Motor neuron disease (MND), also known as amyotrophic lateral sclerosis (ALS), is a progressive neurodegenerative disorder that affects motor neurons in the brain and spinal cord. It leads to muscle weakness and atrophy due to the degeneration of motor neurons. The majority of cases have no clear cause, though some are genetic. There is currently no cure for MND, but riluzole has been shown to modestly extend survival. Symptomatic treatments can help address problems like excessive saliva or emotional lability. Prognosis is generally poor, as most patients die within 3-5 years of diagnosis due to respiratory failure or other complications.

1. Motor neuron disease MND:ALS Dr. Mohammad Shaikhani.

9. MND (ALS):

14. Clinical features: course Signs Signs Symptoms often begin focally in one part & spread gradually but eventually become widespread

15. Clinical features: involvement patterns Signs Signs 1.Progressive muscular atrophy Predominantly spinal motor neurons affected Weakness / wasting of distal limb muscles at first Fasciculation in muscles Tendon reflexes may be absent

18. DD: Signs Signs

27. TYPES OF SPINAL MUSCULAR ATROPHY Good Facial / bulbar weakness, proximal limb weakness, gynaecomastia X-linked Adult life, males only Bulbospinal Good, seldom disabling Distal weakness and wasting of hands and feet Autosomal dominant Early adult life Distal forms Slowly progressive disability Proximal weakness and wasting, EMG shows denervation Autosomal recessive Childhood, adolescence Kugelberg-Welander Poor Severe muscle-wasting/weakness Autosomal recessive Infancy Werdnig-Hoffmann Prognosis Features Inheritance Onset Type