Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clonazepam or 5-(2-chlorphenyl)-1, 3-dihydro-7-nitro-2H-1,4benzodiazepin-2-one, is a close structural and pharmacological relative of nitrazepam. It has a broad spectrum of activity against the various types of epilepsy, and is effective in many patients whose condition has proved resistant to other antiepileptic drugs. Its chief uses are in status epilepticus, in which intravenous clonazepam may replace diazepam as the drug of first choice, and in the minor motor seizures of childhood, particularly petit mal absences, the Lennox-Gastaut syndrome and infantile spasms. Clonazepam is also at least as effective as current treatment in psychomotor and myoclonic epilepsies, but seems unlikely to replace phenytoin and the barbiturates in the treatment of grand mal or focal motor seizures except in patients resistant to standard therapy. Initial success with clonazepam can be followed by loss of effect, but benefit can often be restored, at least initially, by temporary interruption and re-institution of treatment. Side-effects are common with clonazepam. Most patients experience drowsiness and fatigue, which are frequent causes of withdrawal, together with lesser incidences of ataxia, dystonia, hypotonia, and hyperactivity. These effects usually disappear with continued therapy, and are minimised by gradual introduction of the drug over 2-4 weeks. Hypersalivation and excessive bronchial secretion may be a problem in children and infants.
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PMID:Clonazepam: a review of its pharmacological properties and therapeutic efficacy in epilepsy. 97 34

The case is described of a neonate who suffered from withdrawal symptoms within 24 hours after birth as a result of the use of clomipramine by the mother during pregnancy. The symptoms consisted of increased irritability, alternating hyper- en hypotonia, hyperreflexia, cyanosis and hypothermia. He was treated with clonazepam with good result. Prescription of clomipramine during pregnancy should be restricted to specific cases and the doses should be kept as low as possible. Because the symptoms are withdrawal symptoms, phenobarbital should not be used as treatment, as it increases drug metabolism by the liver causing an even faster decrease of plasma concentrations of clomipramine. Clonazepam is the drug of choice.
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PMID:[Neonatal withdrawal symptoms following the use of clomipramine during pregnancy]. 143 66

Menkes' kinky hair disease (trichopoliodystrophy) is a rare inherited X-linked recessive disease with an incidence of about 1:35,000, and is rare reported previously in Taiwan. We present 2 cases with typical features including sparse, coarse and stubby, kinky hair, depigmented skin, pudgy face, arrow-shaped upper lip, hypotonia, Babinski signs bilaterally, profound psychomotor retardation with disability of head control or rolling over, and poorly controlled myoclonic jerks. Both were male infants with a family history of male relatives died in early childhood. Their hairs showed pili torti and trichorrhexis nodosa microscopically. Serum levels of copper were 14 ug/dl and 20 ug/dl. Ceruloplasmin levels were 10.4 mg/dl and less than 7 mg/dl. Their EEG showed abnormal generalized brain polyspike waves. Brain CT scan showed generalized brain atrophy, and chronic subdural hematoma in case 1. Bilateral urinary bladder diverticula and spurs over the distal ends of the femoral diaphysis were found in case 1. Normal urinary bladder was found in case 2 initially, then diverticula developed one year later. They are currently on anticonvulsants (Rivotril) therapy. Repeated attacks of respiratory infection, myoclonic seizure, hypotonia, and static neurologic developmental status are noted.
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PMID:[Menkes' kinky hair disease: report of 2 cases]. 217 69

There is currently little evidence available concerning the risks of foetal exposure to new anti-epileptic drugs such as lamotrigine, vigabatrin, gabapentine, topiramate. A small number of malformations without organ specificity have been described and are not easy to interpret because of the existence of concomitant treatments. We have reported a series of 12 pregnancies with exposure to recent anti-epilepticdrugs and that were reported to the Post-marketing Surveillance office in Tours, France. Five concerned Lamictal of which 2 related to monotherapy, one concerned Epitomax used in monotherapy and there were 6 cases of polytherapy including Sabril. Associated drug therapies were Depakine, Tegretol, Rivotril and Urbanyl. Six of the patients were on folic acid supplements. The average age of the women was 26.5 years. In each case, treatment had been initiated before conception and was continued for at least 3 months. Of the 12 babies born, only one presented with a malformation (aplasia of the muscle of the left lower lip and asymmetrical abduction of the hips) following exposure to Lamictal and Depakine. Four infants, two of whom were premature, showed signs of neonatal stress: transient respiratory distress and difficulty in taking feeding-bottles following exposure throughout the pregnancy to Epitomax; suction disorders, hypotonia and vomiting were observed after exposure to Sabril, Tegretol and Rivotril throughout the pregnancy; respiratory distress and apnoea--bradycardia were observed after exposure throughout the pregnancy to Lamictal and Urbanyl; respiratory distress and thrombocytopaenia were observed after exposure throughout the pregnancy to Lamictal". This small series confirms that the current data concerning the teratogenicity of new anti-epileptic drugs are as yet insufficient to exclude any teratogenic risk. Consequently, strict adherence to current recommendations relating to drug use during pregnancy is essential. The treatment of all patients wishing to become pregnant should be discussed.
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PMID:[New antiepileptic drugs in pregnancy: outcome of 12 exposed pregnancies]. 1242 60