Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Calcium hopantenate (HOPA-Ca), which is obtained by substituting the beta-alanine of pantothenic acid for gamma-amino butyric acid (GABA), is a therapeutic drug for mental retardation and cerebrovascular dementia. HOPA-Ca is known to produce convulsive seizures in some patients although it is also true that this improves EEG abnormalities and suppresses epileptic seizures. Thus, clinical observations suggest that HOPA-Ca exerts a paradoxical influence on epilepsy. In order to gain further insight into the influence of HOPA-Ca on epilepsy, we examined its effects on the generalized seizure-triggering threshold (GST) of kindled amygdaloid (AM) seizure and on the rate of AM kindling in rats. Male Wistar rats weighing 200-250 g were used. Under pentobarbital anesthesia, a bipolar electrode, made of twisted stainless steel wire 0.2 mm in diameter, was stereotaxically implanted into the left AM. Daily electrical stimulation was given at the intensity of afterdischarge threshold (ADT). Electrical stimulation was continued until at least five consecutive generalized convulsions were evoked. Subsequently, the stimulus intensity was daily lowered by 20-microA steps and the last stimulus intensity for evoking generalized convulsion was designated as the GST. Experiment 1. Influence of HOPA-Ca on the GST; HOPA-Ca was dissolved in saline as a vehicle. Injections of 50, 100, 250, 500 mg/kg HOPA-Ca were given intraperitoneally 60 min before the stimulation at the GST. The GST never changed with vehicle injection. The GST increase was never observed with any amount of HOPA-Ca. However, the GST dropped flowing HOPA-Ca infection independent of its amount. The GST reduction was always 20-microA.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Facilitatory effect of calcium hopantenate on amygdaloid kindling in rats]. 322 34

We report a 3-year-old Japanese girl who developed acute encephalopathy while receiving calcium hopantenate (Calcium D-(+)-4-(2, 4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate). She had hyperammonemia, elevated CPK, lactic acidemia and pyruvic acidemia, however, she did not show elevated SGOT or SGPT. Calcium hopantenate has been used in Japan for the treatment of mental retardation with behavior abnormalities. Recently there have been three reports on the occurrence of Reye-like syndrome in patients receiving this drug. Clinical signs and laboratory data of these patients are similar to those of Reye syndrome. Calcium hopantenate causes pantothenic acid deficiency in the young rat, which may reduce the content of coenzyme A. If this drug decreases coenzyme A biosynthesis, it may reduce beta-oxidation of fatty acids and levels of dicarboxylic acids would increase because of increasing omega-oxidation. We suspect that there is a possible relationship between the occurrence of acute encephalopathy and calcium hopantenate therapy.
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PMID:Acute encephalopathy with hyperammonemia and dicarboxylic aciduria during calcium hopantenate therapy: a patient report. 382 51