Motor Neuron Disease Flashcards
What is motor neuron disease?
Untreatable and rapidly progressive neurodegenerative condition
UMN/LMN signs without sensory problems
How does MND present?
Muscle weakness and potentially problems with speech, swallow and breathing
Focal onset and continuous spread, finally generalised paresis
Where is the usual site of onset of MND?
Extremities 70% (upper>lower)
Bulbar 25%
Thoracic 2%
What is the split in MND between UMN and LMN?
UMN 10%
LMN 90%
What percemtage of MND cases are familial?
10% (90% sporadic)
Describe the bulbar variant of MND
F>M
60-80yo
Always generalised into ALS
What therapeutic interventions occur for bulbar MND?
Early communicator
Nutritional support
Care for URT
What are the therapeutic interventions for thoracic MND?
Early non-invasive intubation with compensation for years, but increasing risk of reflux, aspiration, pneumonia
Describe the ALS variant of MND
Flail Arm Syndrome Flail Leg Syndrome Primary lateral sclerosis Focal distal spinal muscular dystrophy Kennedy's disease (SMA variant)
What dementia can present with MND?
Frontotemporal
What is included in the DDx of MND?
Classic MND (or ALS) Progressive Muscular Atrophy Primary lateral sclerosis Syndromes of MND and frontotemporal dementia Progressive bulbar palsy Flail limb
What UMN signs may you see in MND?
Spasticity
Babinski sign
Bulbar UMN signs: exaggerated snout reflex, clonic jaw jerk, emotional lability, forced yawning
Cervical/lumbar: clonic deep tendon reflexes, preserved reflex in weak wasted limb, Hoffmann reflex, hyperreflexia
Abdominal: loss of superficial abdominal reflexes
What is included in the on-going management of MND?
Access to specialist MND services Key worker assessing needs and coordinates care Communication needs Nutritional needs Respiratory needs
