II. Epidemiology

  1. Onset age 50-70 years

III. Pathophysiology

  1. Subtype of Amyotrophic Lateral Sclerosis (ALS)
  2. Cranial Nerve motor nuclear atrophy, glial overgrowth
    1. Cranial Nerve 5
    2. Cranial Nerve 7
    3. Cranial Nerve 9
    4. Cranial Nerve 10
    5. Cranial Nerve 12
  3. Subcortical involvement of corticobulbar tracts

IV. Symptoms

  1. Drooling
  2. Difficult chewing
  3. Dysphagia
  4. Dysarthria
  5. Nasal regurgitation

V. Signs

  1. Arm and leg spasticity
  2. Hyperactive reflexes
  3. Ophthalmoplegia
  4. Fasciculation of Tongue and lip Muscles
  5. Emotional lability

VI. Differential Diagnosis

  1. Acute Bulbar Paralysis
    1. Acute CNS vascular lesion (cerebral Hemorrhage, Ischemic CVA)
    2. Acute Bulbar Polioencephalitis
  2. Chronic Bulbar Paralysis
    1. Syringobulbia
    2. Multiple Sclerosis
    3. Myasthenia Gravis

VII. Course

  1. Progresses to Aspiration Pneumonia, respiratory arrest
  2. Death in 1 to 3 years from onset

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Ontology: Bulbar Palsy, Progressive (C0030442)

Definition (NCI) 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.
Definition (MSH) A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)
Concepts Disease or Syndrome (T047)
MSH D010244
ICD9 335.22
ICD10 G12.22
SnomedCT 230547002, 54304004
English Bulbar Palsies, Bulbar Palsy, Bulbar Paralyses, Bulbar Paralysis, Palsies, Bulbar, Paralysis, Bulbar, BULBAR PALSY, PARALYSIS BULBAR, Palsy, Bulbar, Bulbar palsies, progressive bulbar palsy (diagnosis), progressive bulbar palsy, Paralysis bulbar, Bulbar Palsies, Progressive, Bulbar Palsy, Progressive, Palsies, Progressive Bulbar, Palsy, Progressive Bulbar, Progressive Bulbar Palsies, Progressive Bulbar Palsy, Bulbar Palsy, Progressive [Disease/Finding], bulbar palsies, bulbar palsy progressive, bulbar palsy, bulbar paralysis, Bulbar palsy (disorder), Progressive bulbar palsy, PBP - Progressive bulbar palsy, Bulbar palsy, Bulbar paralysis, Progressive bulbar palsy (disorder), bulbar; paralysis, paralysis; bulbar
French PARALYSIE BULBAIRE, Paralysie bulbaire, Paralysie bulbaire progressive
Portuguese PARALISIA BULBAR, Paresia Bulbar Progressiva, Paralisia bulbar, Paralisia progressiva bulbar, Paralisia Bulbar, Paralisia Bulbar Progressiva
Spanish PARALISIS BULBAR, Paresia Bulbar Progresiva, parálisis bulbar progresiva (trastorno), parálisis bulbar progresiva, parálisis bulbar, Parálisis bulbar progresiva, Parálisis bulbar, Parálisis Bulbar Progresiva, Parálisis Bulbar
Dutch paralyse bulbair, bulbaire paralyse, bulbair; paralyse, paralyse; bulbair, bulbaire verlamming, progressieve bulbairparalyse, Bulbaire paralyse, progressieve, Bulbaire progressieve paralyse, Paralyse, bulbaire, Paralyse, bulbaire progressieve, Progressieve bulbaire paralyse
German Paralyse bulbaer, Bulbaer-Paralyse, BULBAERPARALYSE (DURCH ISCHAEMIE), BULBAERPARALYSE, Bulbaerparalyse, progressive Bulbaerparalyse, Bulbärparalyse, Bulbärparalyse, progressive, Paralyse, Bulbär-
Swedish Bulbär pares, progressiv
Japanese シンコウセイキュウマヒ, キュウマヒ, 延髄球不全麻痺, 延髄不全麻痺, ファチオ-ロンデ症候群, 進行性球麻痺, 麻痺-延髄, ファチオ-ロンデ病, ファチオ-ロンド症候群, ファチオ-ロンデ萎縮, 麻痺-球, ファチオ-ロンド病, ファチオ-ロンド萎縮, 延髄麻痺, 球麻痺-進行性, 延髄球麻痺, Fazio-Londe病, 球麻痺
Czech paralýza bulbární, bulbární obrna progresivní, Bulbární paralýza, Bulbární progresivní obrna, Bulbární obrna
Finnish Etenevä bulbaariparalyysi
Italian Paralisi bulbare, Paralisi bulbare progressiva
Polish Porażenie opuszkowe postępujące, Porażenie opuszkowe, Zespół Fazio-Londe
Hungarian Progressiv bulbaris bénulás, Bulbaris paralysis, Bulbaris paresis, Paralysis bulbaris
Norwegian Progressiv bulbærparese