The term cognitive function refers to the group of functions performed by the brain. These brain functions include attention, short and long term memory, language learning, problem solving, decision making, speech-fluency, auditory and visual functions [1].

What happens to cognitive functions in Spina Bifida?

Spina Bifida is a neurological disorder with physical and neurosychological damage where a large number of individuals with Spina Bifida develop hydrocephalus and other disabilities of posterior cortex, midbrain, cerebellum and white matter. Therefore, intellectual skills among individuals with SB are often found altered or below average [2]. Cognitive deficits in SB may impair the patients’ academic ability, motor ability, verbal memory [3], perception and speech fluency. Thus, it is correct to conclude that SB has a great impact on a person’s behavioural outcomes [4].

In patients with SB who develop hydrocephalus intra-cranial pressure (ICP) may be raised. The raised ICP may further affect the cognitive disabilities. Usually, in SB patients hydrocephalus or raised ICP alone does worsen the situation; however, it only happens when both the conditions exist together. Sometimes, the patients with SB may have a more serious  abnormality called Arnold-Chiari Malformation.

Arnold-Chiari Malformation in Spina Bifida

This is a malformation of the brain where cerebellar tonsils protrude downwards through the foramen magnum due to the lack of development of the posterior fossa. Thus, the condition stops the outflow of cerebrospinal fluid (CSF) due to the compression or blockage in the outflow tract. Such patients report with headache, dizziness, problems in coordination and swallowing and in more severe conditions, paralysis [5]. Other rare problems include hydrocephalus, spinal curvature and connective tissue disorders. Severity of the disability depends upon the elevation of ICP and hydrocephalus in which shunt revision, infections and seizures may develop.

Video submission into the Neuro Film Festival. Very good information about the Arnold-Chiari Malformation.

How to confirm cognitive dysfunction in Spina Bifida?

Cognitive dysfunction can be confirmed by different types of tests:

PCFT: Prudhoe Cognitive Function Test

The PCFT test is a psychological test that is used to assess the intellectual disability of patients.  It can be used for  patients  with Spina Bifida [6]. The PCFT is available in three forms:

  1. PCFT long
  2. PCFT short A
  3. PCFT short B

 

Question based or history related:

  • A patient may have problems with memory and other brain functions such as planning, instruction following and decision making
  • A careful medical history is helpful to confirm that patients’ memory is intact or if it has declined
  • The mini mental status examination (MMSE) also confirms cognitive dysfunction in spina bifida

Neurological Examination

A part of the general examination is neurological   by which cognitive functions can be assessed by testing the reflexes, eye movements, walking and balance.

Lab Tests

Blood tests are also helpful to rule out cobalamin deficiency in Spina bifida which has a detrimental affect on memory.

Brain Imaging

Brain MRI or CT scan is also helpful to confirm any physical problems (Arnold-Chiari malformation) in the brain.

How to minimize the loss of cognitive function in SB?

Cognitive function disabilities in SB can be improved in any age group. Some patients who are treated by certain procedures have normal IQ levels. Three cognitive impairments will be covered:

  1. Attention/memory difficulty
  2. Auditory and visual difficulty
  3. Literacy difficulty

Attention/Memory difficulty

  • Make a habitual pattern for routine work. By using a routine pattern the patient develops a procedural memory for performing  these tasks.
  • Minimize distractions around the patient, especially about his/her own daily schedule. That is, remove all non-essential information about the tasks.

Auditory and Visual difficulty

  • Put the relevant information and objects in the patients’ immediate environment. This will make it easier to access.
  • Use different modalities to provide information (multi modal approach). Using both visual and auditory means of providing information is more helpful than a single way.

Literacy difficulty

  • Use simple and understandable language around the patient.
  • Avoid numerical values and calculations. Calculators can be used for calculation of numerical values.

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Reference

  1.  Sternberg, RJ, Sternberg K. Cognitive psychology (6th Ed.). Belmont, CA: Wadsworth, Cengage Learning. 2009.
  2.   Hannay HJ. Functioning of the corpus callosum in children with early hydrocephalus. J Int Neuropsych Soc. 2000;6:351-61.
  3.  Dennis M, Fletcher JM, Rogers T, Hetherington R, Francis DJ. Object-based and action-based visual perception in children with spina bifida and hydrocephalus. J Int Neuropsych Soc. 2002;8:95-106.
  4.   Hetherington R, Dennis M. Motor function profile in children with early hydrocephalus. Dev Neuropsychol. 1999;15:25-51.
  5.  Chiari malformation: Symptoms. Mayo Clinic. November 13, 2008.
  6.  Kay DWK, Tyrer SP, Margallo-Lana M-L, Moore PB, Fletcher R, Berney TP, et al. Preliminary Evaluation of a Scale to Assess Cognitive Function in Adults with Down’s Syndrome: The Prudhoe Cognitive Function Test. Journal of Intellectual Disability Research, 2003; 47, 155-68.