Motor Neuron Disease Flashcards
What is MND
Umbrella term that encompasses a variety of specific diagnoses.
Types of MND
Amylotrophic lateral sclerosis (ALS), progressive bulbar palsy, progressive muscular atrophy, primary lateral sclerosis
Which is the most common type of MND
ALS - 95%
Pathophysiology behind MND
Progressive degeneration of upper and lower motor neurons. Sensory neurons are spared
Signs of lower motor neuron disease
Muscle wasting, reduced tone, fasciculations, reduced reflexes
Signs of upper motor neuron disease
Increased tone or spasticity, brisk reflexes, upgoing plantar responses
Typical presentation of MND
Late middle aged man, with possible affected relative. Insidious progressive weakness of the muscles throughout the body
How variable is MND
Widely as it depends on the region involved - can be cranial, cervical, or bulbar, and whether UMN or LMN
Presentation of ALS
Signs of UMN and LMN lesions, hypertonia, impaired fine movement, hyperreflexia, cramps, fasciculations, weakness and atrophy.
What type of variant is progressive muscular atrophy
Pure LMN variant
What type of variant is primary lateral sclerosis
Pure UMN variant
Pathology on the anatomy in MND
Degeneration of nerves forming corticospinal and corticobulbar tracts, and anterior horn cells in the spinal cord supplying voluntary muscle
How to diagnose MND
Clinical presentation, excluding other conditions which can cause motor neuron symptoms.
Medical management of MND
Riluzole and Edaravone
Overall management of MND
Pain relief, spasticity treatment, enteral feeding, NIV, support groups and palliative care
