Diabetic-Striatopathy-CT-and-MR-Imaging-Appearance.ppt

Diabetic Striatopathy:CT and MR Imaging Appearance of a Rare Movement Disorder Associated with Uncontrolled Diabetes MellitusS Lin MD,J Dorr MD,R Pandit MDSanta Clara Valley Medical Center,San Jose,CAPresentation#eEdE-02DisclosurevThe authors have no actual or potential conflicts of interest in relation to this presentationGoals and ObjectivesvDescribe and demonstrate the specific CT and MR imaging characteristics of diabetic striatopathyvReview the clinical presentation of hemichorea-hemiballism in diabetic striatopathyvDiscuss the differential for the imaging features of diabetic striatopathyBackgroundvDiabetic striatopathy is the term used to describe the clinical presentation of hemichorea-hemiballism in non-ketotic hyperglycemia with characteristic imaging findings in the basal gangliavThis entity is uncommonly seen,but has distinctive imaging and clinical featuresvIn this presentation,we review these features to facilitate recognition of this entityPathophysiologyThe exact pathophysiology of diabetic striatopathy is unknown,although several hypotheses exist:vSeveral reported cases demonstrate low signal intensity in the basal ganglia on GRE which raises the possibility of petechial hemorrhage.vHypoperfusion and increased lactate peak have been demonstrated on SPECT which suggests that ischemia,presumably due to hyperglycemia and/or hyperviscosity,may play a role in this disease.vFour cases described in literature were biopsied with pathology demonstrating gemistocytes(reactive astrocytosis),gliosis,and neuronal loss.These findings are also suggestive of underlying ischemia.Clinical PresentationvPatients present with neurological symptoms of hemichorea-hemiballismChorea:brief,irregular“dance like”involuntary motions which have been described as“jerky”Ballism:large amplitude involuntary motion described as more of a violent flinging motionvSymptoms are usually unilateralUncommonly,symptoms can be bilateral in up to 11%of patientsvLabs:elevated serum glucose without urinary or serum ketones,increased serum osmolality,elevated HgbA1cImagingvLocation:Imaging abnormalities occur in the basal ganglia with sparing of the internal capsuleMost commonly in the putamen and caudateWhen symptoms are bilateral,imaging findings are bilateralWhen symptoms are unilateral,imaging findings are also unilateral and contralateral to the symptomatic sidevImaging findings on CT:Homogeneous hyperattenuation in the basal gangliavImaging findings on MRI:Homogeneous T1 hyperintensity in the basal gangliavImaging abnormalities and clinical symptoms generally resolve with better glycemic control MRI:Case 1Axial T1-weighted pre-contrast MRI in the same patient demonstrates homogeneous T1 hyperintensity in the right lentiform nucleus with sparing of the internal capsule.No abnormal enhancement was seen on post-contrast images.CT:Case 2History:73 yo diabetic female who presented with involuntary hemiballistic jerking motions of the right lower extremity for 3 daysHgbA1c was 10.1At presentation:Axial non-contrast CT demonstrates homogeneous hyperattenuation in the left putamen.Follow-up:Axial non-contrast CT performed 2 years later,after strict glycemic control was achieved,demonstrates resolution of imaging abnormalities.Patients symptoms had also resolved at this time.PresentationFollow up 2 years laterDifferential DiagnosisvUnilateral T1 hyperintensity in the basal ganglia ddx:Subacute infarct with petechial hemorrhagevBilateral T1 hyperintensity in the basal ganglia ddx:Hypoxic/anoxic injury:Hypodense basal ganglia on CTMay have involvement of the cerebral cortex,hippocampi,and thalamiBasal ganglia hemorrhageManganese toxicity:history of hepatic encephalopathy or total parenteral nutritionWilson disease:low ceruloplasminHypodense basal ganglia on CTUsually T1 hypointense on MRI,but can be hyperintenseCarbon monoxide poisoning:Most commonly occurs in the globus pallidus Hypodense basal ganglia on CTCan have T1 hyperintensity if there is 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