Diabetic striatopathy 2019

BRAIN

Received October 31, 2016; accepted August 30, 2017.

Two Cases of Diabetic Striatopathy: A Rare Movement Disorder Associated with Uncontrolled Diabetes Mellitus

From the Radiology Department at Santa Clara Valley Medical Center (S.L., J.D., R.P., M.P.), San Jose, California.

Presented at the 54th Annual Meeting of the American Society of Neuroradiology, May 23–26, 2016, Washington, DC. Please address correspondence to Stephanie Lin, MD, Radiology Department, Santa Clara Valley Medical Center, 751 S Bascom Ave, San Jose, CA 95128; e-mail: SL457@cornell.edu.

S. Lin, J. Dorr, R. Pandit, and M. Patel

http://dx.doi.org/10.3174/ng.1600046

ABSTRACT

Disclosures Based on information received from the authors, Neurographics has determined that there are no Financial Disclosures or Conflicts of Interest to report.

Diabetic striatopathy is the term used to describe a rare syndrome that occurs in patients with nonketotic hyperglycemia. This entity is uncommonly seen but has a distinctive clinical presentation of hemichorea-hemiballismus, with characteristic imaging findings in the basal ganglia. In this report, we present 2 cases of diabetic striatopathy with a brief review of existing literature.

INTRODUCTION

There are several causes for the clinical presentation of hemichorea-hemiballismus, including stroke, infections, drugs, metabolic derangements, neurodegenerative disorders, neoplasm, and nonketotic hyperglycemia in diabetes mellitus.1 The classic imaging appearance of diabetic striatopathy in the appropriate clinical setting essentially excludes other etiologies and should be recognized by the radiologist. In this report, we present 2 cases of this uncommon syndrome with a brief review of existing literature.

CASE 1

A 56-year-old woman with a history of poorly controlled type 2 diabetes mellitus presented to the emergency department with choreiform involuntary movements of the left upper extremity and left lower extremity, which had begun 3 weeks before presentation and had been progressively worsening. On physical examination, the patient had normal strength and sensation in the left upper and lower extremities, and no additional focal neurologic findings. A noncontrast CT of the 424

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head showed asymmetric hyperattenuation in the right basal ganglia (Fig 1A).

Subsequent noncontrast MR imaging of the brain demonstrated corresponding T1 hyperintensity in the right basal ganglia (Fig 1B). The lesion was isointense to gray matter on T2WI and gradient-echo imaging, and no restricted diffusion was seen (Fig 1D). There was no enhancement on postcontrast T1WI (Fig 2B). Clinically, at presentation, the hemoglobin A1c level was significantly elevated, at 13.7%; fasting blood glucose was mildly elevated, at 168 mg/dL; and results of testing for urine ketones were negative. The patient’s symptoms persisted at her last clinical follow-up 1 year after the initial episode, despite improved glycemic control and symptomatic treatment with risperidone and gabapentin.

CASE 2

A 73-year-old woman with type 2 diabetes mellitus presented to the emergency department with 3 days of involuntary hemiballistic jerking motions of the right lower extremity and associated difficulty with walking. Sensation was normal, and no ad-

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Fig 1. Diabetic striatopathy: Case 1. A, Noncontrast axial CT demonstrates asymmetric hyperattenuation in the right basal ganglia (arrow). B, Axial T1-weighted unenhanced MR imaging in the same patient demonstrates homogeneous T1 hyperintensity in the right basal ganglia (arrow). C, Axial postcontrast T1WI demonstrates no abnormal enhancement. D, Axial DWI demonstrates no restricted diffusion.

Fig 2. Diabetic striatopathy: Case 2. A, Axial noncontrast CT demonstrates hyperattenuation in the left putamen (arrow). B, Axial noncontrast CT performed 2 years later, after strict glycemic control was achieved, demonstrates resolution of imaging abnormalities; the patient’s symptoms had also resolved at this time. C, Axial T1-weighted precontrast MR imaging demonstrates T1 hyperintensity in the left putamen (arrow).

ditional focal neurologic deficits were identified on examination. At presentation, her serum glucose level was elevated to 276 mg/dL, hemoglobin A1c was elevated to 7.9%, and results of testing for urine ketones were negative. Noncontrast CT was performed and demonstrated hyperattenuation of the left putamen (Fig 2A). On MR imaging, there was corresponding T1 hyperintensity in the left putamen (Fig 2C). No signal intensity abnormality was seen on T2WI, gradient-echo imaging, or DWI. The patient was treated with strict glycemic control, with subsequent improvement in her symptoms. A noncontrast CT was performed 2 years later and demonstrated resolution of imaging abnormalities (Fig 2B). The patient’s hemiballismus had also resolved at this time.

