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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 15  |  Issue : 3  |  Page : 266-268

Seizure and hemichorea post renal transplantation: A recipient with heterogeneous risk factors - A case report


1 Department of Nephrology, Bombay Hospital, Mumbai, Maharashtra, India
2 Department of Nephrology, Apex Kidney Care, Sushrut Hospital, Mumbai, Maharashtra, India
3 Gurmeet Imaging, Mumbai, Maharashtra, India

Date of Submission04-Feb-2021
Date of Decision03-Jun-2021
Date of Acceptance03-Jun-2021
Date of Web Publication30-Sep-2021

Correspondence Address:
Dr. Viswanath Billa
Department of Nephrology, Bombay Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijot.ijot_7_21

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  Abstract 

Kidney transplantation is the preferred renal replacement therapy for patients with end-stage kidney disease. However, these patients are vulnerable to multiple complications arising out of immunosuppression, comorbidities, and opportunistic infections. We report a case of hemichorea in a transplant recipient. This patient had concurrent polycythemia, new-onset diabetes after transplantation, and was on calcineurin-based immunosuppression. The magnetic resonance imaging ascribed her hemichorea to Non ketotic hyperglycemia (NKH) which recovered completely with prompt treatment of the hyperglycemia.

Keywords: Hemichorea, hyperglycemia, new-onset diabetes after transplantation, renal transplant


How to cite this article:
Jose A, Usulumarty D, Billa V, Singh K. Seizure and hemichorea post renal transplantation: A recipient with heterogeneous risk factors - A case report. Indian J Transplant 2021;15:266-8

How to cite this URL:
Jose A, Usulumarty D, Billa V, Singh K. Seizure and hemichorea post renal transplantation: A recipient with heterogeneous risk factors - A case report. Indian J Transplant [serial online] 2021 [cited 2021 Nov 29];15:266-8. Available from: https://www.ijtonline.in/text.asp?2021/15/3/266/327395


  Introduction Top


Chorea is a hyperkinetic movement disorder characterized by excessive spontaneous movements that are irregularly timed, randomly distributed, and abrupt. Acquired causes of chorea include vascular disease, postinfective autoimmune central nervous system disorders pediatric autoimmune disorders associated with streptococcal infections, drugs, systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, acquired immunodeficiency syndrome, chorea gravidarum, and polycythemia rubra vera. The treatment of this condition involves identifying the underlying etiology. The pattern of injury on neuroimaging helps identifying the causative factor.[1] We report a case of a 50-year-old female, 2 years postrenal transplant with stable graft function and minimal immunosuppression presenting with chorea, and further evaluation brought to light multiple risk factors.


  Case Report Top


A 50-year-old woman was admitted with choreiform movements along with tingling, numbness, and weakness of her left upper limb for 2 days before presentation. She was diagnosed to have chronic kidney disease due to chronic tubulointerstitial nephritis in 2015 and had undergone a living donor kidney transplant in March 2018 after spending 3 years on hemodialysis. She had been on triple immunosuppression with tacrolimus, mycophenolate, and steroids with a stable graft function, (Se. Cr 0.8 mg/dl). She developed polycythemia in January 2019 for which she was undergoing regular phlebotomies and new-onset diabetes after transplantation (NODAT) was diagnosed during this admission. At presentation, she was afebrile, conscious, and oriented with typical choreiform movements of the left hand with a power grade of 3/5 and reduced sensations. Investigations showed a hemoglobin of 16.7 g/dl, creatinine of 1.1 mg/dl, tacrolimus level of 5.9 ng/ml, and total count was 12,200. Her random blood sugar measured 453 mg/dl with an HbA1c of 11% and urine and plasma acetone were negative. After admission, she had an episode of generalized seizures and the magnetic resonance imaging (MRI) showed patchy confluent T2 and fluid-attenuated inversion recovery hypointensities in the right parietal, pre and postcentral gyrus, and subcortical white matter [Figure 1]. Although classical changes of hyperglycemia in the subcortical white matter were picked up in this patient, the MRI failed to identify similar occult lesions in the basal ganglia probably due to the early imaging. Her electroencephalogram was normal. She was managed with anti-epileptics, insulin, fluids, and phlebotomy. Her sugars normalized within 2 days with concurrent recovery of the neurological symptoms. She was discharged on insulin and antiepileptics. The tacrolimus in her immunosuppression was switched to cyclosporine. She is currently completely asymptomatic and a follow-up MRI scan done a month later, showed complete resolution of the previous MRI findings [Figure 2].
Figure 1: Magnetic resonance imaging showing subcortical hypointensities in the right parieto occipital area (marked by arrow)

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Figure 2: Repeat Imaging shows complete clearance of opacities

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  Discussion Top


There are multiple acquired causes of involuntary movements. The pathophysiology of chorea is most commonly linked to a dysfunction of inhibitory pathways within the basal ganglia. Chorea is an uncommon symptom, especially so in the transplant population. When it occurs there is a need to review the biochemistry, drug history, and other coexisting medical conditions. This patient had three potential risk factors for hemichorea; polycythemia, calcineurin inhibitors (CNI), and nonketotic hyperglycemia.

