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Pseudobulbar palsy

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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84 Pseudobulbar palsy

Instruction

Examine this patient’s cranial nerves.

Salient features

History

• Ask the patient whether there is difficulty in swallowing or nasal regurgitation

• Any changes in speech

• Emotional lability

• History of stroke, multiple sclerosis or motor neuron disease.

Examination

• Spastic tongue

• Donald Duck speech

• Patient is emotionally labile (uncontrollable laughter, crying, irritability or anger)

• Sluggish movements of the palate when the patient is asked to say ‘aah’.

Proceed as follows:

• Check the jaw jerk

• Tell the examiner that you would like to do the following:

• Check the gag reflex
• Look for upper motor neuron signs in the limbs.

Diagnosis

This patient has pseudobulbar palsy (lesion) caused by a stroke (aetiology); he has difficulty in swallowing and emotional lability (functional status).

Questions

What could be the underlying cause?

• Bilateral stroke

• Multiple sclerosis

• Motor neuron disease.

Advanced-level questions

How would you differentiate bulbar palsy from pseudobulbar palsy?

  Pseudobulbar palsy Bulbar palsy
Prevalence Common Rare
Type of lesion Upper motor neuron Lower motor neuron, muscular
Site of lesion Bilateral, usually in the internal capsule Medulla oblongata
Tongue Small, stiff and spastic Flaccid, fasciculations
Speech Slow, thick and indistinct Nasal twang
Nasal regurgitation Not prominent Prominent
Jaw jerk Brisk Normal or absent
Other findings Upper motor neuron lesions of the limbs Lower motor neuron lesions of the limbs
Effect Emotionally labile Normal effect
Causes Stroke, multiple sclerosis, motor neuron disease, Creutzfeldt–Jakob disease Motor neuron disease, poliomyelitis, Guillain-Barré syndrome, myasthenia gravis myopathy

How would you manage the swallowing and speech difficulties?

The patient would initially require assessment by a speech therapist for the difficulty in swallowing and speech deficits. Barium swallow with videofluoroscopy may be required to ‘visualize’ the swallowing.

Is there any therapy for emotional lability?

One study suggested that therapy with a combination of dextromethorphan and quinidine improves symptoms and quality of life in patients with emotional lability (Neurology 2004:63:1363–70).

What do you understand by the term CADASIL?

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable cause of stroke and vascular dementia in adults. Clinical and neuroimaging features resemble those of sporadic small-artery disease, although patients with CADASIL have an earlier age at onset of stroke events, an increased frequency of migraine with aura and a slightly variable pattern of ischaemic white matter lesions on brain MRI. NOTCH3 (Notch homolog 3), the gene involved in CADASIL, encodes a transmembrane receptor primarily expressed in systemic arterial smooth-muscle cells.

Charles Darwin described pseudobulbar affect in The Expression of Emotions in Man and Animals (1872): ‘certain brain diseases, such as hemiplegia, brain wasting and senile decay, have a special tendency to induce weeping’.

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