ABSTRACT
PURPOSE: Paroxysmal kinesigenic dyskinesia (PKD) is one of the movement disorders in which dyskinesia occurs in a part of the body by a sudden movement after a rest under a tension or a stress. This study was aimed to evaluate the clinical features of children and adolescents with PKD in Korea via analysing the patients who have treated in Department of Pediatrics, Kyungpook National University Hospital. METHODS: A total of seven children with PKD was involved in the study and their medial records were retrospectively evaluated. RESULTS: The mean age of the 7 subjects was 15.7 years (10.0-21.4 years old). The male to female ratio was 6:1. They presented with dystonia with the average duration of 10.5 seconds (3.5-17.5 seconds), which triggered by various sudden movements. No accompanying cormorbidities were noted. Their laboratory findings were unremarkable. Six of the patients, except one who refused treatment with medicine, responded well to medication and remained symptom free. The average time response to medication was 3.4 weeks (0.95-7.81 weeks). They were of treated with either oxcarbazepine (n=4, 14.9+/-5.8 mg/kg/day) or lamotrigine (n=2, 1.5+/-0.9 mg/kg/day). There was no significant difference between two groups in terms of age, response, adverse events, and so on. CONCLUSION: This study showed that clinical features of Korean children with PKD are quite similar to those of other countries. They responded well to the medication. In addition, lamotrigine can be an alternative choice for the treatment.
Subject(s)
Adolescent , Child , Female , Humans , Male , Carbamazepine , Chorea , Dyskinesias , Dystonia , Korea , Movement Disorders , Pediatrics , Retrospective Studies , TriazinesABSTRACT
A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.
Subject(s)
Humans , Infant , Anaplasia , Chemotherapy, Adjuvant , Fever , Follow-Up Studies , Hematuria , Kidney , Kidney Neoplasms , Kidney Pelvis , Muscle, Skeletal , Recurrence , Ureter , Vincristine , Vomiting , Wilms TumorABSTRACT
A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.