Dejerine-Sottas syndrome affects many children, but there is a lack of information available for people to access. This article shares key information about DSS that can be used to ensure that DSS becomes a more understood syndrome and that those with DSS can access the proper support that they require.

Individuals with DSS require educational and developmental support, which can include:

• Therapeutic interventions
• Individual educational support plans
• Appropriate advocates
• Occupational therapists

Read on to learn more about Dejerine-Sottas disease and how those with the syndrome can have their educational and developmental needs met.

Introduction

Dejerine-Sottas syndrome (DSS) is a neurological condition inherited from parents that affects the body's peripheral nerves. These are the nerves located outside of the brain and spinal cord. This rare condition causes the nerves to become thickened and enlarged, and slowly affects a person's ability to move.¹ Recognising DSS when it is first expressed allows for appropriate measures and treatments to be provided so that people who have DSS can be properly 

supported with their developmental and educational needs. Supporting people who have DSS allows an improved quality of life and supports learning and development.

Developmental challenges in children with DSS

Motor development

DSS can begin at a very young age, starting as early as age 2. Muscle weakness within the legs tends to be a common indicator that a child could be experiencing DSS. This weakness begins in the back of the legs and can quickly spread to the muscles in the front of the leg. Muscle weakness can cause children to have delays in motor development and may cause a delay in reaching some motor milestones. These milestones may include sitting, standing and walking; some individuals may need to use a wheelchair by their teenage years. Muscle weakness within the legs may result in coordination issues for people experiencing DSS. Other muscles within the body, such as hand and forearm muscles, can also be affected as the condition develops.¹

Sensory challenges

Individuals with DSS may have absent reflexes, creating multiple sensory challenges. Their vision can also be impacted as the condition worsens, and they may experience a loss of heat sensitivity. Damage to motor and sensory nerves can result in numbness and reduced sensation within muscles such as those in the arms and hands, which can result in a delay in the development of fine motor skills.² This can impact an individual's writing abilities, stop them from gripping objects and may affect other daily activities such as brushing teeth or buttoning items of clothing.

Cognitive and emotional development

DSS does not tend to impact the function of the brain; however, cognitive and emotional development may be impacted. This is due to the difficulties that can be faced due to motor restrictions caused by DSS, which could include difficulty walking or writing.¹ Physical limitations may affect school attendance or other educational opportunities, which could include missed lessons and educational activities. Poor attendance may put a strain on educational development and could prevent an individual with DSS from progressing cognitively. Self-esteem may be impacted as individuals with DSS may face challenges and limitations that can become frustrating due to their physical restrictions. This may result in low self-esteem and a poor self-image due to not being able to do the things that they want to achieve. 

Communication and social skills

Muscle weakness may occur within the facial muscles, which could negatively impact the speech of individuals experiencing DSS. This may make it difficult for individuals to socialise with peers as they may find it difficult to communicate effectively and may become frustrated. Additionally, individuals with DSS may face difficulty when walking and are likely to fatigue quickly. This could result in social isolation, as it may be difficult to maintain relationships due to the physical restrictions and limitations that individuals with DSS face.

Educational support needs

Children with DSS will need accessible classrooms, with appropriate measures in place to allow for educational support. Some individuals with DSS require wheelchairs.¹ So classrooms must have the correct environment and accommodating areas for wheelchair access. Adaptive seating should be available to allow for full support and access to all classroom spaces. Extra time should be provided for individuals with DSS for tasks and examinations to allow for full educational support.

Appropriate equipment, such as voice-to-text systems and adaptive technology, should be provided within educational settings for individuals with DSS. Individuals who struggle to write due to muscle weakness should have access to all supporting equipment to allow them to thrive in classrooms and learning environments.

Activities that can aid in the strengthening of muscles could be implemented to allow for the development of fine motor skills. With the improvement of fine motor skills, individuals with DSS can be supported to strengthen their skills, such as writing and drawing.²

Individual education plans should be created to allow for a more suitable education. These plans can make the school curriculum more appropriate and more accessible for children with DSS. Parents, teachers and physiotherapists should work together to ensure a plan is created that ensures maximum support and maximum success for children with DSS in schools. As their condition progresses, there should be regular updates to allow for the best support to be provided. Plans may include breaking down tasks into more manageable steps and encouraging children to try their absolute best so that they can excel in their studies.

Developmental support needs

Therapeutic interventions

Individuals with DSS can have physical therapies and treatments that can help their development. This can include muscle strengthening exercises that can help to keep muscles strong for as long as possible, aerobic activities that can help improve an individual's stamina to reduce their fatigue and also hydrotherapy, which can be used to stretch affected muscles.² Additionally, people with DSS can have alterations made to their homes that can improve their daily living skills and improve their quality of life.

Psychological and emotional support

Counselling may be recommended for individuals with DSS to help improve their self-confidence and self-image. Counsellors may recommend activities and motivational tasks that can help emotionally support people who have DSS. This may also help build social skills for those who struggle to maintain a social life due to the physical restrictions associated with DSS.

Family support

The families of those who have DSS can play a role in supporting the development of individuals with DSS. Families may be given appropriate training and recommendations on how to help support their family member and to help their development overall.

Collaboration and multidisciplinary care

The collaboration of teachers, medical professionals and family members is essential for providing the best support and care for an individual with DSS. Communicating clearly helps to create a trusting and appropriate support team that is capable of giving the best support to an individual with DSS. Family members may be able to act as advocates if an individual struggles to voice their opinions and feelings in regards to the support they are being given. A multidisciplinary team should regularly keep up to date with their care plans and should make any appropriate adjustments if necessary to ensure that the best support is available.

Summary

Individuals who have DSS face many challenges. This includes motor, sensory and social challenges which can have a major impact on their development. Children with DSS tend to struggle with their motor development, facing muscle weakness and walking difficulties.

Appropriate support must be provided for individuals with DSS to ensure that their development continues and they can progress, physically, socially and mentally. Specific education plans can aid in this by providing personal support, as well as therapeutic interventions and accessible environments, so that individuals with DSS don't face any restrictions when it comes to their development. With collaborative care and appropriate strategies in place, individuals with DSS can be fully supported with their development and education.