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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].

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]
  • Offers practical, clinically relevant material for the diagnosis and treatment of musculoskeletal conditions.[books.google.com]
  • Except for the rate of depression, patients with PBP-N did not differ from patients with PBP-A in the basic demographics, time of presentation, clinical course, survival and treatment received.[ncbi.nlm.nih.gov]
  • […] 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]

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]
  • Because the condition is degenerative, the prognosis for PBP is fairly poor.[disability-benefits-help.org]

Etiology

  • The progressive bulbar palsy is to be tackled with basic understanding about the pathogenesis and etiology. Copyright - 2006 - 2018 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]
  • Muscular manifestation of amyloidosis is extremely rare but must be considered in the differential diagnosis of both bulbar paralysis and myopathic syndromes of unknown etiology.[thieme-connect.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

  • This typically involves symptomatic treatments that are frequently used in many lower motor disorders. [ citation needed ] Epidemiology [ edit ] Progressive Bulbar Palsy is slow in onset, with symptoms starting in most patients around 50–70 years of age[en.wikipedia.org]
  • Epidemiology References: [1] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.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]
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]
  • Discusses physical agents and therapeutic exercise in the prevention, diagnosis, treatment and rehabilitation of disorders that produce pain, impairment, and disability.[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]
  • Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. ‎ Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. ‎[books.google.es]

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: 2018-06-21 22:32