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Pseudobulbar Palsy

Paralyses Pseudobulbar

Pseudobulbar palsy is a symptom featuring a failure to control basic movements of the face and occasional abnormal emotional responses. It is not a distinct condition itself, but rather a result of various neurodegenerative conditions. 


Presentation

An individual displaying pseudobulbar palsy gradually loses their ability to regulate the movement of the muscles of the face. The earliest symptoms that may indicate the presence of palsy is slurred speech. Progressively, a patient exhibits profound difficulty to swallow (dysphagia) or chew; with the time, a person will become practically disabled. Other symptoms include tongue spasticity, as the muscle tone increases, jerking of the jaw and a pharyngeal reflex that may be diminished, absent or even accentuated. A distinct symptom observed in people with pseudobulbar palsy is the so-called pseudobulbar affect or labile affect [4], which involves uncontrollable laughter or crying in response to an inappropriate stimulus. Patients are frequently depressed and lack in muscle strength.

It is important to keep in mind the clinical picture of ALS as well, since it is the condition that most commonly causes pseudobulbar palsy. The disease starts as peripheral muscle weakness, typically located in the hands or feet, and then progresses to more central parts of the body. Muscles become atrophic and tone is augmented to the degree that spasticity and stiffness are observed. Fasciculations are observed, alongside weirdly coordinated movements, as the individuals progressively lose the ability to control their muscles, walk and move in general. Muscle stiffness is not always present; some patients have extremely diminished muscle tone.

At a second stage, the symptoms of pseudobulbar palsy emerge. Patients display poor articulation, dysphagia, difficulty to choke or incontrollable laughter. Aspiration is likely, leading to increased possibility of pneumonia. Ultimately, the lungs fail to function properly, as the breathing muscles are affected too, often rendering the individual in need of mechanical support. Half of the patients die within 3 years upon the initial symptoms and almost 90% within 10-20 years. Amyotrophic lateral sclerosis is also known as Lou Gehrig's disease, in the memory of Lou Gehrig, a renowned American baseball player who suffered from it in the 20th century.

