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Progressive Bulbar Palsy

Bulbar Palsies

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.


Presentation

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].

Pathologist
  • 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]
Dentist
  • 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]
Myxedema
  • […] in diseases classified elsewhere G13.0 Paraneoplastic neuromyopathy and neuropathy G13.1 Other systemic atrophy primarily affecting central nervous system in neoplastic disease G13.2 Systemic atrophy primarily affecting the central nervous system in myxedema[icd10data.com]
Chewing Problem
  • 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). Therapy There is no special treatment, so we can just alleviate the symptoms.[wikilectures.eu]
Hyperhidrosis
  • Transcutaneous microwave ablation for severe primary axillary hyperhidrosis - guidance (IPG601) Source: National Institute for Health and Care Excellence - NICE - 20 December 2017 Evidence-based recommendations on transcutaneous microwave ablation for[evidence.nhs.uk]
Normal Hearing
  • The patient had normal early development and also apparently normal hearing at presentation of illness but, after 6 months of the onset of the disease, hearing loss was documented by brainstem auditory evoked potentials (BAEP).[ncbi.nlm.nih.gov]
Dysphasia
  • 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]
Mononeuropathy
  • […] 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]

Workup

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].

Treatment

  • 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]
  • 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]
  • […] to treat muscle spasms, weakness, drooling, sleep problems, pain, and depression. [1] [3] [2] The Robert Packard Center for ALS Research at John Hopkins offers further general information on treatment: The Mayo Clinic provides information on treatment[rarediseases.info.nih.gov]
  • 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]

Prognosis

  • 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]
  • Can empathise with your fear of ability to meet the challenges ahead. you will still be reeling from the diagnosis and prognosis.[forum.mndassociation.org]
  • ICD-9 : 335.22 ONSET AND PROGRESSION The prognosis for PBP is poor. The symptoms of PBP slowly worsen with onset beginning between the ages of 50-70 years.[secure.ssa.gov]
  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • […] 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]
  • 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]
  • […] 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]

References

Article

  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.

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Last updated: 2019-06-28 10:19