Acute bulbar palsy and Motor Neurone disease (MND)

Question 1

What is acute bulbar palsy

Answer 1

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

Question 2

What does the term bulbar mean?

Answer 2

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.

Question 3

Damage to bulbar nerves will affect will result in what deficits? (Thinking generally as what are these cranial nerves involved in)

Answer 3

Speech

Swallowing

Facial muscles

Question 4

Symptoms of acute bulbar palsy reflect what LMN is affected. E.g. damage to cranial nerve IX will result in what symptoms?

Answer 4

Involved in salivation, swallowing and gag reflex

PT: dysphagia, reduced gag reflex

Question 5

What are common signs and symptoms seen in acute bulbar palsy?

Answer 5

Difficulty chewing
Dysphagia
nasal regurgitation
slurred speech
difficulty handling secretions
aspiration of secretions
Dysphonia (altered vocal ability)
Dysarthuria (hard to articulate words)

Question 6

What are some other clinical signs associated with acute bulbar palsy?

Answer 6

Nasal speech (lacks modulation)
Difficulty with consonants
atrophic (wasting) tongue
drooling
weakness of jaw
weakness facial muscles
absent jaw jerk
absent gag reflex

Question 7

How is acute bulbar palsy classified?

Answer 7

Progressive

Non progressive

Question 8

What is progressive bulbar palsy?

Answer 8

more common
affects adults and children

symptoms escalate over time

Question 9

What is non-progressive bulbar palsy?

Answer 9

Does not worsen

Uncommon

Question 10

What is pseudobulbar palsy?

Answer 10

Damage to UPPER MOTOR NEURONES

Question 11

How would you differentiate between pseudobulbar palsy and acute bulbar palsy?

Answer 11

Pseudo -
emotional lability (outbursts of laughing / crying)
Absence of facial emotions
spastic + pointed tongue
exaggerated jaw jerk

Question 12

What are the most common causes of acute bulbar palsy? (2)

Answer 12

Brainstem strokes

Tumour

Question 13

What are some

a) Degenerative
b) Autoimmune
c) Genetic

causes of acute bulbar palsy?

Answer 13

a) Degenerative:
ALS - amyotrophic lateral sclerosis

b) Autoimmune:
Guillain-Barré syndrome

c) Genetic:
Kennedy disease
BVVL syndrome

Question 14

What is the treatment for acute bulbar palsy?

Answer 14

no treatment

supportive treatments

Question 15

Suggest some supportive treatments for acute bulbar palsy?

Answer 15

Drooling - anticholinergic drugs

Dysphagia - feeding tube - NG or PEG

Speech / chewing / swallowing - speech and language therapy

Question 16

What are some investigations for acute bulbar palsy?

Answer 16

CSF analysis (rule out MS that can present similarly)

MRI of brain (stroke / tumour)

Question 17

What are some complications of acute bulbar palsy?

Answer 17

Aspiration pneumonia

Extensive bulbar damage - respiratory centre in brainstem affected

Progressive bulbar palsy can advance to ALS > death of motor neurones > cant breath > death

Question 18

What is Motor Neurone disease

Answer 18

A progressive, degenerative neurological disease with rapid progression and poor prognosis ( av 3 years)

Question 19

Where does the degeneration occur in MND?

Answer 19

Degeneration in both upper and lower motor neurones (anterior horn cells) in spinal cord and brainstem

Question 20

Explain the concept of MND being an umbrella term

Answer 20

Encompasses many specific diagnosis

Question 21

What are the main clincial variants of MND

Answer 21

Amyotrophic Lateral sclerosis (most common / well known (Stephen hawking)

Progressive Bulbar palsy - affects muscles of talking and swallowing

Question 22

Is MND genetic ?

Answer 22

Often sporadic

BUT

inherited as autosomal dominant in 10% of cases

Question 23

Do patients with MND have any sensory, special senses or micturition symptoms?

Answer 23

NO as these pathways are unaffected - only motor

Question 24

Who is our typical pt with MND?

Answer 24

late middle aged e.g. 60 maybe has a relative with MND

male usually = 2:1 ratio (M:F)

Question 25

A pt presents with mixed upper and lower motor neurone symptoms what should you suspect?

Answer 25

MND

Question 26

Compare signs of an UMN and LMN

Answer 26

UMN:
Increased tone / spasticity
Brisk reflexes
Upgoing plantars

LMN:
Muscle wasting
reduced tone
Fasiculations
reduced refleces

Question 27

How is a diagnosis of MND made?

Answer 27

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

Question 28

What is the treatment for MND?

Answer 28

Incurable

Riluzole (glutamate inhibitor) - slow rate of progression

Symptom treatment:
- Respiratory care: Non invasive ventilation e.g. BIPAP

• Nutrition: PEG

Question 29

What are dangerous complications of MND?

Answer 29

dysphagia, respiratory failure and pneumonia

Question 30

Neuro workbook: describe precisely examination findings of a pt with mixed UMN and LMN signs in MND

Answer 30

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

Question 31

What are differentials for MND?

Answer 31

cervical cord compression and myelopathy

Question 32

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?

Answer 32

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)