Motor Neurone Disease Flashcards
Upper motor neurone signs indicate the lesion is where?
Indicates the lesion is above the anterior horn cell.
Lower motor neurone signs indicate the lesion is where?
Indicates the lesion is on the anterior horn cell or distal to the anterior horn cell.
What are some upper motor neurone signs?
Increased muscle tone (spasticity)
Weakness (Weakness of extensors in arms and flexors in legs) (pyramidal pattern of weakness)
Increased reflexes.
Up going planters.
Lower motor neurone signs?
Decreased muscle tone, weakness, wasting (atrophy) in the muscles.
Fasciculations.
Absent / reduced reflexes.
Presentation of MND?
Primary symptom is muscle weakness.
Most commonly affects the limbs first, upper limb being more common.
Majority (90%) start as lower motor neurone degeneration
How can you distinguish MND from other neurological conditions?
It does not affect the eyes (Mysathenia)
No sensory loss or sphincter dysfunction (MS and neuropathies)
What is the most common type of MND?
Amytrophic lateral sclerosis
How does AML present?
Mixed UMN and LMN signs.
Predominantly starts in the hands before spreading to affect all the limbs and bulbar/thorax muscles.
What is a key feature of a pseudo bulbar palsy?
Emotional lability.
How do progressive bulbar and pseudo bulbar palsy’s present?
Progressive bulbar weakness and subsequent limb involvement.
Drooling, dysphasia, dysarthria, aspiration pneumonia. Emotional lability (if pseudobulbar)
Progressive spinal muscle atrophy presents with only what signs?
Only lower motor neurone signs.
Caused by selective loss of anterior horn cells.
Primary lateral sclerosis is associated with loss of cells where?
Loss of Benz cells in the motor cortex.
Primarily affects the limbs and presents with marked spasticity, pseudobulbar palsy, and NO cognitive decline.
What drug is helpful for spasticity in MND?
Baclofen
What drug is used to prolong life in MND?
Riluzole: anti-glutamine drug that prolongs life of MND patients for 3 months. Not all patients want it.
What is Kennedy’s disease?
X linked genetic disorder that may be misdiagnosed as MND.
Associated with lower motor neurone limb and bulbar weakness and erectile dysfunction, infertility, gynaecomastia and atrophic testes.
