Perfusion abnormality of the caudate nucleus in patients with paroxysmal kinesigenic choreoathetosis

Purpose: Previous cerebral blood flow and glucose metabolism studies suggest that the basal ganglia or thalamus is involved in the pathogenesis of paroxysmal kinesigenic choreoathetosis (PKC). However, the underlying cerebral abnormalities in idiopathic PKC have not been elucidated. To localise cerebral perfusion abnormalities in PKC, we performed interictal brain perfusion ^99mTc-ethylcysteinate dimer (ECD) single-photon emission computed tomography (SPECT) in PKC patients and in normal controls. Methods: Sixteen patients with idiopathic PKC and 18 age- and sex-matched normal controls were included. The patients were de novo diagnosed as having PKC, or had not taken medication for at least 3 months; none of them had structural abnormalities on MRI. Patients had a history of PKC attacks of a duration not exceeding 5 min and starting either on one side or on both sides of the body. These attacks were always induced by a sudden initiation of voluntary movement. PKC attacks were recorded in a hospital after being induced by neurology staff in 13 of the 16 patients. Interictal brain perfusion ^99mTc-ECD SPECT was performed in all 16 patients and 18 normal controls. Differences between the cerebral perfusion in the PKC group and the normal control group were tested by statistical parametric mapping. Student's t test was used for inter-group comparisons. Results: Compared with normal controls, patients with idiopathic PKC showed interictal hypoperfusion in the posterior regions of the bilateral caudate nuclei (false discovery rate-corrected P<0.001 with a small volume correction). Conclusion: This study showed that cerebral perfusion abnormality of bilateral caudate nuclei is present in idiopathic PKC.