Muscle: Bulbar

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Bulbar Weakness or Dysfunction

From Bramwell: Atlas of Clinical Medicine

ALS with Bulbar involvement

Nerve
- HMSN
  - 1A: PMP-22 Point mutation
  - 1D: EGR2
  - 2A2A: MFN2
  - 2C: TRPV4
  - 2: JAG1
  - GDAP1
    - CMT 4A
    - Axonal CMT with hoarseness (CMT 2K)
  - 4F, Adult onset: PRX
- Distal HMN VII, Dominant
  - SLC5A7: 2q12
  - Dynactin: 2p13; Dominant
- Scapuloperoneal SMA: TRPV4; 12q24
- Neuromyotonia (Isaacs)
- HSN + Cough & GE reflux: 3p22
- HNA: SEPT9; 17q25
- Recurrent laryngeal nerve lesion
- Vincristine
Other
Laryngeal abductor paralysis
- Ataxia + Laryngeal abductor paralysis & Motor neuropathy
- Multiple system atrophy
Congenital
- Laryngeal nerve palsy
  - Hereditary
  - Associated structural anomalies
    - Bronchogenic cysts
    - Cleft larynx
    - Esophageal atresia
- Causes, Other
  - Idiopathic
  - Brainstem pathology
    - Arnold-Chiari: May have young adult onset
    - Nuclear dysgenesis
  - Myasthenia gravis, Congenital
  - Myotonic dystrophy
  - Spinal muscular atrophy
  - Charcot-Marie-Tooth & HNA
  - Hereditary: Dominant most common
  - Congenital fibrosis of extraocular muscles

Arnold-Chiari:
Bilateral Vocal Cord Paralysis
Cervical syrinx

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6/19/2017