Nervous System Disorders Epilepsy – an intermittent derangement of the nervous system due to sudden, excessive, disorderly discharge of the cerebral neurons

I. Characteristics *convulsive movements, disturbance of sensation, loss of consciousness, or a combination of these effects *highest incidence rates in ≤20 years of age *seizures before the age of 2 years are usually caused by development defects, birth injuries, or a metabolic disease

II. Common Episodes: akinetic, myoclonic, petit mal, psychomotor

III. Treatment A. Anticonvulsant drug therapy with phenobarbital, phenytoin, and premidone B. Ketogenic diet

IV. Nutritional Care

Aim: to produce and maintain a ketonic state in a child by gradually reversing the usual proportions of dietary fat and carbohydrate while meeting the child’s kilocalorie and protein needs Diet A. Traditional

1. Child fasts in the hospital for 24-72 hours until a 4+ ketonuria is produced 2. Kilocalories from fat to kilocalories from protein and carbohydrate are increased from 1:1 to 3:1

(ketogenic to antiketogenic) ratio in 4 days B. MCT based

1. Approximately 50-70% of total kilocalories from MCT oil 2. Maximum of 19% of total kilocalories from carbohydrates 3. Maximum of 29% of total kilocalories from protein and carbohydrates combined 4. Minimum of 11% of total kilocalories from fats exclusive of MCT 5. Vitamin and mineral supplementation as needed due to nutritional effects of anticonvulsant therapy

a. Calcium b. Vitamin D (10-40 µg for children, 5 µg for adults) c. Folic acid

Cerebral Palsy (CP) – variety of neurologic dysfunctions secondary to brain damage as a result of birth injury, cerebral haemorrhage, or prematurity

I. Type of motor disability A. Athetoid CP – characterized by constant, uncontrollable movements B. Spastic CP or ataxia – characterized by limited activity

II. Problem Areas

1. Abnormal oral-motor reflexes: hyperactive gag reflex, inability to coordinate sucking, swallowing and breathing, tonic bite reflex, tongue thrust, lip/tongue retraction, nasal regurgitation, jaw thrusting, delay in developmental feeding skills

2. Dental problems: caries, bruxism, malocclusion 3. Poor weight gain (noted in athetoid CP) 4. Excessive weight gain (noted in spastic CP)

III. Nutritional Care Objective: to assure adequate nutritional intake to promote maximum growth potential and feeding skills A. Feeding evaluation by qualified professional (i.e. speech pathologist, P.T., O.T., etc.) and development of

appropriate feeding program with possible use of adaptive equipment B. Kilocalorie recommendations based on height:age

1. CP with severely restricted activity: 10 kcal/cm of height:age 2. CP with mild to moderate activity: 15 kcal/cm of height:age 3. CP with excessive weight gain: 11.1–13.9 kcal/cm

C. Protein requirements same as normal children of the same height/age D. Fluid intake should be given special attention to avoid dehydration and constipation E. Thickened fluids, such as milkshakes, sherbet, gelatin, and soups may be helpful to ensure adequate fluid

intake Parkinson’s Disease (PD) – a degenerative central nervous system condition characterized by progressive loss of cells within the substantia nigra. These cells release the neurotransmitter dopamine. It is the loss of dopamine that is primarily responsible for the motor deficits.

I. Characteristics: slow and decreased movement, muscular rigidity and tremors, abnormal gait and balance, slurred speech, poor mastication, dysphagia (difficulty in swallowing), and slowed gastric motility “Classic triad” of signs: tremor, rigidity, and bradykinesia

II. Etiology 1. Altered dopamine metabolism from neural injury 2. Exposure to environmental neurotoxins 3. Predisposition

III. Medical treatment

-PD responds to therapy with the drug levodopa or its combination with carbidopa -Levodopa (L-dopa) is made of amino acids. Since dopamine cannot readily cross the blood-brain barrier, L-dopa (dopamine precursor) is administered. It is converted to dopamine by dopa decarboxylase and crosses the blood-brain barrier

IV. Nutritional Side Effects of Treatments 1. Anorexia 2. Nausea 3. Reduced sense of smell 4. Constipation 5. Dry mouth 6. High protein intake interferes with drug absorption 7. Weight loss due to increased energy expenditure related to dyskinesia 8. Postural hypotension

V. Nutritional Care

*Focus on weight maintenance, regulation of bowel function, dysphagia, difficulty in self-feeding, and the nutrient-drug interactions

1. Maintain desirable weight 2. Prevent lessening of therapeutic effect of drug therapy 3. Reduce swallowing difficulties of dry mouth 4. Regulate adequate bowel function 5. Maintain optimal hydration

Diet 1. Kilocalories adequate to maintain weight 2. Protein redistribution: low protein intake for breakfast and lunch; evening meal to provide normal

allowance (1.1g/kg BW for adults) 3. Tyrosine (a precursor of dopamine) may be beneficial 4. Pyridoxine is limited to less than 5 mg/day to make levodopa more effective 5. Sodium should be adequate to meet needs 6. Fluid and fiber increased to improve bowel function 7. Supplemental vitamins and minerals, if diet is inadequate 8. Serve small, frequent meals and use semisolid foods rather than fluids if swallowing is a problem