From the Guidelines
Pseudobulbar palsy, also known as Progressive Bulbar Palsy, is a neurological disorder characterized by difficulty with voluntary control of facial muscles, speech, and swallowing due to damage to the upper motor neurons that connect the brain to the brainstem. This condition typically presents with symptoms including emotional lability (uncontrollable laughing or crying), dysarthria (slurred speech), dysphagia (difficulty swallowing), and a hyperactive gag reflex. It results from bilateral damage to the corticobulbar tracts, often caused by conditions such as stroke, multiple sclerosis, amyotrophic lateral sclerosis, or traumatic brain injury. The most recent and highest quality study on this topic is from 1, which discusses the alterations in the swallowing process in patients with amyotrophic lateral sclerosis (ALS), a condition that can cause pseudobulbar palsy. According to this study, videofluoroscopy can evaluate the physiopathological alterations in the swallowing process and detect silent aspirations in patients with ALS. Treatment typically involves addressing the underlying cause when possible, along with symptomatic management. For emotional lability, medications like dextromethorphan/quinidine (Nuedexta) at a dose of 20mg/10mg twice daily can be effective, as mentioned in the example answer. Speech therapy and swallowing exercises are crucial components of management to improve communication and reduce aspiration risk. Patients may also benefit from assistive devices for communication and modified diets for swallowing difficulties. The condition differs from bulbar palsy, which affects lower motor neurons, by typically presenting with hyperactive rather than diminished reflexes and preserved muscle bulk despite functional impairment. It is essential to note that the evidence provided in 1, 1, and 1 is not directly related to pseudobulbar palsy, as it focuses on Bell's palsy, a different condition. Therefore, the information from these studies is not relevant to the management of pseudobulbar palsy. In real-life clinical practice, it is crucial to prioritize the management of pseudobulbar palsy based on the most recent and highest quality evidence available, which in this case is from 1.
From the Research
Definition and Characteristics of Pseudobulbar Palsy
- Pseudobulbar palsy, also known as progressive bulbar palsy, is a neurological condition characterized by the loss of control over emotions, resulting in involuntary and uncontrollable episodes of laughing and/or crying 2, 3, 4, 5, 6.
- These episodes are often exaggerated or unrelated to the patient's emotional state, and can be socially disabling 3, 5.
- Pseudobulbar palsy is associated with various neurological disorders, including amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), traumatic brain injury, and stroke 2, 4, 5, 6.
Pathophysiology and Prevalence
- The exact pathophysiology of pseudobulbar palsy is unknown, but it is thought to result from a disruption of the cortico-limbic-subcortical-thalamic-pontocerebellar network involved in emotional expression and regulation 3.
- Pseudobulbar palsy is estimated to affect up to 2 million people in the United States, although it is often under-recognized and undertreated 5.
- Studies have shown that pseudobulbar palsy symptoms are common among patients with diverse neurological conditions, including ALS, MS, Parkinson's disease, and stroke 5.
Treatment and Management
- Various treatments are available for pseudobulbar palsy, including selective serotonin reuptake inhibitors, tricyclic antidepressants, and dextromethorphan/quinidine (DM/Q) 2, 4, 6.
- DM/Q has been shown to be effective in reducing the frequency and severity of pseudobulbar palsy episodes, and is approved by the FDA for the treatment of pseudobulbar affect 2, 4.
- Non-pharmacologic therapies, such as cognitive behavioral therapy, may also be beneficial in managing pseudobulbar palsy symptoms, although more research is needed to fully understand their effectiveness 6.