Thinking Changes in ALS

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Thinking Changes in ALS

• Alison Grossman, PhD
• Department of Neurology, Miller School of
  Medicine, University of Miami

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Background

• ALS was traditionally considered a disease which
  affected the motor system exclusively.
• Recent research has shown that some patients with
  ALS experience changes in cognition, although
  these changes are typically very mild.

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• Common difficulties include thinking of the right
  word to say, sustaining attention, and making
  decisions.
• Since this has not been recognized until
  recently, patients and their family members have
  not been consistently informed of this potential
  issue.

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Objectives

• Discuss the common cognitive and behavioral
  changes exhibited by individuals with ALS.
• Explain how they differ from normal, age-related
  cognitive changes and those seen in other
  diseases.
• Provide strategies for handling them.

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Age-Related Cognitive Changes

• Motor slowing
• Attention
• Word finding
• Slowed thinking
• Memory

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Cognitive Changes in Patients with ALS

• Recent studies have reported that approximately
  one-third to one-half of patients with ALS
  experience some changes in thinking.
• These changes are often very subtle and may not
  be noticed without specific neuropsychological
  testing.

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• Patients with speech or swallowing difficulties
  early on in the disease course may be more
  vulnerable to experiencing cognitive difficulties
  compared with patients whose weakness begins in
  the limbs.
• However, those with ALS disease onset in the
  limbs may also develop thinking difficulties.

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Types of Thinking Changes in ALS

• Concentration
• Shifting attention from one thing to another
• Planning, organization skills
• Decision making, judgment, insight
• Language output
• Changes are often noticed by a concerned family
  member and not the patient.

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Behavioral Changes Associated with ALS

• Lack of initiative or interest (often confused
  with depression)
• Decreased self-control
• Loss of emotional warmth, indifference to others,
  loss of empathy and sympathy

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Frontotemporal Dementia (FTD)

• Typically seen in less than 5 of patients with
  ALS.
• Persistent decline in cognition and behavior
  severe enough to interfere with activities of
  daily living.
• The changes that occur in patients with FTD are
  not the same as those in Alzheimers Disease.

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Other Factors That Affect Cognition

• Depression
• Anxiety
• Sleep problems and fatigue
• Respiratory problems
• Excessive alcohol use
• Certain medications

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Pilot Study on the Prevalence of Cognitive
Impairment in Patients with ALS

• 26 patients diagnosed with ALS were consecutively
  recruited for participation in the study from the
  Kessenich Family MDA ALS Center at the University
  of Miami.
• All study participants were older than 21 years,
  had at least some high school education, and were
  English speaking.

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• Patients were administered a comprehensive
  neuropsychological evaluation in addition to
  their routine neurological exams.

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Results

• Fourteen patients (54) exhibited normal
  performance on all tests administered.
• Ten patients (38) had impaired performance on at
  least two neuropsychological tests.
• Two patients (8) met criteria for a diagnosis of
  dementia.

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Symptom Management

• First, communicate any concerns to the
  neurologist and treatment team.
• Medication
• Modify the environment
• Minimize distractions
• Psychological counseling

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Conclusions

• Cognitive and behavioral changes are now
  recognized as being fairly common in individuals
  with ALS.
• These changes are usually very mild and have
  minimal impact on daily life.
• A small percentage of patients with ALS tend to
  develop FTD.

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Conclusions

• Symptoms can be managed by adopting appropriate
  strategies.
• We strongly encourage both patients and family
  members to discuss cognitive, personality or
  behavioral changes with the health care team so
  that interventions and accommodations can be made.