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
Read Summary - More: Guidance Tissue pathways for non-neoplastic neuropathology specimens Source: Royal College of Pathologists - RCPATH - 01 January 2018 - Publisher: Royal College of Pathologists This document gives the Royal College of Pathologists [evidence.nhs.uk]
Journal of Oral Pathology & Medicine : Official Publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology, 31 (5), pp. 277-9. Cerero Lapiedra R, Moreno López LA, Esparza Gómez GC. [unboundmedicine.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]
We would like to emphasize the role of general dentists in the diagnosis of systemic conditions based on an oral examination that should include the oro-facial muscles. [ncbi.nlm.nih.gov]
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 .
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