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.
Entire Body System
- Weakness
Tongue - weak and wasted and sits in the mouth with fasciculations. Drooling - as saliva collects in the mouth and the patient is unable to swallow (dysphagia). Absent palatal movements. [patient.info]
For UMN disease to cause palatal weakness it must be bilateral because there is bilateral UMN innervation for the nucleus ambiguus. [kaltura.com]
Skip to content Bulbar Palsy Relevant physical signs Bulbar palsy (LMN) Nasal speech which lacks modulation Speaks in short phrases Slurred words, especially of the consonants Palatal weakness Wasted, flaccid, fasciculating tongue Absent jaw jerk Reduced [learnhaem.com]
Pseudobulbar palsy is upper motor neuron weakness of the muscles innervated by 9th, 10th and 12th cranial nerves, while in bulbar palsy is a lower motor neuronal weakness of these muscles. [mobilephysiotherapyclinic.in]
- Disability
Treatment There are no cures for pseudobulbar palsy; the symptoms usually progress over the course of several years, leading to complete disability. [encyclopedia.com]
Progressively, a patient exhibits profound difficulty to swallow (dysphagia) or chew; with the time, a person will become practically disabled. [symptoma.com]
Please enable JS and disable any ad blocker [cairn.info]
- Swelling
A 17-year-old man presented with submandibular swelling and pancytopoenia. He was diagnosed with acute lymphocytic leukaemia… One Citation Neurological complications of cancer chemotherapy J. [semanticscholar.org]
The most common adverse reactions in patients taking NUEDEXTA are diarrhea, dizziness, cough, vomiting, weakness, swelling of feet and ankles, urinary tract infection, flu, elevated liver enzymes, and flatulence. NUEDEXTA may cause dizziness. [medmk.com]
Respiratoric
- Pneumonia
Death from pneumonia, uremia, infectious diseases, new bleedings, nephritis, heart failure and other Diagnosis of pseudobulbar palsy is not difficult. [medicalency.com]
Links [ ✎ edit | edit source ] Related articles [ ✎ edit | edit source ] Motorneuron Diseases Motor Neurons Amyotrophic Lateral Sclerosis Myasthenia Gravis Pneumonia Cranial Nerves External links [ ✎ edit | edit source ] NINDS wiseGEEK Bibliography [ [wikilectures.eu]
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]
Some steps can be taken to manage it, but patients usually die within three to four years of diagnosis, typically as a result of a pneumonia infection. Supportive care can be provided to make the patient as comfortable as possible. [wisegeek.com]
- Stridor
The finding of stridor led to a detailed evaluation of the obstruction. [ncbi.nlm.nih.gov]
[…] weakness or spasticity of the muscles innervated by trigeminal, facial, hypoglossal, glossopharyngeal or vagal nerves Laryngospasm, which is defined as a paroxysmal episode with the sensation that air cannot be moved in and out, accompanied by inspiratory stridor [nature.com]
- Snoring
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Gastrointestinal
- Dysphagia
Second, the pathology behind dysphagia was limited to pseudobulbar palsy. Additional studies are necessary to determine whether this maneuver can be successfully used for other types of dysphagia. [cureus.com]
It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability. [pacs.de]
Dysphagia ICD 10 R13 ICD 9 438.82, 787.2 … Wikipedia [translate.academic.ru]
Pseudobulbar palsy is characterized by dysarthria, dysphagia, facial and tongue weakness, and emotional lability. [ncbi.nlm.nih.gov]
- Dysphagia
Second, the pathology behind dysphagia was limited to pseudobulbar palsy. Additional studies are necessary to determine whether this maneuver can be successfully used for other types of dysphagia. [cureus.com]
It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability. [pacs.de]
Dysphagia ICD 10 R13 ICD 9 438.82, 787.2 … Wikipedia [translate.academic.ru]
Pseudobulbar palsy is characterized by dysarthria, dysphagia, facial and tongue weakness, and emotional lability. [ncbi.nlm.nih.gov]
- Gagging
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]
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]
It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability. [pacs.de]
This leads to a variety of clinical problems, including: Difficulty with speech: spastic dysarthria, dysphonia Difficulty with swallowing: dysphagia Brisk jaw jerk and pout reflex; there may be trismus Slow, spastic, tongue movements Gag reflex may be [minclinic.ru]
