0
Get Alert
Please Wait... Processing your request... Please Wait.
You must sign in to sign-up for alerts.

Please confirm that your email address is correct, so you can successfully receive this alert.

1
Letter   |    
Pseudobulbar Palsy and Affect in a Case of Progressive Multifocal Leukoencephalopathy
Kelly C. Graham; David R. Spiegel
The Journal of Neuropsychiatry and Clinical Neurosciences 2008;20:110-111.

To the Editor: Pseudobulbar palsy, with its neuropsychiatric manifestation of pathological laughing and crying or pseudobulbar affect, has been described in a variety of CNS illnesses.1 We present, with interest, a case of Pseudobulbar palsy in a patient with progressive multifocal leukoencephalopathy, a complication of AIDS.2

+

Case Report

Our patient is a 39-year-old African American male whom we were asked to evaluate at our teaching hospital for an acute change of mental status. The patient’s past medical history was significant for AIDS.

On initial presentation, vital signs and physical examination were essentially within normal limits. However, his neurological examination was significant for bilateral upper extremity hyperreflexia; lower extremity reflexes were difficult to elicit due to the patient’s supine position. He had a positive babinski sign on the right and down-going toes on the left. The jaw-jerk reflex and gag reflex were slightly increased.

Our patient was treated for metabolic abnormalities and his mental status improved minimally. The patient received a magnetic resonance imaging (MRI) scan of the head which demonstrated several patches of signal abnormality involving subcortical white matter, brainstem, and cerebellum. The most likely diagnosis was postviral related demyelination due progressive multifocal leukoencephalopathy.

His psychiatric examination was significant for monotone, nasal speech. His affect was labile and he would easily burst into tears and laugh inappropriately to questions. Cognitively, the patient was oriented times one. His working and short-term memory were impaired.

In addition to a nasal dysarthria, our patient also experienced dysphagia. A barium swallow demonstrated aspiration of all consistency liquids. However, there were no abnormalities in relevant anatomy.

Nonetheless, our patient’s medical condition worsened, and unfortunately he expired before we could attempt treatment of his pseudobulbar affect.

+

Discussion

This case demonstrates a clinical picture of pseudobulbar palsy in a patient suffering from progressive multifocal leukoencephalopathy due to AIDS. Given our patient’s clinical symptoms, plus pseudobulbar affect and MRI findings, he seemed to have pseudobulbar palsy. Historically, pseudobulbar palsy is considered in the setting of vascular disorders, neurodegenerative disorders, and other CNS diseases.1 While various lesions of the CNS may present with pseudobulbar palsy, typically corticobulbar fibers are involved.1 Similar to our patient’s MRI, progressive multifocal leukoencephalopathy includes multiple areas of demyelination,3 further substantiating our impression of pseudobulbar palsy.

Two predominate mechanisms have been proposed to explain pseudobulbar affect. One suggests disinhibition of the motor neurons controlling laughter and crying. Newer research suggests a reciprocal pathway exists between the cerebellum and the brainstem that adjusts laughter and crying responses, making them appropriate to context.4

Pharmacologic interventions for pseudobulbar affect include the tricyclic antidepressants, serotonin reuptake inhibitors and a novel approach utilizing dextromethorphan and quinidine sulfate. Dextromethorphan, an N-methyl-D-aspartate receptor antagonist, inhibits glutamatergic transmission in the regions of the brainstem and cerebellum, which are hypothesized to be involved in pseudobulbar symptoms. Dextromethorphan acts as a sigma ligand, binding to the sigma-1 receptors that mediate the emotional motor expression.5

We feel this case report illustrates that pseudobulbar palsy and pseudobulbar affect should be considered across a variety of CNS conditions.

.
Moore D, Jefferson J: Pseudobulbar palsy, in Handbook of Medical Psychiatry, 2nd ed. Mosby, 2004, pp 302—303
 
.
Mamidi A, DeSimone J, Pomerantz R: Central nervous system infections in individuals with HIV-1 infection. J Neurovirol 2002; 8:158—167
 
.
Mark A, Atlas S: Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MR images. Radiology 1989; 173:517—529
 
.
Keister GW: Pseudobulbar affect: keys to its cause and management. Applied Neurology 2006; 2(8)
 
.
Brooks BR, Thisted RA, Appel SH, et al: Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology 2004; 63:1364—1370
 
+

References

.
Moore D, Jefferson J: Pseudobulbar palsy, in Handbook of Medical Psychiatry, 2nd ed. Mosby, 2004, pp 302—303
 
.
Mamidi A, DeSimone J, Pomerantz R: Central nervous system infections in individuals with HIV-1 infection. J Neurovirol 2002; 8:158—167
 
.
Mark A, Atlas S: Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MR images. Radiology 1989; 173:517—529
 
.
Keister GW: Pseudobulbar affect: keys to its cause and management. Applied Neurology 2006; 2(8)
 
.
Brooks BR, Thisted RA, Appel SH, et al: Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology 2004; 63:1364—1370
 
+
+

CME Activity

There is currently no quiz available for this resource. Please click here to go to the CME page to find another.
Submit a Comments
Please read the other comments before you post yours. Contributors must reveal any conflict of interest.
Comments are moderated and will appear on the site at the discertion of APA editorial staff.

* = Required Field
(if multiple authors, separate names by comma)
Example: John Doe



Web of Science® Times Cited: 2

Related Content
Books
Textbook of Traumatic Brain Injury, 2nd Edition > Chapter 35.  >
The American Psychiatric Publishing Textbook of Geriatric Psychiatry, 4th Edition > Chapter 4.  >
Dulcan's Textbook of Child and Adolescent Psychiatry > Chapter 10.  >
The American Psychiatric Publishing Textbook of Geriatric Psychiatry, 4th Edition > Chapter 4.  >
Dulcan's Textbook of Child and Adolescent Psychiatry > Chapter 10.  >
Topic Collections
Psychiatric News
APA Guidelines