Methods of treating amyotrophic lateral sclerosis

A method for reducing disease progression of a neurodegenerative disease treatable with 11,11-D2-linoleic acid in a patient, comprising:

• administering 11,11-D2-linoleic acid or an ester thereof to the patient with a dosing regimen that comprises a primer dose and a maintenance dose thereby reducing the disease progression in the patient, wherein:
• a) the primer dose comprises periodic administration of from about 7 to 12 grams of 11,11-D2-linoleic acid or an ester thereof per day, wherein the primer dose is continued for about 30 days to about 45 days to rapidly achieve a therapeutic concentration of 13,13-D2-arachidonic acid in vivo;
• and
• b) subsequent to completion of the primer dose, periodically administering the maintenance dose of no more than about 65% of the primer dose of 11,11-D2-linoleic acid or an ester thereof per day thereof to maintain the therapeutic concentration of 13,13-D2-arachidonic acid in vivo, such that a rate of disease progression is reduced, wherein the neurodegenerative disease is mediated at least in part by lipid peroxidation of polyunsaturated fatty acids in neurons of the patient;
• wherein the disease is amyotrophic lateral sclerosis, Huntington's Disease, progressive supranuclear palsy (PSP), Friedreich's ataxia, APO-e4 Alzheimer's Disease, corticobasal disorder (CBD), frontotemporal dementia (FTD), nonfluent variant primary progressive aphasia (nfvPPA), other tauopathies, and late onset Tay-Sachs.