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. 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.
Progressive bulbar palsy is one of the patterns of motor neuron disease (MND)    . 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   . In most cases, bulbar symptoms precede limb defects for a few years . 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  . Damage to motor neuron structures responsible for speech results in a slow and distorted speech, while dysphagia usually follows the onset of speech difficulties  . Over time, virtually all patients with progressive bulbar palsy will develop sialorrhea that occurs as a result of severe dysphagia . In addition to true bulbar manifestations, "pseudobulbar" signs in the form of emotional lability and very common yawning are noted in many patients  . Lower motor neuron (LMN) damage can lead to wasting and weakness of the tongue accompanied by fasciculations .
Entire Body System
Jaw & Teeth
- Fasciculation of the Tongue
Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. [ebi.ac.uk]
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]
The patient presented with Broca’s aphasia along with typical symptoms of PBP, including dysarthria, dysphasia, and mild quadriparesis. DTT was performed at five years after symptom onset. [omicsonline.org]
A 74-year-old right-handed man visited the rehabilitation department of a university hospital for evaluation of language disturbance and dysphasia. He had no family history of neurological disease. [nrronline.org]
[…] atrophic diffuse G12.22 (progressive) creeping G12.22 Paralysis, paralytic (complete) (incomplete) G83.9 ICD-10-CM Diagnosis Code G83.9 Paralytic syndrome, unspecified 2016 2017 2018 2019 Billable/Specific Code atrophic G58.9 ICD-10-CM Diagnosis Code G58.9 Mononeuropathy [icd10data.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 .
Imaging studies in the form of magnetic resonance imaging (MRI) are necessary to rule out other pathologies of the central nervous system (CNS) . In making the definite diagnosis, however, electrodiagnostic tests could have a great value . Electromyography (EMG) shows fasciculation potentials and spontaneous denervation discharges  .
Evaluation of dysphagia is conducted through fiber endoscopy and videofluoroscopy .
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]
Offers practical, clinically relevant material for the diagnosis and treatment of musculoskeletal conditions. [books.google.com]
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]
Publisher Full Text Successful treatment of a genetic childhood ataxia due to riboflavin transporter deficiency. [unboundmedicine.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]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.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:  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.  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]
Physical therapy can help the patient to keep their muscles functional and prevent some muscle spasms. Another option is a medication, which includes especially anti-depressant (against emotial lability) or some analgesics. [wikilectures.eu]
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- 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.