Status dystonicus in children and adolescents

Publication: 23/09/2019  
Next review: 15/02/2025  
Clinical Guideline
CURRENT 
ID: 6164 
Approved By: Trust Clinical Guidelines Group 
Copyright© Leeds Teaching Hospitals NHS Trust 2022  

 

This Clinical Guideline is intended for use by healthcare professionals within Leeds unless otherwise stated.
For healthcare professionals in other trusts, please ensure that you consult relevant local and national guidance.

Management of status dystonicus in children and adolescents

Summary of Guideline

This guideline contains information regarding the identification and management of the potentially life-threatening condition of status dystonicus.

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Aims

To improve the diagnosis and management of status dystonicus in children and adolescents

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Background

Dystonia is defined as the sustained involuntary contraction of muscles leading to abnormal postures and movements. It usually affects multiple muscle groups (generalised) but may be limited to a few (focal). Children with cerebral palsy (a static injury to the antenatal or infant brain) are the commonest group of children with dystonia. Children with any illness or injury affecting the brain are a risk of dystonia.

Status dystonicus is a severe and potentially life-threatening condition comprising of increasingly frequent and severe episodes of generalised dystonia which requires urgent (hospital) management. Status dystonicus usually occurs in a child who is already known to have dystonia, although new-onset cases are reported.

Status dystonicus may present with one or more of the following complications:

  • Elevated body temperature
  • Pain / discomfort
  • Exhaustion from sleep deprivation and exertion
  • Rhabdomyolysis leading to myoglobinaemia and raised Creatine Kinase (CK)
  • Dehydration with electrolyte disturbance from excess sweating
  • Acute renal failure as a consequence of myoglobinuria / dehydration
  • Bulbar dysfunction with risk of aspiration
  • Respiratory failure
  • Death 

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Diagnosis

Status dystonicus is a clinical diagnosis based on the identification of severe, generalized dystonic movements. Diagnosis should also involve identification of associated comorbidity (bulbar and respiratory difficulties, metabolic derangements, exhaustion and pain).

Note: Creatinine kinase (CK) is an enzyme that is released by damaged muscle in status dystonicus and can be measured in blood samples. Presence of significantly elevated CK supports a diagnosis of status dystonicus. However, CK can take 24 hours to become elevated in blood, and therefore a normal CK value does not exclude status dystonicus. For the same reason, CK should not be used in isolation to grade severity of status dystonicus.

Dystonia can be graded in terms of the following:

Dystonia Severity Scale[i]

Grade 1:

The child sits comfortably and has regular periods of uninterrupted sleep. Child stable on medication. 

Grade 2:

The child is irritable and cannot settle.

Dystonic posturing interferes with sitting activities.

The child can only tolerate lying despite usual baseline medication.

Grade 3:

Child unable to tolerate lying and/ or unable to get to sleep or sleep disturbed

No evidence of metabolic decompensation, with creatinine kinase (CK) <1000 IU/L.

Grade 4:

Early multi-organ failure: Clinically as above with:

  • Pyrexia (in absence of infection)
  • Evidence of metabolic compromise (e.g. acidosis, elevated potassium, low calcium, evidence of rising creatinine and/or urea)
  • Evidence of myoglobinuria, CK >1000 IU/L.

Grade 5:

Immediate life-threatening: as above with:

Full metabolic decompensation Respiratory, cardiovascular or renal compromise.

Requires intensive care.

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Investigation

The following investigations are used to monitor complications of status dystonicus and should be conducted in every child presenting with grade 3 - 5 severity:

  • Creatine Kinase (repeated 24 and 48 hours later as CK rise may take 24hrs)
  • Renal function (urea and electrolytes)
  • Liver function testing
  • Calcium
  • Capillary / Venous Gas

Status dystonicus is precipitated by a trigger in approximately 2/3 of cases. Identification (through history and relevant testing) and management of a trigger is helpful in managing symptoms. Triggers can include:

  • Intercurrent illness (especially gastroenteritis with dehydration, chest infections)
  • Pain / discomfort from any source, including constipation, urinary retention, gastro-oesophageal reflux, dental caries/ulcers, dislocated hip
  • Recent anaesthesia
  • Recent surgical procedures
  • Change / omission of usual medication therapy
  • Emotional factors such as stress

In children presenting with status dystonicus with no prior dystonia, investigations should be led by paediatric neurology advice and would include imaging of the brain.

