Neuromuscular Disorders Flashcards
What is motor neuron disease?
Progressive degeneration of motor neurons, leading to muscle wasting and weakness
What is the average survival time for a patient with MND?
3 years
90% of MND involves lower motor neuron. True/False?
True
What is the most common phenotype of MND?
Amyotrophic lateral sclerosis (ALS)
What is the main difference between ALS and PLS?
ALS has both UMN and LMN features, PLS UMN only
ALS is associated with which type of dementia?
Frontotemporal dementia
List investigations for MND
Clinical diagnosis (onset: bulbar, chest etc.) Neurophysiology (EMG)
Outline management of MND
Assess needs and organise care
Speech therapy
Nutrition support
Ventilatory support
What is the classic sign of MND in the hands?
Split hand syndrome (preferential wasting of thenar group in the hand)
MND causes does not cause sensory problems. True or false?
True
Describe the basic pattern of symptoms in ALS
Both UMN and LMN features that starts in one limb and spread to other limbs and trunk
Progressive bulbar palsy involves which cranial nerves?
CN IX, X & XII
Progressive muscular atrophy has purely _____ motor neuron features
Lower
PLS has purely _____ motor neuron features
Upper
Botulism causes what symptoms?
Rapid onset weakness without sensory loss