Cognitive Rehabilitation in Patients With Spina Bifida
Condition: Executive Dysfunction; Cognitive Deficits; Spina Bifida
MeSH terms: Spinal Dysraphism; Cognition Disorders; Cognitive Dysfunction
Study type: Interventional

Summary: 
      This study will examine the effects of a theoretically grounded and manualized cognitive
      rehabilitation program on patients with Spina Bifida (SB). SB is often associated with
      deficit in higher order control over cognition, emotion and behaviour; which is typically
      referred to as executive functions. The present study will examine the efficacy of Goal
      Management Training (GMT) in patients with SB that experience executive difficulties. It is
      expected that GMT will have a favourable effect on cognitive executive functioning,
      psychological and health related factors.
    

Detailed description: 
      INTRODUCTION:

      Rationale and purpose:

      Rehabilitation of impaired cognitive processes has come to be a standard component of medical
      care after traumatic brain injury (TBI) or stroke (Cicerone, Dahlberg, Malec, Langenbahn,
      Felicitti, Kneipp, et al., 2005), and sometimes for patients with infections of the brain,
      hypoxic brain damage, and progressive conditions (Wilson, 2008).

      The objective of this study is to determine the effects of a recently developed cognitive
      rehabilitation program; Goal Management Training (GMT) on the executive functioning in people
      with SB that have cognitive complaints. To date, research efforts have only focused on
      categorizing cognitive impairments in SB with no research directed towards rehabilitation for
      these impairments. Given the extent, nature, and ramifications of cognitive impairments in
      SB, studies on compensatory-based cognitive rehabilitation that teaches management strategies
      such as GMT may be beneficial for this population. The research questions in this study are
      (1) What effect does GMT have on cognitive executive functions? Furthermore, what effect does
      GMT have on subjective and informant evaluation of executive functioning? (2) What effect
      does GMT have on mental health and quality of life? (3) What effect does GMT have on coping
      strategies? There will be a 6 months follow-up with regard to the research questions. It is
      expected that GMT will have a favourable effect on executive functioning, psychological and
      health related factors in patients with SB.

      BACKGROUND:

      Medical and cognitive aspects in Spina bifida (SB):

      SB is a birth defect caused by incomplete neural tube development, resulting in a protrusion
      of spinal cord, meninges, and nerve roots through an opening in the spine. SB is a disorder
      associated with a variety of brain abnormalities, usually including a congenital malformation
      of the cerebellum and hindbrain (Chiari II) and in about half the cases, partial dysgenesis
      of the corpus callosum (Barkovich, 2000). Hydrocephalus occurs in 95% of children with this
      disorder, with 80-90% requiring shunting (Fletcher et al., 2005). SB represents a complicated
      series of neural insults that begins prior to birth, with persisting effects on development,
      including problems in the orthopaedic, cognitive, and behavioural domains (Barkovich, 2000;
      Dicianno, Kurowski, Yang, et al., 2008).

      Executive dysfunction:

      Executive functions are higher level cognitive operations involved in the control and
      direction of lower level functions. Patients with executive dysfunction may experience,
      problems in dealing with novel situations, problems forming a reasonable plan that takes into
      account the relevant details, problems inhibiting habitual responses to situations when these
      are inappropriate, increased distractibility, problems in sustaining attention to task over
      time and keeping goals on-line, impaired monitoring and error-correction of behavioural
      output, low motivation, lack of foresight regarding the effects of one's behaviour,
      difficulty in regulating emotional state, and poor insight into one's difficulties (Levine,
      Stuss, Winocur, Binns, et al., 2007). Executive dysfunction will be examined in the present
      study because of the major implications deficits in this cognitive domain have for patients.

      Although executive dysfunction is often associated with frontal lobe damage, it can also
      result from damage to other brain areas. Damage to the frontal-subcortical white matter
      circuits, which commonly occurs in SB, can disrupt communication between the prefrontal
      cortex and other areas of the brain (Dennis et al., 2006).

