JNS-13176; No of Pages 3 Journal of the Neurological Sciences xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Hemichorea in a patient with diabetic ketoacidosis Ahmed M. Yassin a, Sheetal Shroff a,⁎, Shreshta D. Patel a, Asif M. Paker b, Megan A. Berman a, George R. Jackson c a b c
The University of Texas Medical Branch, Galveston, TX, United States Massachusetts General Hospital, Boston, MA, United States Baylor College of Medicine, Houston, TX, United States
a r t i c l e
i n f o
Article history: Received 26 February 2014 Received in revised form 26 March 2014 Accepted 24 April 2014 Available online xxxx Keywords: Chorea Diabetic ketoacidosis Non-ketotic hyperglycemia Hypothyroidism Vitamin B12 deficiency Basal ganglia
a b s t r a c t Background: Chorea is a common presenting feature of metabolic disorders, including nonketotic hyperglycemia in patients with type 2 diabetes mellitus, but rarely has been reported in diabetic ketoacidosis, hypothyroidism and vitamin B12 deficiency. Methods: Review the literature for reported cases of chorea as a presenting manifestation in metabolic disorders. Results: We report a case of hemichorea in a patient with type 2 diabetes mellitus complicated by diabetic ketoacidosis. The patient had a two day history of right sided hemichorea and decreased level of consciousness. Initial laboratory studies revealed hyperglycemia, ketosis and an anion gap metabolic acidosis consistent with diabetic ketoacidosis. Once treatment was started the choreiform movements significantly improved over three weeks. Conclusion: Although DKA has been rarely reported as a trigger for chorea, it should be in the differential diagnosis of a patient presenting with an acute chorea. Given the reversible nature of this disease, early recognition and treatment are imperative. © 2014 Elsevier B.V. All rights reserved.
1. Introduction Chorea is a prototypic hyperkinetic movement disorder. It is a common symptom of neurological diseases that directly affect the basal ganglia, such as Huntington's disease. However, many systemic disorders also present with chorea, including metabolic disorders such as diabetes mellitus. Nonketotic hyperglycemia (NKH) is an established cause of chorea, and many cases have been reported. On the other hand, diabetic ketoacidosis (DKA) has rarely been reported as a cause for chorea. We report a case of DKA that induced a hemichorea. 1.1. Case report A 69 year old Hispanic male presented with two day history of involuntary movements of his arms and legs and decreased level of consciousness. The patient was confused and could not provide further history. Co-morbidities are type 2 diabetes mellitus, hypertension, and hyperlipidemia. He did not have any family history of any neurological diseases. He did not smoke, drink alcohol or use illicit drugs. His medications included insulin, glipizide, enalapril, hydrochlorothiazide and pravastatin. However, previous clinic notes reported that the patient was non-compliant with diabetic regimen.
⁎ Corresponding author at: Department of Neurology, The University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555, United States. Tel.: +1 979 824 2467. E-mail address: [email protected] (S. Shroff).
On examination, his vital signs were as follows: temperature 37 °C, blood pressure 150/90 mm Hg, respiratory rate 20/min, pulse 85 beats per minute, and oxygen saturation 95% on ambient air. He was somnolent and disoriented to time and place. He was unable to follow simple commands. Pupils were equal and reactive to light bilaterally, extraocular movements were normal, face was symmetrical and tongue was in midline. He was moving all his extremities antigravity and was difficult to maintain the effort (4/5). He had choreiform movements of right upper extremity and bilateral lower extremities (R N L). He was unsteady due to choreiform movements that subsided during sleep. Reflexes were difficult to elicit due to impaired relaxation. Babinski sign was absent. His labs were significant for blood glucose 380 mg/dl (70–110 mg/dl), anion gap 21 [12–16], serum ketones 1 + and glycosylated hemoglobin 16.3% (4–6%). One week after admission additional labs revealed TSH 19.9 μIU/ml (0.45–4.7 μIU/ml), free T4 0.85 ng/dl (0.78–2.2 ng/dl), T3 96.8 ng/dl (97–170 ng/dl), vitamin B12 b 159 Pg/ml (240–930 Pg/ml). Computed-tomography (CT) of his head was negative for a stroke, bleeding or mass in the vicinity of the basal ganglia. Further studies including electroencephalogram, cerebrospinal fluid analysis, serum and urine copper, ceruloplasmin, iron, ferritin and antinuclear antibody were unremarkable. Huntington repeat expansion analysis revealed 15 repeats which is within the normal limits. His DKA was treated with intravenous fluids and insulin. DKA resolved the second day of hospitalization, and he then became fully oriented. His glucose continued to be controlled in the range of 100–200,
http://dx.doi.org/10.1016/j.jns.2014.04.038 0022-510X/© 2014 Elsevier B.V. All rights reserved.
Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
2
A.M. Yassin et al. / Journal of the Neurological Sciences xxx (2014) xxx–xxx
and his chorea improved over 2–3 days. One week after admission, he was found to have hypothyroidism and vitamin B12 deficiency. The patient was started on levothyroxine and intramuscular vitamin-B12. On three month follow-up clinic visit he was alert and oriented, with chorea only confined to his right foot. Strength was 5/5 in upper and lower extremities, reflexes were present throughout. Sensory exam was normal. He was able to walk with minimal assistance.
2. Discussion Chorea is defined as brief, non-stereotyped, rapid, involuntary and purposeless movements involving the upper and/or lower extremities [1]. Chorea can be a symptom of neurological diseases affecting basal ganglia. However, many other conditions can present with chorea such as stroke, uncontrolled diabetes, hyperthyroidism and paraneoplastic disorders. Some drugs are also known to induce chorea [2]. Chorea or ballismus has been reported in different states of diabetes mellitus. It has been widely reported in cases of NKH [3,4]. It has been reported in new onset diabetes mellitus [5]. Persistent chorea can be also triggered by hyperglycemic crisis in diabetic patients [6]. Chorea might also be associated with poorly controlled diabetes in elderly patients [7]. Despite the large number of cases of chorea reported in the setting of NKH, a few cases have been reported in DKA. There have been cases of DKA-induced chorea in patients with previously asymptomatic neurological diseases (e.g. Moyamoya disease, Huntington's disease) [8,9]. After excluding other causes, DKA has been reported rarely as the sole trigger of chorea, similar to our patient [4,10]. This was confirmed by the temporal relationship between DKA and the abnormal movements,
and by improvement of chorea in few days to weeks following control of blood glucose and treatment of ketoacidosis. NKH induced chorea is typically associated with unilateral or bilateral lesions of the putamen or caudate nucleus on CT and/or T1-weighted MRI images [3–7]. These findings may be related to petechial hemorrhage or ischemia. Our patient's T1-weighted MRI showed increased signal intensity in the putamen and anterolateral globus pallidus bilaterally (Fig. 1, panels B and E). Although many mechanisms have been proposed for NKH induced chorea, there are no hypotheses to specifically explain DKA induced chorea. Some of the mechanisms reported for NKH induced chorea are: thalamic disinhibition from depletion of inhibitory neurotransmitters, diabetic vasculopathy with consequent vascular insufficiency of the striatum, acute basal ganglia dysfunction secondary to hyperglycemia, hyperosmolarity, hyperviscosity, petechial hemorrhage or inflammatory processes [11–14]. One of the hypotheses suggests that in NKH, both gammaaminobutyric acid (GABA) and acetylcholine are utilized as an alternative source of energy. Depletion of these neurotransmitters leads to a disinhibition of the thalamus resulting in hyperkinesias [11,12]. However, in DKA acetoacetate produced from the liver can be used to produce GABA. This suggests that metabolic derangement alone cannot explain this phenomenon. Diabetic vasculopathy with a consequent cerebrovascular insufficiency is another hypothesized mechanism. A striatal biopsy of a patient showed hyaline degeneration with thickening of the arteriolar walls, patchy necrotic tissue and prominent vascular proliferation. Abe Y et al. described the findings as “diabetic striatopathy” and added that “vulnerability to this obliterative vasculopathy is restricted to the striatum” [13]. We suggest that multiple mechanisms are involved in the development of chorea in DKA. At this time it is unclear
Fig. 1. MRI showing axial DWI, T1, T2, T2/FLAIR sequence and coronal T1, T2 sequence images respectively. T1-weighted magnetic resonance imaging (MRI) showed increased signal intensity in the putamen and anterolateral globus-pallidus bilaterally.
Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
A.M. Yassin et al. / Journal of the Neurological Sciences xxx (2014) xxx–xxx
as to what extent each of the above mentioned mechanisms are contributing. The prognosis is favorable with both NKH- and DKA-induced chorea [4,10,15]. When hyperglycemia is detected and corrected, the movement disorder in most cases improves within a few days to weeks. Our patient showed improvement of chorea as early as 2–3 days and continued to improve after treating his DKA. This suggests that acute chorea caused by hyperglycemia, whether ketotic or non-ketotic, can be reversible to some extent with a good prognosis. Our patient was also found to have vitamin B12 deficiency and hypothyroidism. Rare cases of chorea have been reported in vitamin B12 deficiency [16–18]. However, the case reports which related chorea with vitamin B12 deficiency stated that the chorea resolved after at least 2 months of intramuscular cobalamin therapy [16,19]. This was not the case in our patient, in whom chorea started to improve within 2–3 days after correction of his DKA. Hypothyroidism has been very rarely associated with chorea. There was a case reported in Thailand in 1986, in which a patient with hypothyroidism presented with hemichoreathetoid movements [20]. In this case, after thyroid hormone replacement therapy for a few weeks, the hemichoreathetoid movement decreased dramatically. Chorea has been reported in a case of Hashimoto's encephalopathy [21]. Another case of chorea was recently reported in a woman recovering from myxedema coma [22]. It's unlikely that our patient's chorea was due to hypothyroidism because by the time he was discovered to have hypothyroidism, a week after admission, his chorea had improved with good glycemic control. 3. Conclusion Although DKA has been rarely reported as a trigger for chorea, it should be in the differential diagnosis of a patient presenting with an acute chorea. Our case demonstrated both the reversible and irreversible components of chorea shortly after correcting the metabolic abnormalities associated with DKA and controlling glucose level. Given the reversible nature of this disease, early recognition and treatment are imperative. Although NKH and DKA can share some of the suggested pathophysiologic mechanisms in inducing chorea, it is unclear as to what extent each of these hypotheses play a role. Authors Contribution Ahmed M. Yassin: Manuscript drafting and editing. Sheetal Shroff: Manuscript drafting and editing. Shreshta D. Patel: Manuscript drafting and editing. Asif M. Paker: Manuscript drafting and editing. Megan A. Berman: Supervisor. Goerge R. Jackson: Supervisor. Disclosure The authors report no disclosure.
3
This study does not involve use of any grant funds.
Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.jns.2014.04.038.
References [1] Reich SG. Pearls: hyperkinetic movement disorders. Semin Neurol 2010;30(1):15–22. [2] Barton B, Zauber SE, Goetz CG. Movement disorders caused by medical disease. Semin Neurol Apr 2009;29(2):97–110. [3] Oh SH, Lee KY, Im JH, Lee MS. 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 Aug 15 2002;200(1–2):57–62. [4] Lee SH, Shin JA, Kim JH, Son JW, Lee KW, Ko SH, et al. Chorea-ballism associated with nonketotic hyperglycaemia or diabetic ketoacidosis: characteristics of 25 patients in Korea. Diabetes Res Clin Pract Aug 2011;93(2):e80–3. [5] Ifergane G, Masalha R, Herishanu YO. Transient hemichorea/hemiballismus associated with new onset hyperglycemia. Can J Neurol Sci Nov 2001;28(4):365–8. [6] Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, et al. Persistent chorea triggered by hyperglycemic crisis in diabetics. Mov Disord Sep 2001;16(5):890–8. [7] Suto Y, Mori M, Kagimoto H, Saito J. A case of hemichorea with hyperglycemia presenting with low signal intensity in the striatum on T2*-weighted gradient-echo magnetic resonance imaging. Rinsho Shinkeigaku Feb 2004;44(2):86–90. [8] Watanabe C, Oishi T, Yamamoto T, Sasaki K, Tosaka M, Sato T, et al. Chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as Moyamoya disease. Diabetes Res Clin Pract Feb 2005;67(2):180–5. [9] Hashimoto K, Ito Y, Tanahashi