Conceptual approach to diagnostic delay in ALS: a United States perspective

The mean time from onset of symptoms to confirmation of diagnosis of amyotrophic lateral sclerosis (ALS) in the United States, as elsewhere, is 16-18 months. Delays may arise from the complex referral pathway, caused at least in part by the multiple types of insurance and health-care services available in the United States and also because physicians sometimes attempt to avoid medicolegal responsibility for a very undesirable diagnosis. In addition, initial symptoms are often intermittent and nonspecific and may be denied or not recognized by the patient. In the United States, the primary care physician is increasingly viewed by health maintenance organizations as a gatekeeper, with incentives to keep the diagnosis within the primary care realm. This may lead to misdiagnosis and inappropriate referral. Even after the patient reaches a neurologist, the differential diagnosis of ALS is large and may involve many tests, all of which may incur scheduling and reporting delays. Reluctance to give a bad diagnosis before it is absolutely certain may also cause delay. Delays after diagnosis may be the result of health insurance constraints, the prejudices of the neurologist in favor of or against particular therapies, and the patient's willingness to accept or ability to pay for therapy. Many of these delays may be lessened by both professional and lay educational initiatives to raise awareness of the symptoms of ALS and encourage more rapid presentation and referral to the neurologist. The availability of credible treatment options would undoubtedly encourage physicians to have hope and to seek an earlier diagnosis.