Progressive bulbar palsy is one of the patterns of motor neuron disease (MND) [1] [2] [3] [4]. The term implies the involvement of the brain stem while the disease may be seen either as an isolated bulbar deficit or in conjunction with other upper/lower motor neuron signs [1] [2] [5]. In most cases, bulbar symptoms precede limb defects for a few years [3]. For an undisclosed reason, elderly women seem to be the predominant group of patients that suffer from progressive bulbar palsy and usually the first manifestation is progressive dysarthria (or even anarthria) which could be initially present only after an ingestion of alcohol [1] [2]. Damage to motor neuron structures responsible for speech results in a slow and distorted speech, while dysphagia usually follows the onset of speech difficulties [2] [3]. Over time, virtually all patients with progressive bulbar palsy will develop sialorrhea that occurs as a result of severe dysphagia [3]. In addition to true bulbar manifestations, "pseudobulbar" signs in the form of emotional lability and very common yawning are noted in many patients [2] [4]. Lower motor neuron (LMN) damage can lead to wasting and weakness of the tongue accompanied by fasciculations [3].

• Weakness

  Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. [icd9data.com]

  […] and tongue; and weak palatal movement. [merckmanuals.com]

  The condition results in a weakness in one’s lower limbs and some people also suffer from weakness in their hands and speech issues. [mymed.com]

• Weight Loss

  Fasciculations, spasticity, hyperactive deep tendon reflexes, extensor plantar reflexes, clumsiness, stiffness of movement, weight loss, fatigue, and difficulty controlling facial expression and tongue movements soon follow. [merckmanuals.com]

  In deciding on the timing of gastrostomy the amount of food and liquid taken, the time taken to consume it, recent weight loss, hunger, indices of nutrition and metabolic demand, and whether or not patients wish to continue oral feeding are the most relevant [doi.org]

  rather than a sudden decision based just on weight loss. 20 However, patients, and often their families, find discussion of interventions difficult and may put off decisions. [dovepress.com]

• Dysphagia

  Evaluation of dysphagia is conducted through fiber endoscopy and videofluoroscopy. [symptoma.com]

  Mechanism of dysphagia in the individual patient The dominant mechanism causing neurogenic dysphagia (table 1 ) may differ in two patients with the same disease and can clearly change over time. [doi.org]

  DYSPHAGIA IN PHARYNGEAL PARALYSIS TREATED BY CRICOPHARYNGEAL SPHINCTEROTOMY. The Lancet, Vol. 301, Issue. 7801, p. 455. CrossRef Google Scholar [cambridge.org]

  […] presented of a patient with progressive bulbar palsy, a form of motor neuron disease, which is a progressive degenerative disorder of the motor nuclei in the medulla producing atrophy and fasciculations of the musculature of the tongue, dysarthria, dysphagia [ncbi.nlm.nih.gov]

• Fasciculation of the Tongue

  Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. [icd9data.com]

  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]

• Muscle Weakness

  A 12-month-old boy with progressive cranial nerve palsies followed by ventilatory failure demanding artificial ventilation, generalized muscle weakness, and rapid progression to death at the age of 21 months is described. [ncbi.nlm.nih.gov]

  Symptoms [ ✎ edit | edit source ] muscle weakness (especially pharyngeal, facial and masticatory muscles) loss of speech emotial lability – rapid alternation of laughing and crying tongue muscle atrophy – people are not able to speak or eat, which can [wikilectures.eu]

  Symptoms vary in severity and may include muscle weakness and atrophy, fasciculations, emotional lability, and respiratory muscle weakness. [merckmanuals.com]

  Symptoms are similar to the common form of ALS, though spasticity is absent and muscle weakness is less severe. In addition, individuals… Read More [britannica.com]

• Osteoporosis

  However, patients with PPS commonly develop new disabilities that cause significant morbidity, such as orthopedic complications (eg, progressive instability of joints, osteoporosis, fractures, osteoarthrosis, scoliosis, and spondylosis), respiratory insufficiency [emedicine.medscape.com]

• Muscle Spasticity

  The small muscles of the hands and feet are usually first affected, but muscle spasticity is absent. Primary lateral sclerosis - this is another rare type of MND. It mainly causes weakness in the leg muscles. [patient.info]

• Fracture

  However, patients with PPS commonly develop new disabilities that cause significant morbidity, such as orthopedic complications (eg, progressive instability of joints, osteoporosis, fractures, osteoarthrosis, scoliosis, and spondylosis), respiratory insufficiency [emedicine.medscape.com]