H, Hayashi M, Yamakita N, Yasuda K, et al. Hyperglycemic Chorea-Ballism or Acute Exacerbation of Huntington's Chorea? Huntington's Disease Unmasked by Diabetic Ketoacidosis in Type 1 Diabetes Mellitus. J Clin Endocrinol Metab Sep 2012;97(9):3016–20. [10] Romero Blanco M, João G, Monteiro E. Hemichorea induced by diabetic ketoacidosis and striatal hyperdensity on computerized axial tomography. Rev Neurol Feb 1–15 2002;34(3):256–8. [11] Guisado R, Arieff AI. Neurological manifestations of diabetic comas: correlation with biochemical alterations in the brain. Metabolism May 1975;24(5):665–79. [12] Rector Jr WG, Herlong HF, Moses III H. Nonketotic hyperglycemia appearing as choreoathetosis or ballism. Arch Intern Med Jan 1982;142(1):154–5. [13] Abe Y, Yamamoto T, Soeda T, Kumagai T, Tanno Y, Kubo J, et al. Diabetic striatal disease: clinical presentation, neuroimaging, and pathology. Intern Med 2009;48(13):1135–41. [14] Wang JH, Wu T, Deng BQ, Zhang YW, Zhang P, Wang ZK, et al. Hemichoreahemiballismus associated with nonketotic hyperglycemia: a possible role of inflammation. J Neurol Sci Sep 15, 2009;284(1-2):198–202. [15] Lai PH, Tien RD, Chang MH, Teng MM, Yang CF, Pan HB, et al. Chorea-ballismus with nonketotic hyperglycemia in primary diabetes mellitus. AJNR Am J Neuroradiol JunJul 1996;17(6):1057–64. [16] Edvardsson B, Persson S. Chorea associated with vitamin B12 deficiency. Eur J Neurol Oct 2011;18(10):e138–9. [17] Kumar S. Vitamin B12 deficiency presenting with an acute reversible extrapyramidal syndrome. Neurol India Dec 2004;52(4):507–9. [18] Pacchetti C, Cristina S, Nappi G. Reversible chorea and focal dystonia in vitamin B12 deficiency. N Engl J Med Jul 25 2002;347(4):295. [19] Shyambabu C, Sinha S, Taly AB, Vijayan J, Kovoor JM. Serum vitamin B12 deficiency and hyperhomocystinemia: a reversible cause of acute chorea, cerebellar ataxia in an adult with cerebral ischemia. J Neurol Sci Oct 15 2008;273(1–2):152–4. [20] Chotmongkol V, Bhuripanyo P. Movement disorders in hypothyroidism: a case report. J Med Assoc Thail May 1989;72(5):288–90. [21] Taurin G, Golfier V, Pinel JF, Deburghgraeve V, Poirier JY, Edan G, et al. Choreic syndrome due to Hashimoto's encephalopathy. Mov Disord Sep 2002;17(5):1091–2. [22] Yu CH, Stovel R, Fox S. Chorea—an unusual manifestation in a woman recovering from myxedema coma. Endocr Pract May–Jun 2012;18(3):e43–8.
Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Hemichorea in a patient with diabetic ketoacidosis Ahmed M. Yassin a, Sheetal Shroff a,⁎, Shreshta D. Patel a, Asif M. Paker b, Megan A. Berman a, George R. Jackson c a b c
The University of Texas Medical Branch, Galveston, TX, United States Massachusetts General Hospital, Boston, MA, United States Baylor College of Medicine, Houston, TX, United States
a r t i c l e
i n f o
Article history: Received 26 February 2014 Received in revised form 26 March 2014 Accepted 24 April 2014 Available online xxxx Keywords: Chorea Diabetic ketoacidosis Non-ketotic hyperglycemia Hypothyroidism Vitamin B12 deficiency Basal ganglia
a b s t r a c t Background: Chorea is a common presenting feature of metabolic disorders, including nonketotic hyperglycemia in patients with type 2 diabetes mellitus, but rarely has been reported in diabetic ketoacidosis, hypothyroidism and vitamin B12 deficiency. Methods: Review the literature for reported cases of chorea as a presenting manifestation in metabolic disorders. Results: We report a case of hemichorea in a patient with type 2 diabetes mellitus complicated by diabetic ketoacidosis. The patient had a two day history of right sided hemichorea and decreased level of consciousness. Initial laboratory studies revealed hyperglycemia, ketosis and an anion gap metabolic acidosis consistent with diabetic ketoacidosis. Once treatment was started the choreiform movements significantly improved over three weeks. Conclusion: Although DKA has been rarely reported as a trigger for chorea, it should be in the differential diagnosis of a patient presenting with an acute chorea. Given the reversible nature of this disease, early recognition and treatment are imperative. © 2014 Elsevier B.V. All rights reserved.
