Motor Neuron DiseaseMotor NeuronsNeuromuscular DiseasesBulbar Palsy, ProgressiveAmyotrophic Lateral SclerosisMuscular Atrophy, SpinalNeuronsSurvival of Motor Neuron 1 ProteinSpinal CordSurvival of Motor Neuron 2 ProteinAnterior Horn CellsNerve DegenerationSMN Complex ProteinsSuperoxide DismutaseMuscular Disorders, AtrophicMotor CortexFasciculationBulbo-Spinal Atrophy, X-LinkedGaggingMuscular AtrophyAxonsNeurons, AfferentTDP-43 ProteinopathiesFrontotemporal Lobar DegenerationNeurofilament ProteinsFrontotemporal DementiaElectromyographyToxascariasisbeta-Hexosaminidase beta ChainEvoked Potentials, MotorRNA-Binding Protein FUSPharyngostomyRiluzolePyrrolidonecarboxylic AcidInclusion BodiesNeural ConductionElectric InjuriesDeglutition DisordersMice, TransgenicPick Disease of the BrainMotor ActivityDisease Models, AnimalBrain Injury, ChronicLathyrismMutationDEAD Box Protein 20Ulnar NerveNerve Tissue ProteinsSpinal Cord DiseasesBrainPeripheral NervesNeuromuscular JunctionAction PotentialsSandhoff DiseaseMarchiafava-Bignami DiseaseRats, TransgenicNeurologic ExaminationCricoid CartilageAxonal TransportDementiaDNA Repeat ExpansionParalysisSpinal Muscular Atrophies of ChildhoodRecruitment, NeurophysiologicalElectric StimulationMuscle, SkeletalImmunohistochemistryAge of OnsetAnimals, Genetically ModifiedCells, CulturedSpastic Paraplegia, HereditaryMusclesGastrostomyCell DeathMuscle WeaknessTime FactorsSpinal Nerve RootsMice, Inbred C57BLPhenotypeMolecular Motor ProteinsPedigreeDisease ProgressionCell CountSynapsesThyrotropin-Releasing HormoneGanglia, InvertebrateNervous System DiseasesAtrophyNeuroprotective AgentsNeuroimaging