MOTOR NEURON

DISEASE
The motor neurone diseases (or motor neuron
diseases) (MND)
• are a group of neurological disorders that
selectively affect motor neurones, the cells that
control voluntary muscle activity including
speaking, walking, breathing, swallowing and
general movement of the body.
CLASSIFICATION
Forms of motor neurone disease include:
•amyotrophic lateral sclerosis (ALS)
(sometimes called Lou Gehrig's disease)
•primary lateral sclerosis (PLS)
•progressive muscular atrophy (PMA)
•Bulbar:
pseudobulbar palsy - spastic
progressive bulbar palsy - spastic and
flaccid
•Spinal muscular atrophy
Amyotrophic lateral sclerosis (ALS)

•ALS is a progressive, fatal, neurodegenerative disease

caused by the degeneration of motor neurons, the nerve cells
in the central nervous system that control voluntary muscle
movement.

•The disorder causes muscle weakness and atrophy

throughout the body as both the upper and
lower motor neurons degenerate, ceasing to send messages to
muscles.

•Cognitive function is generally spared except in certain

situations such as when ALS is associated with
frontotemporal dementia.

• Sensory nerves and the autonomic nervous system, which

controls functions like sweating, generally remain functional.
Signs and symptoms

The earliest symptoms are

• weakness and/or muscle atrophy arm or a leg
•Twitching
•cramping, or stiffness arm or a leg
• slurred and nasal speech.
•dysphagia
•Dysarthria
•Spasticity
•hyperreflexia including an overactive gag reflex.
•Babinski's sign +
•Fasciculations
•pseudobulbar affect, also known as "emotional lability“
(uncontrollable laughter, crying or smiling)
 CAUSES

•not known, and scientists do not yet know why

ALS strikes some people and not others
•ALS research scientists are looking at a
number of possible ALS causes, including:
 
Genetics
Glutamate
Autoimmune responses
Environment.
Primary lateral sclerosis (PLS)

•is a rare neuromuscular disease

•characterized by progressive muscle
weakness in the voluntary muscle
•It affects upper motor neurons only
Symptoms and progression

•Onset of PLS usually occurs after age 50

•difficulty with balance
•weakness and stiffness in the legs
•Clumsiness
•spasticity in the hands, feet, or legs
•foot dragging
•speech problems
•The disorder usually begins in the legs,
but it may also start in the tongue or the
hands
Progressive muscular atrophy (PMA)
is a rare subtype of amyotrophic lateral sclerosis
(ALS) or motor neurone disease (MND) which
affects only the lower motor neurones.

Symptoms

•atrophy
•fasciculations
•muscle weakness
Some patients have symptoms restricted only to the arms or
legs (or in some cases just one of either). These cases are
referred to as "Flail Arm" (FA) or "Flail Leg" (FL) and are
associated with a better prognosis.
Bulbar:
•pseudobulbar palsy - spastic
•progressive bulbar palsy - spastic and flaccid

Pseudobulbar palsy results from an

upper motor neuron lesion to the corticobulbar
pathways in the pyramidal tract. Patients have
difficulty chewing, swallowing and demonstrate
slurred speech (often initial presentation).
Individuals with pseudobulbar palsy also
demonstrate inappropriate emotional outbursts.
Symptoms

•Dysphagia (difficulty in swallowing)

•Labile affect
•Dysarthria
•Uncontrollable laughing or crying
Signs

• Speech is slow, thick and indistinct

• Gag reflex is normal, exaggerated or absent
• Tongue is small, stiff and spastic
• Jaw jerk is brisk
• There may be upper motor neuron lesion of the limbs.
• Bulbar palsy is a similar disorder but is caused by
lower motor neuron lesions