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Frontotemporal dysfunction

Management and treatment for frontotemporal dysfunction in motor neurone disease is an emerging area.

Miller and others 2009b

What is the effect of cognitive or behavioral impairment on management of patients with ALS?

  • In patients with ALS with frontal lobe impairment, noncompliance with PEG (72%) and NIV (75%) was greater, and survival was shorter by 11 months, compared to cognitively normal patients with ALS (Class III).
  • Conclusion
    • Insufficient data exist on the effect of cognitive or behavioral impairment on the management of patients with ALS.
  • Recommendation
    • There are insufficient data to support or refute the impact of cognitive and behavioral impairment on management in ALS (Level U).

What treatments are effective for cognitive or behavioral impairment in ALS?

  • No studies were identified that evaluated pharmacologic treatment for cognitive or behavioral impairment in ALS. One Class III study found an improvement in cognition after NIV initiation.
  • Conclusions
    • Data are inadequate regarding the effect of pharmacologic treatment or NIV for cognitive or behavioral impairment in ALS.
  • Recommendation
    • There are insufficient data to support or refute treatment of cognitive or behavioral impairment in ALS (Level U).

Hardiman 2007

Neuropsychological and psychiatric services may be required to manage the implications of evolving frontal dysexecutive syndrome, and to provide appropriate pharmacotherapy for the associated cognitive decline.

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