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Treatment / Management

In any patient who has an intrathecal baclofen (ITB) pump, ITB withdrawal should be presumed to be the cause of an acute presentation with status dystonicus (pump malfunctioning / empty reservoir). The ‘Intrathecal Baclofen use in Children and Adolescents’ Leeds Health Pathways guideline should be consulted and the on-call paediatric neurology consultant should be urgently informed.

Principles of management are based on the level of severity as below:

Dystonia Severity Scale[i] 

Assessment 

Plan 

Grade 1:

The child sits comfortably and has regular periods of uninterrupted sleep. Child stable on medication. 

Nil needed

Continue as normal

Grade 2:

The child is irritable and cannot settle. 

Dystonic posturing interferes with sitting activities.

The child can only tolerate lying despite usual baseline medication.

R/V in next few days

Treat underlying causes.

Adjust medication

Grade 3:

Child unable to tolerate lying and/ or unable to get to sleep or sleep disturbed

No evidence of metabolic decompensation, with creatinine kinase (CK) <1000 IU/L.

Urgent assessment, including finding triggers

Treat underlying causes.

Further adjustments to medication.

Blood screen required to look for signs of decompensation (including renal function, CK); Observe in hospital; be prepared to escalate management.

Ensure adequate hydration (commence intravenous hydration if in doubt)

Grade 4:

Early multi-organ failure: Clinically as above with:

  • Pyrexia (in absence of infection)
  • Evidence of metabolic compromise (e.g. acidosis, elevated potassium, low calcium, evidence of rising creatinine and/or urea)
  • Evidence of myoglobinuria, CK >1000 IU/L.

Emergency hospital admission for organ support and to prevent renal failure and other complications

Treat underlying causes.

Regular ( 4hourly at least) bloods for CK, renal and liver function

Nasogastric, gastrostomy or rectal medication (see below) if tolerated and working, otherwise intravenous medication.

Intravenous fluid support.

It is essential that all children in grade 4 are discussed with PICU as soon as possible

Grade 5:

Immediate life-threatening: as above with:

Full metabolic decompensation Respiratory, cardiovascular or renal compromise.

Requires intensive care.

Need HDU or PICU

Treat underlying causes.

Consider need for :

  • IV Infusion of clonidine and/or midazolam
  • Dialysis/ haemofiltration
  • Invasive ventilation
  • Intrathecal baclofen LP dose or infusion

Specific management options[iii],[iv]

(see appendix 1 for guidance on dosing)

For doses, side effects and route of administration see appendix 1

Additional notes with reference to the management flow chart:

  • IV fluids
    Increased insensible losses through sweating can rapidly lead to dehydration and maintenance fluids may need to be increased by an additional 5-20% each day to compensate for increased insensible losses. Electrolytes should be measured regularly.
  • Positioning and handling
    Positioning (especially adopting a ‘flexed’ posture) can be useful in ‘breaking’ the spasms in some children and nursing and physiotherapy input may provide additional strategies to improve spasm-free periods and sleep. In some children, dystonia may be exacerbated by handling and this should be minimised to necessary cares.
  • Address emotional / behavioural /psychological contributing factors
    Many children with dystonia may be quite physically disabled but with intact cognition. In some of these children psychological / emotional factors can further aggravate their underlying dystonia. This should be considered and appropriate support provided.
  • Clonidine
    Clonidine has a less sedating, non-respiratory depressant effect and may prove effective in achieving control or preventing breakthrough dystonia.

    Clonidine can be administered by continuous enteral infusion via NG tube or gastrostomy if necessary by calculating the total daily dose and dividing by 24 hours to deliver.

    Where the enteral route is unsuitable owing to vomiting, diarrhoea, gastrointestinal bleeding or ileus, the equivalent doses may have to be administered as an IV infusion (needing close observation in HDU setting) or transdermal patch.
  • Specific therapy for Rhabdomyolysis
    (e.g. urine alkalinization, dantrolene, neuromuscular paralysis, and/or dialysis in acute renal failure)
  • Intrathecal Baclofen (ITB)
    It has been tried in a small number (~10%) of patients with refractory status dystonicus with various reports of benefit and failure. Complications and tolerance can potentially limit the use of ITB over long periods. ITB is not without other risks such as over-dosage, withdrawal syndrome, and more commonly catheter migration or breakage, however, it is considered less invasive than brain surgery
  • Deep Brain Stimulation (DBS)
    Deep brain stimulation can be an effective treatment for status dystonicus in the majority of treated patients. The bilateral globus pallidus interna is the current anatomical site of choice for this surgical procedure. DBS is a major neurosurgical intervention and carried risk of morbidity and mortality. DBS is offered at the Evelina Hospital, London and should be discussed with the paediatric neurology team there if felt to be relevant.

