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What is Dejerinesottas syndrome?

Dejerine-Sottas syndrome isa type Charcot Marie Tooth disease characterised by muscle weakness and loss of sensation in the arms and legs due to damage of the peripheral nerves in the body.Dejerine-Sottas syndrome starts in childhood and symptoms are usually more severe and progress more rapidly than other forms of Charcot-Marie-Tooth disease.

Physiotherapy for Dejerine- Sottas syndrome will improve your child’s ability with daily activities by maintaining muscle strength and flexibility for as long as possible and helping you manage their current symptoms.

Neurological physiotherapist closely supervising functional fine motor skill exercises to improve independent livingAbove: Neurological physiotherapist closely supervising functional fine motor skill exercises to improve independent living

Diagnosis of Dejerine-Sottas syndrome

A neurologist will confirm a diagnosis of Dejerine-Sottas syndrome by looking at family history, early onset of the symptoms (usually clumsiness and loss of balance) and nerve conduction tests.

What causes Dejerine-Sottas syndrome?

Dejerine-Sottas syndrome is an inherited neurological disorder caused by a defective gene resulting in damage to the peripheral nerves that are made up of motor and sensory nerve fibres.

What are the effects/symptoms of Dejerine-Sottas syndrome?

Dejerine-Sottas syndrome affects the upper and lower extremities. Damage to the motor and sensory nerves that supply the foot may cause:
  • Muscle weakness
  • Muscle atrophy (wasting)
  • Foot drop
  • Ankle sprains
  • Ataxia (lack of coordination)
  • Reduced balance
  • Difficulty walking
Damage to the nerves that supply the hands may lead to:
  • Numbness and reduced sensation
  • Reduced muscle strength and dexterity
Other symptoms may include:
  • Pain
  • Fatigue
  • Respiratory problems

Physiotherapy for Dejerine-Sottas syndrome

Physiotherapy treatment for Dejerine-Sottas syndrome at will be centred on keeping the muscles strong for as long as possible to promote independence with everyday activities. At our specialised neurological physiotherapists will provide an individualised treatment program specific to your child’s needs. Physiotherapy treatment will be focused on:
  • Promoting muscle strength and length
  • Maintaining healthy joint positions to facilitate walking
  • Improving normal gait pattern to reduce risk of falls
  • Maintaining physical fitness
  • Reducing fatigue
  • Managing pain
  • Promoting independence
  • Improving quality of life
It is important to commence physiotherapy as soon as possible following a diagnosis to delay or reduce muscle weakness and enhance functional ability. Physiotherapy at for Dejerine-Sottas syndrome will depend on your child’s individual needs but may involve:
  • Muscle strengthening exercises that are engaging and appropriate for your child to keep muscles strong for as long as possible
  • Muscle stretching to lengthen tight muscles and keep joints flexible
  • Aerobic activities to build stamina and reduce fatigue
  • Advice on positioning in sitting and lying to increase comfort and prevent muscle tissue shortening
  • Advice on ankle braces or orthoses to improve mobility and minimise injury
  • Hydrotherapy to stretch tight muscles and build up muscle strength
Our physiotherapists also work closely with other health professionals such as occupational therapists and orthotists to provide the best possible treatment.

If you would like more information on how physiotherapy can help your child with Dejerine-Sottas syndrome, or to book an appointment, please call 0330 088 7800.

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