Plethora
  • There is a plethora of tests available to diagnose the various neurological conditions resulting in pseudobulbar palsy, the most widely used of which are Computerized Tomography and Magnetic Resonance Imaging of the brain.[symptoma.com]
Stridor
  • The finding of stridor led to a detailed evaluation of the obstruction.[ncbi.nlm.nih.gov]
Stridor
  • The finding of stridor led to a detailed evaluation of the obstruction.[ncbi.nlm.nih.gov]
Dysphagia
  • A 28-year-old, previously healthy, normotensive woman suddenly developed an acute pseudobulbar palsy with dysarthria, dysphagia, hypernasal voice and mild right arm paresis.[ncbi.nlm.nih.gov]
  • Abstract We reported five cases with sudden onset of severe supranuclear dysarthria and dysphagia following recurrent minor stroke.[jstage.jst.go.jp]
  • The patient presented with sudden onset of dysphagia, anarthria, and glossplegia.[ncbi.nlm.nih.gov]
  • It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability.[radiopaedia.org]
  • Convert to ICD-10-CM : 335.23 converts approximately to: 2015/16 ICD-10-CM G12.8 Other spinal muscular atrophies and related syndromes Clinical Information A syndrome characterized by dysarthria, dysphagia, dysphonia, impairment of voluntary movements[icd9data.com]
Gagging
  • Tests examining jaw jerk and gag reflex can also be performed.[en.wikipedia.org]
  • Pseudobulbar palsy - comment on: gag reflex tongue jaw jerk speech emotions Gag reflex - increased or normal Tongue - spastic. No fasciculations "it cannot be protruded, lies on the floor of the mouth and is small and tight".[quizlet.com]
  • It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability.[radiopaedia.org]
  • Pseudobulbar Palsy Bulbar Palsy degeneration of corticobulbar pathways to V,VII,X,XI,XII disturbance to X, XI, XII,sometimes VII, rather than the corticobulbar tracts lower motor neurone signs absent lower motor neurone signs present gag reflex ( /n)[gpnotebook.co.uk]
  • On clinical examination of the patient, the gag reflex and jaw jerk can be performed and these will be problematic.[xpertdox.com]
Choking
  • Surgical interventions such as placing a feeding tube may have to be performed if the patient can no longer swallow properly and are at risk of choking.[xpertdox.com]
  • Patients display poor articulation, dysphagia, difficulty to choke or incontrollable laughter. Aspiration is likely, leading to increased possibility of pneumonia.[symptoma.com]
  • When the symptoms progress to disability, there is a high risk of choking and aspiration (breathing food or liquids into the lungs), which can lead to severe pneumonia and death.[encyclopedia.com]
Drooling
  • Postural changes can help with drooling of saliva and may prevent aspiration.[patient.info]
  • All patients showed medically intractable seizures, complex partial seizures with secondary generalization and clinical symptoms of pseudobulbar palsy, including dysarthria, limitation of tongue movement and drooling.[ncbi.nlm.nih.gov]
  • […] and spasmodic, mirthless laughter. pseudobulbar palsy Pseudobulbar paralysis, spastic bulbar paralysis A disease of middle age, characterized by bilateral spasticity of the facial and deglutitive muscles, resulting in dysarthria, dysphonia, dysphagia, drooling[medical-dictionary.thefreedictionary.com]
  • The symptoms typically observed in a person with pseudobulbar palsy include difficulty to speak, chew, swallow and drooling. The tongue becomes stiff and reflexes are frequently impaired.[symptoma.com]
Trismus
  • Trismus has not previously been described as the presenting feature of a pseudobulbar palsy.[ncbi.nlm.nih.gov]
  • Correlations with pseudobulbar palsy and parkinsonism. ( 3366147 ) Mancardi G.L....Loeb C. 1988 37 Neoplastic obstruction of the trachea complicating pseudobulbar palsy. ( 3406789 ) Aumentado D.J....Payne C.B. 1988 38 Trismus in pseudobulbar palsy. ([malacards.org]
Emotional Lability
  • Two patients sought treatment for symptoms of bulbar motor dysfunction and the marked emotional lability that characterizes pseudobulbar palsy (PBP).[ncbi.nlm.nih.gov]
  • In this context, emotional lability is a misnomer. "Hyperactive emotional reflexes" would be a more appropriate descriptive term, as the underlying mood appears normal, and only the degree of emotional expression is pathological.[ncbi.nlm.nih.gov]
  • ABSTRACT Two patients sought treatment for symptoms of bulbar motor dysfunction and the marked emotional lability that characterizes pseudobulbar palsy (PBP).[doi.org]
  • A syndrome characterized by DYSARTHRIA, dysphagia, dysphonia, impairment of voluntary movements of tongue and facial muscles, and emotional lability.[ebi.ac.uk]
Dysarthria
  • Synonyms: 'bulbar palsy' - lower motor neurone dysarthria, neuromuscular dysarthria, atrophic bulbar paralysis; 'pseudobulbar palsy' - upper motor neurone dysarthria, spastic dysarthria The components of normal speech Speaking is a voluntary task which[patient.info]
  • A 28-year-old, previously healthy, normotensive woman suddenly developed an acute pseudobulbar palsy with dysarthria, dysphagia, hypernasal voice and mild right arm paresis.[ncbi.nlm.nih.gov]
  • Abstract We reported five cases with sudden onset of severe supranuclear dysarthria and dysphagia following recurrent minor stroke.[jstage.jst.go.jp]
  • It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability.[radiopaedia.org]
Drop Attacks
  • Subsequently, atonic drop attacks became the predominant epileptic pattern. Epileptogenic electrographic abnormalities were secondary generalized or multifocal.[ncbi.nlm.nih.gov]
  • Two patients tried on multiple anticonvulsants continued to have frequent seizures whereas two treated by callosotomy had no subsequent drop attacks and improved behavior.[pediatricneurologybriefs.com]
Nasal Speech
  • speech labile emotions normal emotions bilateral UMN signs in limbs increased; - reduced; n normal Links: pseudobulbar palsy progressive bulbar palsy[gpnotebook.co.uk]

Workup

Pseudobulbar palsy is a symptom and therefore it is clinically diagnosed upon observation of a compatible clinical picture. Diagnostic tests are performed in order to detect the underlying condition, which had lead to the symptom.

