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1、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 rela
2、tion 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 striatopath
3、yBackgroundvDiabetic 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 presentat
4、ion,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 pet
5、echial 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(r
6、eactive 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
7、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 abno
8、rmalities 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 C
9、T: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 homo
10、geneous 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
11、 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 tim
12、e.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 co
13、rtex,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
14、 globus pallidus Hypodense basal ganglia on CTCan have T1 hyperintensity if there is hemorrhagic necrosisReferences1.Lin JJ,Lin GY,Shih C.Presentation of striatal hyperintensity on T1-weighted MRI in patients with hemiballism-hemichorea caused by non-ketotic hyperglycaemia:report of seven new cases
15、and a review of literature.J Neurol 248:7505,2001.2.Abe Y,Yamamoto T,Soeda T,et al.Diabetic Striatal Disease:Clinical Presentation,Neuroimaging,and Pathology.Inter Med 48:1135-1141,2009.3.Lai PH,Tien RD,Chang MH,et al.Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus.AJNR A
16、m J Neuroradiol 17:1057-1064,1996.4.Shan DE,Ho DMT,Chang C,Pan HC,Teng MMH.Hemichoreahemiballism:an explanation for MR signal changes.AJNR Am J Neuroradiol 19:863-870,1998.5.Lai PH,Chen PC,Chang MH,et al.In vivo proton MR spectroscopy of chorea-ballismus in diabetes mellitus.Neuroradiology 43:525-53
17、1,2001.6.Chu K,Kang DW,Kim DE,Park SH,Rho JK.Diffusion weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia:a hyperviscosity syndrome?Arch Neurol 59:448-452,2002.7.Ohara S,Nakagawa S,Tabata K,Hashimoto T.Hemiballism with hyperglyce
18、mia and striatal T1-MRI hyperintensity:an autopsy report.Mov Disord 16:521-525,2001.8.Oh SH,Lee KY,Im JH,Lee MS.Chorea associated with nonketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study:a metaanalysis of 53 cases including four present cases.J Neurol Sci (12):5762,2002.