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Pseudobulbar Palsy

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Last Update: August 9, 2021.

Continuing Education Activity

Pseudobulbar palsy is due to an upper motor lesion caused by bilateral disturbance of the corticobulbar tracts. Pseudobulbar palsy is characterized by dysarthria, dysphagia, facial and tongue weakness, and emotional lability. This activity outlines the evaluation and management of pseudobulbar palsy and highlights the role of the interprofessional team in managing patients with pseudobulbar palsy.

Objectives:

  • Review the etiology of pseudobulbar palsy.
  • Outline the appropriate history, physical, and evaluation of pseudobulbar palsy.
  • Explain the management options available for pseudobulbar palsy.
  • Describe some interprofessional team strategies for improving care coordination and communication to advance the management of pseudobulbar palsy and improve outcomes.
Access free multiple choice questions on this topic.

Introduction

Magnus reported the first case of pseudobulbar palsy in 1837 in a patient having multiple infarcts. Lepine, in 1877 introduced the term pseudobulbar palsy for differentiation purposes. Pseudobulbar palsy is due to an upper motor lesion caused by bilateral disturbance of the corticobulbar tracts. The corticobulbar tracts exert supranuclear control over brainstem motor nuclei and are involved in the muscular movement of the head and neck. They originate from pyramidal cells in the cortex and terminate at cranial nerve nuclei. These nuclei control mastication, deglutition, and speech. Pseudobulbar palsy is characterized by dysarthria, dysphagia, facial and tongue weakness, and emotional lability.[1][2] Any condition which damages bilateral corticobulbar pathways can cause pseudobulbar palsy.

Etiology

Pseudobulbar palsy is not a disease but a clinical constellation. Clinical conditions that involve corticobulbar tracts bilaterally can cause pseudobulbar palsy.[3] Many pathological conditions can lead to pseudobulbar palsy. These include traumatic brain injury, neoplasm, vascular lesions, metabolic abnormality, or neurological disease. Pseudobulbar palsy is one of the severe complications of cerebrovascular diseases.[2][3]

Neurological disorders, such as amyotrophic lateral sclerosis, Parkinson disease, and progressive supranuclear palsy are common causes of pseudobulbar palsy.[1] Other causes include post-stroke pseudobulbar palsy, demyelinating conditions such as multiple sclerosis, and congenital malformations of the opercular or insular cortex.[1][4][5] Consecutive lacunar strokes can involve both corticobulbar tracts and cause pseudobulbar palsy.[6]

Rare causes of pseudobulbar palsy are:

  • Central pontine myelinolysis: Common symptoms include pseudobulbar palsy.[7]
  • Bilateral thalamic infarction: Very rarely infarction of specific nuclei manifests as pseudobulbar palsy.[4][8]
  • Methotrexate-induced neurotoxicity: It is uncommon, but acute or subacute neurological clinical signs and symptoms, including pseudobulbar palsy, have been reported in the literature.[9][10]
  • Progressive multifocal leukoencephalopathy: It rarely causes pseudobulbar symptoms.[11]
  • Cerebral malaria: Pseudobulbar palsy in cerebral malaria may occur probably due to demyelination.[12]
  • Bacterial endocarditis: Cerebral disturbance may occur in bacterial endocarditis and can manifest as pseudobulbar palsy.[13]
  • Syphilis: Requires CSF examination.[13]
  • Large petroclival meningioma: It presents as bulbar motor dysfunction and emotional lability that characterizes pseudobulbar palsy.[1]
  • Neurocysticercosis: It is uncommon.[3]

Autoimmune encephalitis and Hashimoto encephalopathy causing pseudobulbar palsy have been reported; this highlights the importance of early detection of anti-thyroperoxidase antibodies in a patient with pseudobulbar palsy.[5]

Epidemiology

Pseudobulbar palsy epidemiology depends on the cause that has lead to corticobulbar pathway damage. A study on the prevalence of motor neuron disease, including pseudobulbar palsy, reveals that prevalence increases, particularly after 50 years of age. Incidence is higher in males compared to females among all age groups.[14]

History and Physical

Pseudobulbar palsy is due to an upper motor lesion in the corticobulbar pathways in the pyramidal fibers. Pseudobulbar palsy presents as:  