There is a plethora of tests available to diagnose the various neurological conditions resulting in pseudobulbar palsy, the most widely used of which are Computerized Tomography and Magnetic Resonance Imaging of the brain. Speech problems can also be evaluated diagnostically, thanks to newly-emerging techniques [5], such as electropalatography (EPG), electromagnetic articulography (EMA) and pressure-sensing EPG. Depending on the suspected underlying condition, further tests can be performed: electromyography and blood tests. Pseudobulbar palsy is perceived by some researchers to be a result of bilateral damage to the corticobulbar tract [6] and a brain MRI can be safely used to confirm this hypothesis in each patient.

Treatment

Pseudobulbar palsy is a symptom that is irreversible and, unfortunately, so are the diseases that lead to its appearance; symptoms follow a long, gradual exacerbation period that amounts to years and ultimately render a person completely disabled. The underlying condition should be properly treated by a neurologist; in some cases the symptoms may subside to a certain extent. Depending on the requirements of each case, various measures can be supportively applied, such as parenteral nutrition if a patient is unable to eat and runs a risk of aspiration, changes in posture for the same reasons, speech therapy and genetic counseling for the families of patients who present with such a disease early in childhood [7].

A variety of medications have been used to relieve a patient of some of the symptoms involved in pseudobulbar palsy. Baclofen has been used to decrease muscle spasticity, anticholinergic drugs aim at minimizing drooling and other medications such as levodopa, fluoxetine and amantadine have also been administered.

The pseudobulbar affect can be improved with the use of antidepressants (SRIs, tricyclic). Nuedexta is a new medication approved for the treatment of the phenomenon and is composed of dextromethorphan, a substance that functions as a glutamatergic inhibitor in the cerebellum and brainstem.

Prognosis

Pseudobulbal palsy constitutes a poor prognostic marker for two reasons. Firstly, the conditions themselves associated with the symptom are degenerative and incurable and secondly, a progressive loss of facial muscle control is eventually expected to lead to choking or aspiration and death.

Etiology

Pseudobulbar palsy is a set of symptoms that can arise as a result of various neurological conditions. Damaged neuronal fibers that connect the brainstem to the cerebral cortex result in the patient's inability to regulate the muscles of the face, swallow, chew, speak properly or control emotional responses. The disorders that may cause pseudobulbar palsy include:

Epidemiology

Neurodegenerative disorders that lead to pseudobulbar palsy tend to affect men more often than women and usually arise in the age of 50 years old. A considerable percentage of people who suffer from an motor neuron disease (5%-7%) have an inherited disease type. The most common diseases of this group are:

Sex distribution
Age distribution

Pathophysiology

It is believed that a damaged corticobulbar tract, namely the tract that connects the upper motor neurons to cranial motor nerves nuclei results in the symptomatology of pseudobulbar palsy. Regarding the emotional dysfunction, it is thought that the connections between the cerebellum and brainstem are responsible for sensing a stimulus and responding respectively [2], therefore damage located in the tract can lead to out-of-place manifestations of excessive laughter or crying that cannot be reversed. Another theory further proposes that the emotional effect can be a result of an inappropriate stimulation of the subthalamic nucleus [3].

Prevention

Pseudobulbar palsy cannot be prevented at any case.

Summary

Pseudobulbar palsy is a set of symptoms exhibited as a result of a plethora of neurodegenerative diseases, such as Parkinson's disease, amyotrophic lateral sclerosis (ALS), multiple sclerosis or stroke, amongst others.