Remember:

  • Different children may have greater / lesser response to certain agents so often have a tailored management plan
  • If in doubt about any of the above discuss with a senior and/or the on call paediatric neurology consultant
  • Please be aware that once a child or young person has had successful treatment for severe (grade 4/5) status dystonicus they remain acutely unwell with a high risk of respiratory, cardiovascular and renal instability and re-emergence of status dystonicus. It is vital that these individuals are very closely monitored and there is a low threshold for transfer to HDU / PICU

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Provenance

Record: 6164
Objective:

To provide evidence-based recommendations for appropriate diagnosis, investigation and management of status dystonicus

Clinical condition:

Status dystonicus; Dystonia

Target patient group:
Target professional group(s): Secondary Care Doctors
Pharmacists
Registered Nurses Working in Critical Care
Adapted from:

Evidence base

References

[i] Lumsden D, Lundy C, Fairhurst C, Lin J-P. Dystonia Severity Action Plan: a simple grading system for medical severity of status dystonicus and life-threatening dystonia. Developmental Medicine & Child Neurology. 2013; Volume 55(7): 671-672

[ii] Lumsden D, Lundy C, Fairhurst C, Lin J-P. Dystonia Severity Action Plan: a simple grading system for medical severity of status dystonicus and life-threatening dystonia. Developmental Medicine & Child Neurology. 2013; Volume 55(7): 671-672

[iii] Allen, N. M., Lin, J.-P., Lynch, T. and King, M. D. (2014), Status dystonicus: a practice guide. Developmental Medicine & Child Neurology, 56: 105–112. doi: 10.1111/dmcn.12339

[iv] Riney, K., Surtees, R.,  deSousa, C. (2004), Status Dystonicus Guidelines Great Ormond Street Hospital for Children NHS Trust. Available on the World Wide Web: [www.niamhsjourney.com/download/i/mark_dl/u/4007111453/.../Status]

Approved By

Trust Clinical Guidelines Group

Document history

LHP version 2.0

Related information

Appendix 1:

(Note - the below is a combination of BNF dosing + GOSH status dystonicus guideline dosing regime, however in acute setting on-call paediatric neurologist may chose to escalate at a quicker rate)

Drug 

Doses

Side Effects to look for

Alimemazine (Trimeprazine, Vallergan)

ORAL:

2mg/kg/dose (max 60mgs) max BD

(caution with use < 6 months)

Available in liquid or tablet form at LTHT

Antimuscarinic effects (urinary retention, dry mouth, GI disturbance)

Extrapyramidal effects

Mood change, irritability

Liver dysfunction

Arrythmias

Baclofen

ORAL: initially 300 micrograms/kg daily in 4 divided doses, increased gradually at weekly intervals until satisfactory response; usual maintenance dose of 0.75–2 mg/kg daily in divided doses; Child up to 8 years max. total daily dose 40 mg/day, Child 8–18 years max. total daily dose 60 mg/day;

review treatment if no benefit within 6 weeks of achieving maximum dose. (Baclofen is given intrathecally via an intrathecal pump in some patients).

Drooling, drowsiness, loss of head control, respiratory muscle weakness, urinary retention, constipation, irritability, weakness, sleep disturbance, appetite change

Chloral hydrate

ORAL or PR:

30-50mg/kg increasing if necessary up to 100mg/kg (max 1g/dose) tds

(can be given rectally)

Sedation, Respiratory depression, Gastric irritant, Rash, Headache

Ketonuria

Eosinophilia, low WCC

Clonidine

ORAL:

Child 2–17 years

Initially 0.5–1.5 microgram/kg 3 times a day, then increased if necessary up to 25 micrograms/kg daily in divided doses, increase dose gradually; maximum 1.2 mg per day

Increased every 5-7 days as required

IV: As an infusion (doses of 0.25–2.0 micrograms kg/hr), with consideration of higher or bolus doses as tolerated

Transdermal patch: dosing is equivalent to enteral dosing. Patches changed weekly and available as following strengths:

  • 2.5mg (100mcg/24hr) patch
  • 5mg (200mcg/24hr) patch
  • 7.5mg (300mcg/24hr patch)

Can apply multiple patches for higher doses.

