Motor neuron disease (MND) is also known as Lou Gehrig’s disease or amyotrophic lateral sclerosis or ALS. You may also hear some doctors referring to it as Charcot's disease, syndrome or even Charcot's sclerosis. In general, MND is found in some 5 to 7 of 100.000 people and doctors in general practice witness one or two cases during their career. According to some statistics, men over 80 years of age are more likely to develop this condition than elderly women of the same age.
About MND or Lou Gehrig’s Disease
This disease is very rare, but people suffering from it are faced with progressive paralysis and potential death. MND affect motor neurons, causing dysfunction of lower motor neuron (LMN) and upper motor neuron (UMN) and provoking muscular paralysis. Patients usually have mixed LMN/UMN paralysis, although LMN symptoms are usually more dominant.
No one knows why some people get this disease. However, current medical theory believes that abnormality of mitochondrial function can be responsible for oxidative stress in motor neurons and therefore damage of LMN and UMN.
MND symptoms usually include focal onset of ALS, which affects both upper and lower motor neurons. It could have three different patterns, affecting the limbs (limb onset, which is the most common start of this disease) and bulbar onset, which is present in some 20% of all ALS patients. Additionally, patients may also have respiratory onset of this disease, but happens very rarely.
MND may also be presented only with UMN or only with LMN features. Pure upper motor neuron features of the disease are known as primary lateral sclerosis, while patients diagnosed with pure LMN features are known to have progressive muscular atrophy.
Possible Health Complications and End Stage Care
There are numerous complications of MND and patients may suffer from many health-related problems. Constipation, spasticity and cramps of the muscles and urinary tract infections are probably the least serious. However, these patients may also suffer from pneumonia, depression, immobility and attendant disability, some complications because of immobility, such as: bedsores, skin infections or ulcer, as well as some cognitive deterioration and finally respiratory failure and death.
Care in patients diagnosed to be in end stages of this disease usually requires hospice care and symptom management. Opiates may be useful for respiratory distress, lorazepam for choking episodes, while subcutaneous morphine may resolve present dyspnea. Also, some advanced directives may be given from the patient, in order to implement his or her wishes regarding the treatment.