DISCUSSION

Diabetic striatopathy occurs in patients with poorly controlled diabetes in the setting of nonketotic hyperglycemia and is more commonly seen in Asian and elderly populations.2,3 Most patients have a history of known diabetes mellitus at the time of presentation, though occasionally patients present with hemichorea-hemiballismus as the first sign of diabetes.3-5 Most patients with diabetic striatopathy

have type 2 diabetes mellitus, though rare cases of patients with type 1 diabetes mellitus have been reported.6-8 Etiology

The exact pathophysiology of diabetic striatopathy is unknown, though several hypotheses exist. Results of early studies indicate petechial hemorrhage as a possible etiology because gradient-echo (GRE) hypointensity in the basal ganglia on MR imaging was reported in several cases.8-10 However, there have also been multiple reported cases of diabetic striatopathy with normal signal on GRE, including the 2 cases presented here, which argues against this hypothesis.4,6 Additional proposed mechanisms include ischemia, diabetes-related microangiopathy, and depletion of ␥-aminobutyric acid.6,11 An increased lactate peak in the basal ganglia has been demonstrated on MRS, which supports the idea that ischemia, presumably due to hyperglycemia and/or hyperviscosity, may play a role in this disease.4,11,12 Six cases have been described in the literature with biopsy or pathology results.6 One common element of these pathologic findings is reactive astrocytosis and neuronal loss, which also suggests that underlying ischemia plays a role in this disease. However, pathology findings in these

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cases have otherwise been inconsistent and have included infarction with microhemorrhage, infarction without microhemorrhage, and reactive astrocytosis without infarction or microhemorrhage.6,10,11 Clinical Presentation

Patients with diabetic striatopathy typically present with unilateral hemichorea-hemiballismus, though bilateral symptoms are seen in up to 11% of patients. Although most cases in the literature describe symptoms of hemichorea or hemiballismus at the time of presentation,2 5 cases of delayed hemichorea-hemiballismus have been described in a study by Abe et al,6 which suggests a possible spectrum of clinical presentation. These patients had symptoms of unilateral extremity weakness or numbness and developed hemichorea or hemiballismus 2–14 days later on the same side as the weakness.6 Laboratory findings included an elevated serum glucose level, elevated hemoglobin A1c level, and the absence of urinary or serum ketones.6 However, a range of serum glucose levels has been reported, with a serum glucose level as low as 169 mg/dL.2 Ketotic hyperglycemia has also been very rarely reported, with at least 3 cases described in the literature.5,13,14

strate hypoattenuation in the basal ganglia on CT, whereas diabetic striatopathy is characteristically hyperattenuating on CT. In addition, clinical history and laboratory findings are generally sufficient to distinguish these diagnoses. For example, patients with manganese toxicity would be expected to have a clinical history of hepatic encephalopathy or total parenteral nutrition, whereas patients with Wilson disease would be expected to have low ceruloplasmin and other systemic involvement.

Treatment and Prognosis

The primary treatment for diabetic striatopathy is correction of the patient’s hyperglycemia and long-term strict glycemic control. If symptomatic treatment for choreiform and ballistic movements is desired, then haloperidol or secondgeneration antipsychotics and anticonvulsants are generally used. The prognosis is fairly good, with improvement or resolution of symptoms usually seen after treatment of the patient’s acute hyperglycemia. However, symptoms may persist in a significant number of patients. Recurrence of hemichorea-hemiballismus may be seen after resolution of the initial episode in up to 13% of patients, usually in the setting of hyperglycemia.2

Imaging

CONCLUSION

The characteristic imaging findings of diabetic striatopathy occur in the basal ganglia, almost always with sparing of the internal capsule and involvement of the putamen. The caudate is more frequently involved than the globus pallidus. Symptoms are most often unilateral with imaging findings seen in the basal ganglia contralateral to the symptomatic side. When symptoms are bilateral, imaging abnormalities are seen in the bilateral basal ganglia. On CT, the classic appearance is homogeneous hyperattenuation in the affected basal ganglia.2 However, CT examination can also be unremarkable.4,6,11 MR imaging is often the next step in the evaluation of the patient. On MR imaging, homogeneous T1 hyperintensity is always seen in the affected basal ganglia. T2 signal intensity is variable and may be hypointense or isointense to gray matter.

