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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1068–1069
References [1] Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge–Weber syndrome. Neurologist 2011;17:179–84. [2] Gökalp HZ, Ozkal E, Erdogan A, et al. A giant meningioma of the fourth ventricle associated with Sturge–Weber disease. Acta Neurochirugica (Wien) 1981;57:115–20.
[3] Hasselblatt M, Nolte KW, Paulus W. Angiomatous meningioma: a clinicopathologic study of 38 cases. Am J Surg Pathol 2004;28:390–3. [4] Shirley MD, Tang H, Gallione CJ, et al. Sturge–Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 2013;368:1971–9.
http://dx.doi.org/10.1016/j.jocn.2014.11.026
Chorea associated with nonketotic hyperglycemia: An uncommon patient with bilateral movements Lu Wang a,b, Chun-ling Song b,⇑ a b
Department of Neurology, Tianjin Baodi Hospital, 8 Guangchuan Street, Baodi District, Tianjin 301800, China The Second Hospital of Jilin University, Nanguan, Changchun, Jilin, China
a r t i c l e
i n f o
Article history: Received 26 June 2014 Accepted 26 November 2014
Keywords: Chorea Hyperdensity Hyperglycemia Movement disorders
a b s t r a c t We report a 74-year-old woman who presented with bilateral chorea and no known history of diabetes. Movement disorders such as chorea can be observed in patients presenting with hyperglycemia. The occurrence of hemichorea–hemiballism has been increasingly associated with nonketotic hyperglycemia in the medical literature. The chorea disappeared completely after correction of the hyperglycemia. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Movement disorders such as chorea can involve unilateral or sometimes bilateral extremities in association with a nonketotic hyperglycemic state [1]. Several recent studies have documented the characteristic brain imaging feature of hyperdensity in the striate area on CT scans and T1-weighted images (T1WI) of brain MRI. The prognosis is generally good. In this paper, we report a 74-year-old woman who presented with bilateral chorea and brain CT scans which showed hyperdensity in the bilateral striate areas. Her symptoms resolved rapidly after strict glycemic control.
obese habitus she most likely had type 2 diabetes. A CT scan showed hyperdensity in the striate areas bilaterally (Fig. 1). A brain MRI was not performed. Our patient was diagnosed with chorea associated with nonketotic hyperglycemia. The patient was treated with insulin and small doses of haloperidol (1 mg three times daily). A significant reduction in abnormal movements was noted and these movements disappeared within 5 days which prompted discontinuation of the haloperidol treatment. At that time, her blood glucose concentration was 7.1 mmol/L. The patient was discharged from the hospital with instructions for further outpatient management and her glucose was well controlled. After 3 months follow-up the patient remained asymptomatic.
2. Case report 3. Discussion A 74-year-old woman presented to the emergency department with abnormal movements of the head and the upper limbs which had begun approximately 4 days before admission. These movements were involuntary, rapid, irregular and disappeared only during sleep. The movements had not spread to her lower extremities and she was able to walk. She did not complain of any other symptoms, including double vision, dizziness or limb weakness. She had a 10 year history of hypertension but no known diabetic history. On examination, she was conscious and had no apparent cognitive disorders. She had no neurological signs other than the chorea. Laboratory examinations showed that her fasting blood glucose was 30.4 mmol/L (normal range: 3.9–6.1) and her glycohemoglobin level was 14.8% with no ketone bodies in the urine. Her renal and hepatic functions were normal. It was proposed that given her age, highly elevated blood glucose level and morbidly ⇑ Corresponding author. Tel: +86 13920641540. E-mail addresses: [email protected] (L. Wang), [email protected] (C.-l. Song).
