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Complex motor disorders – treatment stages

Information for referrers

When a child is referred to the Evelina London complex motor disorders services, there are three treatment stages.

One: diagnosis and assessment

All patients referred to us will be assessed to determine their suitability for neurosurgery and to establish which neurosurgical procedure would be most suitable.

Children are reviewed to establish their level of motor impairment, disability, quality of life and independence skills. Further diagnostic tests are offered on an individual basis.

Those with normal neuroimaging but no other specific neurogenetic markers are usually offered a trial of L-dopa to rule out dopa-responsive dystonia. This is usually followed by trials of oral trihexyphenydyl and baclofen to attempt to improve dystonia. Those who do not respond well are considered for neurosurgery.

Further information

Tests and investigations

All referred cases undergo:

  • an up-to-date MRI brain scan to assess the suitability for DBS targets
  • an FDG-PET-CT brain scan to look at the metabolic viability of potential DBS targets
  • mfagstim testing of the corticospinal tracts and subcortical structures.

Children with pre-established diagnoses may not need further genetic or neurometabolic investigations, however:

  • we work closely with our paediatric metabolic colleagues to maximise diagnostic yields and to support of a variety of metabolic disorders
  • genetic testing may be undertaken as indicated by imaging and biochemical results. Primary dystonias will be screened for DYT1, DYT5 and DYT11 mutations as appropriate, after discussion with our genetic colleagues.

Clinical assessments

All referrals will have detailed assessments of:

  • gross motor skills
  • fine motor skills
  • speech, language, communication and feeding
  • neuropsychological functioning and emotional state
  • participation
  • quality of life.

Goal setting

The process of goal-setting is an essential component of the decision to go forward with a deep brain stimulator (DBS) or intrathecal baclofen pump (ITB) implant.

Goals of ITB and DBS or other management strategies will be established with the child and carers from the outset.

Multidisciplinary pre-surgical planning

Having established which neurosurgical procedure would be most suitable, the child and family have an opportunity to discuss the implications of surgery with our nurse coordinator and clinical psychologist.

A meeting is arranged with the neurosurgical team to discuss the risks and benefits of neurosurgical implantation of either deep brain stimulation electrodes or, if this is unsuitable, an intrathecal baclofen pump system. Goals are reviewed in detail and any other practical or technical issues are discussed. 

Two: neurosurgery and immediate postoperative care

Neurosurgery is undertaken at King's College Hospital Paediatric Neurosurgical Unit. The patient is then transferred back to Evelina London Children's Hospital.

The patient is admitted the day before surgery. The paediatric neurologist attends the neurosurgery and activates either the intrathecal baclofen pump (ITB) or the deep brain stimulators as indicated.

The day after surgery the child is transferred back to Evelina London. Here they will convalesce and any necessary adjustments to the ITB or DBS systems will be undertaken. They will have 10 day course of intravenous antibiotics following DBS.

Patients are nursed in isolation in the laminar flow cubicles on Savannah Ward to minimise possible infection.

Stitches and clips are removed on day ten and patients are normally discharged 10-14 days after surgery.

Three: post surgical follow-up

Management of intrathecal baclofen pump or a deep brain stimulator systems.

Children implanted with an intrathecal baclofen pump or a deep brain stimulator will require an average of six visits to the hospital in the first year and four in the second year post-implant.

Each visit aims to assess the clinical state of the child, review previously established goals and determine future goals as the child improves.

The number of visits will depend on a number of factors:

  • underlying condition
  • rate of progression/deterioration prior to implantation
  • the need to adjust the rate of baclofen infusions or deep brain stimulator parameters.

Close liaison with local services and therapists is anticipated.

Follow-up in the third or subsequent years will be tailored to individual needs.

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