This chapter provides an overview of an animal model of motor neuron disease in guinea pigs. Guinea pigs injected with bovine motor neurons develop symptoms of neuromuscular degeneration marked by weakness, evidence of denervation by electromyographic and morphological criteria, and the loss of motor neurons within the spinal cord. These guinea pigs develop high serum titers of IgG-class antibodies to motor neuron constituents. Immunohistochemistry describes the presence of IgG within the spinal cord motor neurons and at the end plates of immunized animals. This experimental model offers a relatively specific immune-mediated destruction of motor neurons, which permit detailed study of the mechanism of motoneuron loss and of therapeutic agents that may ameliorate the process. This syndrome of experimental autoimmune motor neuron disease is distinct from experimental allergic encephalomyelitis produced by the inoculation of myelin basic protein and other myelin constituents, and also distinct from experimental allergic neuritis that compromises peripheral nerve function. Only the passive transfer of motor neuron dysfunction with serum derived from guinea pigs with experimental autoimmune motor neuron disease, shed light on the pathogenesis of the disorder.
ASJC Scopus subject areas