Bedwell first described hemichorea associated with nonketotic hyperglycemia (HCNH) in 1960 [2]. Elderly Asian women appear to be predisposed to this disease, suggesting a genetic influence or inadequate diabetes control in this population. Other causes that may need to be ruled out in the relevant clinical setting include Wilson’s disease, Huntington’s chorea and post-streptococcal Sydenham’s chorea [3]. Individuals with no history of diabetes can present with a movement disorder of sudden onset. The combination of hemichorea, nonketotic hyperglycemia and high density in the contralateral striate area of the brain on T1WI obtained by MRI has been considered a unique syndrome. There have been only a few patients with bilateral movements. A metaanalysis of 53 patients found that only six patients exhibited bilateral movements, all others displayed hemichorea and 15 patients also had a focal motor deficit [4]. Our patient had bilateral chorea. The radiological findings of HCNH are unique to this disorder. In addition to brain CT scans that usually show hyperdensity in the striatum contralateral to the affected side and MRI scans that show
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1068–1069
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Fig. 1. Brain CT scans of a 74-year-old woman who presented with bilateral chorea associated with nonketotic hyperglycemia showed hyperdensity in the bilateral striate areas.
hyperintensity on T1WI, single photon emission computed tomography reveals a significant decrease in the ratio of blood flow in affected brain areas in patients compared to that in normal controls, consistent with ischemia. Positron emission computed tomography studies also show reduced rates of brain glucose metabolism in the basal ganglia contralateral to the chorea [5] and diffusion weighted imaging findings suggest the presence of a small focal area of cytotoxic edema in the striate area [6]. Theories proposed to explain these imaging findings include hyperviscosity, petechial hemorrhages, calcium deposition, ischemic infarction, cytotoxic edema and myelin breakdown. That this systemic disorder should cause such localized clinical and radiological findings suggests the possibility that pre-existing basal ganglia disease such as focal small vessel ischemia in addition to hyperglycemia may play a role in causing cellular dysfunction in brain regions affected by HCNH. Hyperglycemia, through the impairment of cerebral autoregulation, may cause hypoperfusion, that is revealed on imaging, and gamma aminobutyric acid depletion in neurons of the basal ganglia as a result of anaerobic metabolism. In addition, hyperglycemia may induce alterations in dopaminergic activity in the striatum of post-menopausal women, as upregulated dopamine receptor activity and decreased dopaminergic catabolism have been observed [7]. This loss of inhibitory neurotransmitters could potentially cause chorea. Chorea associated with nonketotic hyperglycemia generally has a good prognosis. The symptoms tend to improve after blood glucose levels are normalized. Radiological abnormalities may persist for days or weeks after normalization of glycemia [8]. http://dx.doi.org/10.1016/j.jocn.2014.11.026
4. Conclusion Chorea, especially involving bilateral movements, is an unusual complication of uncontrolled diabetes, however, it carries a favorable prognosis in most cases. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Lin JJ, Chang MK. Hemiballism–hemichorea and non-ketotic hyperglycemia. J Neurol Neurosurg Psychiatry 1994;57:748–50. [2] Bedwell SF. Some observations on hemiballismus. Neurology 1960;10:619–22. [3] Kranick SM, Price RS, Prasad S, et al. Reasoning:a 52-year-old woman with subacute hemichorea. Neurology 2008;71:e59–62. [4] Oh SH, Lee KY, Im JH, et al. Chorea associated with non-ketotic 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. [5] Hsu JL, Wang HC, Hsu WC. Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study. J Neurol 2004;251:1486–90. [6] Massaro F, Palumbo P, Falcini M, et al. Generalized chorea-ballism in acute non ketotic hyperglycemia: findings from diffusion-weighted magnetic resonance imaging. Parkinsonism Relat Disord 2012;18:998–9. [7] Fujioka M, Taoka T, Matsuo Y, et al. Magnetic resonance imaging shows delayed ischemic striatal neurodegeneration. Ann Neurol 2003;54:732–47. [8] Pisani A, Diomedi M, Rum A. Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism. J Neurol Neurosurg Psychiatry 2005;76:1717–9.
