Diabetic Striatopathy: CT and MR Imaging Appearance of a Rare Movement Disorder Associated with Uncontrolled Diabetes Mellitus S Lin MD, J Dorr MD, R Pandit MD Santa Clara Valley Medical Center, San Jose, CA Presentation # eEdE-02

Disclosure The authors have no actual or potential conflicts of interest in relation to this presentation

Goals and Objectives Describe and demonstrate the specific CT and MR imaging characteristics of diabetic striatopathy Review the clinical presentation of hemichorea-hemiballism in diabetic striatopathy Discuss the differential for the imaging features of diabetic striatopathy

Background Diabetic striatopathy is the term used to describe the clinical presentation of hemichorea-hemiballism in non-ketotic hyperglycemia with characteristic imaging findings in the basal ganglia This entity is uncommonly seen, but has distinctive imaging and clinical features In this presentation, we review these features to facilitate recognition of this entity

Epidemiology Diabetic striatopathy occurs with poorly controlled diabetes, specifically in the setting of non-ketotic hyperglycemia More common in Asian and elderly patients Occurs predominantly in patients with type 2 diabetes mellitus

Pathophysiology The exact pathophysiology of diabetic striatopathy is unknown, although several hypotheses exist: Several reported cases demonstrate low signal intensity in the basal ganglia on GRE which raises the possibility of petechial hemorrhage. Hypoperfusion 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. Four cases described in literature were biopsied with pathology demonstrating gemistocytes (reactive astrocytosis), gliosis, and neuronal loss. These findings are also suggestive of underlying ischemia. 6

Clinical Presentation Patients present with neurological symptoms of hemichorea- hemiballism Chorea: brief, irregular “dance like” involuntary motions which have been described as “jerky” Ballism: large amplitude involuntary motion described as more of a violent flinging motion Symptoms are usually unilateral Uncommonly, symptoms can be bilateral in up to 11% of patients Labs: elevated serum glucose without urinary or serum ketones, increased serum osmolality, elevated HgbA1c

Imaging Location: Imaging abnormalities occur in the basal ganglia with sparing of the internal capsule Most commonly in the putamen and caudate When symptoms are bilateral, imaging findings are bilateral When symptoms are unilateral, imaging findings are also unilateral and contralateral to the symptomatic side Imaging findings on CT: Homogeneous hyperattenuation in the basal ganglia Imaging findings on MRI: Homogeneous T1 hyperintensity in the basal ganglia Imaging abnormalities and clinical symptoms generally resolve with better glycemic control

CT: Case 1 History: 56 yo female with history of poorly controlled diabetes presented to the ED with worsening choreiform involuntary movements of the left upper and lower extremity for 3 weeks HgbA1c was 13.7 Non-contrast axial CT demonstrates asymmetric hyperattenuation in the right lentiform nucleus (arrow)

MRI: Case 1 Axial 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 2 Presentation Follow up 2 years later History: 73 yo diabetic female who presented with involuntary hemiballistic jerking motions of the right lower extremity for 3 days HgbA1c was 10.1 At 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. Patient’s symptoms had also resolved at this time. Presentation Follow up 2 years later

MRI: Case 2 At presentation: Axial T1-weighted pre- contrast MRI in the same patient demonstrates T1 hyperintensity in the left putamen.

Differential Diagnosis Unilateral T1 hyperintensity in the basal ganglia ddx: Subacute infarct with petechial hemorrhage Bilateral T1 hyperintensity in the basal ganglia ddx: Hypoxic/anoxic injury: Hypodense basal ganglia on CT May have involvement of the cerebral cortex, hippocampi, and thalami Basal ganglia hemorrhage Manganese toxicity: history of hepatic encephalopathy or total parenteral nutrition Wilson disease: low ceruloplasmin Usually T1 hypointense on MRI, but can be hyperintense Carbon monoxide poisoning: Most commonly occurs in the globus pallidus Can have T1 hyperintensity if there is hemorrhagic necrosis Age-related calcification/mineralization: should be GP mostly, if caudate/putamen involved suspect metabolic condition 13

Treatment and Prognosis Primary treatment: Correction of acute hyperglycemia Long term strict glycemic control Symptomatic treatment of choreiform and/or ballistic involuntary movements: 1st generation antipsychotics such as Haloperidol Less commonly used agents include 2nd generation antipsychotics, anticonvulsants, and gabapentin Prognosis: Symptoms and imaging findings usually resolve after treatment of patient’s hyperglycemia There is recurrence of chorea in up to 13% of patients, usually in the setting of hyperglycemia 1st gen: Haloperidol, Fluphenazine 2nd gen antipsychotics: olanzapine, clozapine, quetiapine, and risperidone Anti seizure:  valproic acid, carbamazepine, oxcarbazepine, topiramate, levetiracetam, and gabapentin Tetrabenazine – dopamine depleter 14

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