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Learning and Behaviour in Myelomeningocoele (open spina bifida)

Learning and behaviour in Myelomeningocoele (open spina bifida)

Myelomeningocoele is a condition that affects the development of the brain and spinal cord. This information sheet looks at how differences in brain development in spina bifida can affect thinking, learning and behaviour (cognition).

People with myelomeningocoele often describe similar patterns of strengths and weaknesses in their abilities in everyday life, and formal testing by neuropsychologists often identifies a similar profile of results. By contrast, people with hydrocephalus alone are found to be affected in a wide range of ways.

Summary

Open spina bifida changes the way the brain, as well as the spine, develops.

The ‘main’ part of the brain (cortex) might be thinner and not as organised.

The connection between the two halves of the brain might not form fully.

The cerebellum might not form fully, and might crowd other nerves and parts of the brain at the top of the spinal cord (Chiari II).

Difficulties imagining the future affect decision making, motivation, organisation and planning.

Feeling how time passes in a different way can affect self caring, healthy eating and how we feel about our health.

Information may take a bit longer to ‘sink in’. Having it in small chunks can help.

There may be difficulty getting focused, switching between activities or ignoring distractions, and managing change can be difficult.

Learning facts, routines, and anything that involves ‘rules’, like phonics, can be a strength.

How does brain development differ?

In the womb, around the middle of a pregnancy without spina bifida, CSF (cerebrospinal fluid) which has collected in the ventricles forces two channels to form. This lets CSF flow onto the surface of the brain and spinal cord. The CSF contains important chemicals needed for the development of the cortex (the main part of the brain). The chemicals cause the brain cells to divide, and guide them into their correct positions, in neat layers, in which they form connections.

In spina bifida, the CSF may not flow onto the surface of the brain, so these important chemicals are unable to support cortex development. The cortex may be thinner, and less organised than typical.

People with spina bifida may have changes to the corpus callosum, an important bundle of brain fibres which connects the left and right halves of the cortex and allows the flow of messages around different areas of the brain. The corpus callosum may be absent (callosal agenesis) or thinner than usual (callosal hypoplasia).

In addition, the cerebellum may be smaller, shaped differently (long and thin rather than round) and lower than usual, protruding into the spinal canal (see information sheet on Arnold Chiari).

If the brain is formed differently, it follows that it may function in a different way.  Some people may find many challenges. Different issues may be experienced by some people, none by others.

How may cognition be affected?

Memory

There are many different types of memory, for example, memory for how to carry out tasks, working memory for remembering information that we are using now. Some, but not all, types of memory are affected in spina bifida.

Prospective memory is very important for planning, motivation and goal setting, and decision making. It allows us to imagine future events and situations, comparing the possible outcomes, so we can decide what to do, or how to behave. We can think about what we will be doing later, so we can prepare and organise. If it is difficult to imagine the future, it can be difficult to make changes to let us reach our goals for the future. Decisions we make when we look long-term tend to be different to those we make when we just have ‘now’ to think about. It can be difficult to begin or initiate an activity if we can’t imagine the end result. It can also be difficult to use past experiences to plan what we do in future, meaning a person might repeat the same mistakes again and again, even though those actions might cause a lot of worry and distress to themselves.

Someone with spina bifida may not recognise situations they are struggling with, so may not ask for support, as they don’t have a mental picture of how things could be different.

Episodic memory is our memory for specific events. When this is affected, it is harder to remember events, and to put them into the order they happened. If events are recalled, it can be hard to sense when they happened. For example, a visit from a relative might be remembered as a recent event, yet several years may have passed. Episodic memory also forms part of autobiographical memory, which puts our ‘story’ in order and gives us a sense of ‘self’. When autobiographical memory is affected, it can be harder for a person to identify their strengths and weaknesses, so when choosing subjects to study or careers they might benefit from guidance from someone who knows them well.

Working memory allows us to hold information in our minds whilst we need it or are using it. An example might be remembering a message while we write it down. Working memory can be affected in spina bifida if it is overloaded by too many things at once.

Relative strengths for someone with spina bifida include memory for how to do things (procedural) and concepts like colour and the sounds of letters.

Attention

There are two different types of attention

1)    Where attention is drawn to the loudest, most frightening or exciting things happening around you

2)    Where we choose what we want to concentrate on (goal-directed attention)

People with spina bifida often have difficulty with goal-directed attention, which can mean distractibility while trying to work and needing extra time to really get focused. However, attention can be held once focused, and people may find that once they have settled they find it hard to ‘tear themselves away’, to go on to the next activity.