  • Dysarthria, or anarthria 
  • Dysphagia, drooling
  • Dysphonia, hypernasal voice, slurred speech
  • Glossplegia with paresis of the tongue. Bilateral supranuclear lesions of the hypoglossal nerve in pseudobulbar palsy can produce an inability of the tongue to function.
  • Difficulty in chewing due to weakness of the mastication muscle, and paresis of facial muscles
  • Emotional lability (pseudobulbar affect) is the abnormal expressions of emotion sometimes described in patients with pseudobulbar palsy, such as pathological laughter and emotional outburst, which is characterized by involuntary, uncontrollable laughter, or crying. Pathological laughter has also been reported in association with many tumors of the posterior fossa, particularly those involving pons and midbrain.
  • Trismus: Acute pseudobulbar palsy can present with trismus due to the bilateral infarction of the internal capsule.
  • On physical examination, there is a brisk jaw jerk. Other facial reflexes also become exaggerated. There are retained or increased palatal reflexes. Unlike lower motor neuron bulbar palsy, in pseudobulbar palsy, there is no atrophy or fasciculations of the affected muscles. Moreover, in bulbar palsy, emotions are not affected.[1][3][15]

Evaluation

The diagnosis of pseudobulbar palsy is mainly clinical and based on clinical features. Evaluation of the condition includes identifying the underlying cause. Possible causes of acute pseudobulbar palsy (neoplastic, inflammatory, demyelinating, myasthenic) should merit consideration.[4] Detailed history, including any previous history of stroke, is required. Complete neurological examination with the Mini-Mental State Examination (MMSE) score.[8] Investigations focus on evaluating for stroke, neoplasia, multiple sclerosis, or other demyelination disorders, such as:

  • Complete blood count, metabolic profile, and serology
  • Electroencephalogram
  • CSF analysis (oligoclonal bands)
  • Imaging studies, such as computed tomography (CT) and MRI of the brain
  • Diffusion-weighted MRI/T2-weighted MRI
  • Motor- and sensory-evoked potential tests
  • Videofluoroscopic swallowing study (VFSS)
  • Speech assessment
  • Psychiatric consultation [7]

Treatment / Management

Treatment of pseudobulbar palsy is mainly supportive and includes management of the underlying etiology.

Dysphagia can be managed with oromotor exercises, pharyngeal tactile stimulation, tongue retraction exercises, effortful swallowing exercises, the Mendelsohn maneuver, and shaker exercises. Percutaneous endoscopic gastrostomy tube (PEG) may be considered for patients with severe dysphagia or recurrent aspiration pneumonia.

For dysarthria speech therapy, vocal cord adduction exercises, and respiration-phonation training can be helpful.[8]

Acupuncture by a prompt and deep insertion technique has shown satisfactory results. It may result in the restoration of the swallowing and phonetic functions.[2]

Although it is not a mood disorder, clinicians frequently prescribe antidepressants to address emotional outbursts of excessive laughing or crying. The pseudobulbar effect affects approximately 20 to 50% of patients with amyotrophic lateral sclerosis in pseudobulbar palsy. A fixed-dose combination of dextromethorphan/quinidine for the treatment of pseudobulbar affect has shown to reduce the frequency and severity of laughing and crying behavior.[16]

Methotrexate-induced pseudobulbar palsy can be managed with intravenous immunoglobulins.[9]

Rehabilitation: Patients with pseudobulbar palsy require comprehensive interprofessional care involving a physician, physical therapist, occupational therapist, speech pathologist, social worker, respiratory therapist, and nurse caseworker.

Differential Diagnosis

Alcohol withdrawal seizures and delirium tremens should be distinguished when making a diagnosis of pseudobulbar palsy.[8] Metabolic encephalopathy can have a similar presentation.[7]

Pathological laughter also correlates with large compressive posterior fossa tumors, posterior fossa meningiomas, epidermoid tumors, and trochlear nerve neurinomas. Other tumors associated with pathological laughter include hypothalamic glioblastoma multiforme, pontine glioblastoma multiforme, metastatic melanomas, etc.[1]

Prognosis

The prognosis of pseudobulbar palsy depends on the underlying etiology.

Complications

Pseudobulbar palsy can lead to the following complications:

  • Aspiration pneumonia
  • Severe dysphagia
  • Social withdrawal

Deterrence and Patient Education

Patients and their caregivers require education about the etiology associated with pseudobulbar palsy and its possible outcome. For example, patients with stroke or brain trauma have different treatment requirements. Slow recovery may happen over some time and neurological functions recover. On the other hand, clinical conditions such as multiple sclerosis, Parkinson disease, and amyotrophic lateral sclerosis require prolonged and consistent interprofessional management because of their progressive and irreversible nature.

