Motor Neurone disease- from cheese and onion Flashcards
What is motor neurone disease?
A group of diseases characterised by progressive degeneration of the motor neurons in the motor cortex, anterior horn of the spinal cord and cranial nerve nuclei.
How can motor neurone disease be distinguished from MS and other polyneuropathies?
There is no sensory loss of sphincter dysfunction.
How can motor neurone disease by distinguished from myasthenia?
No issues with eye movements in MND.
Where are the motor neurons degenerating in amyotrophic lateral sclerosis?
They degenerate in both the motor cortex and the anterior horn. This means you get UMN signs due to the motor cortex issue and LMN signs due to the issue in the anterior horn.
What is bulbar palsy?
What signs will this present with?
Disease of the nuclei of cranial nerves IX to XII in the medulla.
Signs are of LMN lesions of the tongue and muscles of talking and swallowing e.g. flaccid, fasciculating tongue, jaw jerk is normal or absent, speech is quiet, hoarse or nasal.
Where is the motor neurone degeneration occurring in progressive bulbar palsy?
In cranial nerve nuclei of IX to XII.
Where is the motor neurone degeneration occurring in progressive muscular atrophy? What signs may you get?
This is an anterior horn cell lesion only, so you only get LMN signs. Distal muscle groups will be affected before proximal muscle groups.
Where is the motor neurone degeneration occurring in primary lateral sclerosis?
Loss of Betz cells in the motor cortex meaning you only get UMN signs.
Definitive diagnosis of ALS
Lower + upper motor neurone signs in 3 regions
Probable diagnosis of ALS
Lower + upper motor neurone signs in 2 regions
Probable diagnosis of ALS with lab support
Lower + upper motor neurone signs in 1 region or upper motor neurone signs in more than 1 region
EMG shows acute denervation in > 2 limbs
Possible diagnosis of ALS
Lower + upper motor neurone signs in 1 region
Which drugs may be suitable in supportive treatment of MND?
Riluzole- prolongs life by approximately 3 months.
What may be used for drooling?
Propantheline
or amitrptilline
What techniques can be used for dysphagia?
Blend food
Nasogastric tube?
