Abstract

AYURVEDIC MANAGEMENT OF SIALIDOSIS TYPE 1 – A CASE STUDY

Smitha Anekal*, A. S. Patil

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Abstract

Background: Sialidosis is an autosomal recessive disorder caused by a mutation in the neuraminidase gene (NEU-1), leading to a deficiency of neuraminidase and resulting in abnormal intracellular accumulation of sialyloligosaccharides. There are two types of Sialidosis: Type 1 (milder) and Type 2 (severe). Sialidosis Type 1, also known as normomorphic or cherry-red spot myoclonus syndrome, usually appears in the second or third decade of life. It is a rare condition with an overall prevalence of about one in every 5 to 10 million. The exact pathophysiology of this disorder is not fully understood, but in Ayurveda, it can be seen as a vata vyadhi. Samprathi and smaprapthi ghatakas can be understood, and it can be managed as anuktha vyadhi focusing on smaprapthi vighatana to help control the progression of the disease. Case report: A 26- year- old male patient presented with intentional tremor and action myoclonus, accompanied by postural instability. He sought care at an allopathic hospital three years ago, where further investigation confirmed a diagnosis of Sialidosis type 1, and he has been undergoing treatment. For additional management, he approached our hospital. Diagnosed as vatavyadi, he received treatment with basti and shamana chikitsa; improvement was observed after basti administration. Conclusion: Sialidosis can be classified as vatavyadhi, resulting from beeja dosha and margavarodha, with sanga and vimargamana of vata leading to chala guna vriddhi. Therefore, the treatment should aim to clear the margavarodha while simultaneously reducing the chala guna of vata. Ayurveda can be beneficial in managing these chronic cases to control disease progression and minimize dependency on medication.

Keywords: Sialidosis, Action myoclonus, Vatavyadhi, Basti, Samprapthi.