Core symptoms: | |
1. Kinesigenic triggers and attacks presenting as dystonia, chorea, ballism, or a combination of them; 2. No impairment of awareness during attacks. | |
Supportive evidence: | |
1. Presence of aura; 2. Attack duration < 1 min; 3. Positive result of high-knee exercise test; 4. Good response to low-dose voltage-gated sodium channel blockers, especially carbamazepine/ oxcarbazepine. | |
Diseases listed in the following should be excluded: 1. Cerebrovascular disease; 2. Demyelinating disease, especially multiple sclerosis; 3. Metabolism disorders: a. Hyperthyroidism; b. Calcium-phosphate metabolism disorders (hypoparathyroidism, pseudoparathyroidism, parathyroid hyperthyroidism, pseudoparathyroid hyperplasia), primary familial brain calcification; c. Glucose metabolism disorder; d. Kernicterus 4. Brain trauma; 5. Psychological disorder. The following red flags may indicate secondary causes or an alternative diagnosis: 1. Duration of attacks > 1 min; 2. Age of onset over 20 years; 3. Abnormalities in brain CT/MRI scanning or the presence of other neurologic/systemic problems; 4. No response to anticonvulsants; 5. Abnormal results of interictal examinations. |