1. 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

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

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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)

10. 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.

11. 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

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

13. 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

14. 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

15. References
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 and a review of literature. J Neurol 248:750–5, 2001.
Abe Y, Yamamoto T, Soeda T, et al. Diabetic Striatal Disease: Clinical Presentation, Neuroimaging, and Pathology. Inter Med 48: , 2009.
Lai PH, Tien RD, Chang MH, et al. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol 17: , 1996.
Shan DE, Ho DMT, Chang C, Pan HC, Teng MMH. Hemichoreahemiballism: an explanation for MR signal changes. AJNR Am J Neuroradiol 19: , 1998.
Lai PH, Chen PC, Chang MH, et al. In vivo proton MR spectroscopy of chorea-ballismus in diabetes mellitus. Neuroradiology 43: , 2001.
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: , 2002.
Ohara S, Nakagawa S, Tabata K, Hashimoto T. Hemiballism with hyperglycemia and striatal T1-MRI hyperintensity: an autopsy report. Mov Disord 16: , 2001.
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 (1–2):57–62, 2002.