Motor Neurone Disease Flashcards
What causes MND?
Selective loss of neurones in motor cortex , cranial nerve nuclei and anterior horn cells
Prognosis with MND?
Average 3 years
Typical onset of MND?
Aged 50-75 years old
Who is MND more common in ?
Males
Feature of motor neurone disease?
UMN and or LMN signs without sensory problems
Muscle weakness -> problems with speech, swallowing, breathing
UMN sings in MND?
Increased tone hyper reflexia Extensors plantar responses - babinski Spastic gait. Exaggerated jaw reflex Slowed movements Hoffman reflex
LMN signs in MND?
Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes
What is split hand syndrome?
Wasting of thenar group - atrophy seen in ALS
Phenotypes of MND?
ALS ALS-frontotemporal dementia Primary lateral sclerosis Progressive muscular atrophy Progressive bulbar palsy
Management of MND?
Special services
MDT
Communication needs
Nutritional needs - dieticians, gastrostomy
Respiratory needs - home ventilation
Riluzole - prolongs life by 3 months
Propantheline/ amitriptyline for drooling
What is riluzole?
An antiglutamatergic drug
Diagnosis of MND?
Clinical criteria
Diagnosis of exclusion
Limb onset, bulbar onset and or cognitive behavioural symptoms
Neuro imaging and lab studies to exclude other diagnoses
What is the diagnostic criteria used for MND?
El Escorial
El Escorial definite criteria?
UMN and LMN in 3 regions
El Escorial probable criteria?
UMN and LMN in 2 regions
El Escorial possible criteria?
UMN and LMN in 1 region
El Escorial suspected criteria?
UMN or LMN in > one region
Which is the most common phenotype of MND?
ALS
Prognosis of ALS?
Poor
Features of ALS?
UMN and LMN features
Prognosis of ALS frontotemporal dementia?
Poor
Features of ALS frontotemporal dementia?
UMN and LMN features
Prognosis of primary lateral sclerosis?
Good : > 5 years
Features of primary lateral sclerosis?
UMN features
Features of progressive muscular atrophy?
LMN features
Prognosis of progressive muscular atrophy?
Variable survival
Features of progressive bulbar palsy ?
Women more than men.
Affects CN IX -XII
effects tongue and muscles of talking and swallowing
