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.

• Anorexia

  Case presentation 60 years old male from Najaf, went to the emergency department with three days history of drowsiness and anorexia after COVID-19 related fever and myalgias. He had significant risk factors. [pesquisa.bvsalud.org]

• 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

  Guide to Molluscum Contagiosum and Related Skin Diseases" "A Simple Guide to Toothache and Related Diseases" "A Simple Guide to Muscle Dystrophy Disease and Related Diseases" "A Simple Guide to Appendicitis and Causative Diseases" "A Simple Guide to Snoring [amazon.ca]

• Dysphagia

  Mechanical Dysphagia  Dysphagia caused by a large bolus or luminal narrowing is called mechanical Dysphagia 2. [slideshare.net]

  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]

  Dysphagia ICD 10 R13 ICD 9 438.82, 787.2 … Wikipedia [translate.academic.ru]

  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]

• Dysphagia

  Mechanical Dysphagia  Dysphagia caused by a large bolus or luminal narrowing is called mechanical Dysphagia 2. [slideshare.net]

  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]

  Dysphagia ICD 10 R13 ICD 9 438.82, 787.2 … Wikipedia [translate.academic.ru]

  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]

• 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]

  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]

  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]

  There is often an exaggerated jaw jerk, a hyperactive gag reflex and frontal release signs such as snout and suck reflexes. Pseudobulbar affect is common.(1) The video by Dr. Paul D. [neurosigns.org]

  Lower motor neuron signs (atrophy and fasciculations of the tongue, absent gag reflex) differentiate bulbar palsy from pseudobulbar palsy, which presents with upper motor neuron signs (spastic tongue, exaggerated gag, and jaw jerk reflexes). [amboss.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]

  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]

• Loss of Appetite

  Also, people with PBA often lack certain features of depression, such as sleep disturbances or a loss of appetite. But depression is common among those who have pseudobulbar affect. [mayoclinic.org]

  […] of appetite Differential diagnoses: Myasthenia gravis lyme disease botulism Brown–Vialetto–Van Laere syndrome Worster-Drought syndrome or congenital suprabulbar paresis X-linked bulbospinal muscular atrophy (Kennedy’s disease) Diagnosis: Diagnosis of [mobilephysiotherapyclinic.in]

• 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]

• Suggestibility

  Our data suggest that pathological laughing and crying can occur without any other manifestation of pseudobulbar palsy. An attempt is made to correlate this patient's clinical and radiological findings. [ncbi.nlm.nih.gov]

  One suggests disinhibition of the motor neurons controlling laughter and crying. [neuro.psychiatryonline.org]

  These findings suggest that the corticobulbar tract is located in the genu of the internal capsule and the junction of the internal capsule with the corona radiata, and that bilateral damage to the corticobulbar tract at these levels could play an important [jstage.jst.go.jp]

  The authors suggest that the clinical and imaging features of these patients indicate a distinct and specific syndrome and the malformations appear to result from specific derangement of neuronal migration. [1] COMMENT. [pediatricneurologybriefs.com]

• 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]

• Social Isolation

  Complications Severe symptoms of pseudobulbar affect (PBA) can cause embarrassment, social isolation, anxiety and depression. [mayoclinic.org]

  It can cause embarrassment and anxiety, leading to withdrawal and social isolation. How common is pseudobulbar affect? Researchers estimate that 2 million to 7 million people in the United States have PBA. [my.clevelandclinic.org]

  This may improve with age, but equally may cause increasing distress as it is socially isolating, interferes with school work and using computers, and leaves chronically irritated skin. [gosh.nhs.uk]

• Fear

  Recurrent stroke seems to be common in patients with APS and a first stroke, recurrent stroke and vascular dementia are feared consequences of APS. [ncbi.nlm.nih.gov]

  […] and Related Diseases" "A Jab in the Face, Injections and Aspirations of Joints, Bursa and polyps" "The Malaria Patient, Diagnostic Challenges and Related Diseases" "A Simple Guide to Pineal Gland (The Third Eye) and Its Functions" "A Simple Guide to Fear [amazon.ca]

• Behavior Disorder

  Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. Parkinsonism Relat Disord. 2009 Jan. 15(1):59-61. [Medline]. [emedicine.medscape.com]

• 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]

  Dysarthria and dysphagia without other neurologic deficits are indications for screening for lacunar infarction. [e-arm.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]

  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]

  Pseudobulbar [Nom commun] Dysarthrias, Pseudobulbar [Nom commun] Pseudobulbar Dysarthria [Nom commun] Pseudobulbar Dysarthrias [Nom commun] Mutisme pseudobulbaire [Nom commun] Mutisme pseudo-bulbaire [Nom commun] Dysarthrie pseudobulbaire [Nom commun [idref.fr]

• 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]

• 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]

• Cranial Nerve Involvement

  Like spinal nerves these two cranial nerve nuclei receive only contralateral innervation from the pyramidal tract. [csuchico.edu]

• Slow Speech

  […] gag reflex Pseudobulbar palsy (UMN) High-pitched, slow speech Imprecise articulation, sounds forced Palatal weakness Small, spastic, poorly mobile tongue Brisk jaw jerk Exaggerated gag reflex Pseudobulbar affect – emotional lability Other signs Nasogastric [learnhaem.com]

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.

• White Matter Lesions

  "Subcortical white matter lesions in osmotic demyelination syndrome". AJNR. American Journal of Neuroradiology. 16 (7): 1495–1497. PMC 8338057. PMID 7484639.] ^ a b Graham KC, Spiegel DR (2008). [en.wikipedia.org]

  Relationship between periventricular and deep white matter lesions and depressive symptoms in older people. The LADIS study. Int J Geriatr Psychiatry 21(10):983–989 PubMed CrossRef Google Scholar 22. [link.springer.com]

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.

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.

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].

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

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].

Pseudobulbar palsy cannot be prevented at any case.

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.

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.

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.