|Year : 2022 | Volume
| Issue : 3 | Page : 305-308
Diabetic striatopathy: A rare entity
Prabhat Kumar Agrawal, Akhilesh Kumar Singh, Nikhil Pursnani, Upendra Kumar Verma
P.G. Department of Medicine, S.N. Medical College, Agra, India
|Date of Submission||18-Apr-2022|
|Date of Decision||14-Jun-2022|
|Date of Acceptance||25-Jun-2022|
|Date of Web Publication||26-Sep-2022|
Dr. Nikhil Pursnani
P.G. Department of Medicine, S.N. Medical College, Agra.
Source of Support: None, Conflict of Interest: None
Diabetic striatopathy is a rare clinico-radiological phenomenon characterized by hemichorea/hemiballismus, hyperglycemia, hyperosmolarity in the absence of ketoacidosis. It is due to lesion in contralateral basal ganglia which is evident as T1 hyperintensity in MRI brain study. Here we are reporting a case of hemichorea /hemiballismus which was associated with hyperglycemia. Our patient improved significantly once blood glucose was controlled adequately. As it carries good prognosis, we are reporting this rare case to illustrate the importance of recognition of this rare syndrome.
Keywords: Hemichorea, hyperglycemia, striatopathy
|How to cite this article:|
Agrawal PK, Singh AK, Pursnani N, Verma UK. Diabetic striatopathy: A rare entity. J Diabetol 2022;13:305-8
| Introduction|| |
Diabetic striatopathy is a rare syndrome which is characterised by a variety of hyperkinetic movement disorder, hyperglycemia, hyperosmolarity in the absence of ketoacidosis. Hemichorea is an involuntary, non purposeful, irregular and non rhythmic movement affecting one side of body due to lesion in contralateral striatum. Hemiballismus is violent, sudden, flinging, involuntary large amplitude movement predominantly affecting the proximal extremities, involving one side of body due to lesion in contralateral subthalmic nucleus. Therefore, hemichorea hemiballismus (HCHB) is a spectrum of continuous, involuntary and non patterned movements that involve one side of the body.
The common causes of HCHB consist of ischemic or hemorrhagic stroke, head trauma, brain tumor, systemic lupus erythematosus (SLE), Hyperglycemic Hyperosmolar Nonketotic Coma (HONK), Wilson’s disease and thyrotoxicosis. HCHB has been described mainly in elderly females of Asian ethnicity. Females being more affected could be related to post menopausal estrogen induced alterations of mainly two inhibitory neurotransmitters: Gamma Amino Butyric Acid (GABA) and dopamine.
The pathophysiology of this syndrome is not very well known and there are many theories. Cerebral autoregulation of blood flow is altered by hyperglycemia resulting in hypoperfusion and anaerobic metabolism which causes reduction in inhibitory neurotransmitter GABA in basal ganglia resulting in involuntary movements., Hyperviscosity due to high blood glucose level alters blood brain barrier resulting in vascular insufficiency of striatal neurons leading to irregular movements.
Histopathological studies in these patients have shown neuronal loss and gliosis with no evidence of infarction or haemorrhage in striatum.
| Case Presentation|| |
A 45 -year-old female with Type II diabetes for two years presented with one month history of involuntary and unpatterned movements involving right side of the body. Her movements could not be stopped by voluntary control but it stopped while patient was asleep. These movements were not associated with urinary incontinence, tongue bite, eye ball uprolling, altered sensorium or faecal incontinence. There was no past history of cerebrovascular accidents or head trauma. There was no significant drug addiction or family history. Her dietary history revealed that she was taking carbohydrate rich diet, no physical exercise and was not taking antidiabetic drugs regularly.
On examination she was conscious and oriented to time, place and person. Her higher mental functions were intact. All cranial nerves were found to be within normal limits, pupils bilaterally symmetrical normally reacting to light, sensory and motor examination were found to be within normal limits. There were no cerebellar signs or signs of meningial irritation.
Her vitals were normal. Her random blood glucose was 410 mg/dL. Serum potassium was 3.8 mEq/L while serum sodium was 146 mEq/L. Patient’s HbA1c was found to be 9.1%. Her lipid profile was abnormal and showed total cholesterol of 268 mg/dL, LDL level of 179 mg/dL, triglycerides level of 205 mg/dL, and HDL level of 34 mg/dL. Urinary ketones were negative.
Chest X-ray and electrocardiogram were normal. On Slit lamp examination for Kayser-Fleischer ring More Detailss was negative. MRI Brain demonstrated hyperintensity on T1 weighted image in left putamen and left globus pallidus [Figure 1] and [Figure 2]. Blooming was also noted in left globus pallidus and left putamen on Gradient recalled echo (GRE) image. After admission to hospital her blood glucose was monitored and controlled with Human insulin.
|Figure 1: Blooming is noted in left globus pallidus and putamen on GRE image|
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|Figure 2: MRI brain showing T1 hyperintensity of left globus pallidus and putamen|
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To control above mentioned chorieform movements oral administration of Haloperidol 0.5 mg at night was started by third day, her involuntary movements were alleviated. She was discharged on seventh day after hospitalization on haloperidol 0.5 mg at night, rosuvastatin 20 mg at night and a strict insulin regimen. Her blood sugar was well undercontrolled as shown in ambulatory glucose monitoring graph. [Figure 3]
|Figure 3: Her blood sugar was well undercontrolled as shown in ambulatory glucose monitoring graph|
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| Discussion|| |
In this case diabetic striatopathy was diagnosed with help of typical clinical and radiological findings. The literature shows that T1 weighted hyperintensity occurs because of ischemic insult due to hyperviscosity secondary to uncontrolled hyperglycemic states. Cerebral autoregulation of blood flow is altered by hyperglycemia resulting in hypoperfusion and anaerobic metabolism which causes reduction in inhibitory neurotransmitter GABA in basal ganglia resulting in involuntary movements., In uncontrolled diabetic patients metabolic disturbances which are associated with hyperglycemia and vascular insult cause regional cerebral metabolic failure. This event produces metabolic derangement leading to rapid escalation of swollen astrocytes knows as gemistocytes. In diabetic striatopathy MRI brain study shows T1 weighted hyperintensity in caudate nucleus, globus pallidus/striatum. Diffusion weighted study shows restricted diffusion. Blooming in caudate nucleus, globus pallidus and putamen is also seen on GRE image.
Literature shows that these involuntary movements resolve when the blood glucose levels are controlled. Chorea has a temporal relation with blood glucose levels, once blood glucose comes to normal these movements shows resolution. The cornerstone of management is aggressive control of blood glucose level. In some refractory cases medication such as haloperidol and tetrabenazine have been shown to be effective in controlling these movements. Our patient also improved significantly with appropriate treatment which is consistent with literature of this rare disease.
| Conclusion|| |
This case illustrates the importance of recognition of this rare clinico-radiological syndrome because involuntary movements are easily improved once blood glucose level is controlled, hence carries good prognosis. When clinicians come across these involuntary movements, screening for hyperglycemia is essential even in the absence of past history of diabetes.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]