# Recurrent Fevers, Dysautonomia, and Dehydration in a Patient With Lesch-Nyhan Syndrome

## TL;DR

A 26-year-old man with Lesch-Nyhan syndrome experienced severe symptoms due to autonomic instability causing dehydration.

## Contribution

This case report highlights a rare, life-threatening manifestation of Lesch-Nyhan syndrome involving autonomic instability and dehydration.

## Key findings

- The patient presented with fever, hypotension, and hypernatremia linked to autonomic instability.
- Excessive free water loss was suspected as the cause of the patient's severe symptoms.
- This case underscores the importance of considering autonomic dysfunction in LNS management.

## Abstract

Lesch-Nyhan syndrome (LNS) is a disease characterized by a reduced ability to recycle purines, leading to increased de novo purine synthesis and uric acid production. Patients classically present with an array of hyperuricemic, neurologic, and behavioral symptoms. In this report, we describe a 26-year-old male with a history of LNS and recurrent fevers of unknown origin who presented to the emergency department (ED) with a fever, hypotension, and hypernatremia. We suspect that our patient's presentation was caused by autonomic instability in the setting of LNS leading to excessive free water loss. This report highlights a rare but life-threatening manifestation of LNS.