Use of Remote Monitoring by E-mail for Long-Term Management of the Classic Ketogenic Diet

Cinzia Ferraris; Monica Guglielmetti; Elisa Tamagni; Claudia Trentani; Valentina De Giorgis; Ludovica Pasca; Costanza Varesio; Ottavia Eleonora Ferraro; Anna Tagliabue

doi:10.3390/nu12061833

Use of Remote Monitoring by E-mail for Long-Term Management of the Classic Ketogenic Diet

Nutrients ◽

10.3390/nu12061833 ◽

2020 ◽

Vol 12 (6) ◽

pp. 1833

Author(s):

Cinzia Ferraris ◽

Monica Guglielmetti ◽

Elisa Tamagni ◽

Claudia Trentani ◽

Valentina De Giorgis ◽

...

Keyword(s):

Ketogenic Diet ◽

Remote Monitoring ◽

Glucose Transporter ◽

Deficiency Syndrome ◽

Drug Resistant Epilepsy ◽

Nutritional Monitoring ◽

Age Range ◽

E Mail

The classic ketogenic diet (cKD) requires constant nutritional monitoring over time both to ensure its effectiveness and to reduce the likelihood of short- and long-term adverse effects. We retrospectively described the use of remote monitoring by e-mail during the first year of follow-up on cKD in 34 children (47% males; age range: 2−17 years) diagnosed with drug-resistant epilepsy (DRE; n = 14) or glucose transporter type 1 deficiency syndrome (GLUT1-DS; n = 20). All the e-mails were evaluated analyzing their frequency and content at 3, 6 and 12 months. Three families never sent e-mails. A median of 36.0 (IQR 23.0–64.0) e-mails per family were sent during the 12 follow-up months by the 31 patients. GLUT1-DS patients sent a greater number of e-mails than the DRE group (median 39.0 (IQR 25.5–56.5) vs. median 26.0 (IQR 19.0–65.0)). At the end of the follow-up period, a greater number of e-mails had been exchanged between the nutritional team and the families belonging to the group that increased its linear growth (median 83.5; IQR 48.0–102.0), compared to the other ones. Constant remote monitoring by e-mail could be a feasible and effective way for a better cKD management.

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Impact of the Ketogenic Diet on Linear Growth in Children: A Single-Center Retrospective Analysis of 34 Cases

Nutrients ◽

10.3390/nu11071442 ◽

2019 ◽

Vol 11 (7) ◽

pp. 1442 ◽

Cited By ~ 9

Author(s):

Ferraris ◽

Guglielmetti ◽

Pasca ◽

De Giorgis ◽

Ferraro ◽

...

Keyword(s):

Ketogenic Diet ◽

Growth Retardation ◽

Glucose Transporter ◽

Linear Growth ◽

Deficiency Syndrome ◽

Drug Resistant ◽

Drug Resistant Epilepsy ◽

Age Range ◽

The Impact

Data on the impact of the ketogenic diet (KD) on children’s growth remain controversial. Here, we retrospectively investigated the occurrence of linear growth retardation in 34 children (47% males; age range: 2−17 years) diagnosed with drug-resistant epilepsy (DRE; n = 14) or glucose transporter type 1 deficiency syndrome (GLUT1-DS; n = 20) who had been treated with the KD for 12 months. The general characteristics of children with and without growth retardation were also compared. All participants received a full-calorie, traditional KD supplemented with vitamins, minerals, and citrate. Most children (80%; 11/14 in the DRE subgroup and 16/20 in the GLUT1-DS subgroup) treated with the KD did not show growth retardation at 12 months. Although participants with and without delay of growth did not differ in terms of baseline clinical characteristics, dietary prescriptions, or supplementation patterns, marked ketosis at 12 months tended to occur more frequently in the latter group. Altogether, our results indicate that growth retardation may occur in a minority of children treated with the KD. However, further research is required to identify children at risk and to clarify how increased ketones levels may affect endocrine pathways regulating growth during KD administration.

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Long-term effects of the ketogenic diet on growth in children with resistant drug epilepsy and Glucose Transporter Type 1 Deficiency Syndrome

Nutrition Metabolism and Cardiovascular Diseases ◽

10.1016/j.numecd.2019.05.047 ◽

2019 ◽

Vol 29 (8) ◽

pp. 884

Author(s):

Cinzia Ferraris ◽

Valentina De Giorgis ◽

Ilaria Brambilla ◽

Monica Guglielmetti ◽

Claudia Trentani ◽

...

