© 2017, GENASSIST, Inc. 

By Keith S. Wexler, MBA, Maternal Fetal Medicine, Prenatal Diagnosis and Biotech/Life Sciences Consultant,

Paul Wexler, M.D., F.A.C.O.G., Medical Director, GENASSIST, Inc.

Clinical Professor, Department of OB/GYN, University of Colorado Health Sciences Center

Clinical Professor, Division of Genetics/Dept. of Pediatrics, Univ. of Colorado/The Children’s Hospital

Definitions:

  • Dementia: A chronic or persistent disorder of the mental processes caused by brain disease or injury or marked by memory disorder, personality change or impaired reasoning.

Incidence:

  • Approximately 1 in 14 after 65 years of age
  • Approximately 1 in 8 after 80 years of age

Many risk factors increase likelihood of Dementia:

  • Increasing age
  • High blood pressure
  • Stroke
  • Heart disease
  • Diabetes
  • High cholesterol
  • Smoking
  • Decreased exercise
  • Alcohol
  • Depression

Fronto-Temporal Dementia (PICK Disease): First described by Dr. Arnold Pick in 1892.  It is only one type of Fronto-Temporal Dementia.

Symptoms: Affects areas of the brain associated with personality, behavior, speech, language problems, movement disorders. Memory loss as seen as a prominent symptom in Alzheimer’s may not develop until later in the course of the disease. It often presents earlier than classical Alzheimer’s, often in the 40’s or 50’S. Although life span may be shortened, a relatively long course is common.

Incidence: Approximately 4-15/100,000

  • 20-50% risk if a 1st degree relative has the disease
  • 50-80% of cases are sporadic

Treatment:

  • Selective Serotonin Reuptake Inhibitors (SSRI’s) e.g. fluoxetine, citalopram, sertraline
  • Occupational Therapy (OT)
  • Physical Therapy (PT)
  • Psychotherapy
  • Antidepressants

Genes That May Be Involved:

  • MAPT (17q21.1) – Microtubule Associated Protein Tau
  • GRN [PGRN] (17q21.32) – Granulin (Progranulin Protein)
  • TARDBP (1p36.22) – Transactive Response DNA Binding Protein
  • VCP (9p13.3) – Valosin Containing Protein
  • CHMP2B (3p11.2) Charged Multi Vesicular Body Protein 2B/ Chromatin Modifying Protein 2B