Motor neurone disease Flashcards
Prognosis MND
2-3 year live after diagnosis
Risk facotrs MND
Genetic
Life style and environemnt:
Mechanical/electrical trauma
Military service
High levels of exercise
Exposure to agricultural chemicls
Exposure to variety of heavy metals
MND presentation
Mixed UMN+LMN signs WITHOUT sensory symptoms
Clinical features of MND
Asymmetric weakness + loss of muscle bulk + hyperreflexia + extensor plantars
Start in one limb eg hand wasting, foot drop Possible bulbar onset - dysarthria + swallowing problems
Eye movements and sphincter function preserved
Examination findings MND
Fasciculation multiple sites - resting tongue or stiff slow tongue
Gnereal asymmetrical progressive weakness
No sensory findings
Brisk reflexes, wasting of muscles
High stepping gait of foot drop
UMN signs
increased tone, clonus, reflexes (brisk), extensor plantars
History qs MND
- Muscle wasting
- Pain/discomfort
- Emotional lability
- breathing problems
- Weakness inlc foot drop
- Speech and swallowin issues
Drooling
Cramping
Morning headaches
Behaviour change - bulbar
Differentials for MND
Cervical cord compression - sensory
Myasthenia gravis - limb weakness on repetition, speach + swallowing
Fatigue
No fasciculation/UMN
Benign fasciculation syndrome
- NO EMG denervation
- Worsened in stress
- Healthcare worker
Bloods in MND
FBC, TFTs, U+Es, LFTs, bone profile, aa screen, CK (inflam myositis)
Malginancy, hyperPTHism
Investigations for MND
Bloods
CXR for underlying malignancy
Nerve conduction test
#EMG
TMS - Transcranial magnetic stimulation
MRI
Types of MND
ALS
Bulbar onset/PSP
Progressive muscular atrophy
Primary lateral sclerosis
Kennedys/spinobulbar palsy - not an MND
What would weakness/clumsy hands suggest about MND type
Progressive muscuar artrophy/PMA
Primary lateral sclerosis features
- Mainly effects lower limbs
- Usually not life shortening (v slow progression
EMG shows in MDN
Reduced APS of increased amplitude
