Central Nervous System - DM2

Patterns:

  • Specific cognitive defects may be seen in DM2, but they are believed to be milder than those seen in all forms DM1. In addition to the primary alteration in brain function thought to be caused by the DM2 genetic mutation, there may be contributions from the disordered sleep patterns or the hormonal or other systemic abnormalities seen in the disorder.

  • Reduced blood flow in the frontal and temporal lobes of the brain.

Symptoms:

  • Cognitive and behavioral abnormalities can involve deficits in intelligence, executive function, visual-spatial construction, thinking and problem solving, and attention. The scope and degree of these symptoms may vary.

  • Lack of executive function which can cause great difficulty in planning and organizing one’s life, affecting areas such as paying bills, keeping appointments, and arranging schedules.

  • Mental health disorders including depression.

  • Apathy.

Diagnosis:

  • See a mental health care professional (psychologist or psychiatrist) in order to diagnose psychiatric or behavioral abnormalities.

  • Test for psychiatric or behavioral issues and cognitive changes as a part of your annual exam with your physician.

  • A baseline neuropsychological evaluation is recommended with your doctor. Additional testing can be dictated by your physicians recommendations for your clinical course.

Treatment:

  • Discuss the possible use of psychostimulants with your doctor if you have apathy and an impairing level of fatigue or excessive daytime sleepiness.

  • Discuss the possible use of anti-depressive medication with your doctor as well. Before starting treatment your doctor should do a cardiac examination including a 12 lead ECG (electrocardiogram).

© Myotonic Dystrophy Foundation. All rights reserved.