Acute bulbar palsy and Motor Neurone disease (MND) Flashcards
What is acute bulbar palsy
Set of signs and symptoms related to the dysfunction of lower cranial nerves.
Damage can be to the LMN or the cranial nerve itself.
These nerve arise from brainstem: IX, X, XI, XII
What does the term bulbar mean?
Group of muscles in the head and neck involved in speaking, swallowing, chewing, and holding the jaw in place.
The nerves that control these muscles are found in the bulbar region of the brainstem.
Damage to bulbar nerves will affect will result in what deficits? (Thinking generally as what are these cranial nerves involved in)
Speech
Swallowing
Facial muscles
Symptoms of acute bulbar palsy reflect what LMN is affected. E.g. damage to cranial nerve IX will result in what symptoms?
Involved in salivation, swallowing and gag reflex
PT: dysphagia, reduced gag reflex
What are common signs and symptoms seen in acute bulbar palsy?
Difficulty chewing
Dysphagia
nasal regurgitation
slurred speech
difficulty handling secretions
aspiration of secretions
Dysphonia (altered vocal ability)
Dysarthuria (hard to articulate words)
What are some other clinical signs associated with acute bulbar palsy?
Nasal speech (lacks modulation)
Difficulty with consonants
atrophic (wasting) tongue
drooling
weakness of jaw
weakness facial muscles
absent jaw jerk
absent gag reflex
How is acute bulbar palsy classified?
Progressive
Non progressive
What is progressive bulbar palsy?
more common
affects adults and children
symptoms escalate over time
What is non-progressive bulbar palsy?
Does not worsen
Uncommon
What is pseudobulbar palsy?
Damage to UPPER MOTOR NEURONES
How would you differentiate between pseudobulbar palsy and acute bulbar palsy?
Pseudo -
emotional lability (outbursts of laughing / crying)
Absence of facial emotions
spastic + pointed tongue
exaggerated jaw jerk
What are the most common causes of acute bulbar palsy? (2)
Brainstem strokes
Tumour
What are some
a) Degenerative
b) Autoimmune
c) Genetic
causes of acute bulbar palsy?
a) Degenerative:
ALS - amyotrophic lateral sclerosis
b) Autoimmune:
Guillain-Barré syndrome
c) Genetic:
Kennedy disease
BVVL syndrome
What is the treatment for acute bulbar palsy?
no treatment
supportive treatments
Suggest some supportive treatments for acute bulbar palsy?
Drooling - anticholinergic drugs
Dysphagia - feeding tube - NG or PEG
Speech / chewing / swallowing - speech and language therapy
What are some investigations for acute bulbar palsy?
CSF analysis (rule out MS that can present similarly)
MRI of brain (stroke / tumour)
What are some complications of acute bulbar palsy?
Aspiration pneumonia
Extensive bulbar damage - respiratory centre in brainstem affected
Progressive bulbar palsy can advance to ALS > death of motor neurones > cant breath > death
What is Motor Neurone disease
A progressive, degenerative neurological disease with rapid progression and poor prognosis ( av 3 years)
Where does the degeneration occur in MND?
Degeneration in both upper and lower motor neurones (anterior horn cells) in spinal cord and brainstem
Explain the concept of MND being an umbrella term
Encompasses many specific diagnosis
What are the main clincial variants of MND
Amyotrophic Lateral sclerosis (most common / well known (Stephen hawking)
Progressive Bulbar palsy - affects muscles of talking and swallowing
Is MND genetic ?
Often sporadic
BUT
inherited as autosomal dominant in 10% of cases
Do patients with MND have any sensory, special senses or micturition symptoms?
NO as these pathways are unaffected - only motor
Who is our typical pt with MND?
late middle aged e.g. 60 maybe has a relative with MND
male usually = 2:1 ratio (M:F)
A pt presents with mixed upper and lower motor neurone symptoms what should you suspect?
MND
Compare signs of an UMN and LMN
UMN:
Increased tone / spasticity
Brisk reflexes
Upgoing plantars
LMN:
Muscle wasting
reduced tone
Fasiculations
reduced refleces
How is a diagnosis of MND made?
Clinical diagnosis - needs to be made by a specialist
Electromyography (EMG) : reduced number of action potentials with increased amplitude.
TO rule out:
nerve conduction: normal motor conduction to exclude a neuropathy.
MRI exclude cervical cord compression and myelopathy
What is the treatment for MND?
Incurable
Riluzole (glutamate inhibitor) - slow rate of progression
Symptom treatment:
- Respiratory care: Non invasive ventilation e.g. BIPAP
- Nutrition: PEG
What are dangerous complications of MND?
dysphagia, respiratory failure and pneumonia
Neuro workbook: describe precisely examination findings of a pt with mixed UMN and LMN signs in MND
Mixed:
Wasting and fasciculation in muscles at the same location of brisk reflexes
MND - bulbar palsy:
tongue wasting and fasiculating (bag of worms) but as same time tongue is slow, stiff and spastic +/- a positive jaw jerk
What are differentials for MND?
cervical cord compression and myelopathy
A 63-year-old man is seen in the neurology clinic with a 4-month history of recurrent falls and feeling clumsy. His only history is gastroenteritis 6 months ago.
He has significant leg muscle atrophy and reduced power in both the upper and lower limbs which are more severe on the right. Spontaneous involuntary muscle contractions and relaxations are seen in the upper and lower limbs. Reflexes are absent in the upper limb but brisk in the lower limb. Sensation and coordination are intact. Upgoing plantar reflexes are present bilaterally.
What is the most likely diagnosis?
Motor Neurone Disease - Amyotrophic lateral sclerosis (ALS)
- ‘Fasiculations’ think MND
- motor symptoms, fasiculations, UMN and LMN signs and few sensory - think MND
- ALS: LMN signs in arms and UMN in legs and asymmetry (.i.e. worse on one side is charachteristic for ALS)
