pseudobulbar palsy (spastic bulbar palsy, supranuclear palsy)
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Introduction
A syndrome characterized by involuntary laughter & crying. Emotional expression may be at variance with mood.
Pathology
- bilateral lesions in the descending corticobulbar tracts[2]
- generally secondary to vascular disease (stroke)
- lesions in the frontothalamopontomedullary tract may affect emotional lability[3]
Clinical manifestations
- involuntary laughter & crying (emotional disinhibition)
- emotional expression may be at variance with their mood[2]
- spastic pseudobulbar paralysis[2][3]
- dysarthria
- dysphagia
- dysphonia
- increased gag response
- bilateral facial weakness
- lower face & tongue weakness
- extraocular, upper facial, pharyngeal & jaw muscles are generally spared
- upper motor neuron signs
- spasticity, unless acute
- incoordination
- dysdiadochokinesis
- Cheyne-Stokes respirations may be observed[3]
- often occurs in the setting of multiple strokes
Differential diagnosis
- amyotrophic lateral sclerosis
- multiple sclerosis
- later stages of vascular dementia
- traumatic brain injury
- brain stem or skull base tumor
Management
- dextromethorphan 20-30 mg plus 10 mg quinidine BID[4]
More general terms
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 108, 2318
- ↑ 2.0 2.1 2.2 2.3 Jordan & Cummings, Mental status and neurologic examination in the elderly In: Principles of Geriatric Medicine, 4th ed, Hazzard et al (eds), McGraw-Hill, NY, 1999, 1211
- ↑ 3.0 3.1 3.2 3.3 Principles of Neurology, 4th edition, Adams & Victor, McGraw- Hill, New York, 1989
- ↑ 4.0 4.1 Pioro EP et al. Dextromethorphan plus ultra low-dose quinidine reduces pseudobulbar affect. Ann Neurol 2010 Nov; 68:693. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20839238