      Cognitive rehabilitation:

      Cognitive rehabilitation can be defined as a process whereby people with brain injury work
      together with professional staff and others to remediate or alleviate cognitive deficits
      arising from a neurological insult (Wilson, 2008). Treatment goals may vary, but the major
      goal of cognitive rehabilitation is to enable people with disabilities to function as
      adequately as possible in their own environments (Wilson, 2008). The effectiveness of
      cognitive rehabilitation is well documented within some domains for patients with stroke and
      TBI (e.g., Cicerone et al., 2000, 2005; Wilson, 2008; Rees et al., 2007).

      The cognitive domain in focus of the present study will be executive functioning.
      Interventions within this domain, such as Goal Management Training (GMT), include those
      explicitly directed towards bridging the gap between intention and action, a deficit
      described as "goal neglect" with interventions targeted towards re-establishing endogenous
      control of behaviour.

      Goal Management Training (GMT):

      The present study has translated, and will use an intervention protocol that was originally
      developed to teach patients with brain injury a strategy to improve their ability to plan
      activities and to structure intentions; GMT. GMT aims to increase participants' understanding
      of their own goal management problems, to give them a vocabulary to describe the problems,
      and to give them a set of techniques to compensate for them.

      GMT has been evaluated in 30 patients with mild to severe brain injury, who were randomly
      assigned to groups who received GMT or motor skill training. Participants who followed the
      GMT showed significant gains on everyday paper-and-pencil tasks designed to mimic tasks that
      are problematic for patients with deficits in executive functioning (Levine, Robertson,
      Clare, Carter, Hong, & Wilson, et al., 2000). Furthermore, Levine et al. (2007) have also
      applied a version of this protocol in a sample of 49 elderly with subjective cognitive
      complaints where results indicated improvements in simulated real-life tasks and self-rated
      executive deficits. These gains where maintained at long-term follow-up. Moreover, in a Dutch
      study (van Hooren et al., 2007) involving 37 older adults with executive difficulties, the
      participants in the intervention group were significantly less annoyed by their cognitive
      failures, were better able to manage their executive failures and reported less anxiety
      symptoms than those in the waiting list control group after receiving GMT.

      METHODS:

      The study is an experimental repeated measures design with one treatment group (n=24) and one
      control group (n=14), total (n=38).

      Procedure:

      All the patients between the age of 20 and 45 registered at TRS national resource centre for
      rare disorders in Norway have been asked to participate in the study (n=201). Along with the
      invitation was a self-report questionnaire, Behavior Rating Inventory of Executive
      Function-Adult Version (BRIEF-A), which the respondents had to fill out and return. Inclusion
      of patients was based upon one or more elevated scales on the Metacognition Index (MI).
      Fifty-three (n=53) subjects responded and returned the BRIEF-A questionnaire, and all
      fulfilled the inclusion criteria.

      The baseline measurement of fifty-three (n=53) subjects in this randomised controlled trial
      (RCT) have been done. At baseline the participants filled out questionnaires concerning
      cognitive functioning, mental health, quality of life, and coping. Furthermore,
      neuropsychological assessments were conducted. Additionally, the Dysexecutive Questionnaire
      and BRIEF-A informant report form were filled out by an adult informant who were familiar
      with the rated individual's everyday functioning. Six subjects were excluded at baseline
      because they met the exclusion criteria, six subjects met the inclusion criteria but could
      not follow the programme at the time being because of hospitalization/illness, and three
      subjects could not follow the programme at the time being because of school/education. After
      the baseline measurement the participants were randomly assigned to GMT or control group
      (waiting list). The method of randomisation was a block design with block size 2, with
      stratification for age and education.

      Twenty-four (n=24) subjects have been assigned to GMT, with six subjects in each GMT training
      group. The GMT is structured into seven modules. Each module is designed to run for
      approximately two hours. As such, the subjects will stay at TRS for three days and go through
      module 1 and 2. Then they go home for a month, come back and stay for three days while going
      through modules 3, 4 and 5. Once more, they go home for a month, come in and go through
      modules 6 and 7. GMT consists of tasks performed during training designed to illustrate goal
      management concepts in action, and homework assignments designed to facilitate transfer of
      the concepts to real life. Both the control group and the intervention group will be assessed
      immediately after the intervention group has completed the intervention, and after 6 months.
    

Intervention name(s): Goal management training (GMT)
Intervention type(s): Behavioral
Intervention description(s): Fourteen hours of GMT during a time period of three months.
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Primary purpose: Treatment