Keyword(s):

Ketogenic Diet ◽

Glucose Transporter ◽

Deficiency Syndrome ◽

Long Term Effects

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Long-term use of the ketogenic diet in drug resistant epilepsy syndromes during childhood: Differences between responders and non-responders in seizure types, EEG, and outcome

Neuropediatrics ◽

10.1055/s-0029-1215729 ◽

2008 ◽

Vol 39 (05) ◽

Author(s):

A Dressler ◽

E Reithofer ◽

B Stöcklin ◽

F Benninger ◽

M Freilinger ◽

...

Keyword(s):

Ketogenic Diet ◽

Drug Resistant ◽

Drug Resistant Epilepsy

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Primary Calvarial Ewing Sarcoma: A Case Series

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0041-1722900 ◽

2021 ◽

Author(s):

Sandeep Mohindra ◽

Manjul Tripathi ◽

Aman Batish ◽

Ankur Kapoor ◽

Ninad Ramesh Patil ◽

...

Keyword(s):

Ewing Sarcoma ◽

Tumor Resection ◽

Case Series ◽

Radical Excision ◽

Term Outcome ◽

Long Term Outcome ◽

Intradural Tumor ◽

Age Range

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.

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Classic Ketogenic Diet and Modified Atkins Diet in SLC2A1 Positive and Negative Patients with Suspected GLUT1 Deficiency Syndrome: A Single Center Analysis of 18 Cases

Nutrients ◽

10.3390/nu13030840 ◽

2021 ◽

Vol 13 (3) ◽

pp. 840

Author(s):

Jana Ruiz Herrero ◽

Elvira Cañedo Villarroya ◽

Luis González Gutiérrez-Solana ◽

Beatriz García Alcolea ◽

Begoña Gómez Fernández ◽

...

Keyword(s):

Movement Disorder ◽

Glucose Transporter ◽

Deficiency Syndrome ◽

Laboratory Assessment ◽

Modified Atkins Diet ◽

Atkins Diet ◽

Glut1 Deficiency ◽

Glut1 Deficiency Syndrome ◽

The Impact

Background: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is caused by mutations in the SLC2A1 gene and produces seizures, neurodevelopmental impairment, and movement disorders. Ketogenic dietary therapies (KDT) are the gold standard treatment. Similar symptoms may appear in SLC2A1 negative patients. The purpose is to evaluate the effectiveness of KDT in children with GLUT1DS suspected SLC2A1 (+) and (-), side effects (SE), and the impact on patients nutritional status. Methods: An observational descriptive study was conducted to describe 18 children (January 2009–August 2020). SLC2A1 analysis, seizures, movement disorder, anti-epileptic drugs (AEDS), anthropometry, SE, and laboratory assessment were monitored baseline and at 3, 6, 12, and 24 months after the onset of KDT. Results: 6/18 were SLC2A1(+) and 13/18 had seizures. In these groups, the age for debut of symptoms was higher. The mean time from debut to KDT onset was higher in SLC2A1(+). The modified Atkins diet (MAD) was used in 12 (5 SLC2A1(+)). Movement disorder improved (4/5), and a reduction in seizures >50% compared to baseline was achieved in more than half of the epileptic children throughout the follow-up. No differences in effectiveness were found according to the type of KDT. Early SE occurred in 33%. Long-term SE occurred in 10, 5, 7, and 5 children throughout the follow-up. The most frequent SE were constipation, hypercalciuria, and hyperlipidaemia. No differences in growth were found according to the SLC2A1 mutation or type of KDT. Conclusions: CKD and MAD were effective for SLC2A1 positive and negative patients in our cohort. SE were frequent, but mild. Permanent monitoring should be made to identify SE and nutritional deficits.

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Long-term effectiveness and adverse effects of ketogenic diet therapy in infants with drug-resistant epilepsy treated at a single center in Argentina

Epilepsy Research ◽

10.1016/j.eplepsyres.2021.106793 ◽

2021 ◽

pp. 106793

Author(s):

Marisa Armeno ◽

Antonella Verini ◽

Eugenia Caballero ◽

Araceli Cresta ◽

Gabriela Reyes Valenzuela ◽

...

Keyword(s):

Adverse Effects ◽

Ketogenic Diet ◽

Diet Therapy ◽

Drug Resistant ◽

Single Center ◽

Drug Resistant Epilepsy

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Long-Term Effects of a Classic Ketogenic Diet on Ghrelin and Leptin Concentration: A 12-Month Prospective Study in a Cohort of Italian Children and Adults with GLUT1-Deficiency Syndrome and Drug Resistant Epilepsy

Nutrients ◽

10.3390/nu11081716 ◽

2019 ◽

Vol 11 (8) ◽

pp. 1716 ◽

Cited By ~ 2

Author(s):

Ramona De Amicis ◽

Alessandro Leone ◽

Chiara Lessa ◽

Andrea Foppiani ◽

Simone Ravella ◽

...