Flexibility of thinking

Many people with spina bifida find it very difficult to manage change, which can cause a lot of anxiety; things change and this is often outside our control. Sometimes people become anxious about trying new things, or try to control all aspects of their life with rituals or routines. This can lead to a very narrow, dull lifestyle, and won’t prevent change from happening anyway.

It might be very difficult to shift attention away from something you’re doing, or to cope when rules or expectations change. Sometimes people find it difficult to adapt how they behave to suit different situations. For example, we might speak to our friends in a different way to how we speak to a police officer.  

Managing change well needs us to have a clear idea of how that change will affect the future, and adapting our behaviour needs us to be able to ‘think ahead’ to see how each different possibility might end up. This prospective memory (as above) is an important part of flexibility and motivation; if we can’t think ahead to a goal, we can’t plan our behaviour to get the results we want.  

Processing information

Information needs to be processed by the brain so that we can react to what is going on or store it as memories. Different areas of the brain are responsible for dealing with different information, movement or emotions. Pathways develop in the brain starting when we’re babies, linking brain cells together in complex networks, so that information can be easily passed from area to area. If pathways are less developed, or if the corpus callosum is not fully formed, it may take longer for information to be processed. The cerebellum also seems important for processing information.

Associative processing is a relative strength for people with spina bifida, and is concerned with processing in a rule-based, ‘concrete’ way. This leads to strengths in learning facts, word recognition, routines, and rule-based concepts such as phonics.

Assembled processing, however, is a relative weakness. This type of processing is concerned with linking information from many sources (assembling), such as making inferences, transferring skills to different settings, selecting relevant information from irrelevant information, and linking information from different sources.

There can also be a problem generating new ideas or creating something from scratch, rather than seeing an example first.

Passing time

People with spina bifida may not sense time passing in the same way as other people. This becomes important with self-care routines. For example, you may not notice how long has passed since the last catheterisation, shower or drink of water. You may remember that things need to be done, but not recognise that ‘now’ is the time to do them.  Things may not be done at the right time, leading to severe health problems like UTIs, dehydration and pressure sores. It can also make it harder to plan to eat a healthy diet, as getting meals ready may not begin until the person is already really hungry. It can cause difficulties with hospital or doctor’s appointments, for example, if you don’t have a ‘feel’ for how long you’ve had symptoms, how long it is since your medicine ran out etc.

Also, care assessments might not be very accurate if you’re not sure how often you actually do certain things, like showering, rather than how often you intend to.

Planning, organising, sequencing and motivation

Planning and organising can be very difficult for people with spina bifida.

This can stem from difficulty in looking ahead (prospective memory) which helps us ‘see’ what we’re aiming at, and what we’re trying to achieve. It’s very hard to get motivated when we can’t imagine the end result of something we might want to do.

It can come from trouble designing a system in your head, sorting things into categories (strategy formation). Or it can come from difficulty getting things in the right order (sequencing). If we can’t work out the first step of the task, where to start, we can’t get started and may ‘put it off’.

What can help?

Attention

Give yourself extra time to settle and really focus on what you need to do, and give yourself time to finish completely if you can (easier than trying to pick up where you left off at a later date).

Find  a quiet place to do more challenging things, away from distractions or interruptions, as your focus would be drawn to these rather than what you’re trying to do.

Allow yourself a little time (maybe 5 minutes), to switch from one thing to the next, if you find it hard to ‘switch off’ from one thing to start another.

Try to do one thing at a time, avoiding multitasking if you can. The more things your brain tries to do at the same time, the less well it will do any of them.

Processing

Allow a little time for one piece of information to be absorbed and processed, before the next piece is given, so that the information can ’sink in’, in the right order. It’s more likely to be remembered if bits aren’t missing.

Use the strength of rote-learning to learn different ways of doing similar things. For learning new activities, clear instructions and demonstrations are useful. Even if the new activity is very similar to one you know, you may not recognise the part you already know, or be able to guess or ‘work out’ what to do based on similar tasks. It might be better to film the steps of something new on a smartphone, so you can watch again as many times as you need.

Taking notes at the same time as watching is difficult, as the brain has to try to do too many things at the same time, such as watching, trying to remember, deciding what words to use in the notes, and physically writing the notes. Having a checklist or written instructions ready lets you focus on the job in hand. 

Using templates or examples of what you’re aiming to do can be easier than trying to create something from scratch.

Managing Time

Routines can help you to structure your day, and you may need support and prompting to begin doing something at the right time, and support to get back to a routine after a break, such as a holiday or hospital stay. Some people use alarms or smartphone apps to plan their day and give prompts. Setting an extra alarm, to go off 5-10 minutes after the first, gives a little time to switch off the attention from what you’re doing before you go onto the next, aiding flexibility. This way, you might be less likely to put off the new thing ‘just for a minute’ but never get back to it.

