Motor neurone disease
Motor neurone disease (MND) is a term used to cover a number of illnesses of the motor neurone: amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), progressive bulbar palsy (PBP) and progressive lateral sclerosis (PLS). MND is the term used internationally while ALS is often used in the United States (where it is also known as Lou Gehrig's disease, after a famous patient) to cover all forms of MND. It was first described by Jean-Martin Charcot, a French neurologist, in 1869 and in France the disease is therefore known as Maladie de Charcot (Charcot's disease).
Signs and symptoms
The onset of symptoms is usually between 40-70 years of age (average 55). The disorder is characterized by the progressive loss of voluntary muscle contraction due to the destruction of nerve cells in the brain and the spinal cord that are responsible for the stimulation of the voluntary muscles.
While the initial symptoms are subtle, the disease causes progressive physical disability. Mental functioning and physical sensation is spared, although many patients exhibit depression as their symptoms worsen.
Diagnosis
The diagnosis is established on both clinical grounds and an electromyography (EMG) examination, which is obligatory to demonstrate the diffuse loss of nervous stimulation of muscles of extremities, face and abdomen.
Clinically, upper motor neuron damage signs (such as spasticity, lively reflexes and Babinski signs) can be found, while the lower motor neurones demonstrate weakness and muscle atrophy. Weakness of bulbar musculature can also be seen (difficulty breathing, swallowing, coughing or speaking).
Neuroimaging examinations are usually performed to rule out alternative causes, such as a mass lesion of upper parts of spinal cord.
Common Neurological Disorders
|