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1068–1069
References [1] Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge–Weber syndrome. Neurologist 2011;17:179–84. [2] Gökalp HZ, Ozkal E, Erdogan A, et al. A giant meningioma of the fourth ventricle associated with Sturge–Weber disease. Acta Neurochirugica (Wien) 1981;57:115–20.
[3] Hasselblatt M, Nolte KW, Paulus W. Angiomatous meningioma: a clinicopathologic study of 38 cases. Am J Surg Pathol 2004;28:390–3. [4] Shirley MD, Tang H, Gallione CJ, et al. Sturge–Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 2013;368:1971–9.
http://dx.doi.org/10.1016/j.jocn.2014.11.026
Chorea associated with nonketotic hyperglycemia: An uncommon patient with bilateral movements Lu Wang a,b, Chun-ling Song b,⇑ a b
Department of Neurology, Tianjin Baodi Hospital, 8 Guangchuan Street, Baodi District, Tianjin 301800, China The Second Hospital of Jilin University, Nanguan, Changchun, Jilin, China
a r t i c l e
i n f o
Article history: Received 26 June 2014 Accepted 26 November 2014
Keywords: Chorea Hyperdensity Hyperglycemia Movement disorders
a b s t r a c t We report a 74-year-old woman who presented with bilateral chorea and no known history of diabetes. Movement disorders such as chorea can be observed in patients presenting with hyperglycemia. The occurrence of hemichorea–hemiballism has been increasingly associated with nonketotic hyperglycemia in the medical literature. The chorea disappeared completely after correction of the hyperglycemia. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction Movement disorders such as chorea can involve unilateral or sometimes bilateral extremities in association with a nonketotic hyperglycemic state [1]. Several recent studies have documented the characteristic brain imaging feature of hyperdensity in the striate area on CT scans and T1-weighted images (T1WI) of brain MRI. The prognosis is generally good. In this paper, we report a 74-year-old woman who presented with bilateral chorea and brain CT scans which showed hyperdensity in the bilateral striate areas. Her symptoms resolved rapidly after strict glycemic control.
obese habitus she most likely had type 2 diabetes. A CT scan showed hyperdensity in the striate areas bilaterally (Fig. 1). A brain MRI was not performed. Our patient was diagnosed with chorea associated with nonketotic hyperglycemia. The patient was treated with insulin and small doses of haloperidol (1 mg three times daily). A significant reduction in abnormal movements was noted and these movements disappeared within 5 days which prompted discontinuation of the haloperidol treatment. At that time, her blood glucose concentration was 7.1 mmol/L. The patient was discharged from the hospital with instructions for further outpatient management and her glucose was well controlled. After 3 months follow-up the patient remained asymptomatic.
2. Case report 3. Discussion A 74-year-old woman presented to the emergency department with abnormal movements of the head and the upper limbs which had begun approximately 4 days before admission. These movements were involuntary, rapid, irregular and disappeared only during sleep. The movements had not spread to her lower extremities and she was able to walk. She did not complain of any other symptoms, including double vision, dizziness or limb weakness. She had a 10 year history of hypertension but no known diabetic history. On examination, she was conscious and had no apparent cognitive disorders. She had no neurological signs other than the chorea. Laboratory examinations showed that her fasting blood glucose was 30.4 mmol/L (normal range: 3.9–6.1) and her glycohemoglobin level was 14.8% with no ketone bodies in the urine. Her renal and hepatic functions were normal. It was proposed that given her age, highly elevated blood glucose level and morbidly ⇑ Corresponding author. Tel: +86 13920641540. E-mail addresses: [email protected] (L. Wang), [email protected] (C.-l. Song).
