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Adult-acquired Neurology - MND

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ness37's version from 2018-04-30 10:06

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DefinitionA group of diseases that affect the motor neurones in either: UMN (cortex and brain stem) or LMN (brain stem and spinal cord) - symptoms depend on type of nerves affected
Types of MNDALS, PBP, PLS, PMA, FTD
ALS Most common, affects LMN and UMN: limbs, bulbar and respiratory muscles, mixed flaccid-spastic dysarthria
PBPAffects LMN, 25% of all cases, flaccid dysarthria and dysphagia
PLSAffects UMN, 1-5% of all cases, early symptoms = lower limbs, spastic dysarthria
PMALMN features, 10% of all cases, progressive limb wasting
FTDRecently recognised, changes in behaviour, personality (language and cognition may precede motor symptoms)
SymptomsMotor, LMN, UMN and cognitive
Motor symptomsLimb weakness, 25% have weakness in bulbar muscles, breathing difficulties
LMN symptomsMuscle cramps, fasciculations (visible and involuntary muscle contractions in the tongue), muscle atrophy, dysphagia and dysarthria
UMN symptomsSpasticity, hyperactive reflexes, spastic dysarthria, dysphagia, hyper-salivation (don't swallow enough so saliva builds up)
Cognitive symptomsNow more recognised symptom. 10% of all cases fulfil criteria for fronto-temporal dementia. Emotional consequences - grief, depression, anxiety, fear, denial, anger
Incidence and prevelenceMore men affected than women, 5% familial, 80% develop symptoms between 40-70 years (peak rate occurrence 60-70%)
PrognosisLife span varies (mean duration is 3 years, PBP has worse prognosis 2 1/2 years) Death is usually due to respiratory failure
MND involvement Get in touch with patient within 4 weeks of diagnosis - helpful to discuss prognosis (AAC involved early). Best seen in a coordinated team = timely referrals.
MedicationOnly 1 licensed drug in UK that prolongs ALS survival by 3 months
Respiratory symptom managementNon-invasive ventilation: improves QoL, prolongs survival, portable/lightweight. Or, tracheostomy: may be considered for prolonged ventilation support
Implications for swallowReduced oral control (tongue mobility, lip closure), nasal regurgitation, weak jaw, pharyngeal residue, delayed swallow trigger = aspiration, weight loss, decreased appetite, fatigue and difficulty taking medication
Dysphagia managementJoint working with dietician, regular review - modified consistencies, education/ information for patient and family
PEGSProlong survival for approx 8 months, procedure can impair respiratory function so RIG is considered (done without sedation and can be sat up)
Implications for speechMixed dysarthria, dysphonia, hyper-nasality/breathy/mono-pitch/mono-loudness, imprecise articulation, reduced speech rate
Assessment for speechTongue and lips (range, strength, endurance, alternate motion), palate (nasalisation of vowels, nasal emission, puff cheeks), vocal quality, breathing, articulation, speech rate, intelligibility)
Causes90-95% no known cause. Possibilities: high level of exercise, familial (20% due to gene mutation)
Management of communicationImpairment therapy not appropriate due to rapid progression, early referral to AAC (voice banking, text to speech devices)
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