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Bulbar and Pseudobulbar Palsy

Synonyms: 'bulbar palsy' - lower motor neurone dysarthria, neuromuscular dysarthria, atrophic bulbar paralysis;
'pseudobulbar palsy' - upper motor neurone dysarthria, spastic dysarthria

The components of normal speech

Speaking is a voluntary task which is taken for granted but is a highly specialised activity. In order to speak, the
following parts of the oral cavity need to be used:
Larynx
Pharynx
Palate
Tongue
Lips
Along with this, controlled expiration is required, so that air can be released at the appropriate speed and in
appropriate amounts.

Neurological control of normal speech

The above structures required for speech are controlled by the nervous system. Corticobulbar tracts from both of
the motor cortices send signals down to the nuclei of the following nerves:
Vagal nerve
Facial nerve
Hypoglossal neve
Phrenic nerve
The motor aspects of speech, like other movements, are also influenced by the extrapyramidal system via the
basal ganglia and the cerebellum. There is ongoing research into which areas of the cortex are involved in
speech and especially in recovery of speech after a stroke. [1]

Phonation and articulation

Speech has two elements: phonation and articulation.

Phonation: the production of sounds, a result of the vocal cords in the larynx.
Articulation: contractions of the muscles of the various other structures involved in speech, ie the
pharynx, palate, tongue and lips. These muscle contractions change the vocal sounds from the larynx,
thus resulting in noises recognised as words.
Larynx - produces vowels and some consonants.
Lips - produce m, b and p.
Lingula - l and t.
Throat and soft palate (guttural) - nk and ng.

Disorders of articulation [2]

This is also called dysarthria or anarthria. There are several causes:
Bulbar palsy
Pseudobulbar palsy

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Cerebellar-ataxic
Hypokinetic
Hyperkinetic
Cerebellar-ataxic, hypokinetic and hyperkinetic dysarthria are discussed elsewhere.

Bulbar palsy
Description
Bulbar relates to the medulla. Bulbar palsy is the result of diseases affecting the lower cranial nerves (VII-XII). A
speech deficit occurs due to paralysis or weakness of the muscles of articulation which are supplied by these
cranial nerves. The causes of this are broadly divided into:
Muscle disorders.
Diseases of the motor nuclei in the medulla and lower pons.
Diseases of the intramedullary nerves of the spinal cord.
Diseases of the peripheral nerves supplying the muscles.
Importantly, these lesions do not affect speech in isolation. The bulbar nerves also innervate muscles involved in
swallowing and facial muscles.
Bulbar palsy is sometimes also classified as non-progressive or progressive. Non-progressive bulbar palsy is an
uncommon condition of uncertain aetiology and there are few reports of it in the literature. [3] Progressive bulbar
palsy can occur in children or adults and form a spectrum of severity, based around the common feature of
bulbar dysfunction and motor neurone degeneration. Genetic abnormalities have been identified in some cases
presenting in childhood. Brown-Vialetto-Van Laere and Fazio-Londe syndromes are the most recent childhood
forms of progressive bulbar palsy to be genetically defined. [4]

Presentation
Lips - tremulous.
Tongue - weak and wasted and sits in the mouth with fasciculations.
Drooling - as saliva collects in the mouth and the patient is unable to swallow (dysphagia).
Absent palatal movements.
Dysphonia - a rasping tone due to vocal cord paralysis; a nasal tone if bilateral palatal paralysis.
Articulation - difficulty pronouncing r; unable to pronounce consonants as dysarthria progresses.
If the pathology progresses then speech becomes slurred and eventually becomes indistinct. There may also be
neurological deficits in the limbs - eg, flaccid tone, weakness with fasciculations.

Aetiology [5]
There is a wide range of causes. The following list is not exclusive:
Diphtheria.
Poliomyelitis .
Motor neurone disease - eg, progressive bulbar palsy (features of pseudobulbar palsy may also be
present).
Syringobulbia.
Cerebrovascular events of the brainstem.
Brainstem tumours.
After radiotherapy for nasopharyngeal carcinoma.
After surgery for acoustic neuroma.
Guillain-Barr syndrome. [6]

Pseudobulbar palsy

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Pseudobulbar palsy
Description
Pseudobulbar palsy results from disease of the corticobulbar tracts. Bilateral tract damage must occur for
clinically evident disease as the muscles are bilaterally innervated.

