Browse Items · Pediatric Palliative Care Library

The use of Melatonin in children with Neurodevelopmental Disorders and impaired Sleep: a randomised, double-blind, placebo-controlled, parallel study (MENDS)

Background: Difficulties in initiating and maintaining sleep are common in children with neurodevelopmental disorders. Melatonin is unlicensed in children yet widely prescribed for sleep problems. Objective: To determine whether or not…

Melatonin treatment in individuals with intellectual disability and chronic insomnia: a randomized placebo-controlled study

BACKGROUND: While several small-number or open-label studies suggest that melatonin improves sleep in individuals with intellectual disabilities (ID) with chronic sleep disturbance, a larger randomized control trial is necessary to validate these…

Melatonin for sleep problems in children with neurodevelopmental disorders: randomised double masked placebo controlled trial

OBJECTIVE: To assess the effectiveness and safety of melatonin in treating severe sleep problems in children with neurodevelopmental disorders. DESIGN: 12 week double masked randomised placebo controlled phase III trial. SETTING: 19 hospitals across…

Melatonin ineffective in neuronal ceroid lipofuscinosis patients with fragmented or normal motor activity rhythms recorded by wrist actigraphy

Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg).…

Constipation in epidermolysis bullosa: successful treatment with a liquid fiber-containing formula

In epidermolysis bullosa (EB), chronic constipation, painful defecation, and fecal impaction frequently contribute to malnutrition and growth failure. Standard treatments for constipation, such as increased intake of conventional dietary fiber and…

A favorable response to antiparkinsonian treatment in juvenile neuronal ceroid lipofuscinosis

To study the effect of dopaminergic drugs on the parkinsonism in juvenile neuronal ceroid lipofuscinosis, the authors conducted an open study of 21 patients. According to the motor Unified PD Rating Scale (UPDRS) score, treatment was initiated with…

Tags: 2001, Aberg L E, levodopa, NCL3, NCL6, Neurology, pharmacologic intervention, Rajantie I, Rinne J O, Santavuori P, selegiline, tone and motor problems

The effect of L 5 hydroxytryptophan on self mutilation in Lesch Nyhan disease: a negative report

Four patients with Lesch Nyhan disease were treated with L 5 hydroxytryptophan, alone, or in combination with carbidopa, and their effect on self mutilatory behavior was compared to that observed during periods in which placebo was administered.…

Levodopa is not a useful treatment for Lesch-Nyhan disease

Lesch-Nyhan disease (LND) is characterized by dystonia, cognitive abnormalities, and self-injurious behavior. No effective therapies are available. LND is associated with a presynaptic dopaminergic deficit, but the reported effects of dopamine…

Tags: 2011, behavior problems, Bloem B R, carbidopa, Jinnah H A, Lesch-Nyhan syndrome, levodopa, levodopa-dopa, Movement Disorders, pharmacologic intervention, Schretlen D J, tone and motor problems, Visser J E

Successful treatment of cataplexy in patients with early-infantile Niemann-Pick disease type C: use of tricyclic antidepressants

Cataplexy is a brief episode of bilateral loss of muscle tone with intact consciousness, triggered by a variety of strong emotions such as anger, laugh, humor or surprise and it is considered to represent the physiologic atonia of rapid eye movement…

Tags: 2014, Cak H T, cataplexy, Duzgun G, European Journal of Paediatric Neurology, Haliloglu G, Imipramine, NPC, pharmacologic intervention, tone and motor problems, Topcu M, Yuce A

Melatonin appears ineffective in children with intellectual deficits and fragmented sleep: six "N of 1" trials

Prolonged-release melatonin for children with neurodevelopmental disorders

Previous studies demonstrated the efficacy and safety of prolonged-release melatonin in children and adolescents with neurodevelopmental and behavioral disorders. The long-term effectiveness and safety of prolonged-release melatonin treatment were…

Tags: 2011, De Leersnyder H, Laudon M, melatonin, Pediatric Neurology, pharmacologic intervention, PR melatonin, prolonged-release melatonin, Rett syndrome, sleep disturbance/disorders, Zisapel N

Melatonin for treatment of sleep disorders in children with developmental disabilities

This study explored the safety and efficacy of synthetic melatonin in the treatment of sleep problems in 20 children with developmental disabilities, in a randomized, double-blind, placebo-controlled 6-week trial of melatonin versus placebo. All but…

