Anesthetic Management in Marinesco-Sjogren Syndrome

Abstract

Marinesco-Sjogren syndrome (MSS) is a very rare autosomal recessive disorder characterized clinically by muscle weakness, mental retardation, cerebellar ataxia and cataracts. The most prominent neurological feature is the cerebellar ataxia with hypotonicity and mental retardation is apparent from infancy. Patients with MSS may be posted for surgery early in the age for cataract aspiration, which requires unique anesthetic considerations involving myopathy, hypotonicity, growth and mental retardation. Till now only one case report of anesthetic management in MSS has been reported by Kouichiro Minami et al. Here we report a case of MSS and its anesthetic management coming for cataract surgery.

Keywords

• marinesco-sjogren syndrome
• myopathy
• hypotonia