OBJECTIVE: Diabetic striatopathy (DS) is characterized by a hyperglycemic state associated with chorea/ballism, and/or striatal hyperdensity on computed tomography, or hyperintensity on T1-weighted magnetic resonance imaging. To date, there have been only a few case series reported in the literature on this topic. In the present study, we report four cases of DS associated with movement disorders. METHODS: The patients were recruited based on the presence of hyperglycemia associated with chorea/ballism or striatal hyperintensity on T1-weighted magnetic resonance imaging. RESULTS: Four patients with DS (two men and two women), with a mean age of 61 years, were included in our study. Three out of the four patients had a previous diagnosis of type 2 diabetes mellitus. The mean blood glucose level on admission and glycated hemoglobin were 390.25 mg/dl and 12.45%, respectively. Hemiballism was present in two patients: one patient had dystonia and the other had choreiform movement at presentation. The putamen was affected in all patients, with involvement of the globus pallidus and caudate nucleus in one patient. All patients had resolution of their abnormal movements after glucose-lowering therapy, with additional use of anti-chorea medication in three patients. CONCLUSION: DS should be considered in elderly patients who present with chorea/ballism/dystonia and should be accordingly managed with resolution of abnormal movements. In addition, dystonia can be a presenting symptom in DS.
Keywords: Chorea hyperglycemia basal ganglia syndrome, diabetic hemiballism/hemichorea, dystonia, diabetic striatopathy, hyperglycemic non-ketotic hemichorea/hemiballism
|How to cite this URL:|
Ranjan A, Mudassir S, Sinha N, Kumar A. Diabetic striatopathy: A case series of rare and treatable movement disorder. Ann Mov Disord [Epub ahead of print] [cited 2023 Jun 1]. Available from: https://www.aomd.in/preprintarticle.asp?id=368621
| Introduction|| |
Diabetic striatopathy (DS) is a rare neurological condition characterized by uncontrolled hyperglycemia associated with chorea/ballism and abnormality of the basal ganglia on computed tomography/magnetic resonance imaging (CT/MRI). In addition, it is known as chorea hyperglycemia basal ganglia syndrome, hyperglycemic non-ketotic hemichorea/hemiballism, or diabetic hemiballism/hemichorea. It is a reversible condition with resolution of abnormal movements and radiological manifestations after the correction of hyperglycemia, commonly reported to occur in elderly women of Asian origin. Acute cerebrovascular insult, infectious/immunological diseases, toxins, and malignant diseases are other known causes of hemichorea/hemiballism. Here, we present a report on four cases of DS with varied manifestations.
| Methods|| |
Four patients with DS who fulfilled the criteria of a hyperglycemic state associated with both or one of the two following conditions were included in the study: (1) chorea/ballism and (2) striatal hyperintensity on T1-weighted MRI. Demographic, clinical, biochemical, and neuroimaging findings of these patients were noted. None of the patients had previous or present history of cerebrovascular insult or movement disorders, exposure to potentially offending drugs, or family history of movement disorders.
| Results|| |
Two men and two women with a mean age of 61 years (range: 52–70 years) were included in the study [Table 1]. The mean blood glucose level on admission and glycated hemoglobin were 390.25 mg/dl and 12.45%, respectively. Three patients had non-ketotic hyperglycemia (NKH) on admission. One patient that is, third patient, (patient in video 3) had a blood glucose level of 177 mg/dl on admission but had history of NKH (blood glucose: 584 mg/dl) 1 month prior to onset of the involuntary movements. Urinalysis for ketones and blood gas analysis for acidosis were negative in all patients.
In two patients (patients in video 1 and 4 Video 1), involuntary movement was classified as left arm–leg hemiballism. Choreiform movement involving the face–arm–leg was observed in patient 3, and patient 2 had left arm dystonic tremor [Video 1]. T1-weighted MRI showed contralateral striatal hyperintense signals in all patients. The putamen was involved in all cases, with additional involvement of the globus pallidus and caudate nucleus in one patient [video 4] [Figure 1]. Susceptibility-weighted imaging showed blooming in the bilateral putamen in only one patient [video 3; [Figure 2]]. All cases were managed using anti-chorea drugs, in addition to hydration and control of blood sugar. Patients 2 and 3 completely recovered on day 7, while patients in v1 and 4completely recovered at the 1-month follow-up, and their symptomatic drugs were withdrawn [Video 1, 2, 3 and 4] [Additional file 1][Additional file 2] [Additional file 3] [Additional file 4].
