ABSTRACT
BACKGROUND: Primary periodic paralysis (PPP) are rare inherited neuromuscular disorders including Hypokalemic periodic paralysis (HypoPP), Hyperkalemic periodic paralysis (HyperPP) and Andersen-Tawil syndrome (ATS) characterised by attacks of weakness or paralysis of skeletal muscles. Limited effective pharmacological treatments are available, and avoidance of lifestyle related triggers seems important. OBJECTIVE: Our aim was to search and assess the scientific literature for information on trigger factors related to nutrition and physical activity in PPP. METHODS: We searched Ovid Medline and Embase database for scientific papers published between January 1, 1990, to January 31, 2020. RESULTS: We did not identify published observation or intervention studies evaluating effect of lifestyle changes on attacks. Current knowledge is based on case-reports, expert opinions, and retrospective case studies with inadequate methods for description of nutrition and physical activity. In HypoPP, high carbohydrate and salt intake, over-eating, alcohol, dehydration, hard physical activity, and rest after exercise are frequently reported triggers. Regarding HyperPP, fasting, intake of potassium, alcohol, cold foods or beverages, physical activity, and rest after exercise are frequently reported triggers. No nutrition related triggers are reported regarding ATS, exercise can however induce ventricular arrhythmias. CONCLUSIONS: Our results support that dietary intake and physical activity may play a role in causing paralytic attacks in PPP, although the current scientific evidence is weak. To provide good evidence-based patient care, several lifestyle aspects need to be further assessed and described.
Subject(s)
Andersen Syndrome/physiopathology , Diet , Exercise , Paralyses, Familial Periodic/physiopathology , Paralysis/etiology , Adolescent , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young AdultSubject(s)
Exercise/physiology , Neuromuscular Diseases , Adult , Contracture/etiology , Creatine Kinase/blood , Exercise Therapy , Fatigue/etiology , Heart Diseases/etiology , Humans , Lung Diseases/etiology , Mitochondrial Myopathies/complications , Mitochondrial Myopathies/therapy , Muscle Weakness/etiology , Muscular Dystrophies/complications , Muscular Dystrophies/therapy , Myopathies, Structural, Congenital/complications , Myopathies, Structural, Congenital/therapy , Myotonic Disorders/complications , Myotonic Disorders/therapy , Neuromuscular Diseases/complications , Neuromuscular Diseases/genetics , Neuromuscular Diseases/therapy , Pain/etiology , Physical Therapy Modalities , Resistance TrainingABSTRACT
BACKGROUND: Prader-Willi syndrome (PWS) is a rare genetic disorder resulting in obesity. The diets for young children with PWS must balance the importance of preventing development of obesity with the need to supply sufficient energy and essential nutrients. OBJECTIVE: To investigate the nutritional intake for children with PWS 2, 3, and 4 years of age and compare it with Nordic Nutritional Recommendations (NNR) and intake of healthy controls. DESIGN: Assessments of food intake for six children 2-4 years of age were performed twice a year. At the age of 2 and 3 years data was obtained by using food recall interviews and at 4 year of age a pre-coded food-diary was used. RESULTS: The energy intake for the 2-year-old children was 3.25 MJ/day (SD 0.85) and for the 3- and 4-year olds 3.62 MJ/day (SD 0.73) and 4.07 MJ/day (SD 0.39 MJ), respectively. These intakes are 61%, 68%, and 77% of the estimated energy requirements in NNR for healthy 2-, 3- and 4-year-old children, respectively, and 60% and 66% of the energy intakes of 2- and 4-year-old children in reference populations. The children's BMI-for-age score and length growth was within the normal range during the study period. The intake of fat was about 25 E% in all age groups and reduced when compared with reference populations. In 25% of the assessments the fat intake was 20 E% or below. The intake of iron was below recommendations in all age groups both with and without supplementation. The mean intake of vitamin D and tocopherol was below recommendations when intakes were determined excluding dietary supplementations. CONCLUSIONS: More large-scale investigations on nutritional intake are needed to further investigate dietary challenges for this patient group.