The defect lies in the function of the upper motor neuron (UMN) and particularly of the corticobulbar tract, which connects the UMN to the nuclei of the cranial motor nerves controlling facial muscles. Pseudobulbar palsy mainly results in the patient's inability to control those muscles which leads to an inability to swallow, speak properly and chew. The tongue exhibits increased tone (spasticity) and accentuated reflexes. Another symptom that often accompanies the clinical picture involves laughing or crying intensely without a respective stimulation. This paradox is known as the pseudobulbar affect and depicts the patient's inability to control emotion, which is also a result of a defective corticobulbar pathway.

The type of palsy in question is not observed, in most of the cases, from the very beginning stages of the disease. Diseases of the motor neuron system, such as amyotrophic lateral sclerosis, start with weakness felt in the muscles of the extremities (hands, feet) and progress to encompass muscles of the central part of the body. The patient's inability to control facial muscles and associated complications (pseudobulbar palsy) are observed when the disease has already progressed.

Treatment is targeted at alleviating some of the symptoms experienced by patients; baclofen is frequently used for muscle spasticity, alongside anticholinergic drugs and antidepressants. Pseudobulbar palsy is an incurable symptom, because it arises as a result of equally incurable neurodegenerative conditions.

Patient Information

Pseudobulbar palsy is not a disease itself, but a symptom that results from many neurological diseases. A person with this type of palsy cannot control their facial muscles and gradually lose the ability to speak, chew and swallow. It is most often a symptoms that is included in the clinical picture of conditions affecting the transmission of nerve signals that control movement, such as Lou Gehrig's disease, multiple sclerosis, stroke etc.

The symptoms typically observed in a person with pseudobulbar palsy include difficulty to speak, chew, swallow and drooling. The tongue becomes stiff and reflexes are frequently impaired. Another aspect of the phenomenon involves outbursts of laughter or crying when something insignificant happens-this is also caused by neurological defects (labile affect).

Pseudobulbar palsy is diagnosed when a doctor observes the manifestations. Often, it is confused with depression, when the labile affect is the first symptom to emerge. The underlying condition must be diagnosed, since the palsy originated from it. A CT or MRI scan, an electromyographic study and various other tests are available in order to diagnose the condition a patient suffers from and design an appropriate treatment plan. Pseudobulbar palsy and the underlying conditions are incurable, but there is a wide range of medications which can be administered supportively, namely to alleviate some of the symptoms or delay the disease progress.

The way pseudobulbar palsy affects an individual can be debilitating. It is important for patients and their families to learn to understand the aspects of the symptom and the underlying condition, in order to relieve the patient from the stress that a neurodegenerative disease causes. Patients can achieve communication with various other ways and can benefit from medications that will lessen the impact of their symptomatology. At any case, psychological support is a crucial part of the treatment and should not be disregarded by patients.

References

Article

  1. Bourgouin PM, Chalk C, Richardson J, Duang H, Vezina JL. Subcortical white matter lesions in osmotic demyelination syndrome. AJNR Am J Neuroradiol. 1995 Aug;16(7):1495-7.
  2. Graham KC, Spiegel DR. Pseudobulbar palsy and affect in a case of progressive multifocal leukoencephalopathy. J Neuropsychiatry Clin Neurosci. 2008 Winter;20(1):110-1.
  3. Okun MS, Raju DV, Walter BL, et al. Pseudobulbar crying induced by stimulation in the region of the subthalamic nucleus. J Neurol Neurosurg Psychiatry. 2004 Jun;75(6):921-3.
  4. McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base. 2002 May;12(2):67-71.
  5. Murdoch BE; Physiological investigation of dysarthria: recent advances. Int J Speech Lang Pathol. 2011 Feb;13(1):28-35.
  6. Asfora WT, DeSalles AA, Abe M, Kjellberg RN. Is the syndrome of pathological laughing and crying a manifestation of pseudobulbar palsy? J Neurol Neurosurg Psychiatry. 1989 Apr;52(4):523-5.
  7. Manole A, Fratta P, Houlden H. Recent advances in bulbar syndromes: genetic causes and disease mechanisms. Curr Opin Neurol. 2014 Oct;27(5):506-14.

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Last updated: 2019-07-11 21:28