|Figure 1: (a–c) - T1- and T2-weighted magnetic resonance imaging (MRI) of the brain in axial sections demonstrates hyperintense signals in the right putamen, caudate, and globus pallidus in the fourth patient (blue arrows). (d and e) - Small areas of hypointense signal are observed on T2-weighted and fluid-attenuated inversion recovery images (green arrows). (f) Diffusion restriction not appreciated on diffusion-weighted imaging|
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|Figure 2: (a). Hyperintense signal is observed in the right putamen on T2-weighted MRI of the brain (orange arrows). (b). T2-weighted MRI of the brain displays corresponding mild hypointensity (yellow arrows). (c) Blooming artifact is evident in the bilateral basal ganglia on the axial susceptibility-weighted imaging sequence in the third patient (green arrows).|
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| Discussion|| |
Uncontrolled hyperglycemia is associated with various neurological manifestations such as seizures, encephalopathy, and movement disorders. DS is characterized by uncontrolled hyperglycemia with striatal abnormalities on neuroimaging and contralateral movement disorders.
In addition, DS is usually associated with NKH, as shown in our study. In NKH, the metabolism of the brain changes from the Krebs cycle to an alternative anaerobic pathway, leading to depletion of the inhibitory neurotransmitter gamma-aminobutyric acid into succinic acid, causing disinhibition of the thalamus by the globus pallidus. On the other hand, in diabetic ketoacidosis, acetoacetate produces gamma-aminobutyric acid, leading to decreased incidence of striatopathy. Cerebrovascular pathology in the form of inflammation, necrosis, and neovascularization in the striatum, similar to diabetic retinopathy, is another hypothesis proposed for DS. DS is associated with uncontrolled hyperglycemia for a long duration; however, it has also been reported as a presentation of new-onset type 2 diabetes mellitus or after the correction of hyperglycemia as observed in our case report. DS occurring 1 month after an episode of hyperglycemia could be due to the delayed ischemic effect of uncontrolled hyperglycemia. Chorea/ballism is described as unilateral in >90% of the patients with DS, similar to that observed in our study, where three patients had unilateral left-sided involvement. Although one of our patients had dystonia in the left upper limb and only two cases have been previously reported, dystonia is rarely associated with uncontrolled hyperglycemia.
To date, only few case series from India showing the association of striatopathy with NKH have been published in the literature [Table 1]. In study by Misra et al., all three patients had complete recovery of abnormal movements with hydration and control of blood sugar. Hemichorea completely subsided in a single patient with insulin therapy and hydration, while the remaining two patients required additional anti-chorea drugs for suppression of abnormal movements in study by Cherian et al.
One of the hallmark features of DS is the involvement of the striatum on neuroimaging with the putamen being the most common, followed by the caudate nucleus, globus pallidus, and sparing of the internal capsule. Isolated putamen involvement is more common than the combined involvement of the putamen–caudate nucleus, as shown in our study. Striatal abnormalities are commonly observed as T1 hyperintensity, while blooming is reported on susceptibility-weighted imaging, as found in our patient, and diffusion restriction is reported on diffusion-weighted imaging. Striatal abnormalities in DS may be due to the deposition of mineral, destruction of myelin, petechial hemorrhage, or gemistocyte accumulation. Calcification, bleed, toxins, and metabolic and genetic diseases are the other known causes of striatal hyperintensity on MRI. Adequate hydration and management of hyperglycemia remains the principal line of management for DS. Patients who continue to have chorea may require to be treated with anti-chorea drugs, as was the case in three of our patients.
In conclusion, DS can be a presenting symptom in patients with new-onset type 2 diabetes mellitus. In addition to chorea/ballism, dystonia can be present in DS, and patients may present with symptoms weeks after correction of hyperglycemia with a favorable outcome.
Consenting details and publishing
Written informed consent was taken from the patient for publication and dissemination.
I want to acknowledge my patient for their cooperation and consent for this Publication.
- (1) Abhay ranjan- Conception and design, analysis and interpretation of data, drafting the article or revising it critically for important intellectual content; and final approval of the version to be published
- (2) Sanaullah Mudassir- Acquisition of data, analysis of data, 1st draft of the article
- (3) Neetu Sinha- Acquisition of data, analysis and interpretation of data, Drafting of article.
- (4) Abhishek Kumar- Acquisition of data and analysis and interpretation of data.
Ethical compliance statement
Written informed consent was taken from the patient for online publication and dissemination. The authors confirm that the approval of an institutional review board was not required for this work. They also confirm that they have read the Journal’s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines
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Conflicts of interest
There are no conflicts of interest.
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Department of Neurology, Indira Gandhi Institute of Medical Sciences, Sheikhpura, Patna - 800 014, Bihar
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]