Sometimes people with dystonic symptoms develop a rare condition called status dystonicus which is intense and frequent episodes of severe generalised dystonia. An individual episode of status dystonicus is called a ‘dystonic storm’ or ‘dystonic attack’. They usually occur in individuals who already have generalised dystonia.
Although the exact numbers are not known, it is thought that mild to moderate dystonic storms are a fairly regular experience for some individuals with generalised dystonia, especially if it is an acquired dystonia. Most severe cases of status dystonicus occur when generalised dystonia appears in combination with other neurological condition or metabolic conditions or if it is has been caused by a traumatic or infective event or trigger.
If a person’s swallowing or breathing is affected by a dystonic storm, they may require emergency medical attention. In very severe cases, there may be renal involvement and individuals may be admitted to an intensive care unit setting where they may be sedated with medication or need temporary mechanical ventilation to support breathing and allow relaxation of dystonic muscles.
Childhood dystonic storms, brittle dystonia and status dystonicus
In this situation, the child is in an unstable motor condition in which the dystonia suddenly flares up in severity, interrupting all normal activities and often requiring additional medication or initiation of an emergency care plan.
A simple scheme for dystonia severity assessment and planning (DSAP) has recently been published (Lumsden et al 2013) in which SAP also stands for ‘soon as possible’ to denote the urgency of getting the dystonia under control.
Five DSAP levels exist:
Level 1: Child sits comfortably and can participate as usual in their normal activities of the day
Level 2: The child cannot sit comfortably or tolerate sitting at all
If the child is constantly slipping from level 1 to level 2 we consider this to be unstable dystonia and the background management needs to be adjusted at home following telephone and outpatient consultations in the local hospital with usual paediatric team. If this happens several times a month, the dystonia may be described as ‘brittle’ in the same way as asthma may be referred to as ‘brittle’, necessitating emergency plans and frequent hospital reviews for advice to adjust medication.
Level 3: The child has difficulty lying and sleeping
This is a potentially very dangerous condition and needs to be managed urgently in a children’s ward where underlying precipitating factors such as respiratory or urinary infections are excluded or if present, are managed with antibiotics. Dystonia-relieving medication, fluids and nutrition are also adjusted and the goal is to achieve good periods of sleep with medication where possible without depressing respiration.
Level 4: Excessive dystonic muscle contractions lead to sweating, fever, muscle damage, dehydration and poor urinary output and hypotension (low blood pressure).
The child is managed in a High Dependency Unit (HDU). Nutrition, fluid and medication may need to be delivered by intravenous infusion or via nasogastric tube if the child is too sick to cooperate. In this condition, oxygen is often required by mask or nasal prongs.
Level 5: Multi-organ failure requiring support.
There is marked muscle breakdown (known as rhabdomyolysis). Multi-organ support is required such as oxygen, mechanical ventilation, renal dialysis, management of clotting disorders and liver support, usually in the setting of a Paediatric Intensive Care Unit (PICU). Deep sedation is essential and support of breathing as a consequence of this is almost invariable. Severe muscle breakdown can lead to renal and hepatic failure as well as disorders of blood clotting referred to as disseminated intravascular coagulation. There may be a need to completely paralyse muscles temporarily and artificially ventilate the child to prevent further muscle breakdown.
A practical guide to managing the child with Status Dystonicus or severe dystonia has recently been reported using the DSAP grades (described above) for guidance (Allen 2014). This guide helps management as the dystonia severity dictates but more work is required determine the best way
of terminating status dystonicus, a condition which may last days, weeks or even months and may be fatal in up to 10% of cases (Fasano 2012).
Last reviewed April 2014
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