MOTOR NEURON DISEASE Flashcards
(11 cards)
define motor neuron disease
describes a group of rare, fatal, neurodegenerative disorders. They are devastating conditions that cause the loss of the upper and/or lower motor neurons, leading to a variety of progressive neurological symptoms that can develop over months to years.” (Knight, 2020)
prevalence/incidence
als being the most common variant
male to female ratio is 1:1
median age of onset of 55
cases and risk factors of MND
- genetic causes
- environmental factors (predisposing the development)
classification of MND
- inherited - autosomal dominant, autosomal recessive, x-linked inheritance
- sporadic
- affect upper or lower motor neurons or both
pathomechanics of MND
symptoms of MND
- early symptoms
- limb-onset
- bulbar-onset
- respiratory-onset - advanced
- muscular
- speaking and swallowing difficulties
- saliva problems
- excessive yawning
- emotional changes
- changes to cognitive abilities
- breathing difficulties - end stage symptoms
- increasing body paralysis
- significant shortness o breath - secondary
- depression
- insomnia
- anxiety
types of MND
- Amyotrophic lateral sclerosis (ALS)
- Progressive bulbar palsy (PBP)
- Progressive muscular atrophy (PMA)
- Primary lateral sclerosis (PLS)
- Spinal muscular atrophy
- Kennedy’s disease
- Post-polio Syndrome
Classification of spinal muscle atrophy
SMA type 0 - 4
- earlier the symptoms appear, the greater the impact on the motor function
assessment of MND
- no specific tests to diagnose
- gene tests to know the type
following tests for extension
- electromyography
- nerve conduction study done with EMG
- laboratory tests
- magnetic resonance imaging (MRI)
- magnetic resonance spectroscopy
management of MND
- no cure or standard treatment
- riluzole
- edaravone
- nusinersen
- Onasemnogeme abeparovec-xioi (Zolgensmat™) gene therapy
- muscle relaxers
slp management for mnd
- overarticulation
- supportive therapies
- non-invasive ventilatory support
