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Progressive bulbar palsy

MedGen UID:
18290
•Concept ID:
C0030442
•
Disease or Syndrome
Synonyms: Bulbar Palsies, Progressive; Bulbar Palsy, Progressive; Palsies, Progressive Bulbar; Palsy, Progressive Bulbar; Progressive Bulbar Palsies
SNOMED CT: Bulbar palsy (54304004); PBP - Progressive bulbar palsy (54304004); Progressive bulbar palsy (54304004); Bulbar paralysis (54304004)
 
Monarch Initiative: MONDO:0008890

Definition

A progressive motor neuron disorder affecting the muscles which are innervated by cranial nerves of the lower brain stem. Signs and symptoms include difficulties in chewing and swallowing, dysarthria, and weakness of the facial muscles and tongue. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive bulbar palsy

Professional guidelines

PubMed

Immunosuppressive treatment of patients with amyotrophic lateral sclerosis.
Werdelin L, Boysen G, Jensen TS, Mogensen P
Acta Neurol Scand 1990 Aug;82(2):132-4. doi: 10.1111/j.1600-0404.1990.tb01602.x. PMID: 2256442

Recent clinical studies

Etiology

Riboflavin in Neurological Diseases: A Narrative Review.
Plantone D, Pardini M, Rinaldi G
Clin Drug Investig 2021 Jun;41(6):513-527. Epub 2021 Apr 22 doi: 10.1007/s40261-021-01038-1. PMID: 33886098
Autophagy and Motor Neuron Diseases.
Zhang X, Yang K, Le W
Adv Exp Med Biol 2020;1207:53-74. doi: 10.1007/978-981-15-4272-5_3. PMID: 32671738
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients.
Hu Q, Li H, Tian J, Zhang B
Neurol Sci 2018 Feb;39(2):305-311. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3184-2. PMID: 29124441
Recent advances in bulbar syndromes: genetic causes and disease mechanisms.
Manole A, Fratta P, Houlden H
Curr Opin Neurol 2014 Oct;27(5):506-14. doi: 10.1097/WCO.0000000000000133. PMID: 25159929

Diagnosis

Benefit of high-dose oral riboflavin therapy in riboflavin transporter deficiency.
Fennessy JR, Cornett KMD, Burns J, Menezes MP
J Peripher Nerv Syst 2023 Sep;28(3):308-316. Epub 2023 Aug 24 doi: 10.1111/jns.12587. PMID: 37537696
Herbal medicine and acupuncture relieved progressive bulbar palsy for more than 3 years: A case report.
Peng S, Chang W, Tian Y, Yang Y, Li S, Ni J, Zhu W
Medicine (Baltimore) 2022 Nov 11;101(45):e31446. doi: 10.1097/MD.0000000000031446. PMID: 36397351Free PMC Article
Riboflavin in Neurological Diseases: A Narrative Review.
Plantone D, Pardini M, Rinaldi G
Clin Drug Investig 2021 Jun;41(6):513-527. Epub 2021 Apr 22 doi: 10.1007/s40261-021-01038-1. PMID: 33886098
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Brown-Vialetto-Van Laere syndrome.
Sathasivam S
Orphanet J Rare Dis 2008 Apr 17;3:9. doi: 10.1186/1750-1172-3-9. PMID: 18416855Free PMC Article

Therapy

Benefit of high-dose oral riboflavin therapy in riboflavin transporter deficiency.
Fennessy JR, Cornett KMD, Burns J, Menezes MP
J Peripher Nerv Syst 2023 Sep;28(3):308-316. Epub 2023 Aug 24 doi: 10.1111/jns.12587. PMID: 37537696
Herbal medicine and acupuncture relieved progressive bulbar palsy for more than 3 years: A case report.
Peng S, Chang W, Tian Y, Yang Y, Li S, Ni J, Zhu W
Medicine (Baltimore) 2022 Nov 11;101(45):e31446. doi: 10.1097/MD.0000000000031446. PMID: 36397351Free PMC Article
Riboflavin in Neurological Diseases: A Narrative Review.
Plantone D, Pardini M, Rinaldi G
Clin Drug Investig 2021 Jun;41(6):513-527. Epub 2021 Apr 22 doi: 10.1007/s40261-021-01038-1. PMID: 33886098
Recent advances in bulbar syndromes: genetic causes and disease mechanisms.
Manole A, Fratta P, Houlden H
Curr Opin Neurol 2014 Oct;27(5):506-14. doi: 10.1097/WCO.0000000000000133. PMID: 25159929
Brachial-plexus injuries.
Leffert RD
N Engl J Med 1974 Nov 14;291(20):1059-67. doi: 10.1056/NEJM197411142912005. PMID: 4606284

Prognosis

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.
Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244
Riboflavin in Neurological Diseases: A Narrative Review.
Plantone D, Pardini M, Rinaldi G
Clin Drug Investig 2021 Jun;41(6):513-527. Epub 2021 Apr 22 doi: 10.1007/s40261-021-01038-1. PMID: 33886098
Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients.
Hu Q, Li H, Tian J, Zhang B
Neurol Sci 2018 Feb;39(2):305-311. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3184-2. PMID: 29124441
Emergencies in motoneuron disease.
Finsterer J, Stöllberger C
Intern Emerg Med 2017 Aug;12(5):641-650. Epub 2017 Mar 9 doi: 10.1007/s11739-017-1644-6. PMID: 28280980
Brown-Vialetto-Van Laere syndrome.
Sathasivam S
Orphanet J Rare Dis 2008 Apr 17;3:9. doi: 10.1186/1750-1172-3-9. PMID: 18416855Free PMC Article

Clinical prediction guides

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.
Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244
Recent advances in riboflavin transporter RFVT and its genetic disease.
Jin C, Yonezawa A
Pharmacol Ther 2022 May;233:108023. Epub 2021 Oct 15 doi: 10.1016/j.pharmthera.2021.108023. PMID: 34662687
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population.
Zhang HG, Chen L, Tang L, Zhang N, Fan DS
Chin Med J (Engl) 2017 Aug 5;130(15):1768-1772. doi: 10.4103/0366-6999.211538. PMID: 28748847Free PMC Article
AALSS.
Yen A, Simpson E, Haverkamp L, Appel S
Amyotroph Lateral Scler Other Motor Neuron Disord 2004 Sep;5 Suppl 1:94-8. doi: 10.1080/17434470410019708. PMID: 15512884

Recent systematic reviews

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.
Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244
Hypothalamic-Bulbar MRI Hyperintensity in Anti-IgLON5 Disease with Serum-Restricted Antibodies: A Case Report and Systematic Review of Literature.
Tagliapietra M, Frasson E, Cardellini D, Mariotto S, Ferrari S, Zanusso G, Plebani M, Monaco S
J Alzheimers Dis 2021;79(2):683-691. doi: 10.3233/JAD-201105. PMID: 33337376
Secondary hypokalemic paralysis with bulbar weakness and reversible electrophysiologic abnormalities: A case report and systematic review.
Ignacio KHD, Bagnas MAC, Espiritu AI, Reyes JPBT
J Clin Neurosci 2019 Dec;70:254-257. Epub 2019 Aug 19 doi: 10.1016/j.jocn.2019.08.063. PMID: 31439490

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