Tags: 2001, Developmental Disabilities, Dodge N N, Journal of Child Neurology, melatonin, Neurosciences & Neurology, Pediatrics, pharmacologic intervention, sleep disturbance/disorders, unspecified developmental disabilities, Wilson G A

Effect of melatonin dosage on sleep disorder in tuberous sclerosis complex

We report a randomized, double-blind, controlled, crossover trial investigating the response to oral melatonin using two dose regimens in patients with sleep disorders associated with tuberous sclerosis complex. Eight outpatients with tuberous…

Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group

Sixty patients with disabling myoclonus excluding mainly spinal myoclonus were treated by piracetam as an open-labeled study, and myoclonus score, neurological symptoms, functional disability, and intensity of myoclonus were scored before and after…

Tags: 1996, Epilepsy, Ikeda A, Kimura J, Mizuno Y, Movement Disorders, Myoclonus, pharmacologic intervention, piracetam, Seizures, Shibasaki H, Tashiro K, tone and motor problems

Clinical overview of children with mucopolysaccharidosis type III A and effect of Risperidone treatment on children and their mothers psychological status

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disorder characterized by progressive mental deterioration and severe behavioral problems. We conducted an open-label, crossover study of the efficacy and safety of Risperidone on…

Tags: 2010, behavior problems, Brain and Development, Coker M, Demiral N, Erermis S, hyperactivity, Kalkan U S, Ozbaran B, pharmacologic intervention, Risperidone, sleep disturbance, Yuncu Z

Effect of baclofen on emesis and 24-hour esophageal pH in neurologically impaired children with gastroesophageal reflux disease

OBJECTIVES: Gastroesophageal reflux disease (GERD) is difficult to control with medical therapy in neurologically impaired children. The gamma-aminobutyric acid type B receptor agonist baclofen was recently reported to reduce reflux in adult patients…

Tags: 2004, baclofen, De Lange syndrome, emesis, feeding difficulties, Hirayama S, Ida S, Journal Of Pediatric Gastroenterology And Nutrition, Kawahara H, Kawai M, pharmacologic intervention, West syndrome, Yoshimura N

A double-blind, placebo-controlled, crossover trial of the selective dopamine D1 receptor antagonist ecopipam in patients with Lesch-Nyhan disease

Lesch-Nyhan disease (LND) is a genetic disorder that has characteristic metabolic, neurologic, and behavioral features. There are multiple behavioral problems including impulsivity, aggressiveness, and severe recurrent self-injurious behavior (SIB).…

Tags: 2016, behavioral problems, Chipkin R, ecopipam, Jinnah H A, Khasnavis T, Lesch-Nyhan syndrome, Molecular Genetics and Metabolism, pharmacologic intervention, Puig J G, self-injury, Sommerfeld B, Torres R J

Effective treatment of spasticity using dronabinol in pediatric palliative care

BACKGROUND: Cannabis extracts have a wide therapeutic potential but in many countries they have not been approved for treatment in children so far. OBJECTIVE: We conducted an open, uncontrolled, retrospective study on the administration of dronabinol…

Tags: 2016, Balzer S, Borkhardt A, chromosome 18q deletion, dronabinol, European Journal of Paediatric Neurology, Gagnon G, Hoell J I, Janßen G, Kuhlen M, Lissencephaly Type 1, Metachromatic Leukodystrophy, NCL3, Oommen P T, pharmacologic intervention, Spasticity, tone and motor problems

Gabapentin can significantly improve dystonia severity and quality of life in children

INTRODUCTION: Gabapentin has been used in the management of neuropathic pain, epilepsy and occasionally movement disorders. METHODS: A four-year retrospective, observational study analysed the use of gabapentin for severe dystonia in children at the…

Tags: 2016, European Journal of Paediatric Neurology, Gabapentin, Gimeno H, IND, involuntary muscle contractions, Kaminska M, Lin J S, Liow N Y, Lumsden D E, Marianczak J, pharmacologic intervention, Tomlin S, tone and motor problems

Sleep dysfunction in Rett syndrome: a trial of exogenous melatonin treatment

Nine girls with Rett syndrome (mean age, 10.1 years) were monitored 24 hours a day over a period of 10 weeks using wrist actigraphy. Baseline sleep-wake patterns were assessed for 1 week. Subsequently, patients underwent a 4-week melatonin treatment…

Tags: 1998, Budden S S, Developmental Medicine and Child Neurology, McArthur A J, melatonin, pharmacologic intervention, Rett syndrome, sleep disturbance/disorders