Enhancing Healthcare Team Outcomes

Pseudobulbar palsy requires interprofessional management involving physicians, social workers, physical therapists, speech pathologists, respiratory consultants, pharmacists, and nurses. Other healthcare professionals can be integrated depending upon the etiology of pseudobulbar palsy. Patients with pseudobulbar palsy who suffer from pathological laughter and emotional outburst may be more prone to depression. A psychiatric evaluation can be of help. Neuroscience and rehabilitation nurses care for patients and coordinate care with the team. These patients and their caregivers need to have counsel to improve perceptions, and this will help reduce social withdrawal. Patients and caregivers should understand the available pharmacological management. Pharmacists should assist the interprofessional team with a review of medications, check for drug interactions, and educate patients about the importance of compliance and potential side effects.[Level 5]

Review Questions

References

1.
McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base. 2002 May;12(2):67-71. [PMC free article: PMC1656925] [PubMed: 17167648]
2.
Hong C. Thirty-six cases of pseudobulbar palsy treated by needling with prompt and deep insertion. J Tradit Chin Med. 2006 Sep;26(3):184-5. [PubMed: 17078444]
3.
Kumar AS, Subrahmanyam DK. Neurocysticercosis presenting as pseudobulbar palsy. J Neurosci Rural Pract. 2014 Jan;5(1):76-7. [PMC free article: PMC3985368] [PubMed: 24741260]
4.
Karacostas D, Artemis N, Giannopoulos S, Milonas I, Bogousslavsky J. Bilateral thalamic infarcts presenting as acute pseudobulbar palsy. Funct Neurol. 1994 Sep-Oct;9(5):265-8. [PubMed: 7750810]
5.
Oz Tuncer G, Teber S, Kutluk MG, Albayrak P, Deda G. Hashimoto's encephalopathy presenting as pseudobulbar palsy. Childs Nerv Syst. 2018 Jun;34(6):1251-1254. [PubMed: 29368307]
6.
Stocker A, Struffert T, Engelhorn T, Huttner HB. Consecutive Lacunar Strokes Mimicking Brainstem Symptoms in a Patient With Pseudobulbar Palsy. Neurologist. 2015 Jul;20(1):16-7. [PubMed: 26185957]
7.
Schneider P, Nejtek VA, Hurd CL. A case of mistaken identity: alcohol withdrawal, schizophrenia, or central pontine myelinolysis? Neuropsychiatr Dis Treat. 2012;8:49-54. [PMC free article: PMC3280106] [PubMed: 22347796]
8.
Lee HY, Kim MJ, Kim BR, Koh SE, Lee IS, Lee J. Acute Pseudobulbar Palsy After Bilateral Paramedian Thalamic Infarction: A Case Report. Ann Rehabil Med. 2016 Aug;40(4):751-6. [PMC free article: PMC5012989] [PubMed: 27606284]
9.
Kinirons P, Fortune A, Enright H, Murphy RP. Acute pseudobulbar palsy due to methotrexate with rapid response to intravenous immunoglobulin. J Neurol. 2005 Nov;252(11):1401-3. [PubMed: 16314998]
10.
Cruz-Carreras MT, Chaftari P, Shamsnia A, Guha-Thakurta N, Gonzalez C. Methotrexate-induced leukoencephalopathy presenting as stroke in the emergency department. Clin Case Rep. 2017 Oct;5(10):1644-1648. [PMC free article: PMC5628205] [PubMed: 29026563]
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Almeida V, Mestre T, De Carvalho M. Pseudobulbar syndrome in two patients with human immunodeficiency virus infection. Amyotroph Lateral Scler. 2010;11(1-2):220-2. [PubMed: 20184520]
12.
Mohapatra MK, Sethy G, Mohanty SC. Pseudobulbar paralysis--a sequelae of cerebral malaria. J Assoc Physicians India. 2004 Apr;52:324-5. [PubMed: 15636339]
13.
Shafar J. Bacterial endocarditis presenting as pseudobulbar palsy. Br Med J. 1967 Nov 11;4(5575):338. [PMC free article: PMC1748731] [PubMed: 6053619]
14.
GBD 2016 Motor Neuron Disease Collaborators. Global, regional, and national burden of motor neuron diseases 1990-2016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet Neurol. 2018 Dec;17(12):1083-1097. [PMC free article: PMC6234315] [PubMed: 30409709]
15.
Lai MM, Howard RS. Pseudobulbar palsy associated with trismus. Postgrad Med J. 1994 Nov;70(829):823-4. [PMC free article: PMC2397823] [PubMed: 7824418]
16.
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC., Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73(15):1227-33. [PMC free article: PMC2764728] [PubMed: 19822873]
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Bookshelf ID: NBK553160PMID: 31985953

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