ALS - Part 4 - Diagnosis & Standard Medical Practice

  Diagnosis

To diagnose a patient with ALS, the doctor will perform a physical examination. The patient will be given an electromyography (EMG) test which tests nerve impulses within muscles. A weak response can indicate ALS (Gerics). An MRI (Magnetic Resonance Imaging) test can rule out some other neurological problems such as stroke, Alzheimer’s disease, Parkinson’s disease and others (Nordqvist). Blood tests can rule out autoimmune diseases like lupus or multiple sclerosis. The nerve conduction velocity test (NCV) shows the speed at which nerve communication is occurring. The doctor may conduct a spinal tap to analyze the cerebrospinal fluid (Nordqvist). A muscle biopsy may be performed to rule out a muscle disease (Nordqvist).

Most of these tests are done to rule out other diseases that can mimic ALS (McCarthy). To date there is no ALS-specific diagnostic test, although researchers are actively looking for one. Before confirming a diagnosis of ALS, some neurologists may want to wait a couple of months to see if there is progression in symptoms such as muscle shrinking, muscle weakness, twitching, or alterations in normal muscles reflexes (Nordqvist).

Standard Medical Practices

Currently standard medicine only offers one drug, Rilutek (Riluzole), that is approved for use with ALS patients, and it has been shown to extend life span by up to four to six months (Zoccolella, Santamato and Lamberti). This drug is well tolerated although it can have side effects of nausea, vomiting, weakness, dizziness and coughing (Rhodes). Also offered in standard medical care are foot braces, hand splints, wheelchairs, and counseling. Treatment is basically palliative. With swallowing difficulties, pureeing food if often necessary, and eventually a gastrostomy tube (feeding tube) may be used. Some drugs can also be prescribed to help with bulbar difficulties (swallowing or speech difficulties) and with muscle cramping (Segala). The most common cause of death in ALS is respiratory insufficiency and is present in almost all cases at some stage of the illness (Hardiman).

A bi-level positive pressure ventilator (BiPAP) is recommended for ALS patients. A BiPAP applies a lighter expiratory than inspiratory pressure so that the work of breathing is not increased during expiration. Those patients that tolerate and can utilize the device can show an increase of life expectancy by up to 9 months (Hardiman).

A patient with the symptom of drooling (sialorrhea) can be given one of two drugs or radiation to help with this condition. A review of 40 studies done with ALS patients showed that one drug did show useful improvement – butulinum toxin type B (BTxA) (Miller, Jackson and Kasarskis).

Another symptom of ALS can be excessive laughing or crying or other involuntary emotional expression (called pseudobulbar affect). The episodes can last for seconds to minutes, and can occur several times per day, with onset being unpredictable. The response can either be appropriate for the situation, although quite excessive, or the outburst can be incongruent such as a joke eliciting weeping. Currently there are two drugs available for the patient with this symptom and the combination of the two is considered more effective (Miller, Jackson and Kasarskis). Unfortunately, 24% of the people prescribed the combination drug terminated treatment due to side effects like dizziness, nausea, and drowsiness (Miller, Jackson and Kasarskis).

As difficulties in communicating become an issue for ALS patients, standard medical practice offers an alphabet board, computer devices, utilization of the anal sphincter and eye movement devices to allow communication when needed (Miller, Jackson and Kasarskis).

Muscle cramping and muscle spasticity (abnormal stiffness) are common ALS symptoms. There is currently no recommended or standard medical intervention for the symptom of muscle cramping (Miller, Jackson and Kasarskis). And there is no medication that has been studied that ameliorates the symptom of muscle spasticity. However, in one study, moderate exercise was found to help slightly (Miller, Jackson and Kasarskis).

Depression is another potential symptom in ALS patients. There are no studies that establish any medical treatment that is effective (Miller, Jackson and Kasarskis). Insomnia is also common in ALS patients possibly due to respiratory weakness, anxiety, depression, or cramping. Again, there are no studies of standard medical treatment for insomnia associated with ALS (Miller, Jackson and Kasarskis).

A study done in 2009 reviewed current and emerging therapeutic agents used for ALS during the previous 23 year period as to their effectiveness and safety (Zoccolella, Santamato and Lamberti). Forty eight compounds were analyzed, of which seven were a vitamin, mineral, amino acid or other supplement generally available to alternative practitioners and the general public. The conclusion of the study was that “none of them significantly prolonged survival or improved quality of life of ALS patients.” Discussion in the body of the article did reflect that some of the seven supplements showed some interesting results that will be discussed later in this paper.

This paper is in honor of Warren Schaeffer, an exceptional
person, adventurous being and a first-class gentleman.