• Myalgia

  Postpolio syndrome Most patients with postpolio syndrome (PPS) present with new, slowly progressive muscle weakness, frequently accompanied by muscle pain (myalgias) and fatigue, which can occur in both previously affected and unaffected muscles. [emedicine.medscape.com]

• Dysarthria

  Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. [icd9data.com]

  Progressive bulbar palsy refers to the onset of brain stem-related symptoms such as dysarthria or swallowing difficulties. The diagnosis rests on a thorough clinical assessment, imaging studies, and neurophysiological testing. [symptoma.com]

  A case is presented of a patient with progressive bulbar palsy, a form of motor neuron disease, which is a progressive degenerative disorder of the motor nuclei in the medulla producing atrophy and fasciculations of the musculature of the tongue, dysarthria [ncbi.nlm.nih.gov]

  Jaw jerk – increased Speech – spastic: “a monotonous, slurred, high-pitched, ‘Donald Duck’ dysarthria” that “sounds as if the patient is trying to squeeze out words from tight lips”. [lifeinthefastlane.com]

• Slurred Speech

  Progressive bulbar palsy usually leads to slurred speech and difficulty swallowing, as Rutgers University details. [reference.com]

  The symptoms are often those of slurred speech and difficulty in swallowing. The life expectancy of a sufferer is between six months to three years from the onset of the initial symptoms. [mymed.com]

  It also includes similar symptoms as PBP – affection of head and neck muscles, swallowing or chewing problems and slurred speech (usually the first symptoms from all). [wikilectures.eu]

  Typical symptoms of bulbar palsy include slowed or paralyzed eye movements, facial weakness, slurred speech (dysarthria or nasal), gait disturbance and dizziness. [dizziness-and-balance.com]

  Yes, I have a son who is an otolaryngologist and a daughter who is a Speech/Language Pathologist who first discovered my condition. Mine began with slurred speech. [medhelp.org]

• Confusion

  This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII).This disorder should not be confused with pseudobulbar palsy or progressive spinal muscular atrophy. [en.wikipedia.org]

  Doctors and patients often use the term motor neurone disease as an umbrella term to describe the condition rather than the actual sub-type of motor neurone disease, which can make things confusing. [leedsth.nhs.uk]

  The corneal stroma contains linear deposits which are more discrete, more peripheral, more delicate, and more radial than those in lattice type I with which it is sometimes confused. [disorders.eyes.arizona.edu]

  First, the diagnosis of progressive bulbar palsy is usually conducted by physical examination and history taking and often confused with other types of MND (such as ALS, primary lateral sclerosis, and progressive muscular atrophy), thus accurate diagnosis [nrronline.org]

  If a patient obviously faces prolonged recovery or is confused and keeps pulling a nasogastric tube out a gastrostomy is more acceptable and allows a far higher proportion of the prescribed diet to be delivered. 14 47-50 In chronic neurodegenerative conditions [doi.org]

• Flaccid Paralysis

  […] diseases characterized by episodic flaccid paralysis or muscular weakness. progressive bulbar paralysis the chronic form of bulbar paralysis; called also Duchenne's disease or paralysis. [medical-dictionary.thefreedictionary.com]

The diagnosis of progressive bulbar palsy mandates a comprehensive workup, starting with a detailed patient history and a thorough physical examination. It is imperative to find out about the onset, the course, and the progression of symptoms in order to raise the clinical suspicion.

During a complete neurological examination, which presents an extremely important part of the workup, physicians can reveal various findings that indicate either upper and/or lower motor neuron damage, such as speech or swallowing difficulties.

Laboratory studies comprised of basic biochemistry, inflammatory markers, serum and urine protein electrophoresis, a hormonal check up (particularly the thyroid gland hormones), and if necessary, analysis of the cerebrospinal fluid (CSF) and heavy metal testing, should be conducted [3].

Imaging studies in the form of magnetic resonance imaging (MRI) are necessary to rule out other pathologies of the central nervous system (CNS) [3]. In making the definite diagnosis, however, electrodiagnostic tests could have a great value [3]. Electromyography (EMG) shows fasciculation potentials and spontaneous denervation discharges [3] [5].

Evaluation of dysphagia is conducted through fiber endoscopy and videofluoroscopy [6].

