Table 4. Trigger avoidance.
| Disorder | Cause (gene) | Movement disorder | Other clinical features | Common triggers to avoid or treat | Other treatment | Outcome |
|---|---|---|---|---|---|---|
| Alternating hemiplegia of childhood | Sodium-potassium pump (ATP1A3) | Paroxysmal weakness, dystonia, chorea | Developmental delay, seizures, abnormal eye movements | Stress, fatigue | Flunarizine, sleep | Symptoms can be prevented or attenuated45 |
| Biotin-thiamine responsive basal ganglia disease | Thiamine transporter (SLC19A3) | Episodic or progressive dystonia, parkinsonism, ataxia | Encephalopathy, seizures | Metabolic decompensation caused by fasting, infection or fever | Biotin plus thiamine | Early treatment prevents or reverses symptoms33 |
| Episodic ataxia type 2 | Cacnala | Ataxia (episodic and progressive), dystonia | Migraines, epilepsy | Stress, fasting, fatigue | 4-aminopyridine, acetazolamide | Prevention or reduction of attacks51 |
| Glutaric aciduria type 1 | Glutaryl coa dehydrogenase (GCDH) | Dystonia, parkinsonism | Developmental delay, encephalopathic crises | Metabolic decompensation caused by fasting, infection or fever | Dietary lysine restriction, L-carnitine | Treatment of triggers mitigates long-term deficits42,43 |
| Maple syrup urine disease | Branched chain alpha-ketoacid dehydrogenase complex (BCKDHA, BCKDHB, DBT) | Dystonia, tremor, ataxia (may be paroxysmal) | Developmental delay, episodic encephalopathy | Dietary non-compliance, or metabolic decompensation caused by fasting, infection or fever | Dietary leucine restriction | Treatment of triggers mitigates episodes85 |
| Methylmalonic aciduria | Methylmalonyl coa mutase (MUT) | Dystonia, chorea | Developmental delay, auditory impairment, encephalopathic crises | Dietary non-compliance, or metabolic decompensation caused by fasting infection, or fever | Dietary protein restriction, L-carnitine | Treatment of triggers may attenuate crises44 |
| Paroxysmal kinesigenic dyskinesia | Multiple causes (eg, Prrt2) | Dystonia, chorea, tremor | Sudden movement, stress, fatigue | Carbamazepine, other anticonvulsants | Prevents symptoms in many cases52 | |
| Paroxysmal non kinesigenic dyskinesia | Multiple causes | Dystonia, chorea, tremor | Stress, alcohol, caffeine | Prevents symptoms in many cases52 | ||
| Propionic acidemia | Propionyl coa carboxylase (PCCA, PCCB) | Dystonia, chorea | Developmental delay, encephalopathic crisis, seizures optic atrophy | Dietary non-compliance, or metabolic decompensation caused by fasting, infection or fever | Dietary protein restriction, L-carnitine | Early treatment prevents or mitigates symptoms44 |
| Rapid onset dystonia-Parkinsonism1 | Sodium-potassium pump (ATP1A3) | Dystonia, parkinsonism, tremor, ataxia | Cognitive or psychiatric impairment, seizures | Emotional or physical stress, fatigue, alcohol | Complete or partial prevention of symptoms45 |
Many of these disorders have variable or mixed phenotypes. The most common or most dominant movement disorder shown in boldface type. The term metabolic decompensation refers to a state in which the body's consumption of a metabolite (e.g, energy.) exceeds supply.