Serum melatonin kinetics and long-term melatonin treatment for sleep disorders in Rett syndrome

We studied the circadian rhythm of serum melatonin levels in two patients with classical Rett syndrome having severe sleep disorders; serum melatonin levels were measured before and during melatonin treatment using radioimmunoassay. Patient 1 had a…

Tags: 1999, Brain and Development, melatonin, Miyamoto A, Oki J, Okuno A, pharmacologic intervention, Rett syndrome, sleep disturbance/disorders, Takahashi S

Prophylactic effect of L 5 hydroxytryptophan on self mutilation in the Lesch Nyhan syndrome

Four cases of the Lesch Nyhan syndrome found in the Tokyo area were given L 5 hydroxytryptophan orally. L 5 hydroxy tryptophan relieved self mutilation effectively without causing any side effects. However this substance did not alleviate…

Tags: 1975, behavioral problems, choreoathetosis, L-5-Hydroxytryptophan, L-Dopa, L-tryptophan, Lesch-Nyhan syndrome, Mizuno T, Neuropadiatrie, pharmacologic intervention, self-injury, tone and motor problems, Yugari Y

Feed-induced Dystonias in Children with Severe Central Nervous System Disorders

Dystonias can arise from any painful stimuli in neurologically disabled children. Classically, feed-induced dystonias from mediastinal pain due to severe gastroesophageal reflux disease are described as Sandifer spasm. We report a case series of 12…

Tags: 1134-47-0 (baclofen), 2017, acquired brain injury, Adolescent, Article, Baclofen/dt [drug Therapy], baclofen/tl [Intrathecal Drug Administration], Campbell D I, Case Study, central nervous system disease, Cerebral Palsy, Child, Clinical Article, Constipation, Dystonia, dystonia/dt [Drug Therapy], feed induced dystonia, feed induced dystonia/dt [Drug Therapy], feeding difficulties, Female, Gastroesophageal Reflux, Gastroscopy, GERD, Human, Hydrocephalus, Hyperglycinemia, Infant, infantile spasm, intestinal dysmotility, intestine motility, Intestine Obstruction, Journal Of Pediatric Gastroenterology And Nutrition, Male, microcephaly, Mordekar S R, motor dysfunction, MPSIII, onset age, Parenteral Nutrition, Percutaneous Endoscopic Gastrostomy, peristalsis, pharmacologic intervention, Practice Guideline, Preschool Child, Priority Journal, reflux esophagitis, Retrospective Study, Rett syndrome, Sanfilippo syndrome, School Child, tone and motor problems, total parenteral nutrition, Velayudhan M, West syndrome

Supplementation with carnitine reduces the severity of constipation: A retrospective study of patients with severe motor and intellectual disabilities

Carnitine is an essential nutrient for the mitochondrial transport of fatty acids. Carnitine deficiency causes a variety of symptoms in multiple organs. Patients with severe motor and intellectual disabilities often have carnitine deficiency. This…