There usually is no restricted diffusion, which is useful for excluding acute stroke from the differential diagnosis. GRE signal intensity is also variable, with hypointensity occasionally reported in the literature.4,9 Imaging abnormalities usually resolve after symptom resolution.2

Diabetic striatopathy is a rare syndrome with distinctive imaging findings of T1 hyperintensity on noncontrast MR imaging and homogeneous hyperattenuation on noncontrast CT in the basal ganglia contralateral to the symptomatic side. In the appropriate clinical setting of hemichoreahemiballismus in a patient with nonketotic hyperglycemia, diabetic striatopathy should be the primary diagnostic consideration.

Differential Diagnosis

The differential diagnosis for unilateral homogeneous T1 hyperintensity in the basal ganglia is limited to subacute infarction with petechial hemorrhage. This can be differentiated from diabetic striatopathy with the characteristic clinical context of hemichorea-hemiballismus in a patient with diabetes and nonketotic hyperglycemia. The differential diagnosis for bilateral T1 hyperintensity in the basal ganglia is broader and includes entities such as hypoxic injury, manganese toxicity, chronic liver disease, Wilson disease, and carbon monoxide poisoning. However, most of these entities demon426

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REFERENCES 1. Dewey RB Jr, Jankovic J. Hemiballism-hemichorea: clinical and pharmacologic findings in 21 patients. Arch Neurol 1989; 46:862– 67 2. Oh SH, Lee KY, Im JH, et al. Chorea associated with nonketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. J Neurol Sci 2002;200:57– 62.

10.1016/S0022-510X(02)00133-8 3. 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 2001;248:750 – 55. 10.1007/s004150170089 4. Lai PH, Tien RD, Chang MH, et al. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol 1996;17:1057– 64 5. Lee P, Kek P, Soh A. Hyperglycemia-associated hemichoreahemiballism: the spectrum of clinical presentation. Intern Med 2015;54:1881– 84. 10.2169/internalmedicine.54.4289 6. Abe Y, Yamamoto T, Soeda T, et al. Diabetic striatal disease: clinical presentation, neuroimaging, and pathology. Intern Med 2009;48:1135– 41. 10.2169/internalmedicine.48.1996 7. Aquino JH, Spitz M, Pereira JS. Hemichorea-hemiballismus

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as the first sign of type 1b diabetes during adolescence and its recurrence in the setting of infection. J Child Neurol 2015;30: 1362– 65. 10.1177/0883073814553972 8. Altafullah I, Pascual-Leone A, Duvall K, et al. Putaminal hemorrhage accompanied by hemichorea-hemiballism. Stroke 1990;21:1093–94. 10.1161/01.STR.21.7.1093 9. Suto Y, Mori M, Kagimoto H, et al. A case of hemichorea with hyperglycemia presenting with low signal intensity in the striatum on T2*-weighted gradient-echo magnetic resonance imaging. Rinsho Shinkeigaku 2004;44:86 –90 10. Mestre TA, Ferreira JJ, Pimentel J. Putaminal petechial haemorrhage as the cause of non-ketotic hyperglycaemic chorea: a neuropathological case corelated with MRI findings. J Neurol Neurosurg Psychiatry 2007;78:549 –50

11. Shan DE, Ho DM, Chang C, et al. Hemichorea hemiballism: an explanation for MR signal changes. AJNR Am J Neuroradiol 1998;19:863–70 12. Lai PH, Chen PC, Chang MH, et al. In vivo proton MR spectroscopy of chorea-ballismus in diabetes mellitus. Neuroradiology 2001;43:525–31. 10.1007/s002340100538 13. Iwata A, Koike F, Arasaki K, et al. Blood brain barrier destruction in hyperglycemic chorea in a patient with poorly controlled diabetes. J Neurol Sci 1999;163:90 –93. 10.1016/ S0022-510X(98)00325-6 14. Nabatame H, Nakamura K, Matsuda M, et al. Hemichorea in hyperglycemia associated with increased blood flow in the contralateral striatum and thalamus. Intern Med 1994;33: 472–75. 10.2169/internalmedicine.33.472

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