Amyotrophic lateral sclerosis and hospice care.

Abstract

Although the vast majority of patientsadmittedtohospiceshavecancer, some4 to 5 percentsuffer from otherterminaldiseases, the mostnotable of which are neuromuscularin nature.Without a doubt,patientswith this groupof illnessescanbeverydifficult for thehospiceteamto carefor sincethey can havea wide constellation of terminal symptoms.Furthermore, thesepatientsoften live longer than cancerpatients,thus, further taxingnot only thefamily but alsothe hospiceteamaswell. This reviewwill focus on the most commonform of theseneuromusculardisordersencounteredin hospicepractice,namely amyotrophiclateralsclerosis(ALS) or Lou Gehrig’sdisease. Motor neurondisease(MND) is a heterogeneous groupof disorderswith varyingsignsandsymptoms, all affecting in somemannerthe anteriorhorn cells. The most common subsetof MND in adults is ALS which affects notonlytheanteriorhorncellsbutalso the corticospinaltracts.Theneteffect of theALS lesionis to causeprogressive wasting and weaknessof those muscleswhich havelost their nerve supplyinadditionto corticospinal tract signsof spasticityandpathologicreflexes in the absenceof sensoryfindings. In mostcasesof ALS, intellectual function remainsintact. Variants of classicalALS includespinalmuscular atrophy,progressivebulbarpalsy,primarylateralsclerosisandbenignfocal amyotrophy.Spinal muscularatrophy presentsa clinical picture of limb weaknessandwastingwithvaryingdegreesof cranialnerveinvolvement.Progressivebulbar palsy is dominatedby weaknessandatrophyof cranialnerves ofthelowermotorneurontype,whereas, primarylateralsclerosisismanifestedby uppermotorneuronsignsalone.Finally, benign focal amyotrophyis a clinical syndromein whichthe signsof muscle weaknessandatrophymaybeginin a singlelimb but notprogress. Theworldwideincidenceof ALS is 0.4to 1.8per100,000population.1The incidenceof familial ALS is approximately5 to 10percentof all ALS with the modeof inheritanceunclear. Of interest, thereis an unexpectedly high incidenceof ALS inGuamwhich hasbeenextensivelystudied.Themale to femaleratio is 1.0to 1.6with theage of onsetbetween50 and60 years.