This paper is only available as a PDF. To read, Please Download here.
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 β-oxidation of fatty acids and levels of dicarboxylic
acids would increase because of increasing ω-oxidation.
We suspect that there is a possible relationship between the occurrence of acute encephalopathy
and calcium hopantenate therapy.
Key words
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Acute encephalopathy in siblings, Reye's syndrome vs. salicylate intoxication.Am J Dis Child. 1976; 130: 295-297
- Reye-like syndrome associated with valproic acid therapy.J Pediatr. 1979; 95: 142-144
- Margosa oil poisoning as a cause of Reye's syndrome.Lancet. 1981; 1: 487-489
- A double-blind controlled study of Ca hopantenate and placebo in conformity to a rating list for the evalution of the abnormal behaviors in children (III) (in Japanese).Rinshou Hyoka (Tokyo). 1974; 2: 375-384
- Studies on the influence of homopantothenic acid on the hepatic coenzyme A and its precursors contents in young albino rats (in Japanese).Vitamins (Kyoto). 1966; 34: 505-507
- A case of Reye-like syndrome with dicarboxylic aciduria (in Japanese).Proc Jap Soc Med Mass Spectrom (Kurume). 1982; 7: 153-156
- Three cases of acute encephalopathy associated with calcium hopantenate administration (in Japanese).No To Hattatsu (Tokyo). 1983; 15: 258-259
- Diagnosis and treatment of Reye' syndrome.JAMA. 1982; 246: 2441-2444
- Gluundescribed metabolic disorder.Clin Chim Acta. 1976; 66: 227-239
- The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features.Neurology. 1975; 25: 16-24
- Urinary excretion of C6-C10 dicarboxylic acids in glycogen storage disease type I and III.Clin Chim Acta. 1974; 51: 93-101
- Mass spectrometric identification of 3-hydroxy-1, 10-decane-dioic acid and 3-hydroxy-1, 12-dodecanedioic acid in urine of ketotic patients (in Japanese).Proc Jap Soc Med Mass Spectrom (Kurume). 1980; 5: 131-134
- Calcium hopantenate therapy in psychiatric practice (in Japanese).Kiso To Rinshou (Tokyo). 1979; 13: 3935-3955
- Two autopsied cases of sudden death associated with calcium hopantenate.Brain Dev. 1984; 6: 230
- An autopsy case of Reye's syndrome occurred during administration of calcium hopantenate (in Japanese).Saishin-Igaku (Osaka). 1984; 39: 1679-1685
Article info
Publication history
Accepted:
March 25,
1986
Received:
December 25,
1985
Identification
Copyright
© 1986 Published by Elsevier Inc.
