While neurofibromatosis often appears in children as visible spots, freckles or tiny bumps, the genetic disorder can also cause learning disabilities, including problems with attention, working memory, inhibition and planning. Up to 40% of children with neurofibromatosis type 1 (NF1) are diagnosed with Attention Deficit/Hyperactivity Disorder (ADHD). These symptoms can often cause issues at home and school, yet there are few evidence-based interventions to improve these cognitive dysfunctions. In a recent pilot study, the Cogmed learning program was evaluated as a possible method to counteract these deficits.
Caused by a genetic defect on chromosome 17, NF1 causes tumors to form on nerve tissue anywhere in the central nervous system, including the brain, spinal cord and nerves. On a healthy gene, a protein called neurofibromin manages normal cell growth but the mutated NF1 does not produce enough neurofibromin allowing cells to multiply and tumors to form.
Typically, the tumors are benign with mild aesthetic symptoms. However, because of their location on the CNS, they can affect other important functions, including hearing, vision, heart and blood vessels, pain, as well as cognitive impairments.
As an initial pilot test to see if a larger clinical trial is warranted, researchers reported on a study measuring the effectiveness of Cogmed working memory exercises on core cognitive functioning. They also used resting state fMRI imaging to assess changes in functional connectivity to understand the neural correlates of working memory and the changes caused by working memory training.
Sixteen children between the ages of 8 and 15 years with diagnoses of NF1 and working memory issues were enrolled in the study, with their parents’ consent. First, they underwent fMRI scans and baseline cognitive testing using the Cogstate battery (not affiliated with Cogmed). This collection of six computerized tests, Detection, Identification, One Back, One Card Learning, Continuous Paired Associate Learning and Groton Maze Learning, was chosen “due to its high test-retest reliability and negligible practice effects in healthy individuals across different exam settings.” By using a standardized, independent cognitive test battery with a large normative database of age-matched participants, the efficacy of the Cogmed training program could be measured before and after.
Cogmed is a computer-based exercises program focused on improving attention by increasing working memory capacity. The Cogmed training consisted of approximately 25 sessions at home, each lasting 30-45 minutes, over 6-10 consecutive weeks.
At the conclusion of Cogmed training, the children were assessed again with resting fMRI and Cogstate testing. The imaging study showed, significant regional differences in two of the four tested resting state measures: fractional amplitude of low frequency fluctuations (fALFF) and regional homogeneity (ReHo). Improvement was seen in two of the Cogstate tests, Identification and Groton Maze Learning, indicating progress in attention and executive function.
The research has been published in Psychiatry Research: Neuroimaging.
While the sample size was small, the pilot test achieved its goal of initial findings. “This pilot investigation revealed preliminary evidence of Cogmed training effects on neural measures and cognitive performance in a small sample of children with NF1,” concluded the researchers.
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