What’s Up Doc column: Is the disease on NBC-TV comedy ‘Zoey’s Extraordinary Playlist’ real?

Dr. Jeff Hersh More Content Now USA TODAY NETWORK

Columns share an author’s personal perspective.


Q: Is the disease on the NBC-TV comedy “Zoey’s Extraordinary Playlist” real?

A: If you mean the condition where Zoey hears and sees people dancing and singing to express their innermost feelings, then no, that is not a real identified medical condition. However, if you are referring to the progressive bulbar palsy (PBP) that her TV father died from, then yes, that is a real condition.

The neurons that send messages directly from the brain stem to the bulbar muscles - that control swallowing, chewing, speaking and movement of the facial muscles - are called upper motor neurons, and those that send signals from the brain’s cerebral cortex via the spine to the muscles of the rest of the body are called lower motor neurons.

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease (after the famous baseball player who died from it in 1941), is a degenerative neurological condition that may affect both upper and lower motor neurons, causing weakness, loss of muscle tone, fasciculations, loss of reflexes and/or other symptoms. In about three-quarters of ALS patients, symptoms begin with insidious asymmetric weakness in the limbs (often the hands or feet, subsequently moving up the affected arm or leg).

ALS is a rare condition affecting about 50 per 1 million Americans, with about 6,500 newly diagnosed cases per year. About 10% of cases have an inherited component, the remaining 90% are sporadic, occurring randomly. ALS usually strikes people in their 30s through 60s, although it can occur at essentially any age.

PBP is possibly a subtype of ALS that initially affects the bulbar muscles. Most cases of PBP eventually develop symptoms in other areas of the body, similar to those of other ALS patients.

PBP is a very rare condition, with some cases with onset in infancy/childhood and some cases in adults.

There are many diseases that can cause muscle weakness/compromise such as acute infections (West Nile, polio, Lyme and others), poisonings (including lead, mercury, others), monoclonal gammopathies, muscular dystrophy, Parkinson’s disease, multiple sclerosis, myasthenia gravis, spinal muscular atrophy, myopathies and many others. After other possible causes of the patient’s symptoms have been ruled out or deemed unlikely, the diagnosis of ALS is suspected based on the patient’s history and physical exam (one example clinical scoring system to aid in the diagnosis is discussed below). The diagnosis is supported by specialized tests such as an EMG (electromyography, a study of the electrical activity in the muscles) and/or nerve conduction studies.

When ALS is considered there are four regions of the body that are specifically evaluated:

• The bulbar region discussed above.

• The cervical region that controls the arms.

• The thoracic region that controls the chest, back and abdomen, including the breathing muscles.

• The lumbosacral region that controls everything from the waist down.

In one clinical scoring system, the diagnosis of ALS is thought to be:

• Definite if there are ALS symptoms in three regions.

• Probable if there are symptoms in two regions or if there are symptoms in one region with positive electromyography (EMG) findings in at least two limbs.

• Possible if there are symptoms in one region with no EMG findings.

• Suspected if there are other more subtle symptoms that could be early ALS but could be from other conditions as well.

The cause of ALS/PBP is not understood. Treatment is supportive, addressing the patient’s nutritional needs, respiration and other activities of daily living, as well as avoiding complications such as pressure sores. The medication Riluzole was approved in 1995 to help slow the progression of symptoms. Although no cure has yet been identified, there is ongoing research trying to develop one.

ALS and PBP are progressive diseases, and the muscle weakness typically worsens (both in severity and in the number of muscles affected) over months to years. Because of this, they often lead to death within five years as the progressive muscle weakness eventually includes the breathing muscles. About 15% of patients with ALS live five years after diagnosis, and about 5% survive for more than 10 years. Long-term survival is more likely in those who are younger at diagnosis, male and with limb (rather than bulbar) symptom onset. For example, the brilliant physicist Stephen Hawking who was diagnosed with ALS at age 21 lived to age 77.

Jeff Hersh, Ph.D., M.D., can be reached at