[P3-78] A Case of Paroxysmal Kinesigenic Dyskinesia with Moyamoya Disease
[Introduction] Paroxysmal kinesigenic dyskinesia (PKD) is a disease characterized by abnormal involuntary movement, such as chorea, athetosis and dystonia, induced by onset of motion. We experienced a case of ten-year-old girl with Moyamoya disease diagnosed PKD.
[Case presentation] She caused multiple cerebral infarctions at four years old, and was diagnosed Moyamoya disease by magnetic resonance imaging (MRI). She had bilateral indirect anastomoses surgery but remained mild right paralysis and dysarthria. At ten years old, she visited our hospital for symptoms of difficulty of speech and gait. She showed dystonia of left lower limb, chorea of left upper limb and tic of left eyelid and mouth precipitated by onset of movement, with no familial history of PKD. Her electroencephalogram showed no epileptic discharge, and furthermore, there is no abnormality on MRI and no abnormal value of blood examination. Single photon emission computed tomography (SPECT) was normal. These symptoms always occurred immediately following movement at awaking state, and didn’t reveal at resting state.We suspected PKD and started carbamazepine 5mg/kg. A few days after, involuntary movement gradually diminished for two years.
[Conclusion] We could diagnose PKD for characteristics of movement and effectiveness of antiepileptic medication. The neural mechanisms of PKD have not been fully unexplained. This case with multiple cortical infarction and imbalance or unstable of intracranial blood flow shows possibility hypothesis that imbalance of cortical-cortical and cortical-basal ganglia circuitry leads involuntary movement as PKD. PKD should be suspected in the case of appearance of involuntary movement with postoperative Moyamoya disease.
[Case presentation] She caused multiple cerebral infarctions at four years old, and was diagnosed Moyamoya disease by magnetic resonance imaging (MRI). She had bilateral indirect anastomoses surgery but remained mild right paralysis and dysarthria. At ten years old, she visited our hospital for symptoms of difficulty of speech and gait. She showed dystonia of left lower limb, chorea of left upper limb and tic of left eyelid and mouth precipitated by onset of movement, with no familial history of PKD. Her electroencephalogram showed no epileptic discharge, and furthermore, there is no abnormality on MRI and no abnormal value of blood examination. Single photon emission computed tomography (SPECT) was normal. These symptoms always occurred immediately following movement at awaking state, and didn’t reveal at resting state.We suspected PKD and started carbamazepine 5mg/kg. A few days after, involuntary movement gradually diminished for two years.
[Conclusion] We could diagnose PKD for characteristics of movement and effectiveness of antiepileptic medication. The neural mechanisms of PKD have not been fully unexplained. This case with multiple cortical infarction and imbalance or unstable of intracranial blood flow shows possibility hypothesis that imbalance of cortical-cortical and cortical-basal ganglia circuitry leads involuntary movement as PKD. PKD should be suspected in the case of appearance of involuntary movement with postoperative Moyamoya disease.