Neurological manifestations of polycythemia include headache, vertigo, stroke, visual symptoms, tinnitus, and paresthesia.[2] Chorea is an infrequently reported complication of the disease (0.5%–5%).[3] It is usually generalized with predominant involvement of the orofaciolingual muscles. Hyperviscosity of the blood which reduces oxygen transport, and increases platelet contact and adhesion to the vessel wall particularly in the basal ganglia, plays an important part in its pathogenesis.[2] MRI in patients with polycythemia complicated by chorea are most often normal. Advanced imaging such as fluorodeoxyglucose-positron emission tomography and Tc99 labeled tropane dopamine transporter imaging (TRODAT-single-photon emission computed tomography) may pick up the transient alterations in the metabolic activity of the basal ganglia.[4]

CNIs are another group of drugs that can cause chorea. Calcineurin is expressed in several areas of the brain: Cerebral cortex, striatum, substantia nigra, cerebellum, and hippocampus. They access astrocytes and cerebrovascular smooth muscle, causing vasospasm. CNI also causes neurotoxicity by altering mitochondrial function, activation of anaerobic glycolysis, proteases, phospholipases, and the generation of free radicals. This leads to apoptotic or necrotic cell death.[5] Neuroimaging features of CNI neurotoxicity include bilateral, symmetric T2 hyperintense signal abnormalities in subcortical regions of the parietal and occipital lobes, corresponding to a typical manifestation of posterior reversible encephalopathy syndrome.[6]

NKH is a clinical syndrome characterized by severe hyperglycemia, hyperosmolality, and intracellular dehydration without ketoacidosis. This is commonly described in the elderly, long-standing diabetic patients but has also been described as an initial presentation of type 2 diabetes mellitus. Our patient had NODAT and presented with severe hyperglycemia. Although most metabolic disorders are bilateral, NKH presents unilaterally as hemichorea or hemiballism. Other manifestations include seizures, hemianopsia, dysphagia, hemisensory loss, and hemiparesis. The main mechanism is thought to be secondary to the depletion of Gamma-aminobutyric-acid (GABA) and acetylcholine neurotransmitters. During hyperglycemia, glucose metabolism through the Krebs cycle is inhibited and the brain reverts to an anaerobic metabolism wherein it uses GABA to produce energy, thereby depleting it and also causing metabolic acidosis.[7] Hyperglycemia can also lead to hyperviscosity resulting in ischemia and disruption of blood-brain barrier causing intracellular acidosis and regional metabolic failure.[8]

Classic MRI features of chorea caused by NKH are the presence of hypointense signals on T2-weighted sequences, predominantly involving unilateral basal ganglia. However, these findings may not be picked up by an early MRI. In our patient, these changes were seen instead in the subcortical white matter which has been reported in NKH which completely reverse on control of hyperglycemia.[9] Other conditions which can cause focal T2 hypointense appearance are venous congestion due to chronic cerebral venous thrombosis and focal meningoencephalitis.[10] Venous congestion was ruled out due to the absence of any dilated venous collaterals or evidence of chronic venous sinus thrombosis. There was no focal leptomeningeal or brain parenchymal enhancement which ruled out focal meningoencephalitis. Other differentials for hypointensities on T2 imaging include leptomeningeal metastasis, multiple sclerosis, and  Sturge- Weber syndrome More Details More Details. The clinical history and progress were clearly suggestive of hyperglycemia-induced involuntary movements. The patient had a very rapid response to correction of blood sugar, a follow-up MRI done 1 month later showed complete clearance of all the abnormalities both of which supports a metabolic rather than a structural etiology.


  Conclusion Top


Transplant patients should be carefully monitored for the development of NODAT. Once diagnosed, sugars should be managed aggressively and modification of the immunosuppressive regimen should be considered. NODAT can also have varied neurological presentations. The early identification is important as managing sugar is the mainstay of treatment and it poorly responds to antiepileptics. An MRI appearance is fairly classical and helps make a quick and accurate diagnosis.

Declaration of patient consent

The authors certify that the patient has obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Wild EJ, Tabrizi SJ. The differential diagnosis of chorea. Pract Neurol 2007;7:360-73.  Back to cited text no. 1
    
2.
Nazabal ER, Lopez JM, Perez PA, Del Corral PR. Chorea disclosing deterioration of polycythaemia vera. Postgrad Med J 2000;76:658-9.  Back to cited text no. 2
    
3.
Midi I, Dib H, Köseoglu M, Afsar N, Günal DI. Hemichorea associated with polycythaemia vera. Neurol Sci 2006;27:439-41.  Back to cited text no. 3
    
4.
Bhargava R, Dass J, Singh S, Vaid A. Hemichorea, a rare presenting feature of polycythemia vera. Neurol India 2013;61:86-7.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Meena P, Bhargava V, Rana D, Bhalla A, Ashwani. Approach to neurological disorders in a kidney transplant recipient. Gupta Kidney360;1:837-44.  Back to cited text no. 5
    
6.
Wu Q, Marescaux C, Qin X, Kessler R, Yang J. Heterogeneity of radiological spectrum in tacrolimus-associated encephalopathy after lung transplantation. Behav Neurol 2014;2014:931808.  Back to cited text no. 6
    
7.
Guisado R, Arieff AI. Neurologic manifestations of diabetic comas: Correlation with biochemical alterations in the brain. Metabolism 1975;24:665-79.  Back to cited text no. 7
    
8.
Chu K, Kang DW, Kim DE, Park SH, Roh JK. Diffusion-weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: A hyperviscosity syndrome? Arch Neurol 2002;59:448-52.  Back to cited text no. 8
    
9.
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 2002;200:57-62.  Back to cited text no. 9
    
10.
Kamble JH, Parameswaran K. “Venous congestion” as a cause of subcortical white matter T2 hypointensity on magnetic resonance images. Ann Indian Acad Neurol 2016;19:411-3.  Back to cited text no. 10
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