Bilateral striopallidodentate calcinosis with paroxysmal kinesigenic dyskinesia.
Bilateral striopallidodentate calcinosis is characterized by calcification of the basal ganglia and other gray matter structures. We describe a 16-year-old boy with paroxysmal kinesigenic dyskinesia. He exhibited mineralization in the basal ganglia, posterior thalami, and dentate nuclei bilaterally, and was diagnosed with sporadic bilateral striopallidodentate calcinosis. The paroxysmal kinesigenic dyskinesia responded to low-dose treatment with carbamazepine (200 mg/day).
Diaz
Gloria E
GE
Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, MN, USA. diazmedina.gloria@mayo.edu
Wirrell
Elaine C
EC
Matsumoto
Joseph Y
JY
Krecke
Karl N
KN
D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D001479:Basal Ganglia / Q000473:pathology*; D002114:Calcinosis / Q000473:pathology*; D002220:Carbamazepine / Q000627:therapeutic use; D002819:Chorea / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male
D000927:Anticonvulsants; D002220:Carbamazepine