General Anesthesia Plus Muscle Relaxant in a Patient with Kugelberg Welander Disease: A Case Report

Abstract

Spinal muscular atrophies (SMAs) represent a rare group of inherited disorders that cause progressive degeneration of the anterior horn cells of the spinal cord. The exact cause of the degeneration is unknown. Loss of these cells results in a progressive lower motor neuron disease that has no sensory involvement and that is manifested as hypotonia, weakness, and progressive paralysis. Kugelberg Welander spinal muscular atrophy (also known as Wohlfart-Kugelberg-Welander syndrome or mild SMA) is a milder form of SMA, with symptoms typically presenting after age 18 months. Here in we report a case of anesthetic management of a patient with Kugelberg Welander disease who was refered for squint surgery and also we reviewed some other cases of SMA patients receiving different types of anesthesia.

Keywords

• spinal muscular atrophy
• squint surgery
• muscle relaxant