Motor Neuron disease

Dr. Nabin Simkhada

• Irreversible loss of motor neuron and gliosis
o Motor cortex,
o Motor nuclei of brainstem
o Anterior horn of spinal cord

Chronic and progressive in nature

ALS (Lou Gehrig’s disease)
• Neurodegenerative disorder
• Both UMN and LMN features
• Majority –sporadic (no family history)
• Familial cases- AD pattern of inheritance
• Missense mutation of SOD1 gene (20% of ALS)
Multiple cellular mechanism converge to
cause motor neuron dysfunction and death
Morphological features
• Reactive gliosis
• In UMNs, Pyramidal and betz cells are lost Wallerian degeneration of
Coticospinal and corticobulbar cells reactive astrocytic gliosis

• Thinning of anterior nerve roots

• Due to progressive degeneration of anterior horn cells

• Bunina bodies ( PAS+ eosinophilic intracytoplasmic inclusions in

spared LMNS)
Symptoms
Diagnosis
ALS is fast progressing
Treatment
• No cure for MND, so treatment focus on relieving the symptoms,
slowing the progression, and maximizing the patient’s independence
and comfort.
• This can include the use of breathing, feeding, mobility and
communication appliances and devices.
• Rehabilitation therapy may include physical, occupational and speech
therapy.
Approved products for ALS
Prognosis