Thorough coverage for each neurological disease clearly defines age at onset, course of illness, clinical features, and treatment options. Differential diagnosis tables and treatment algorithms expedite clinical decision making. [books.google.com]

http://www.alscenter.org/what-is-als/treatment/index.html The Mayo Clinic provides information on treatment of ALS in general, which may be helpful: http://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/diagnosis-treatment/treatment [rarediseases.info.nih.gov]

Progressive Bulbar Palsy. ‘’The Treatment of Diseases of the Nervous System: A Manual for Practitioners’’. W. Wood and Company. (1900) Swash M, and Desai J. Motor Neuron Disease: Classification and nomenclature. [en.wikipedia.org]

Unfortunately, there is no treatment or cure for this condition. It is a progressive disorder and a fairly aggressive type of condition with little scope for improvement. [tandurust.com]

Is the prognosis any worse for the bulbar palsy type? Dr. Belsh answers: Patients with ALS generally present with symptoms referable either to the limb or bulbar muscles. [web.archive.org]

Progressive bulbar palsy, also known as bulbar onset amyotrophic lateral sclerosis (ALS), is a variant of motor neuron disease (MND) that carries a poor prognosis and significant reduction in the quality of life. [symptoma.com]

The prognosis of the condition is also fairly poor, primarily because the causative factor of this condition is yet not known. Most of the treatment would be symptomatic and there is little hope. [tandurust.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

The progressive bulbar palsy is to be tackled with basic understanding about the pathogenesis and etiology. Copyright - © 2006 - 2019 Privacy Policy - Disclosure [cerebralpalsytreating.com]

Usually, etiology is unknown. Nomenclature and symptoms vary according to the part of the motor system most affected. Myopathies have similar features but are disorders of the muscle membrane, contractile apparatus, or organelles. [msdmanuals.com]

CSF analysis and MRI of the brain help identify the etiology. Treatment is mainly supportive. Prognosis is poor, with a life expectancy of 1–3 years after diagnosis. [amboss.com]

Etiology PSP is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). [orpha.net]

Epidemiology References: [1] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Summary Epidemiology Prevalence is conservatively estimated at about 1/16,600. Clinical description PSP usually manifests during the sixth or seventh decade of life. [orpha.net]

There may be several causes for such oxidative damage to motor neurons and the disease may just represent an end-stage phenotypic expression of these abnormalities. [ 4 ] Epidemiology MND is relatively uncommon with an annual incidence of about 2 cases [patient.info]

Epidemiologic data suggest that smoking may be an established risk factor for sporadic ALS. [8] The peak age of onset is between 55 and 75 years. [emedicine.medscape.com]

Ertekin C et al. (2000) Pathophysiological mechanism of oropharyngeal dysphagia in amyotrophic lateral sclerosis. Brain 123 : 125–140 35. Sasaki CT and Suzuki M (1997) Laryngeal spasm: a neurophysiologic redefinition. [nature.com]

Pathophysiology This is a degenerative condition that affects motor neurons, namely the anterior horn cells of the spinal cord and the motor cranial nuclei. [patient.info]

[…] of self-inflicted trauma: dental intervention to prevent chronic lip chewing by a patient with a diagnosis of progressive bulbar palsy. ( 2978766 ) Rover B.C....Morgano S.M. 1988 10 Progressive bulbar palsy and gammopathy. ( 3658181 ) Smith D.B....Alvarez [malacards.org]

The encyclopedia also explores its subject from a wellness perspective, explaining actions that can prevent neurological disorders and injuries and promote general nervous system health. [books.google.com]

Their methodology is to anticipate the progressive problems and delay them as long as possible, and prevent her from getting hurt (falls, etc.) which is great. [inspire.com]

[…] sudden cardiac death - guidance (IPG603) Source: National Institute for Health and Care Excellence - NICE - 20 December 2017 Evidence-based recommendations on subcutaneous implantable cardioverter defibrillator insertion for preventing sudden cardiac [evidence.nhs.uk]

1. Turner MR, Talbot K. Mimics and chameleons in motor neurone disease. Pract Neurol. 2013;13(3):153-164.
2. Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 2009;4:3.
3. Zarei S, Carr K, Reiley L, et al. A comprehensive review of amyotrophic lateral sclerosis. Surg Neurol Int. 2015;6:171.
4. McDermott CJ, Shaw PJ. Diagnosis and management of motor neurone disease. BMJ. 2008;336(7645):658-662.
5. Sathasivam S. Motor neurone disease: clinical features, diagnosis, diagnostic pitfalls and prognostic markers. Singapore Med J. 2010;51(5):367-372.
6. Kühnlein P, Gdynia HJ, Sperfeld AD, et al. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol. 2008;4(7):366-374.