Careful Consideration Should Be Preceded Before Intrathecal Baclofen Pump Implantation in a Patient with Amyotrophic Lateral Sclerosis: A Case Report

Abstract

Amyotrophic Lateral Sclerosis (ALS) is characterized by progressive degeneration of both upper and lower motor neurons. Predominant upper motor neuron involvement causes clinical manifestations of spasticity. We report a case of ALS presenting progressive spastic paraplegia treated with intrathecal baclofen (ITB) test trials before ITB pump implantation. Electrodiagnostic studies confirmed the diagnosis of ALS. After administration of 50 mcg and 25 mcg of ITB separately, severe spasticity of both legs was relieved effectively within 2 hours after a ITB test trial. However, weakness of both lower extremities appeared which eventually led to functional decline in the item “turning in bed and adjusting bed clothes” of the ALS Functional Rating Scale-Revised. Desaturation event also occurred four hours after 50 mcg ITB bolus injection. Therefore, careful consideration is necessary for in a patient with ALS presenting spastic paraplegia before ITB pump implantation. In particular, ITB test trial should be preceded when considering ITB pump implantation.