1. Introduction Chorea is a prototypic hyperkinetic movement disorder. It is a common symptom of neurological diseases that directly affect the basal ganglia, such as Huntington's disease. However, many systemic disorders also present with chorea, including metabolic disorders such as diabetes mellitus. Nonketotic hyperglycemia (NKH) is an established cause of chorea, and many cases have been reported. On the other hand, diabetic ketoacidosis (DKA) has rarely been reported as a cause for chorea. We report a case of DKA that induced a hemichorea. 1.1. Case report A 69 year old Hispanic male presented with two day history of involuntary movements of his arms and legs and decreased level of consciousness. The patient was confused and could not provide further history. Co-morbidities are type 2 diabetes mellitus, hypertension, and hyperlipidemia. He did not have any family history of any neurological diseases. He did not smoke, drink alcohol or use illicit drugs. His medications included insulin, glipizide, enalapril, hydrochlorothiazide and pravastatin. However, previous clinic notes reported that the patient was non-compliant with diabetic regimen.
⁎ Corresponding author at: Department of Neurology, The University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555, United States. Tel.: +1 979 824 2467. E-mail address: [email protected] (S. Shroff).
On examination, his vital signs were as follows: temperature 37 °C, blood pressure 150/90 mm Hg, respiratory rate 20/min, pulse 85 beats per minute, and oxygen saturation 95% on ambient air. He was somnolent and disoriented to time and place. He was unable to follow simple commands. Pupils were equal and reactive to light bilaterally, extraocular movements were normal, face was symmetrical and tongue was in midline. He was moving all his extremities antigravity and was difficult to maintain the effort (4/5). He had choreiform movements of right upper extremity and bilateral lower extremities (R N L). He was unsteady due to choreiform movements that subsided during sleep. Reflexes were difficult to elicit due to impaired relaxation. Babinski sign was absent. His labs were significant for blood glucose 380 mg/dl (70–110 mg/dl), anion gap 21 [12–16], serum ketones 1 + and glycosylated hemoglobin 16.3% (4–6%). One week after admission additional labs revealed TSH 19.9 μIU/ml (0.45–4.7 μIU/ml), free T4 0.85 ng/dl (0.78–2.2 ng/dl), T3 96.8 ng/dl (97–170 ng/dl), vitamin B12 b 159 Pg/ml (240–930 Pg/ml). Computed-tomography (CT) of his head was negative for a stroke, bleeding or mass in the vicinity of the basal ganglia. Further studies including electroencephalogram, cerebrospinal fluid analysis, serum and urine copper, ceruloplasmin, iron, ferritin and antinuclear antibody were unremarkable. Huntington repeat expansion analysis revealed 15 repeats which is within the normal limits. His DKA was treated with intravenous fluids and insulin. DKA resolved the second day of hospitalization, and he then became fully oriented. His glucose continued to be controlled in the range of 100–200,
http://dx.doi.org/10.1016/j.jns.2014.04.038 0022-510X/© 2014 Elsevier B.V. All rights reserved.
Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
2
A.M. Yassin et al. / Journal of the Neurological Sciences xxx (2014) xxx–xxx
and his chorea improved over 2–3 days. One week after admission, he was found to have hypothyroidism and vitamin B12 deficiency. The patient was started on levothyroxine and intramuscular vitamin-B12. On three month follow-up clinic visit he was alert and oriented, with chorea only confined to his right foot. Strength was 5/5 in upper and lower extremities, reflexes were present throughout. Sensory exam was normal. He was able to walk with minimal assistance.
2. Discussion Chorea is defined as brief, non-stereotyped, rapid, involuntary and purposeless movements involving the upper and/or lower extremities [1]. Chorea can be a symptom of neurological diseases affecting basal ganglia. However, many other conditions can present with chorea such as stroke, uncontrolled diabetes, hyperthyroidism and paraneoplastic disorders. Some drugs are also known to induce chorea [2]. Chorea or ballismus has been reported in different states of diabetes mellitus. It has been widely reported in cases of NKH [3,4]. It has been reported in new onset diabetes mellitus [5]. Persistent chorea can be also triggered by hyperglycemic crisis in diabetic patients [6]. Chorea might also be associated with poorly controlled diabetes in elderly patients [7]. Despite the large number of cases of chorea reported in the setting of NKH, a few cases have been reported in DKA. There have been cases of DKA-induced chorea in patients with previously asymptomatic neurological diseases (e.g. Moyamoya disease, Huntington's disease) [8,9]. After excluding other causes, DKA has been reported rarely as the sole trigger of chorea, similar to our patient [4,10]. This was confirmed by the temporal relationship between DKA and the abnormal movements,
