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Table 1 Revised clinical diagnostic criteria for primary PKD

From: Recommendations for the diagnosis and treatment of paroxysmal kinesigenic dyskinesia: an expert consensus in China

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.