IMPROVEMENT IN HURLER SYNDROME WITH AYURVEDIC MANAGEMENT: A CASE REPORT

Abstract

Hurler syndrome is a rare lysosomal storage disorder occurring due to the deficiency of the lysosomal enzyme alpha L Iduronidase and has an autosomal recessive inheritance [1]. Ayurveda explains this condition as aavaranajanya vatavyadhi (obstructive pathology) [2]. This is the case of Hurler Syndrome of a female child of 13yrs with a relevant family history (her sister had the same syndrome). The patient had dysmorphic facies, pain and stiffness over multiple joints, dysmorphic facies, difficulty in walking, and mild speech impairment. She visited the outpatient department of our tertiary Ayurvedic health centre three times. She took OP and IP management along with dietary recommendations for about two months and ten days, and significant improvement was noted such as a reduction in contractures, ability to walk independently, self-feeding and the ability for normal schooling. The assessment was done with Cerebral Palsy Quality of Life (CPQOL). The values changed from 330 to 445 before and after treatment, respectively and gradings of spasticity as per the Modified Ashworth scale changed from 2+ to 1+ in both upper limbs and from 3+ to 2+ in both lower limbs.