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Progressive Bulbar ParalysisAka: Progressive Bulbar Palsy, Duchenne's Syndrome, Duchenne's Paralysis, Labioglossopharyngeal Paralysis, Labioglossolaryngeal Paralysis, Bulbar Paralysis

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  1. Epidemiology
    1. Onset age 50-70 years
  2. 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
  3. Symptoms
    1. Drooling
    2. Difficult chewing
    3. Dysphagia
    4. Dysarthria
    5. Nasal regurgitation
  4. Signs
    1. Arm and leg spasticity
    2. Hyperactive reflexes
    3. Ophthalmoplegia
    4. Fasciculation of Tongue and lip muscles
    5. Emotional lability
  5. Differential Diagnosis
    1. Acute bulbar paralysis
      1. Acute CNS vascular lesion (hemorrhage or thrombosis)
      2. Acute Bulbar Polioencephalitis
    2. Chronic bulbar paralysis
      1. Syringobulbia
      2. Multiple Sclerosis
  6. Course
    1. Progresses to Aspiration Pneumonia, respiratory arrest
    2. Death in 1 to 3 years from onset

Bulbar Palsy, Progressive (C0030442)

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)
ConceptsDisease or Syndrome (T047)
ICD9335.22
MSHD010244
EnglishBulbar Palsies, Bulbar Palsy, Bulbar Paralyses, Bulbar Paralysis, PARALYSIS BULBAR, PBP - Progressive bulbar palsy, Progressive Bulbar Palsies, Progressive Bulbar Palsy
Spanishparalisis bulbar, paralisis bulbar progresiva
Parent ConceptsMotor Neuron Disease (C0085084), Brain stem disorder (C0151532), Motor Neuron Disease, Upper (C0521659), Multiple cranial nerve lesions and linked syndromes (C0553759), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, CST, DXP, ICD9CM, MSH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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