UKMLA PLAB AMC USMLE Prometric MCQ

Correct Answer: B

Explanation:
This patient was eventually diagnosed with Motor Neurone Disease .Age onset is usually after 50 years, occasionally in 30-50 years group. This is a progressive disorder of unknown cause, in which there is degeneration of spinal and cranial motor neurones and pyramidal neurones in the motor cortex

Motor Neurone Disease (MND) is the name given to a group of diseases in which the nerve cells (neurones) controlling the muscles that enable us to move around, speak, breathe and swallow fail to work normally. With no nerves to activate them, muscles gradually weaken and waste. The patterns of weakness vary from person to person. Motor neurones are classified as upper and lower motor neurones. Upper motor neurones descend from the brain to the brain stem and the spinal cord; lower motor neurones extend from the brain stem and the spinal cord to the muscles

When the upper motor neurones alone are affected the disease has two types: if limb involvement is predominant the disease is called progressive lateral sclerosis; if brain stem involvement is predominant the disease is called progressive pseudobulbar palsy. When lower motor neurones alone are affected the disease is called progressive muscular atrophy. When both upper and lower motor neurones are involved the disease is called amyotrophic lateral sclerosis. This is the commonest form .

Motor neurone disease is progressive, most patients dying within 3-5 years of the onset of symptoms. Young patients and those with early bulbar symptoms tend to show a more rapid course, wheras older patients and those with mainly peripheral involvement may survive 10 years. Death is usually from respiratory infection & failure and the complications of immobility