Keyword(s):

Body Composition ◽

Nutritional Status ◽

Body Fat ◽

Deficiency Syndrome ◽

Drug Resistant ◽

Z Score ◽

Glut1 Deficiency ◽

Glut1 Deficiency Syndrome ◽

Drug Resistant Epilepsy

The classical ketogenic diet (cKD) is an isocaloric, high fat, very low-carbohydrate diet that induces ketosis, strongly influencing leptin and ghrelin regulation. However, not enough is known about the impact of a long-term cKD. This study evaluated the effects of a 12-month cKD on ghrelin and leptin concentrations in children, adolescents and adults affected by the GLUT1-Deficiency Syndrome or drug resistant epilepsy (DRE). We also investigated the relationship between the nutritional status, body composition and ghrelin and leptin variations. We carried out a longitudinal study on 30 patients: Twenty-five children and adolescents (15 females, 8 ± 4 years), and five adults (two females, 34 ± 16 years). After 12-monoths cKD, there were no significant changes in ghrelin and leptin, or in the nutritional status, body fat, glucose and lipid profiles. However, a slight height z-score reduction (from −0.603 ± 1.178 to −0.953 ± 1.354, p ≤ 0.001) and a drop in fasting insulin occurred. We found no correlations between ghrelin changes and nutritional status and body composition, whereas leptin changes correlated positively with variations in the weight z-score and body fat (ρ = 0.4534, p = 0.0341; ρ = 0.5901, p = 0.0135; respectively). These results suggest that a long-term cKD does not change ghrelin and leptin concentrations independently of age and neurological condition.

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Epilepsy surgery in children with drug-resistant epilepsy, a long-term follow-up

Acta Neurologica Scandinavica ◽

10.1111/ane.12154 ◽

2013 ◽

Vol 128 (6) ◽

pp. 414-421 ◽

Cited By ~ 17

Author(s):

T. Hallböök ◽

P. Tideman ◽

I. Rosén ◽

J. Lundgren ◽

E. Tideman

Keyword(s):

Epilepsy Surgery ◽

Drug Resistant ◽

Long Term Follow Up ◽

Drug Resistant Epilepsy

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WP1-21 Maximum safe resection of large medial temporal intrinsic tumours: does the outcome justify the risk?

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-abn.21 ◽

2019 ◽

Vol 90 (3) ◽

pp. e7.1-e7

Author(s):

A Kumaria ◽

A Paterson ◽

M Sitaraman ◽

S Basu

Keyword(s):

Case Series ◽

Csf Leak ◽

Visual Field Defects ◽

Long Term Outcomes ◽

Karnofsky Score ◽

Female Age ◽

Grade 3 ◽

Age Range

ObjectivesTo analyse on the long-term outcomes in patients undergoing maximum safe resection (MSR) for large intrinsic temporal tumours.DesignCase seriesSubjectsAll patients undergoing MSR of large medial temporal intrinsic tumours between May 2006 and February 2012 at a tertiary neurosurgical centre with a minimum follow up of 6 years.MethodsRetrospective review of hospital records.ResultsFifty-one patients underwent MSR (28 male, 23 female); age range 20–80 years (mean age 55.3). There was no difference in laterality, although dysphasia was a feature in 32% of left-sided lesions. Presenting features in general included seizures (46%), headaches (27%), hemiparesis (12%) and visual field defects (6%). Surgery was generally well tolerated (median post-operative Karnofsky score 92.5). No patients developed new dysphasia or weakness, but there was transient worsening of existing hemiparesis (n=4) and dysphasia (n=2). Other complications included CSF leak/pseudomeningocoele (n=2), oculomotor palsy (n=1) and wound infection (n=1). Histopathological casemix was GBM (50%), WHO 3 gliomas (14%), WHO 2 gliomas (10%) and metastases (4%). In total, 57% of patients received radiotherapy and 35% received chemotherapy. Survival correlated with pathology; in glioblastoma patients it ranged from 2–19 months (mean 10.4 months). Survival in grade 3 tumours ranged from 10–38 months (mean 24.4 months). 60% of patients with Grade 2 tumours are surviving symptom free with no histological upscale at 8–10 years follow-up. No patient required a second debulking procedure.ConclusionsMSR did not result in survival benefit in glioblastoma. MSR is justified in lesions with pre-operative radiological features of Grade 2 glioma.

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