Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Benign neonatal sleep myoclonus is a self-limited movement disorder characterized by neonatal-onset myoclonic jerks only during sleep, abrupt and consistent cessation with arousal, and absence of concomitant electrographic changes suggestive of seizures. It has a good outcome and was included in the differential diagnosis of neonatal seizures. A presumed transient serotonin imbalance and genetic factors may play a role in the pathogenesis of this disorder. We report a case of benign neonatal sleep myoclonus mimicking status epilepticus in an infant with a family history of nocturnal myoclonus, tic disorder, and sleep disturbance. We suggest that this benign entity should be included in the differential diagnosis of status epilepticus during the newborn period.
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PMID:Benign neonatal sleep myoclonus mimicking status epilepticus. 1503 88

Differential diagnosis between epileptic and nonepileptic paroxysmal disorders is fundamental not only to allow correct management of patients but also to avoid the burden of unnecessary antiepileptic medication. The focus of this chapter is limited to imitators of idiopathic generalized epilepsies (IGE) which are expressed through myoclonic, tonic-clonic, tonic, atonic, and absence seizures. Apparent losses of consciousness and drop attacks also have to be considered. Benign myoclonus of early infancy is the main nonepileptic disorder in the differential diagnosis of infantile spasms, but is not dealt with here because West syndrome is not an IGE. Hyperekplexia, metabolic disorders, hypnagogic myoclonus, and disturbed responsiveness caused by the use of drugs are listed in Table 1. Other conditions that may imitate more focal epileptic seizures are omitted. Benign neonatal sleep myoclonus, apnea and apparent life-threatening events in infants, cyanotic and pallid breath-holding spells, syncope, staring spells, psychogenic seizures, hyperventilation syndrome, and narcolepsy have been selected based on frequency or difficulties in differential diagnosis with the intention to cover the most conspicuous imitators of IGE in different ages.
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PMID:Nonepileptic disorders imitating generalized idiopathic epilepsies. 1630 79

Benign neonatal sleep myoclonus is a nonepileptic syndrome characterized by myoclonic jerks during sleep. It occurs in the first weeks of life and disappears, in most cases, within 3 months. There are no sequelae, and psychomotor and cognitive development are normal. The syndrome is usually sporadic; only a few familial cases have been reported in the literature. This case report describes three members of a single family with benign neonatal sleep myoclonus and discusses its differentiation from other types of myoclonus.
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PMID:Familial benign neonatal sleep myoclonus. 1750 67

Sleep disorders are classified in dyssomnias, parasomnias, sleep disorder associated with medical and psychiatric disorders and proposed sleep disorders. Only the parasomnias have been studied as such in the newborn period. The parasomnias that occur in this age group are infant sleep apnea, congenital central hypoventilation syndrome, sudden infant death syndrome, and benign neonatal sleep myoclonus. Infant sleep apnea includes three entities: (1) apnea of prematurity, (2), apparent life threatening episodes with apnea and (3) obstructive sleep apnea. Congenital central hypoventilation syndrome can be associated with other autonomic system illness, such as Hirschsprung disease (Haddad syndrome) and neuroblastoma. The implementation of the supine sleep position and smoking free homes has diminished the frequency of sudden infant death syndrome. Benign neonatal sleep myoclonus should be considered in all newborns with a normal exam between the episodes when they always occur during sleep. This entity may be mistaken for status epilepticus, because it is associated with increases in heart rate. Benzodiazepines prolongs the duration of the episodes.
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PMID:[Sleep disorders in the newborn]. 1842 81

Benign neonatal sleep myoclonus is a condition characterized by myoclonus occurring during sleep. Reported here are 18 cases of infants who had electroencephalographic monitoring, with myoclonic events recorded in 17 cases. In all cases, onset was within days to a few weeks of birth. In no case was the diagnosis of sleep myoclonus considered by the referring primary care physician. Myoclonus was evident during sleep in all 18 infants, but in two of them it was observed also during the transition from sleep to awakening. The behavioral features of the disorder were more varied than previously described: four of the infants had lateralized myoclonus and two had myoclonus involving the head and face. In most of the children, the duration of myoclonus was brief, but in two cases the paroxysmal events lasted more than 30 minutes. None of the children had electroencephalographic abnormalities during the myoclonus. The condition was short-lived, with the myoclonus resolving in most children within 3 months of onset. Benign neonatal sleep myoclonus is an under-recognized condition but one that has a consistently favorable outcome. Infants with the condition should not be treated with antiepileptic drugs.
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PMID:Benign neonatal sleep myoclonus: history and semiology. 1938 69

Benign neonatal sleep myoclonus is an uncommon, nonepileptic disorder characterized by myoclonic jerks appearing in the neonatal period that occur predominantly during sleep. Although self-limiting, the disorder is frequently confused with epileptic neonatal seizures. A few familial cases have been reported; however the genetics has not been studied. We ascertained 3 families with 2 or more affected individuals and analyzed the pedigrees. We used microsatellite markers to determine if the disorder was possibly linked to KCNQ2 or KCNQ3, the 2 genes that cause most cases of benign familial neonatal seizures, a disorder that it could be easily confused with. The 3 pedigrees, including one with 4 affected individuals, were suggestive of autosomal dominant inheritance. The loci for KCNQ2 and KCNQ3 were excluded in the 2 larger families. We conclude that benign neonatal sleep myoclonus can show autosomal dominant inheritance and is not allelic with benign familial neonatal seizures.
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PMID:Benign neonatal sleep myoclonus: an autosomal dominant form not allelic to KCNQ2 or KCNQ3. 2244 48

We describe acute movement disorders in 92 children, aged 5 days to 15 years, from an Indian tertiary hospital. Eighty-nine children had hyperkinetic movement disorders, with myoclonus in 25, dystonia in 21, choreoathetosis in 19, tremors in 15, and tics in 2. Tetany and tetanus were seen in 5 and 2 children, respectively. Hypokinetic movement disorders included acute parkinsonism in 3 children. Noninflammatory and inflammatory etiology were present in 60 and 32 children, respectively. Benign neonatal sleep myoclonus in 16 and opsoclonus myoclonus syndrome in 7 accounted for the majority of myoclonus cases. Vitamin B12 deficiency in 13 infants was the most common cause of tremors. Rheumatic fever and encephalitis were the most common causes of acute choreoathetosis. Acute dystonia had metabolic etiology in 6 and encephalitis and drugs in 3 each. Psychogenic movement disorders were seen in 4 cases only, although these patients may be underreported.
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PMID:Acute movement disorders in children: experience from a developing country. 2529 19

18 neonates aged 5-60 days with Benign neonatal sleep myoclonus were identified. Fifteen neonates had been misdiagnosed as neonatal seizures before referral. All treatments were withdrawn once the diagnosis of benign neonatal sleep myoclonus was made. Benign neonatal sleep myoclonus should be considered early in the differential diagnosis of neonatal seizures.
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PMID:Benign neonatal sleep myoclonus: frequently misdiagnosed as neonatal seizures. 2638 39