months. The primary outcome measure was health-related quality of life, with seizure frequency and blinded rated myoclonus severity as secondary outcome measures. Ketosis was achieved within 2 weeks and all patients completed the 3 months on the Modified Atkins Diet. The Diet was well tolerated by all four patients. Health-related quality of life improved considerably in one patient and showed sustained improvement during long-term follow-up, despite the progressive nature of the disorder. Health-related quality of life remained broadly unchanged in the other three patients and they did not continue the Diet. Seizure frequency remained stable and blinded rating of their myoclonus showed improvement, albeit modest, in all patients. This observational, prospective study shows that some North Sea Progressive Myoclonus Epilepsy patients may benefit from the Modified Atkins Diet with sustained health-related quality of life improvement. Not all our patients continued on the Diet, but nonetheless we show that the Modified Atkins Diet might be considered as a possible treatment in this devastating disorder.

Tiina Muurinen - One of the best experts on this subject based on the ideXlab platform.

  • Modified Atkins Diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathy patients.
    EMBO molecular medicine, 2016
    Co-Authors: Sofia Ahola, Mari Auranen, Vidya Velagapudi, Pirjo Isohanni, Satu Niemisalo, Niina Urho, Jana Buzkova, Nina Lundbom, Antti Hakkarainen, Tiina Muurinen
    Abstract:

    Mitochondrial myopathy (MM) with progressive external ophthalmoplegia (PEO) is a common manifestation of mitochondrial disease in adulthood, for which there is no curative therapy. In mice with MM, ketogenic Diet significantly delayed progression of the disease. We asked in this pilot study what effects high‐fat, low‐carbohydrate “Modified Atkins” Diet (mAD) had for PEO/MM patients and control subjects and followed up the effects by clinical, morphological, transcriptomic, and metabolomic analyses. All of our five patients, irrespective of genotype, showed a subacute response after 1.5–2 weeks of Diet, with progressive muscle pain and leakage of muscle enzymes, leading to premature discontinuation of the Diet. Analysis of muscle ultrastructure revealed selective fiber damage, especially in the ragged‐red‐fibers (RRFs), a MM hallmark. Two years of follow‐up showed improvement of muscle strength, suggesting activation of muscle regeneration. Our results indicate that (i) nutrition can modify mitochondrial disease progression, (ii) Dietary counseling should be part of MM care, (iii) short mAD is a tool to induce targeted RRF lysis, and (iv) mAD, a common weight‐loss method, may induce muscle damage in a population subgroup. ![][1] High‐fat, low‐carbohydrate Modified Atkins Diet (mAD) is a common weight‐loss method, found to ameliorate mitochondrial myopathy in mice. In human patients, mAD induces muscle damage, especially of ragged‐red fibers, the most affected by the disease. [1]: /embed/graphic-1.gif

Muurinen Tiina - One of the best experts on this subject based on the ideXlab platform.

  • Modified Atkins Diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathypatients
    'EMBO', 2016
    Co-Authors: Ahola Sofia, Auranen Mari, Isohanni Pirjo, Niemisalo Satu, Urho Niina, Buzkova Jana, Velagapudi Vidya, Lundbom Nina, Hakkarainen Antti, Muurinen Tiina
    Abstract:

    Mitochondrial myopathy (MM) with progressive external ophthalmoplegia (PEO) is a common manifestation of mitochondrial disease in adulthood, for which there is no curative therapy. In mice with MM, ketogenic Diet significantly delayed progression of the disease. We asked in this pilot study what effects high-fat, low-carbohydrate Modified Atkins Diet (mAD) had for PEO/MM patients and control subjects and followed up the effects by clinical, morphological, transcriptomic, and metabolomic analyses. All of our five patients, irrespective of genotype, showed a subacute response after 1.5-2weeks of Diet, with progressive muscle pain and leakage of muscle enzymes, leading to premature discontinuation of the Diet. Analysis of muscle ultrastructure revealed selective fiber damage, especially in the ragged-red-fibers (RRFs), a MM hallmark. Two years of follow-up showed improvement of muscle strength, suggesting activation of muscle regeneration. Our results indicate that (i) nutrition can modify mitochondrial disease progression, (ii) Dietary counseling should be part of MM care, (iii) short mAD is a tool to induce targeted RRF lysis, and (iv) mAD, a common weight-loss method, may induce muscle damage in a population subgroup.Peer reviewe

Hoon Chul Kang - One of the best experts on this subject based on the ideXlab platform.

  • Ketogenic Diet for Children with Epilepsy: A Practical Meal Plan in a Hospital.
    Clinical nutrition research, 2016
    Co-Authors: Eunjoo Lee, Hoon Chul Kang, Heung Dong Kim
    Abstract:

    A ketogenic Diet (KD) is a Dietary approach to treat intractable epilepsy. The KD begins with hospitalization and the child and their parents can adapt to the KD for 1-2 weeks. Recently, various type of Dietary intervention such as the Modified Atkins Diet (MAD) and the low glycemic index treatment (LGIT) have been performed. Since 2010, we carried out the KD, MAD, and LGIT for total of 802 patients; 489 patients (61%) for the KD, 147 patients (18.3%) with the MAD, and 166 patients (20.7%) for the LGIT. In this report, application of these Dietary practices in Severance Hospital is shared.

