Alteration of Ictal and Interictal Perfusion in Patients with Paroxysmal Kinesigenic Dyskinesia

Abstract

Although previous cerebral blood flow studies have suggested that the basal ganglia or thalamus are involved in the pathogenesis of paroxysmal kinesigenic dyskinesia (PKD), the precise anatomic substrate or pathophysiological networks associated with PKD remain unclear. Here, ictal and interictal single photon emission computed tomography (SPECT) in 2 patients with idiopathic PKD compared to 6 age-matched normal controls and the perfusion findings of subtraction ictal SPECT co-registered to MRI (SISCOM) in 1 patient are reported. The interictal and ictal perfusion changes were different in each of the patients and there were no consistent anatomic substrates observed. 2 patients had significant perfusion changes in the left frontal/temporal cortices compared to controls, whereas the others showed an increased uptake of ^99mTc-ethyl cysteinate dimer (ECD) in the left occipital area on subtraction SPECT imaging. The results of this study suggest that the pathophysiology of PKD cannot be simply explained by lesions of the basal ganglia or thalamus, and that other associated areas of the cortex are likely involved in these movement disorders.

Key words

paroxysmal kinesigenic dyskinesia (PKD) - cerebral blood flow - SPECT

• References

• 1 Demirkiran M, Jankovic J. Paroxysmal dyskinesias: clinical features and classification. Ann Neurol 1995; 38: 571-579
• 2 Kertesz A. Paroxysmal kinesigenic choreoathetosis. An entity within the paroxysmal choreoathetosis syndrome. Description of 10 cases, including 1 autopsied. Neurology 1967; 17: 680-690
• 3 Guerrini R. Idiopathic epilepsy and paroxysmal dyskinesia. Epilepsia 2001; 42: S36-S41
• 4 Hayashi R, Hanyu N, Yahikozawa H et al. Ictal muscle discharge pattern and SPECT in paroxysmal kinesigenic choreoathetosis. Electromyogr Clin Neurophysiol 1997; 37: 89-94
• 5 Ko CH, Kong CK, Ngai WT et al. Ictal ^99mTc ECD SPECT in paroxysmal kinesigenic choreoathetosis. Pediatr Neurol 2001; 24: 225-227
• 6 Houser MK, Soland VL, Bhatia KP et al. Paroxysmal kinesigenic choreoathetosis: A report of 26 patients. J Neurol 1999; 246: 120-126
• 7 Shirane S, Sasaki M, Kogure D et al. Increased ictal perfusion of the thalamus in paroxysmal kinesigenic dyskinesia. J Neurol Neurosurg Psychiatry 2001; 71: 408-410
• 8 Joo EY, Hong SB, Tae WS et al. Perfusion abnormality of the caudate nucleus in patients with paroxysmal kinesigenic choreoathetosis. Eur J Nucl Med Mol Imaging 2005; 32: 1205-1209
• 9 Mir P, Huang YZ, Gilio F et al. Abnormal cortical and spinal inhibition in paroxysmal kinesigenic dyskinesia. Brain 2005; 128: 291-299
• 10 Van Paesschen W. Ictal SPECT. Epilepsia 2004; 45: S35-S40