Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a series of 53 fenfluramine intoxications (15 taken from the literature), 10 were lethal after doses of 28.7--70 mg/kg of body weight. Cardiac arrest occurred 1--4 hr after ingestion in 9 cases; all these 9 patients died. Two out of 3 patients with more than 15 mg/kg had coma and convulsions. Other frequent signs were mydriasis, tachycardia, and rubor of the face. The additional signs of nystagmus, hypertonia, trismus, hyperreflexia, clonus, excitation, hyperthermia, and sweating define the clinical syndrome of fenfluramine intoxication. Symptoms begin 30--60 min after ingestion and can persist during several days. Early gastric lavage, instillation of activated charcoal, diazepam in case of seizures, chlorpromazine for malignant hyperthermia, propranolol for extreme tachycardia, and lidocaine in the event of ventricular extrasystoles are recommended. If trismus is a prominent sign, muscle relaxants must be given before gastric lavage can be done. The relatively benign course after survival of the first 4 hr suggests supportive therapy only in the later phase of intoxication.
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PMID:Fenfluramine poisoning. 43 87

Hemiplegic migraine (HM) is a rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. Hemiplegic migraine has two main forms according to the familial history: patients with at least one first- or second-degree relative who has aura including motor weakness have familial hemiplegic migraine (FHM); patients without such familial history have sporadic hemiplegic migraine (SHM). The prevalence of HM is one in 10,000 with FHM and SHM being equally frequent. Typical HM attacks include a motor weakness that is always associated with other aura symptoms, the most frequent being sensory, visual and speech disorders. In addition, basilar-type symptoms occur in up to 70% of the patients. Severe attacks may occur in FHM as well as in SHM with prolonged hemiplegia, confusion, coma, fever and seizures. The clinical spectrum also includes permanent cerebellar signs (nystagmus, ataxia, dysarthria) and less frequently various types of seizures and intellectual deficiency. FHM is the only variety of the autosomal dominant migraine and all three know genes encode ion-transporters. A genetic diagnosis is now possible by screening the three known genes involved in FHM (CACNA1A, ATP1A2 and SCNA1). Prognosis is usually good. Treatment is similar to approaches used in other varieties of migraine with aura, excepted for triptans that are contraindicated in MHF/MHS. Based on new pathophysiological insight, preventive treatments by various antiepileptic agents seem promising.
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PMID:[Familial and sporadic hemiplegic migraine]. 1840 71