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pseudobulbar palsy (spastic bulbar palsy, supranuclear palsy)
A syndrome characterized by involuntary laughter & crying. Emotional expression may be at variance with mood.
Pathology:
1) bilateral lesions in the descending corticobulbar tracts [2]
-> generally secondary to vascular disease (stroke)
2) lesions in the frontothalamopontomedullary tract may affect emotional lability [3]
Clinical manifestations:
1) involuntary laughter & crying (emotional disinhibition)
2) emotional expression may be at variance with their mood [2]
3) spastic pseudobulbar paralysis [2,3]
a) dysarthria
b) dysphagia
c) dysphonia
d) increased gag response
4) bilateral facial weakness
a) lower face & tongue weakness
b) extraocular, upper facial, pharyngeal & jaw muscles are generally spared
5) upper motor neuron signs
a) spasticity, unless acute
b) incoordination
c) dysdiadochokinesis
7) Cheyne-Stokes respirations may be observed [3]
8) often occurs in the setting of multiple strokes
Differential diagnosis:
1) amyotrophic lateral sclerosis
2) multiple sclerosis
3) later stages of vascular dementia
4) traumatic brain injury
5) brain stem or skull base tumor
Management:
- dextromethorphan 20-30 mg plus 10 mg quinidine BID [4]
Related
bulbar paralysis (palsy)
corticobulbar tract
emotional lability; asthenic disorder
General
syndrome
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 108, 2318
- 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
- Principles of Neurology, 4th edition, Adams & Victor, McGraw-
Hill, New York, 1989
- Pioro EP et al.
Dextromethorphan plus ultra low-dose quinidine reduces
pseudobulbar affect.
Ann Neurol 2010 Nov; 68:693.
PMID: 20839238