ALS - Part 3 - ALS Symptoms & Progression

   Symptoms and Progression

The first symptoms of ALS noticed by the patient are usually vague. There may be weakness in one leg, hand, face or the tongue (Rhodes). The symptoms of ALS vary according to which muscle group is affected. The disease usually starts in the hands and generally proceeds up the arm. It can also start in the legs making walking difficult or with the throat making swallowing difficult. ALS may progress more quickly on one side of the body than the other. Symptoms of ALS include abnormal fatigue in the limbs, dropping things, tripping, slurred speech, inability to talk loudly, muscle cramps and twitching (Segala). WebMD offers this list of symptoms:

• Weakness or clumsiness in the hands and feet.
  
  
• Gradual loss of strength of the arms and legs.
  
  
• Inability to control the use of the arms and legs.
  
  
• Muscle twitches (fasciculations).
  
  
• A stiff, clumsy walking style.
  
  
• Difficulty swallowing, speaking, and breathing. The ALS patient may notice a problem with drooling.
  
  
• Fatigue.
  
  
• Trouble controlling emotions. The ALS patient may burst into tears or start laughing for no reason.
  
  
• Muscle cramps, especially at night, which may occur late in the course of the disease.
  
  
• Pain during the late stages of the disease.
  
  

ALS is a progressive disease. The spread of weakness into all muscle groups is the defining characteristic of ALS (LEF Protocol for ALS). Deterioration of muscle (muscle wasting or amyotrophy) is a prominent symptom with ALS, resulting in tetany - a condition that is characterized by periodic painful muscular spasms and tremors due usually to faulty calcium metabolism and loss of enzymes. The rate of muscle loss can vary significantly between patients. As motor neurons are destroyed, the signals to the muscles involved cease to be stimulated and wasting occurs.

According to WebMD, “walking, speaking, eating, swallowing, breathing, and other basic functions become more difficult with time.” Due to the development of muscle weakness, respiratory problems are the most serious complication. Swallowing and coughing become more difficult, with the likelihood that aspiration of food and saliva into the lungs can occur (Rhodes). WebMD further states that “pneumonia, pulmonary embolism, lung failure, and heart failure (probably due to lack of adequate breathing as the chest muscles weaken) are the most common causes of death among people with ALS. In most cases, death will occur within 3-5 years after symptoms begin, although some people live for many years, even decades.”

The variability in survival times is due to unknown factors. Some factors that are associated with less survival time include older age at onset, female sex, bulbar onset (a part of the brain stem - shaped like a bulb - that is involved with the process of swallowing), reduced Forced Vital Capacity (FVC), and decreased time from symptom onset to diagnosis (Kamel, Umbach and Stallone). Note: Forced Vital Capacity (FVC) is the volume of air that can forcibly be blown out after full inspiration, measured in liters. FVC is the most basic maneuver in spirometry tests and gives an indication of muscle weakness involving the lungs. Weakness in the muscles of the trunk that control breathing develops slowly over months or years (McCarthy).

ALS results in several communication problems. Patients may suffer from agraphia or loss of writing ability. Writing errors include unfinished sentences, mismatch between a subject and its verb, letter omissions, substitutions, and displacements (Tsuji-Akimoto, Hamada and Yabe). This disability in writing can be compounded by mild cognitive impairment with about 23% of ALS patients showing mild dementia (Tsuji-Akimoto, Hamada and Yabe). This study also reported that if frontotemporal lobar dysfunction is found to be present, then as many as 50% of ALS patients show adverse cognitive changes (Tsuji-Akimoto, Hamada and Yabe). The Tsuji-Akimoto study suggests that agraphia might have some correlation with mild cognitive impairment. In order to assess a patient, this study recommends an analysis of a patient’s writing ability. This could then correlate as to whether the brain’s abilities are being impacted by ALS in addition to a motor neuron impact.

The term dysarthria is also used in describing ALS symptoms (Tsuji-Akimoto, Hamada and Yabe). Dysarthria is difficulty in speech articulation, manifesting as slurring, slowness of speech, and difficulty with producing words. The patient may also have problems controlling the pitch, loudness, rhythm, and voice qualities of their speech. Dysarthria should be distinguished from Aphasia. Aphasia is a condition characterized by a partial or total loss of the ability to communicate verbally or in writing where the loss is due to lack of language comprehension rather than lack of the physical ability to communicate. In dysarthria, the brain is working but the muscles are not working well enough for good communication. ALS patients suffer from dysarthria and not aphasia unless there are cognitive issues in addition to the ALS.

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