Dystonia/ Muscle Spasms/ Spasticity - Paediatric Palliative Care
Description General and supportive treatment Medication and treatment Referral DESCRIPTION Dystonia A movement disorder caused by diseases of the basal ganglia, in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements as well as abnormal posturing that is often painful. Dystonia can be a primary disorder (e.g. brain tumours, metabolic conditions,
General and supportive treatment
DESCRIPTION
Dystonia
A movement disorder caused by diseases of the basal ganglia, in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements as well as abnormal posturing that is often painful. Dystonia can be a primary disorder (e.g. brain tumours, metabolic conditions, neurodegenerative disorders and demyelinating diseases or, secondary to diseases (e.g. TB meningitis) and injury (e.g. hypoxic or traumatic brain injury) of the CNS. Acute dystonia may also be drug induced.
Muscle spasms and Spasticity
Spasticity is velocity-dependent increased resistance to stretch observed in upper motor neuron damage. The increased tone can cause muscle injury resulting in pain secondary to inflammation. Intermittent muscle spasms may also occur secondary to pain, acute illness, positioning or constipation.
GENERAL AND SUPPORTIVE TREATMENT
- Prevent secondary trauma that can be caused by the child hurting herself during dystonic movements – this can be achieved by padding cot sides or nursing on the floor.
- Do not restrain the child with dystonia.
- A weighted blanket may help.
- If feeding is problematic and there is a risk for aspiration, consider nasogastric or feeding gastrostomy.
- Physiotherapy and massage.
- A warm bath may help unlock spasms.
- Supportive counselling for parents and caregivers as this can be very distressing for them, especially if it is difficult to control.
- Identify and treat triggers for muscle spasticity, especially pain.
- Involve physiotherapists and occupational therapists for exercises, splints and positioning to prevent contractures.
MEDICATION AND TREATMENT
If drug dystonia suspected, discontinue offending medication.
- Diazepam, oral, 12 hourly.
- Child 1–11 months: Initial dose of 250 mcg/kg twice a day.
- Child 1–4 years: Initial dose of 2.5 mg twice a day.
- Child 5–11 years: Initial dose of 5 mg twice a day.
- Child 12–17 years: Initial dose of 10 mg twice a day. Maximum total daily dose of 40 mg.
For pain:
- Ibuprofen, oral, 5–10 mg/kg 8 hourly.
If severe, use morphine short-term:
- Morphine, oral [Immediate release morphine (liquid).]
- Starting dose:
- If 0–1 month: 50 mcg/kg/dose 6 hourly.
- If > 1–12 months: 100 mcg/kg/dose 4 hourly.
- If > 12 months: 200–400 mcg/kg/dose 4 hourly.
- Starting dose:
Neuropathic pain and/or neuro-irritability is common in ‘evolving cerebral palsy’, refer to tertiary level for consideration of a gabapentinoid.
REFERRAL
- To Paediatric Neurology where dystonia is difficult to manage to consider use of other dystonia medications (such as carbidopa/levodopa) and to pain specialists for unresolved neuro-irritability (to consider gabapentin). Also see: Pain Control.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter: 21. Palliative Care, 21.1 Symptom Control, 21.1.3 Neuropsychiatric Symptoms, 21.1.3.3 Dystonia/Muscle Spasms/Spasticity