Tags: 102767-28-2 (levetiracetam), 1069-66-5 (valproic acid), 1309-48-4 (magnesium oxide), 1317-74-4 (magnesium oxide), 14093-02-8 (iron), 14378-32-6 (zinc), 146-22-5 (nitrazepam), 1622-61-3 (clonazepam), 2017, 22316-47-8 (clobazam), 298-46-4 (carbamazepine), 461-06-3 (carnitine), 50-06-6 (phenobarbital), 53858-86-9 (iron), 541-15-1 (carnitine), 56-99-5 (carnitine), 57-30-7 (phenobarbital), 57-88-5 (cholesterol), 59-30-3 (folic acid), 6484-89-5 (folic acid), 68291-97-4 (zonisamide), 7439-89-6 (iron), 7440-66-6 (zinc), 7782-49-2 (selenium), 8028-68-0 (phenobarbital), 8047-84-5 (carbamazepine), 84057-84-1 (lamotrigine), 99-66-1 (valproic acid), acute brain disease, Adult, albumin, albumin blood level, anticonvulsant therapy, Aomatsu T, Article, bacterial meningitis, bed rest, body mass, brain infarction, carbamazepine/dt [Drug Therapy], carnitine, carnitine deficiency, carnitine deficiency/dt [Drug Therapy], carnitine/dt [Drug Therapy], Cerebral Palsy, Child, cholesterol blood level, cholesterol/ec [Endogenous Compound], Clinical Article, Clinical Trial, clobazam/dt [Drug Therapy], clonazepam/dt [Drug Therapy], Constipation, constipation/dt [Drug Therapy], constipation/pc [Prevention], Controlled Study, copper blood level, cupric ion/ec [Endogenous Compound], diet supplementation, Disease Severity, Endogenous Compound, enema/dt [Drug Therapy], enteric feeding, epilepsy/dt [Drug Therapy], Female, folic acid, folic acid blood level, folic acid/ec [Endogenous Compound], Gene Expression, groups by age, Hospital, Human, Human Tissue, infantile spasm, Inoue K, Intellectual Impairment, iron blood level, iron/ec [Endogenous Compound], Journal of Clinical Biochemistry and Nutrition, lamotrigine/dt [Drug Therapy], levetiracetam/dt [Drug Therapy], magnesium oxide/dt [Drug Therapy], Male, Medical Record Review, Middle Aged, motor dysfunction, Murata S, NCL3, neuronal ceroid lipofuscinosis, nitrazepam/dt [Drug Therapy], Pelizaeus Merzbacher disease, Pelizaeus- Merzbacher disease, periventricular leukomalacia, pharmacologic intervention, phenobarbital/dt [Drug Therapy], prealbumin, prealbumin/ec [Endogenous Compound], Preschool Child, Retrospective Study, School Child, selenium blood level, selenium/ec [Endogenous Compound], sex difference, Tamai H, trace element, Tuberous Sclerosis, valproic acid, valproic acid/dt [Drug Therapy], West syndrome, Yoden A, Young Adult, zinc/ec [Endogenous Compound], zonisamide/dt [Drug Therapy]

Naltrexone therapy of apnea in children with elevated cerebrospinal fluid beta-endorphin

Previous studies have indicated increased immunoreactivity of the endogenous opioid peptide beta-endorphin in the cerebrospinal fluid (CSF) of infants under 2 years of age with apnea. To assess the role of endogenous opioids in the pathogenesis of…

Botulinum Toxin Type A for the Treatment of Equinus Deformity in Patients With Mucopolysaccharidosis Type II

Mucopolysaccharidoses are lysosomal storage disorders that are caused by a deficiency in the enzymes that degrade glycosaminoglycans. The accumulation of glycosaminoglycans affects multiple systems, resulting in coarse facial features, short stature,…

Movement disorder in ataxia-telangiectasia: treatment with amantadine sulfate

Ataxia-telangiectasia is a cerebellar neurodegenerative disorder presenting with ataxia, chorea, myoclonus, and bradykinesia. Literature on treatment of movement disorders is scarce. We treated 17 children (aged 11.2 +/- 3.9 years) for 8 weeks with…

Serotonergic approaches to the modification of behavior in the Lesch-Nyhan syndrome

In a series of patients with the Lesch-Nyhan syndrome a pharmacologic attempt was made to alter the balance of biogenic amines in the central nervous system by increasing the level of serotin. Patients were treated with 5-hydroxytryptophan in…

Tags: 1980, 5-hydroxy-tryptophan, Applied Research in Mental Retardation, behavioral problems, Johnson H G, Jones K L, Kaufman I A, Lesch-Nyhan syndrome, Nyhan W L, pharmacologic intervention, Self-Injurious behavior

Use of melatonin to treat sleep disorders in tuberous sclerosis

The results of a therapeutic trial of the use of melatonin in patients with tuberous sclerosis complex who also have severe sleep problems are reported. We used a randomized double-blind placebo-controlled crossover design. Seven patients with…

Tags: 1999, Clarke A, Developmental Medicine and Child Neurology, Hancock E, Hunt A B A, melatonin, O'Callaghan F J K, Osborne J P, pharmacologic intervention, sleep disturbance/disorders, Tuberous Sclerosis

Systematic review of melatonin treatment in children with neurodevelopmental disabilities and sleep impairment

Sleep disturbances in children with neurodevelopmental disabilities are common and frequently difficult to treat with conventional pharmacological and behavioural methods. Melatonin is a pineal hormone known to be important in the regulation of the…

Tags: 2004, Antioxidants/therapeutic use, Appleton R E, Brain/physiopathology, Child, Developmental Disabilities/drug therapy/epidemiology/physiopathology, Developmental Medicine and Child Neurology, Humans, melatonin, Melatonin/therapeutic use, pharmacologic intervention, Phillips L, Rett syndrome, sleep disturbance/disorders, Sleep Wake Disorders/epidemiology, Wakefulness/physiology

Browse Items (33 total)