Bulbar onset motor neurone disease occurs in about 20% of those affected. The first sign is usually slurring of the speech, caused by impaired tongue movement, which may be accompanied by obvious wasting and fasciculation of the tongue (fig 1 (bottom)).
Bulbar onset MND or Progressive bulbar palsy (PBP):
Early symptoms may include slurring of speech or difficulty swallowing. Life expectancy is between six months and three years from onset of symptoms.
With MND, you may get thin or thick saliva. If you have swallowing difficulties, thin saliva can pool in the mouth and be difficult to clear. Thick saliva can cause your mouth to feel dry or sticky, and make swallowing harder.
Motor neurone disease (MND) is a rare neurological condition that causes the degeneration (deterioration and loss of function) of the motor system (the cells and nerves in the brain and spinal cord which control the muscles in our bodies). This results in weakness and wasting of the muscles.
A person with MND will usually die between two to three years after diagnosis, but this can vary from person to person.
MND often begins with weakness of the muscles in the hands, feet or voice, although it can start in different areas of the body and progress in different patterns and at different rates. People with MND become increasingly disabled.
Someone with MND may experience difficulties with thin, runny saliva that drools out of the mouth, thick tenacious saliva or phlegm. Thick saliva and phlegm can be particularly difficult to clear if the person has a weakened cough. 2 Some people may experience all of these difficulties at different times.
Sialorrhoea, the unintentional loss of saliva through the mouth, is the frequent complication of neurological disorders affecting strength or coordination of oropharyngeal muscles, such as motor neuron disease/amyotrophic lateral sclerosis (MND/ALS) or Parkinson's disease.
Conclusions: Tongue fasciculations are visible, spontaneous and intermittent contractures of muscle fibers, that are often a neurological finding of concern, reportedly related to motor neuron disorders, most often ALS.
Eventually, the muscles become weak and tight, limiting tongue, lip and/or jaw movement. Perhaps the most obvious result is slow, slurred, and difficult-to-understand speech. A person with bulbar ALS will have poor articulation and difficulty forming words.
One of the common symptoms of ALS is a gradual weakening and loss of control of the muscles in the mouth and throat. These muscles are known as “bulbar muscles,” and some of the “bulbar symptoms” of ALS include difficulty speaking or swallowing.
Early Signs of Bulbar Disease in ALS May Be Evident in Tongue's Movement While Talking.
The symptoms of MND start gradually over weeks and months. They tend to appear on one side of the body first and get progressively worse.
It's caused by a problem with cells in the brain and nerves called motor neurones. These cells gradually stop working over time. It's not known why this happens. Having a close relative with motor neurone disease, or a related condition called frontotemporal dementia, can sometimes mean you're more likely to get it.
The list of existing factors is as following: bulbar features—dysarthria, dysphagia, sialorrhoea or excessive salivation and tongue fasciculations; limb or muscle features—focal weakness, falls, foot drop, muscle wasting, muscle fasciculations or twitching, cramps, sensory impairment and muscle stiffness; respiratory ...
A dry mouth can occur when the salivary glands in your mouth don't produce enough saliva. This is often the result of dehydration, which means you don't have enough fluid in your body to produce the saliva you need. It's also common for your mouth to become dry if you're feeling anxious or nervous.
Examination in such cases may show atrophic, shriveled and weak tongue (Figure 2) with fasciculation and saliva drooling from the angle of the mouth. Figure 2. Bulbar onset ALS with tongue atrophy weakness.
Although MND does not directly affect the teeth and gums, the progressive physical disabilities can lead to poor oral health and a higher rate of extraction.
This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy's disease, and post-polio syndrome.
Living With a Motor Neuron Disease
Some are milder and progress more slowly than others. Although there is no cure for motor neuron diseases, medicines and therapy can ease symptoms and improve your quality of life.
Amyotrophic Lateral Sclerosis and the Eye. As discussed above, ALS affects not only motor neurons, the spinal cord, the cerebellum, and large areas of the brain but also the visual system, including the oculomotor and visual pathways. However, patients usually do not have visual complaints.
Over time, weakness progresses to total lack of control over movement. People with MND lose the ability to walk, talk, and look after themselves without considerable help and support. The muscles that control breathing and swallowing are affected just as much as those that control mobility.