Patients with bilateral UMN weakness or dysfunction will lack voluntary movement of the palate but will have a hyperactive gag reflex (just like a hyperactive DTR with UMN disease). [kaltura.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]
There may be dysphagia, nasal regurgitation, choking, and drooling. Patients may keep food in the mouth for prolonged periods. Although the tongue may be strikingly immobile, atrophy and fasciculations do not develop. [neurosigns.org]
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]
Patients display poor articulation, dysphagia, difficulty to choke or incontrollable laughter. Aspiration is likely, leading to increased possibility of pneumonia. [symptoma.com]
- Nausea
Side effects were dizziness, nausea, and somnolence, which accounted for termination of treatment in 24% with DM/Q compared to 6% with DM and 5% with Q. DM/Q is not yet approved by the US Food and Drug Administration (FDA). [mndcare.net.au]
The most commonly reported adverse events from this Phase III study were dizziness, nausea, diarrhea, fatigue and somnolence, which were mild to moderate in nature. [medmk.com]
[…] open-label, 52-week, multicenter study.43 A total of 553 patients were recruited (40.3% had MS; 31.8%, ALS; 8.3%, stroke; 3.8%, TBI) and were treated with dextromethorphan/quinidine 30/30 mg twice daily.43 The most frequent AEs during treatment were nausea [ajmc.com]
Treatment-related side effects including nausea, dizziness, somnolence, and loose stools were relatively common however; about one quarter of patients withdrew from treatment, the majority within 1 week (102). [frontiersin.org]
Cardiovascular
- Hypertension
Conditions mentioned include: pseudo-bulbar palsy; paresis of facial muscles; spastic paresis of limbs; essential hypertension; spasm in cerebral arteries; and cerebral thrombosis. No treatment given. Patient discharged. [archives.collections.ed.ac.uk]
Hypertension, cardiopathy, smoking habit, dyslipemia and diabetes are the most frequent risk factors in both forms. © 1990 S. [karger.com]
Hypertension, cardiopathy, smoking habit, dyslipemia and diabetes are the most frequent risk factors in both forms. [scienceopen.com]
Kannel WB (1995) Framingham study insights into hypertensive risk of cardiovascular disease. Hypertens Res 18(3):181–196 PubMed CrossRef Google Scholar 14. [link.springer.com]
Patient was not a diagnosed case of hypertension, diabetes mellitus, ischemic heart disease or rheumatic heart disease. Initial examination revealed emotional lability and spastic speech. Cranial nerve examination was normal. [neurologyindia.com]
- Heart Disease
Your recognised location is United States (US) Coronary heart disease This article is freely available only to users in the UK. Your recognised location is United States (US) Melanoma This article is freely available only to users in the UK. [evidence.nhs.uk]
Patient was not a diagnosed case of hypertension, diabetes mellitus, ischemic heart disease or rheumatic heart disease. Initial examination revealed emotional lability and spastic speech. Cranial nerve examination was normal. [neurologyindia.com]
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Heart disease and stroke statistics — 2005 update. http://www.americanheart.org/downloadable/heart/1105390918119HDSStats2005Update.pdf. Accessed March 23, 2011. 43. [dx.doi.org]
Jaw & Teeth
- Hypersalivation
Conditions mentioned include: hemiparesis; pseudo-bulbar palsy; cerebral vascular catastrophes; dysarthria; emotional over-reaction; hypersalivation; and arterial hypertension. Nonsurgical treatment given. Patient discharged. [archives.collections.ed.ac.uk]
- Dental Caries
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Musculoskeletal
- Muscle Spasticity
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. [symptoma.com]
Conditions mentioned include: spasticity of all the muscles; pseudo-bulbo-paralysis; bilateral supranuclear lesions; hysteria; pyelitis; dysarthria; and dysphagia. Nonsurgical treatment given. Patient discharged. [archives.collections.ed.ac.uk]
Psychiatrical
- Emotional Outbursts
Definition Pseudobulbar palsy refers to a group of symptoms—including difficulty with chewing, swallowing, and speech, as well as inappropriate emotional outbursts—that accompany a variety of nervous system disorders. [web.archive.org]
