MND Flashcards
What is the other names for MND?
ALS (amyotrophic lateral sclerosis)
Lou Gehrig disease
What is required for a diagnosis of ALS?
Mixed UMN and LMN signs with an ABSENCE of sensory signs
What is the median survival for MND?
3 years
What is an UMN predominant form of MND?
PLS; primary lateral sclerosis
What is a LMN predominant form of MND?
PMA; progressive muscular atrophy
Which neurodegenerative condition is MND associated with?
FTD
Where can MND begin?
Predominantly spinal/ limbal
Bulbar; speech and swallowing
Cognitive; FTD
Respiratory
Which genes are assoc with MND?
C9orf
SOD1
What are the different forms of motor neurone disease?
ALS PLS Progressive bulbar palsy PMA FTD
Which is the best form of MND in terms of survival?
PLS
Describe an upper motor neurone lesion
Increased tone Hyperreflexia Extensor plantar response Spastic gate Exaggerated jaw jerk Slowed movement Pseudobulbar effect
Describe a lower motor neurone lesion
Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes
What is predominantly affected in a bulbar onset MND?
Wasting, fasciculations of tongue
Facial muscles affected
Pharyngeal muscles affected; difficult phonation
What are the therapeutic interventions utilised in bulbar onset MND?
Early communicator
Nutritional support
Care for URT
Ensure not malnutritioned
What will spinal dysfunction result in?
Muscle wasting
Loss of tone OR contractures
Preferential wasting of thenar group
What interventions can be put into place in the context of spinal dysfunction in MND?
Consider help with washing, dressing, feeding and turning in bed
Mobility aids or hoists required
What investigations are performed for diagnosis of MND?
EMG
What criteria is utilised in the diagnosis of MND?
El Escorial
Diagnosis of MND
Consensus clinical criteria with UmN/LMN signs and absence of sensory symptoms
Diagnosis of exclusion
Limb onset, bulbar onset and/or cognitive
Clinical neurophysiology; el escorial criteria inclusion of EMG findings
Neuroimaging and lab studies to rule out other diseases
What are frequent misdiagnoses of MND?
Carpal tunnel syndrome
Stroke
Neuropathy
What are common false positives for MND?
Multifocal motor neuropathy
Kennedy’s disease
Myopathy (inclusion body myopathy)
Cervical spondylotic radiculomyelopathy
What is the tx for MND?
Riluzole
BiPAP
Gastrotomy; PEG, RIG, PIGG or NG
What are the specific treatments for bulbar MND?
Dysarthria: communication aids
Dysphagia: hydration, nutrition, saliva, gastronomy
What is the association between weight loss and prognosis in MND?
More than 10% weight loss is indicator of mortality within 6 months
What is the treatment for sialorrhoea?
Hyoscine/buscopan
Glycopyrronium
Botox
Suction
What is the treatment for muscle cramps in MND?
Baclofen
Quinine (not commonly used now)
What is the treatment for muscle spasms in MND?
Baclofen Tizanidine Dantrolene Gabapentin Medicinal cannabis
What are the red flag resp signs in MND?
Breathlessness Orthopnoea Recurrent chest infections Disturbed sleep Nightmares Daytime sleepiness Poor concentration Non-refreshed sleep
What is the treatment for SOB/anxiety in MND?
Lorazepam
What is once of the main causes of death in MND?
Weakness of respiratory muscles
