[22] Neurodegenerative Conditions

• Motor Neuron disease

  • atrophy of neurons in
  • precentral gyrus
  • frontal lobe/ventral lobe of spinal cord
• MND

  mean disease: 3 years

  prolongs by: 3 month

  associated in FTD
• TDP-43

  • commonly found in surviving motor neurons
  • pathological cause of FTD
• Amyotrophic Lateral Sclerosis

  • lateral and anterior columns of the spinal cord
  • demyelination of axons
  • TDP-43: C9orf43
  • Sporadic and hereditary
  • 55-75 years old
  • men > women
  • caucasians and non-Hispanics
• Manifestation of ALS

  • muscle weakness & atrophy
  • fasciculation (twitching)
  • distal to proximal; limbs first
• Swallowing Difficulties of ALS in Oral Prep

  • saliva
  • chewing food
  • drooling
  • residue in mouth
  • food spilling outside mouth
• Swallowing Difficulties of ALS in Preparatory Stage

  • food sticks in throat
  • coughing/choking
  • shortness of breath
  • spilling through nose
• Swallowing Difficulties of ALS in Esophageal Stage

  • food stick in throat and back of the neck
  • regurgitation
• direct pathways inhibits IGP

  indirect pathways inhibits EGP, prevent subthalamic nucleus, activates IGP
• dopamine encourages direct, and inhibits indirect

  acetylcholine encourage indirect, and inhibits direct
• Parkinson's Disease

  • common movement disorder
  • loss of dopamine in substantia nigra
  • mean duration: 12.4 years
• PD Etiology

  • multifactorial disease
  • mean onset: 60% years old
  • sporadic
  • 10-15% genetic
  • alpha-synuclein protein found in 40% Lewy body dementia
• PD Speech and Swallowing Difficulties

  • dysphonia; aphonia
  • hypokinetic dysarthria
  • swallowing
  • cognitive dysfunction: heterogenous
• PD Specific Speech Intervention

  • LSVT: Lee Silverman Voice Treatment