Sudden emotional outbursts, in which the patient spontaneously and without cause begins to laugh or cry, are also a characteristic of pseudobulbar palsy. [encyclopedia.com]
Pseudobulbar affect, which involves uncontrollable emotional outbursts, has been reported in diseases including Alzheimer's and Parkinson's. [psychiatryadvisor.com]
- Flat Affect
affect–simulating apathy or severe depression, become enmeshed in trivialities, or have inappropriate responses to environmental cues, aka 'laughing sickness' for the characteristic pathologic–ie, inappropriate laughing–or crying Etiology Multifocal [medical-dictionary.thefreedictionary.com]
Neurologic
- 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]
It also results in dysarthria and dysphagia, but with a decreased gag reflex and absence of emotional lability. [pacs.de]
Larsen from the University of Utah collection demonstrates severe dysarthria, slow tongue movements and a hyperactive gag reflex.(2) The most common cause is multiple cerebral infarctions. [neurosigns.org]
This leads to a variety of clinical problems, including: Difficulty with speech: spastic dysarthria, dysphonia Difficulty with swallowing: dysphagia Brisk jaw jerk and pout reflex; there may be trismus Slow, spastic, tongue movements Gag reflex may be [minclinic.ru]
Dysarthria and dysphagia without other neurologic deficits are indications for screening for lacunar infarction. [e-arm.org]
- Slurred Speech
The signs and symptoms of pseudobulbar palsy include the following: Slowed or slurred speech Facial and tongue weakness Difficulty chewing or swallowing Unintentional weight loss Drooling Spasms of the vocal cords, which can cause changes in tone of voice [painscale.com]
Facial muscle weakness, emotional lability, dysarthria (slowed or slurred speech), dysphagia (difficulty swallowing), dysphonia (vocal cord muscle spasms) and progressive immobility of the tongue are also symptoms of pseudobulbar palsy. [newyorkfacialparalysis.com]
The first noticeable symptom is often slurred speech. Over time, speech, chewing, and swallowing become progressively more difficult, eventually becoming impossible. [encyclopedia.com]
- Seizure
Two children are described who suddenly developed an encephalitic illness with intractable bilateral facial seizures. [ncbi.nlm.nih.gov]
We report a 38-year-old female patient who presented with partial seizures and pseudobulbar palsy. The MRI brain showed multiple small cysts with scolex in both the cerebral hemispheres and a giant intraparenchymal cyst. [doaj.org]
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]
- Apathy
[…] muscles, resulting in dysarthria, dysphonia, dysphagia, drooling, facial weakness, hyperreflexia of extremities, and shuffling–parkinsonian gait; PP is remarkable for the variable psychiatric component, in which the Pts may have a flat affect–simulating apathy [medical-dictionary.thefreedictionary.com]
[…] and emotional apathy. [ncbi.nlm.nih.gov]
- 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]
Skip to content Bulbar Palsy Relevant physical signs Bulbar palsy (LMN) Nasal speech which lacks modulation Speaks in short phrases Slurred words, especially of the consonants Palatal weakness Wasted, flaccid, fasciculating tongue Absent jaw jerk Reduced [learnhaem.com]
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. [neuro.psychiatryonline.org]
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:
- Head traumatization
- Neurosyphilis
- Demyelinating conditions, such as multiple sclerosis
- Upper motor neuron disease
- Strokes
- Lesions located in the brainstem
- Osmotic demyelination syndrome and other metabolic conditions [1].
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:
- Primary lateral sclerosis
- Amyotrophic lateral sclerosis
- Postpolio syndrome
- Progressive muscular atrophy
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
- 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.
- 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.
- 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.
- McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base. 2002 May;12(2):67-71.
- Murdoch BE; Physiological investigation of dysarthria: recent advances. Int J Speech Lang Pathol. 2011 Feb;13(1):28-35.
- 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.
- 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.