and by improvement of chorea in few days to weeks following control of blood glucose and treatment of ketoacidosis. NKH induced chorea is typically associated with unilateral or bilateral lesions of the putamen or caudate nucleus on CT and/or T1-weighted MRI images [3–7]. These findings may be related to petechial hemorrhage or ischemia. Our patient's T1-weighted MRI showed increased signal intensity in the putamen and anterolateral globus pallidus bilaterally (Fig. 1, panels B and E). Although many mechanisms have been proposed for NKH induced chorea, there are no hypotheses to specifically explain DKA induced chorea. Some of the mechanisms reported for NKH induced chorea are: thalamic disinhibition from depletion of inhibitory neurotransmitters, diabetic vasculopathy with consequent vascular insufficiency of the striatum, acute basal ganglia dysfunction secondary to hyperglycemia, hyperosmolarity, hyperviscosity, petechial hemorrhage or inflammatory processes [11–14]. One of the hypotheses suggests that in NKH, both gammaaminobutyric acid (GABA) and acetylcholine are utilized as an alternative source of energy. Depletion of these neurotransmitters leads to a disinhibition of the thalamus resulting in hyperkinesias [11,12]. However, in DKA acetoacetate produced from the liver can be used to produce GABA. This suggests that metabolic derangement alone cannot explain this phenomenon. Diabetic vasculopathy with a consequent cerebrovascular insufficiency is another hypothesized mechanism. A striatal biopsy of a patient showed hyaline degeneration with thickening of the arteriolar walls, patchy necrotic tissue and prominent vascular proliferation. Abe Y et al. described the findings as “diabetic striatopathy” and added that “vulnerability to this obliterative vasculopathy is restricted to the striatum” [13]. We suggest that multiple mechanisms are involved in the development of chorea in DKA. At this time it is unclear
Fig. 1. MRI showing axial DWI, T1, T2, T2/FLAIR sequence and coronal T1, T2 sequence images respectively. T1-weighted magnetic resonance imaging (MRI) showed increased signal intensity in the putamen and anterolateral globus-pallidus bilaterally.
Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
A.M. Yassin et al. / Journal of the Neurological Sciences xxx (2014) xxx–xxx
as to what extent each of the above mentioned mechanisms are contributing. The prognosis is favorable with both NKH- and DKA-induced chorea [4,10,15]. When hyperglycemia is detected and corrected, the movement disorder in most cases improves within a few days to weeks. Our patient showed improvement of chorea as early as 2–3 days and continued to improve after treating his DKA. This suggests that acute chorea caused by hyperglycemia, whether ketotic or non-ketotic, can be reversible to some extent with a good prognosis. Our patient was also found to have vitamin B12 deficiency and hypothyroidism. Rare cases of chorea have been reported in vitamin B12 deficiency [16–18]. However, the case reports which related chorea with vitamin B12 deficiency stated that the chorea resolved after at least 2 months of intramuscular cobalamin therapy [16,19]. This was not the case in our patient, in whom chorea started to improve within 2–3 days after correction of his DKA. Hypothyroidism has been very rarely associated with chorea. There was a case reported in Thailand in 1986, in which a patient with hypothyroidism presented with hemichoreathetoid movements [20]. In this case, after thyroid hormone replacement therapy for a few weeks, the hemichoreathetoid movement decreased dramatically. Chorea has been reported in a case of Hashimoto's encephalopathy [21]. Another case of chorea was recently reported in a woman recovering from myxedema coma [22]. It's unlikely that our patient's chorea was due to hypothyroidism because by the time he was discovered to have hypothyroidism, a week after admission, his chorea had improved with good glycemic control. 3. Conclusion Although DKA has been rarely reported as a trigger for chorea, it should be in the differential diagnosis of a patient presenting with an acute chorea. Our case demonstrated both the reversible and irreversible components of chorea shortly after correcting the metabolic abnormalities associated with DKA and controlling glucose level. Given the reversible nature of this disease, early recognition and treatment are imperative. Although NKH and DKA can share some of the suggested pathophysiologic mechanisms in inducing chorea, it is unclear as to what extent each of these hypotheses play a role. Authors Contribution Ahmed M. Yassin: Manuscript drafting and editing. Sheetal Shroff: Manuscript drafting and editing. Shreshta D. Patel: Manuscript drafting and editing. Asif M. Paker: Manuscript drafting and editing. Megan A. Berman: Supervisor. Goerge R. Jackson: Supervisor. Disclosure The authors report no disclosure.
3
This study does not involve use of any grant funds.
Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.jns.2014.04.038.
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Please cite this article as: Yassin AM, et al, Hemichorea in a patient with diabetic ketoacidosis, J Neurol Sci (2014), http://dx.doi.org/10.1016/ j.jns.2014.04.038
