MOTOR NEURON DISEASE

• Progressive disorder of unknown cause in which

there is degeneration of motor neurons
• Spinal cord & Cranial nerve nuclei
• Pyramidal neurons in the motor cortex.

•The prevalence of the disease is about 5/100 000.

• death of lower motor neurons
(consisting of anterior horn cells in the spinal cord
and their brainstem homologues innervating bulbar
muscles)
• upper or corticospinal, motor neurons
(originating in layer five of the motor cortex and
descending via the pyramidal tract to synapse with
lower motor neurons, either directly or indirectly via
interneurons)
MOTOR NEURON DISEASE
 MOTOR NEURON DISEASE

5%- familial 95%

• autosomal dominant inheritance. • viral infection
• genetic defect lies on chromosome 21 poliomyelitis
• superoxide dismutase (SOD1). herpes zoster
• trauma
• exposure to toxins
lead, aluminum
• electric shock
Clinical features

• Combination of lower and upper motor neuron signs

• without sensory involvement.
• The presence of brisk reflexes in wasted fasciculating

limb muscles is typical.

Onset
• Usually after the age of 50 years
• Very uncommon before the age of 30 years
• Males > Females
Course
Symptoms often begin focally in one part and spread
gradually but relentlessly to become widespread
Symptoms
• Limb muscle weakness, cramps, occasionally
fasciculation
• Disturbance of speech/swallowing
(dysarthria/dysphagia)
Signs
• Wasting and fasciculation of muscles
• Weakness of muscles of limbs, tongue, face, palate
• Pyramidal tract involvement causes spasticity,
exaggerated tendon reflexes, extensor plantar
responses
• External ocular muscles and sphincters usually
remain intact
• No objective sensory deficit
• No intellectual impairment
PATTERNS OF INVOLVEMENT OF MND
Amyotrophic lateral sclerosis
• Pyramidal tract features may predominate
• Combination of distal and proximal muscle-wasting
and weakness, fasciculation
• Spasticity, exaggerated reflexes, extensor plantars
• Bulbar and pseudobulbar palsy follow eventually
Progressive muscular atrophy
• Predominantly spinal motor neurons affected
• Weakness and wasting of distal limb muscles at first
• Fasciculation in muscles
• Tendon reflexes may be absent
Progressive bulbar palsy
• Early involvement of tongue, palate and pharyngeal
muscles
• Dysarthria/dysphagia
• Wasting and fasciculation of tongue
• May be pyramidal signs as well

• Primary lateral scelrosis

• Familial spastic paraplegia
Investigations
• Clinical features are highly suggestive
• EMG to confirm the presence of fasciculation and
denervation
• Sensory nerve conduction and motor conduction
studies are normal
• Spinal & brain scan: to exclude focal spinal or
cerebral disease.
• CSF examination- usually normal
Management
RILUZOLE
• The glutamate antagonist

• 100 mg per day

• Prolongs survival for patients by about 2 months.

• Ceftriaxone- may augment astroglial glutamate

transport
Management

• Psychological and physical support

• Occupationals/ peech therapists/ physiotherapists

• Mechanical aids- splints, walking aids, wheelchairs

and communication devices

• Feeding by percutaneous gastrostomy

• Non-invasive ventilatory support

• Use of opiates and sedative drugs

Prognosis
• Progressive
• mean time from diagnosis to death is 1 year
• Younger patients and those with early bulbar
symptoms tend to show a more rapid course.
• Death is usually from respiratory infection and
failure, and the complications of immobility.
END