Motor neuron disease / Anterior horn cell disease (Basic)

In fornt of patient, use the terminology, anterior horn cell disease

Types

• Amyotrophic Lateral Sclerosis(ALS) = UMNL+LMNL    ( most common presentation)
• UMNL= upper motor meuron lesion, LMNL = lower motor neuron lesion
• Primary Lateral Scerosis = UMNL
• Progressive Muscular Atrophy= LMNL
• Bulbar palsy

ALS= UMNL+LMNL @same limb

(but, Predominant LMNL= Upperlimb+ predominant UMNL=Lower Limb) 

• LMNL= Muscle WASTING + Weakness + FASCICULATION
• UMNL=Tone↑+ ↑ refelex + Extensor plantar

D/D :

1.        Cervical myelopathy(CM)

2.        Syringomyelia (SM)

3.        Multiple Sclerosis(MS)

Investigations:

1.       Nerve conduction study+ EMG

2.       MRI Brain And Cervical cord

Nerve conduction study= Normal in MND,(D/D =  Multifocal Motor Neuropathy/ MMN, it is delayed/reduced)

MRI Brain and spinal cord = to exclude demyelination + cervical myelopathy ( D/D = CM, SM, MS)

(If, demyelination, then it is MS, so go for CSF study)

Rx:

1.       Riluzole = Anti Glutamate , prolong life expectancy 3 months

2.       NIV (BIPAP) = prolong life expectancy 7 months

Prognosis: 

Poor, 50% die within 3 years