  • Lower fat and better quality Diet therapy for children with pharmacoresistant epilepsy
    Korean Pediatric Society, 2013
    Co-Authors: Jung-rim Yoon, Heung Dong Kim, Hoon Chul Kang
    Abstract:

    The ketogenic Diet (KD) is an established, effective, nonpharmacologic treatment for children with pharmacoresistant epilepsy. Although the KD is the most well-established Dietary therapy for epilepsy, it is too restrictive and is associated with serious complications; therefore, alternative lower-fat Diets, including a Modified Atkins Diet and low-glycemic index Diet, have been developed. Recent ongoing clinical evidence suggests that other Dietary therapies have an efficacy almost comparable to that of the KD. In addition, a Diet rich in polyunsaturated fatty acids appears to increase the efficacy of Diet therapy and reduce the complications of a high-fat Diet. Here, we review the systematic information about lower-fat Diets and better-quality Dietary therapies and the current clinical status of each of these Dietary approaches

  • Various indications for a Modified Atkins Diet in intractable childhood epilepsy
    Brain & development, 2011
    Co-Authors: Yoon Mi Kim, Heung Dong Kim, Eunjoo Lee, Varsha Viranchi Vaidya, Timur Khusainov, Shin-hye Kim, Young Mock Lee, Joon Soo Lee, Hoon Chul Kang
    Abstract:

    Abstract Purpose: We reviewed retrospectively our experiences with children with intractable epilepsy who were indicated for a Modified Atkins Diet (MAD). Methods: Twenty children (8 female, 12 male) who were aged 2–17 years with intractable epilepsy and tried the MAD between September 2008 and December 2010 were enrolled. Outcome measures included seizure frequency, adverse reactions and tolerability of the Diet. Results: Finally 9 patients maintained the MAD with favorable seizure outcomes (a reduction of seizure frequency by over 50%) or successfully completed the Diet therapy. Two patients who required a long-term trial of the Diet therapy respectively due to Leigh’s syndrome and uncategorized mitochondrial cytopathy derived from cytochrome c oxidase defect, respectively, successfully maintained the Diet treatment without any significant complications. In 7 patients, the ketogenic Diet (KD) was not only effective but also too restrictive or caused serious unwanted events. Five of them maintained the seizure outcome previously achieved by the KD with the MAD. Ten patients began the MAD because they were reluctant to start the KD. Unfortunately, only 2 patients maintained the MAD with favorable seizure outcomes. One patient who chose the MAD to bridge the KD and complete discontinuation of the treatment successfully completed the Diet therapy. Conclusion: A long-term treatment with the MAD was well tolerated. Moreover, the MAD can successfully substitute the classic KD in patients who showed improvement in seizure outcomes by the KD but could not tolerate it.

  • will seizure control improve by switching from the Modified Atkins Diet to the traditional ketogenic Diet
    Epilepsia, 2010
    Co-Authors: Eric H. Kossoff, Jennifer L. Bosarge, Maria J Miranda, Hoon Chul Kang, Adelheid Wiemerkruel, Heung Dong Kim
    Abstract:

    It has been reported that children can maintain seizure control when the ketogenic Diet (KD) is transitioned to the less-restrictive Modified Atkins Diet (MAD). What is unknown, however, is the likelihood of additional seizure control from a switch from the MAD to the KD. Retrospective information was obtained from 27 patients who made this Dietary change from four different institutions. Ten (37%) patients had ≥10% additional seizure reduction with the KD over the MAD, of which five became seizure-free. The five children who did not improve on the MAD failed to improve when transitioned to the KD. A higher incidence of improvement with the KD occurred for those with myoclonic-astatic epilepsy (70% vs. 12% for all other etiologies, p = 0.004), including all who became seizure-free. These results suggest that the KD probably represents a "higher dose" of Dietary therapy than the MAD, which may particularly benefit those with myoclonic-astatic epilepsy.

  • Will seizure control improve by switching from the Modified Atkins Diet to the traditional ketogenic Diet
    Epilepsia, 2010
    Co-Authors: Jennifer L. Bosarge, Maria J Miranda, Adelheid Wiemer-kruel, Hoon Chul Kang
    Abstract:

    SUMMARY It has been reported that children can maintain seizure control when the ketogenic Diet (KD) is transitioned to the less-restrictive Modified Atkins Diet (MAD). What is unknown, however, is the likelihood of additional seizure control from a switch from the MAD to the KD. Retrospective information was obtained from 27 patients who made this Dietary change from four different institutions. Ten (37%) patients had ‡10% additional seizure reduction with the KD over the MAD, of which five became seizurefree. The five children who did not improve on the MAD failed to improve when transitioned to the KD. A higher incidence of improvement with the KD occurred for those with myoclonic‐astatic epilepsy (70% vs. 12% for all other etiologies, p = 0.004), including all who became seizurefree. These results suggest that the KD probably represents a ‘‘higher dose’’ of Dietary therapy than the MAD, which may particularly benefit those with myoclonic‐