Some people find they need something outside themselves to break attention and structure their day. For example, using timer switches to turn off a TV or computer at a chosen time, if they know they’ll find it hard to switch off once they’re engrossed.

Visual cues (calendars, visual timetables, day clocks…) can help keep track of time.

Keep a record of what you have done and when, or events that have happened, as well as what you need to do in the future. This will help at hospital appointments etc. to help you keep in touch with family and friends, or to keep events in the right order.

Planning, organising, sequencing and motivation

Talking with someone before you start doing something can help you get the steps straight in your mind. Help to get a system in place, with a detailed timetable (what to do, and when to do it) and breaking jobs down into small steps can get you started. Many people find colour-coding can help. Record voice messages on your phone, reminding yourself what to do and when, and use them as alarms to motivate you and keep you to time. We need motivation to do the things we have to do, but not what we want to do. Find the ‘want to’ in your activity, such as looking your best, living in a tidy home, rather than the ‘have to’, like showering, housework.

SMART goals (Specific, Measureable, Achievable, Relevant and Time specific) can help to identify very small, specific steps towards the goal, in the right order, with specific time limits. It can be hard for people with spina bifida to break down large tasks into smaller parts. If you can’t work out the first steps of anything you want to do, you can’t get started at all, and are likely to feel overwhelmed, and put off the job until another time. Practising breaking jobs down into small steps using SMART goal setting, with support, can bring real benefits in ‘getting things done’.


Based on several papers by Dr Jack M Fletcher, Dr M Dennis et al including

Attention Problems and Executive Functions in Children With Spina Bifida and Hydrocephalus Fletcher, Dennis 2011.

www.waisman.wisc.edu/~rowley/sb-kids/articles/Attention%20problems%20and%20executive%20functions%20in%20children%20with%20spina%20bifida%20and%20hydrocephalus..pdf

http://spinabifidaassociation.org/wp-content/uploads/sites/10/2017/03/Neuropsychology.pdf

Dr J Miyan (2009) CSF circulation and cerebral cortex development

https://fluidsbarrierscns.biomedcentral.com/articles/10.1186/1743-8454-6-S2-S13

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Did You Know?

Some babies with spina bifida are now operated on before they are born, via keyhole surgery.

Hydrocephalus can be congenital or acquired.

NPH (Normal Pressure Hydrocephalus) is an excessive build-up of fluid in the head.

Hydrocephalus is a build up of excess fluid in the brain.

Some 11 - 35% of people with Intracranial Hypertension recover spontaneously.

Most babies with spina bifida undergo surgery within 48 hours of birth.

“Every effort should be made to ensure that all children are immunized, no opportunity to immunize should be missed.”

If you have spina bifida +/or hydrocephalus you should receive the same vaccinations as any others, when going abroad.

Hydrocephalus comes from the Greek "hydro" meaning water and "cephalie", meaning brain.

Some forms of hydrocephalus require no specific treatment.

Medical advice should always be sought if shunt infection is suspected.

Shunt: a device that diverts accumulated cerebro-spinal fluid around the obstructed pathways back to the bloodstream.

Possible signs of chronic shunt blockage include: fatigue, general malaise or behavioural changes.

A shunt alert card should be carried at all times by people with hydrocephalus treated by a shunt.

Possible signs of acute shunt blockage may include: visual disturbances, drowsiness and seizures.

Symptoms of Normal Pressure Hydrocephalus are similar to Alzheimer's, Parkinson's disease or simply increasing age.

NPH (Normal Pressure Hydrocephalus) occurs most often in people aged over 60.

Benign Intracranial Hypertension aka Idiopathic Intracranial Hypertension affects about one or two in every 100,000 people!

Symptoms associated with raised intracranial pressure; headache, visual disturbances, photophobia, vomiting, problems with balance...

Diagnosis of Intracranial Hypertension is by scan + measurement of the CSF pressure.

Babies born prematurely are at increased risk of developing hydrocephalus.

Shine can raise money by recycling your used inkjet cartridges, toners or CDs and DVDs.

Para-athletes with spina bifida and hydrocephalus compete in sports ranging from cycling to dressage.

Hydrocephalus may affect memory, concentration and behaviour.

The usual treatment for hydrocephalus is to insert a shunt into the brain.

CSF stands for cerebro-spinal fluid.

Benny Bear is a teddy with hydrocephalus who helps children understand the condition.

Shine is always looking for Marathon Runners to help with fundraising.

Spina bifida occulta is a hidden form of spina bifida.

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