Can be converted from enteral or IV (please refer to APPM fomulary or seek guidance from pharmacist / neurologist) or can be started directly with option for gradual increasing dose by occluding a portion of the patch from contact with skin (e.g. ¼ in contact with skin, ¾ occluded)

Hypotension (monitor BP when starting), drowsiness.

Avoid acute withdrawal.

In addition, transdermal patches may cause skin irritation. May use budesonide spray or topical steroid cream before application and after patch removal to treat this. Rotating patch site is advised.

Dantrolene

ORAL:

Child 5–11 years

Initially 500 micrograms/kg once daily for 7 days, then increased to 500 micrograms/kg/dose 3 times a day, then increased in steps of 500 micrograms/kg/dose every 7 days (max. per dose 2 mg/kg 3–4 times a day) until satisfactory response; maximum 400 mg per day.

Child 12–17 years

Initially 25 mg once daily for 7 days, then increased to 25 mg 3 times a day, then increased in steps of 500 micrograms/kg/dose every 7 days (max. per dose 2 mg/kg 3–4 times a day) until satisfactory response; maximum 400 mg per day

Abdominal pain; anorexia; asthenia; chills; diarrhoea (withdraw if severe, discontinue treatment if recurs on re-introduction); dizziness; drowsiness; fatigue; fever; headache; hepatotoxicity; nausea; pericarditis; pleural effusion; rash; respiratory depression; seizures; speech disturbances; visual disturbances; vomiting

 

Diazepam oral and rectal

RECTAL (PRN: can repeat dose x1)

Neonate 1.25–2.5 mg, then 1.25–2.5 mg after 10 minutes if required.

Child 1 month–1 year 5 mg, then 5 mg after 10 minutes if required.

Child 2–11 years 5–10 mg, then 5–10 mg after 10 minutes if required.

Child 12–17 years 10–20 mg, then 10–20 mg after 10 minutes if required

ORAL

<1yr 250micrograms/kg BD

1-4 yrs 2.5mg BD

5-12 yrs 5mg BD

>12 yrs 10mg BD (max 40mg per day)

Long half life

Doses may be cumulative

Drowsiness, irritability

Respiratory depression

Tolerance may occur

Dihydrocodeine

ORAL:

Child 1–3 years 500 micrograms/kg every 4–6 hours. Child 4–11 years 0.5–1 mg/kg every 4–6 hours (max. per dose 30 mg). Child 12–17 years 30 mg every 4–6 hours

Biliary spasm; bradycardia; confusion; constipation; dependence; difficulty with micturition; dizziness; drowsiness; flushing; hallucinations; muscle rigidity (larger doses); nausea, rash; respiratory depression (larger doses); sleep disturbances;

Gabapentin

ORAL: under 12 yrs 10 mg/kg once daily (max. per dose 300 mg) on day 1, then 10 mg/kg twice daily (max. per dose 300 mg) on day 2, then 10 mg/kg 3 times a day (max. per dose 300 mg) on day 3 (then can be slowly increased if needed up to max 70 mg/kg daily in 3 divided doses), >12yrs 300mg OD and increase by one dose every 3 days as tolerated until 300mg TDs is reached and can slowly increase further to max. 1.6 g 3 times daily

Drowsiness, emotional lability, headaches, dizziness, gastrointestinal disturbance, change in appetite / weight gain, tremor, ataxia, visual disturbance

Midazolam

BUCCAL: 500microgram/kg (max 10mgs)

IV:

Slow iv injection of 100-200microgram/kg then infusion of 30microgram/kg/hr increasing according to response up to a maximum of 120micrograms/kg/hr for 6mth-11yr olds and to a maximum of 200micrograms/kg/hr for 12-17yr olds

Respiratory depression

Cardiovascular depression (severe hypotension)

Potentiated by erythromycin and other drugs

Lorazepam

IV: Used PRN

50 micrograms/kg/dose (max 4mgs)

Can repeat x1 if required

Max dose of 100microgram/kg or 8mgs in 12 hours

Respiratory depression

Hypotension

S/P Liver disease

Trihexyphenidyl (Benzhexol)

Start 1mgs BD (<8yrs) or 2mgs BD (>8yrs). Increase total dose by 1mg (<8yrs) or 2mgs (>8yrs) every 7 days until clinical effect or side effects intervene or max dose 1mg/kg/dose BD.

Anti-cholinergic effects (urinary retention, dry mouth, dry eyes, blurred vision, Gi disturbance etc.)

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