Poor prognostic factors for survival also include bulbar onset, older age of onset, shorter interval from symptom onset to diagnosis and rapid disease progression [1, 9].
Most studies have reported that age, bulbar-onset ALS, and cervical weakness are factors associated with poor prognosis and lower survival, whereas longer diagnostic delay and use of riluzole were positively correlated with increased survival [14–17].
slurred speech, which may develop into difficulty swallowing some foods. a weak grip – you might drop things, or find it hard to open jars or do up buttons. muscle cramps and twitches. weight loss – your arms or leg muscles may have become thinner over time.
Bulbar onset MND or Progressive bulbar palsy (PBP):
PBP affects a smaller number of people than typical ALS, and mainly affects the muscles of the face, throat and tongue. Early symptoms may include slurring of speech or difficulty swallowing.
People who have MND may: develop generalised paralysis (paralysis of both sides of the body) lose speech and have difficulty swallowing. become breathless and experience sleep disturbance.
The usual cause of death is respiratory failure, often associated with infection. There are now two drugs licensed for MND – riluzole, which has been shown to slow the progression in some patients,3 and edaravone, which has been shown to help certain patient groups and is licensed in the United States.
Inherited MND affects up to 1 in 10 people with MND and means they probably have a family history of the disease. Where this is the case, it is impossible to predict when or if a family history means MND will happen. Other triggers may still be needed for the disease to begin.
End-stage symptoms
As MND progresses to its final phase, you might experience: increasing body paralysis, which means you'll need help with most daily activities. significant shortness of breath.
Bulbar-onset ALS generally starts with symptoms like slurred speech, difficulty chewing and swallowing, excessive choking and weakness or twitching in the muscles of the face, jaw, throat, and voice box, particularly the tongue.
The latency from symptom onset to diagnosis in MND documented in the literature has shown little improvement or change over the last 40 years and figures range from 10.6–17.5 months3–13.
Middle stage signs and symptoms
difficulty moving. joint pain. drooling, due to problems with swallowing. uncontrollable yawning, which can lead to jaw pain.
The most important prognostic factor in all human cancers is the stage at presentation, which is the anatomic extent of the disease.
Prognostic or predictive factors may include patient characteristics such as age, ethnicity, sex, or smoking status, disease characteristics such as disease stage or nodal status, and molecular markers such as HER2 amplification and K ras mutation.
Proven risk factors for ALS are genetic variants, male gender, and advanced age. The only environmental factor that is generally accepted to be associated with ALS is smoking.
Symptoms Of End Stages of ALS
Paralysis of voluntary muscles. Inability to talk, chew and drink. Difficulty breathing. Potential heart complications.
“Calculations assuming a uniform riluzole protective benefit indicate an improved median survival from onset by almost 2 months, with about 4% more patients surviving at 2 [years] from onset, if treatment is started at 6 months from onset rather than at 18 months from onset,” the scientists wrote.
Late-stage ALS
In advanced ALS, most voluntary muscles are paralyzed, including those of the mouth and throat. So are those involved in breathing. Poor respiration can lead to fatigue, headaches and impaired thinking. It also increases their susceptibility to pneumonia.
Palliative care for MND
Palliative care is active holistic care of people with advanced progressive illness. It involves: management of pain and other symptoms. psychological support.
Tiredness and MND
Fatigue is common with MND. Factors that may lead to fatigue include immobility, overexertion, sleep disruption, pain, weakened breathing, stress, anxiety, smoking, alcohol and some medications.
Spontaneous remission of non-symptomatic MND is extremely rare.
Behavioural impairment is a recognised feature of MND and problems may include socially inappropriate behaviour, disinhibited comments, impulsivity, apathy and inertia, loss of sympathy and empathy for others, and perseverative, rigid, stereotyped or compulsive behaviour.
A small proportion (5-10%) of people with motor neurone disease (MND) have a family history of the disease. This form of MND is known as familial, or inherited, MND.
In amyotrophic lateral sclerosis (ALS, also known as motor neuron disease) stressors could increase the uptake of neurotoxins, such as mercury, into a stress-activated locus ceruleus, with a subsequent decrease in noradrenaline output to the brain and spinal cord [12].