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Indian Journal of Psychiatry
Medknow Publications
Publication Date
  • Brief Communications
  • Chemistry
  • Medicine


Indian Journal of Psychiatry, April 1987, 29(2), pp. 169-170 BRIEF COMMUNICATIONS S O M E P A R A D O X I A L O B S E R V A T I O N S N O T E D IN T H E PREVALENCE OF H U R L E R ' S A N D H U N T E R ' S S Y N D R O M E H.S.NARAYANAN1 K. S.MOHAN2 N. JAYASIMHA3 B.S. SRIDHARA RAMA RAO4 Hurler's and Hunter's syndromes be- long to the group of lyosimal storage di- sorders where the mucopolysaccharides (MPS) are the storage substances (Stanbury et al. 1983). Both Hurler's and Hunter's cases show the excretion of dermatan and heparan sulphates in the urine. Despite this similarity in biochemical feature the two differ in some other respects. For instance corneal opacity is noted only in subjects with Hurler's but not in Hunters synd- rome. Also the nature of inheritance is au- tosomal recessive in Hurler's while it is sex linked in Hunter's. During our studies on subjects with cases with MPS excretion we noted a variation in the prevalence of the two types and the present paper briefly re- ports the same. Table The age and sex distribution of the cases Age Group (in years) 0 - 3 4 - 6 7 - 9 10 - 14 Total Hurler M 14 8 6 - 28 F 6 3 2 1 12 Hunter M F 1 - 7 - 2 - 4 - 14 - Total M F 15 6 15 3 8 2 4 1 42 12 The subjects of this study were obtained from patients attending the Mental Retar- dation clinic at this centre. Based on clinical and biochemical investigations about eigh- ty cases were found to belong to the group of mucopolysaccharidoses. Among them forty were Hurler's type and fourteen were Hunter's type. From the clinical observations it was noted that cases of Hunter lived somewhat longer than Hurler. It might, therefore be expected that the prevalence of Hunter's should be more frequent than Hurler's. In order to test this the Handy-Wein- berg formula was used to find out the gene frequency. In the case of Hunter's the gene frequency was similar to the

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