Presentation
Tongue - paralysed; no wasting initially and no fasciculations; 'Donald Duck' speech; unable to
protrude.
Palatal movements absent.
Dribbling persistently.
Facial muscles - may also be paralysed.
Reflexes - exaggerated (eg, jaw jerk).
Nasal regurgitation may be present.
Dysphonic.
Dysphagic.
Emotional lability may also be present.
There may also be neurological deficits in the limb - eg, increased tone, enhanced reflexes and weakness.

Aetiology [7]
Cerebrovascular events - eg, bilateral internal capsule infarcts.
Demyelinating disorders - eg, multiple sclerosis.
Motor neurone disease.
High brainstem tumours.
Head injury.
Neurosyphilis.
In motor neurone disease it is common to see both bulbar and pseudobulbar palsies.

Investigations
New developments in technology have led to the use of neurophysiological investigations to assess various
aspects of speech dysfunction. [8] These include electromagnetic articulography (EMA), electropalatography
(EPG) and pressure-sensing EPG. Other tests will depend on the suspected underlying cause but will involve
routine blood tests, imaging of the brain and brainstem (either CT scan or MRI) and electromyography.

Management [9]
All patients should be referred to neurologists. Patients will need admission if dysphagia is present or
symptoms are rapidly progressive.
Treatment will be directed to the underlying cause.
Postural changes can help with drooling of saliva and may prevent aspiration.
Supportive measures may include baclofen for spasticity, anticholinergics for drooling, treatment of
aspiration pneumonia if it occurs and attention to nutrition - eg, enteral feeding. [10]
Management should involve speech and language therapists and the dietician.
Genetic analysis may be appropriate for cases presenting in childhood. [4]

Complications
Poor dentition.
Poor nutrition.
Psychological dysfunction.
Progression of underlying disease.

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Prognosis
This depends on the underlying cause.

Further reading & references

Basiri K, Ansari B, Okhovat AA; Life-threatening misdiagnosis of bulbar onset myasthenia gravis as a motor neuron
disease: How much can one rely on exaggerated deep tendon reflexes. Adv Biomed Res. 2015 Feb 23;4:58. doi:
10.4103/2277-9175.151874. eCollection 2015.
1. Thiel A, Hartmann A, Rubi-Fessen I, et al ; Effects of noninvasive brain stimulation on language networks and recovery in
early poststroke aphasia. Stroke. 2013 Aug;44(8):2240-6. doi: 10.1161/STROKEAHA.111.000574. Epub 2013 Jun 27.
2. McCaffrey P; Neuropathologies of Swallowing and Speech, Chapter 12. Types of Dysarthria: Upper Motor Neuron Damage.
3. Worster-Drought C; Proceedings of the Royal Society of Medicine, 1927.
4. Manole A, Fratta P, Houlden H; Recent advances in bulbar syndromes: genetic causes and disease mechanisms. Curr
Opin Neurol. 2014 Oct;27(5):506-14. doi: 10.1097/WCO.0000000000000133.
5. Neurology, ophthalmology and psychiatry; Royal College of Physicians, 2008.
6. Kumar M, Aroor S, Mundkur S, et al; Guillain-barre syndrome: a clinical study of twenty children. J Clin Diagn Res. 2015
Jan;9(1):SC09-12. doi: 10.7860/JCDR/2015/8344.5491. Epub 2015 Jan 1.
7. Kumar P et al; Kumar and Clark's Clinical Medicine, 2012.
8. Murdoch BE; Physiological investigation of dysarthria: recent advances. Int J Speech Lang Pathol. 2011 Feb;13(1):28-35.
doi: 10.3109/17549507.2010.487919.
9. Barber C et al; Managing Bulbar Symptoms In ACommunity Patient With MND, Royal College of Nursing
10. Kindsley L et al; Amyotrophic Lateral Sclerosis Overview, Gene Reviews, 2001 (updated 2009).

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical
conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its
accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions.
For details see our conditions.
Original Author:
Dr Gurvinder Rull

Current Version:
Dr Laurence Knott

Peer Reviewer:
Prof Cathy Jackson

Document ID:
1721 (v23)

Last Checked:
31/07/2015

Next Review:
29/07/2020

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