MOTOR NEURON DISEASE

ULOKO A.R
 INTRODUCTION
 MND results from selective loss of function of lower and
or upper motor neurons controlling the voluntary muscles
of the limbs and bulbar regions
Its used to describe a family of diseases

The differential diagnosis requires clinical and

electrophysiologic classification as to whether the disease
effects the upper or lower neurones or Both
The anatomical differenciation is augmented by:
 age of onset
rate of deterioration and
familial occurance

Sensation and cognition are normal on simple clinical

assessment in MND
SIGNS OF LMN
Muscle wasting
Fasciculations
Flaccid weakness
Tendon reflexes are retained until profound
denervation or fibrous tissue replacement of the
muscle
SIGNS OF UMN
Spasticity
Clonus
Extensor plantar responses
Weakness
Plantar response or clonus could be obscured by coexisting
muscle atrophy

Abdominal reflexes are preserved involving the UMN( in

contrast to sc dx by tumour, compression, or
demyelinating dx)
Sphincteric control and sexual function are preserved
Weakness in the trunk and abdominal wall makes
excretion slow

It is an incurable diseases treatment is aimed at

minimise the sources of disability
 AMYOTROPHIC LATERAL SCLEROSIS
It occurs world wide
Incidence of 1 to 1.5/100,000 population
Prevalence of 4 to 6/100,000
Commoner in males
Incidence increases with increasing age
Unusual before the 5th decade of life
Unknown cause in the sporadic ALS
RISK FACTORS
Exposure to

Pesticides

Insecticides

smoking

service in the military

 PATHOLOGY
LMN are lost from the clinically affected areas of the SC and
brain stem

Surviving neurons show intracytoplasmic inclusions(bumina

bodies)

Proximal axonal accumulations of neutrofilaments(spheriods)

Motor cortex depleted of Betz cells

Pyramidal tract degenerates

Other pop of neurones can degenerate include:
The peripheral sensory neurones

Clarke’s column neurones

Up to 10% may develop dementia frontal lobe

FEATURES
Bulbar or spinal symptoms evident as disease progresses

Bulbar form:
Dysphagia
Dysphonia
Inhalation of food due to weakness of the tongue,
pharynx,and larynx
Tongue is wasted, weak, and fasciculating palatal
movement are reduced and inability to cough explosively
due to vocal cord palsy

This palsy usually accompanies or preceeded by various

degrees of pseudobulbar involvement

Spasticity of the tongue, immobile with ‘hot potatoe’ speech

Difficulty in inhibiting emotional responses
Respiratory failure due to weaken diaphram and
intercoastal muscles

Spinal form:
Wasting and weakness of one limb
As intrinsic hand muscle wasting or foot drop
occassionally of the shoulder girdle

It is impt to demonstrate umn and lmn signs in atleast 2

limbs
With time the limbs are useless from progressive
denervation

Decubitus ulcers are uncommon as a result of unaffectation

the autonomic regulation of the skin blood flow and
secretion
INVESTIGATION
Electrophysiological investigation to confirm
denervation and exclude other treatable myopathy or
demyelinating neuropathy
TREATMENT
No treatment

Antiglutamate-riluzole reduces tracheostomy free

survival 18 months by 35%

Multidisciplinary management
Dysphagia- Ng tube feeding
Respiratory muscle weakness- oxygen
Limb spasticity- drugs
Inadequate limb function- wheelchair
Communication devices for incomprehensive speech
Depression
 Modification of the enviroment
 PROGNOSIS
Relentless both in severity and extent of muscle
involvement
Death from respiratory complications

Median survival from first symptoms with bulbar onset

about 20mth with only 5% surviving 5 years
Thank you