40 - Neuromuscular rehabilitation: diseases of the motor neuron, peripheral nerve and neuromuscular junction

Summary

Introduction

In this chapter, we focus on rehabilitation of patients with diseases of the motor neuron like amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA) and polio and diseases of the peripheral nervous system, especially acute and chronic inflammatory polyneuropathies Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) and other neuropathies (Fig. 40.1). Additionally, we include diseases of the neuromuscular junction (myasthenia gravis and Lambert–Eaton myasthenic syndrome (LEMS)). We describe the current knowledge on the pathogenesis, the clinical symptoms, and deficits and current therapy concepts during the acute disease state and for rehabilitation including long-term impairment of activities of daily living.

Quality of life of patients is affected not only by the physical disability as measured in neurologic disability scores but also by spiritual, religious and psychologic factors (Simmons et al., 2000). During the course of the disease ALS patients may accept the disease with its presumed fatal outcome leading to a maintained quality of life despite a deterioration of physical strength (Simmons et al., 2000). The situation seems to be different in myasthenic patients: Although their physical conditions are essentially preserved during most time the knowledge of possible crises with life-threatening muscle weakness and the uncertainty whether or when a crisis may occur severely affects their quality of life (Padua et al., 2001). There may be also differences in the acceptance of the chronic disease between the patient and their caregivers (Amosun et al., 1995; Mitsumoto and Del Bene, 2000).