Bedwell first described hemichorea associated with nonketotic hyperglycemia (HCNH) in 1960 [2]. Elderly Asian women appear to be predisposed to this disease, suggesting a genetic influence or inadequate diabetes control in this population. Other causes that may need to be ruled out in the relevant clinical setting include Wilson’s disease, Huntington’s chorea and post-streptococcal Sydenham’s chorea [3]. Individuals with no history of diabetes can present with a movement disorder of sudden onset. The combination of hemichorea, nonketotic hyperglycemia and high density in the contralateral striate area of the brain on T1WI obtained by MRI has been considered a unique syndrome. There have been only a few patients with bilateral movements. A metaanalysis of 53 patients found that only six patients exhibited bilateral movements, all others displayed hemichorea and 15 patients also had a focal motor deficit [4]. Our patient had bilateral chorea. The radiological findings of HCNH are unique to this disorder. In addition to brain CT scans that usually show hyperdensity in the striatum contralateral to the affected side and MRI scans that show
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1068–1069
1069
Fig. 1. Brain CT scans of a 74-year-old woman who presented with bilateral chorea associated with nonketotic hyperglycemia showed hyperdensity in the bilateral striate areas.
hyperintensity on T1WI, single photon emission computed tomography reveals a significant decrease in the ratio of blood flow in affected brain areas in patients compared to that in normal controls, consistent with ischemia. Positron emission computed tomography studies also show reduced rates of brain glucose metabolism in the basal ganglia contralateral to the chorea [5] and diffusion weighted imaging findings suggest the presence of a small focal area of cytotoxic edema in the striate area [6]. Theories proposed to explain these imaging findings include hyperviscosity, petechial hemorrhages, calcium deposition, ischemic infarction, cytotoxic edema and myelin breakdown. That this systemic disorder should cause such localized clinical and radiological findings suggests the possibility that pre-existing basal ganglia disease such as focal small vessel ischemia in addition to hyperglycemia may play a role in causing cellular dysfunction in brain regions affected by HCNH. Hyperglycemia, through the impairment of cerebral autoregulation, may cause hypoperfusion, that is revealed on imaging, and gamma aminobutyric acid depletion in neurons of the basal ganglia as a result of anaerobic metabolism. In addition, hyperglycemia may induce alterations in dopaminergic activity in the striatum of post-menopausal women, as upregulated dopamine receptor activity and decreased dopaminergic catabolism have been observed [7]. This loss of inhibitory neurotransmitters could potentially cause chorea. Chorea associated with nonketotic hyperglycemia generally has a good prognosis. The symptoms tend to improve after blood glucose levels are normalized. Radiological abnormalities may persist for days or weeks after normalization of glycemia [8]. http://dx.doi.org/10.1016/j.jocn.2014.11.026
4. Conclusion Chorea, especially involving bilateral movements, is an unusual complication of uncontrolled diabetes, however, it carries a favorable prognosis in most cases. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Lin JJ, Chang MK. Hemiballism–hemichorea and non-ketotic hyperglycemia. J Neurol Neurosurg Psychiatry 1994;57:748–50. [2] Bedwell SF. Some observations on hemiballismus. Neurology 1960;10:619–22. [3] Kranick SM, Price RS, Prasad S, et al. Reasoning:a 52-year-old woman with subacute hemichorea. Neurology 2008;71:e59–62. [4] Oh SH, Lee KY, Im JH, et al. Chorea associated with non-ketotic 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. [5] Hsu JL, Wang HC, Hsu WC. Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study. J Neurol 2004;251:1486–90. [6] Massaro F, Palumbo P, Falcini M, et al. Generalized chorea-ballism in acute non ketotic hyperglycemia: findings from diffusion-weighted magnetic resonance imaging. Parkinsonism Relat Disord 2012;18:998–9. [7] Fujioka M, Taoka T, Matsuo Y, et al. Magnetic resonance imaging shows delayed ischemic striatal neurodegeneration. Ann Neurol 2003;54:732–47. [8] Pisani A, Diomedi M, Rum A. Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism. J Neurol Neurosurg Psychiatry 2005;76:1717–9.
