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      Acute Generalized Chorea as Presenting Manifestation of Uremic Encephalopathy

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          Abstract

          Sir, Renal dysfunction results in a clinical metabolic condition known as uremia. It causes altered mental status due to involvement of cerebral cortex termed as uremic encephalopathy. Acute hyperkinetic or hypokinetic extrapyramidal disorder in patients with uremia is a very rare syndrome. It was first described by Wang et al. due to bilateral basal ganglia lesions in uremia.[1] Hereby, we report an elderly patient with diabetic nephropathy who presented with acute-onset generalized chorea. Brain magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. Choreiform movements got ameliorated with hemodialysis. CASE REPORT A 72-year-old male was brought to the emergency department with a history of disabling abnormal involuntary movements involving face and all limbs of 1-day duration. The movements were random, arrhythmic, continuous, nonstereotyped, partially suppressible that increased on activity. He had speech difficulty due to facial involuntary movements. There was no history of fever, altered mental status, seizures, myoclonus, diarrhea, or vomiting. He was diabetic and hypertensive of 7 years duration. He was diagnosed to have diabetic nephropathy for 1 year on medical management. On examination, his tongue was dry, pulse rate of 102/min, blood pressure of 146/86 mmHg, and respiratory rate of 20 breaths/min. Neurologically, he was conscious and responding to verbal commands with mild slurred speech. Fundus examination was normal. There was generalized choreiform movement involving face and all limbs. Asterixis could not be made out due to choreiform movements of limbs. Tone in limbs was decreased with sluggish reflexes. Plantar response was withdrawal. There was no neck rigidity. Complete hemogram showed raised total leukocyte counts (16,000 cells per cumm), normal platelet count, and hemoglobin. Serum electrolytes, random blood glucose, serum ammonia, and liver and thyroid function test were normal. Blood urea nitrogen was 76.4 mg/dl, creatinine of 6.2 mg/dl, and glycosylated hemoglobin of 8.2%. Blood gas analysis showed pH: 7.314, bicarbonate: 17.6 mmol/L, and lactate: 2.1 mmol/L. Brain MRI showed hypointense on T1 and hyperintense lesions on T2 and fluid-attenuated inversion recovery sequences in bilateral basal ganglia. Diffusion-weighted imaging showed no restriction in bilateral putamen [Figure 1]. Electroencephalogram showed mild slowing of background rhythm. He was started on sodium valproate (1 g/day) but had no improvement. He underwent hemodialysis with correction of metabolic acidosis. Choreiform movements gradually improved with concomitant decrease in creatinine level. Figure 1 Brain magnetic resonance imaging T1 coronal view (a) showed hypointense lesions (red arrow); T2 coronal (b), and axial (c) fluid-attenuated inversion recovery (d) showed hyperintense lesions in bilateral putamen (red arrow); diffusion-weighted (e) and apparent diffusion coefficient (f) showed no restriction (red arrow) DISCUSSION Uremic encephalopathy is an organic brain syndrome involving the cerebral cortex that is characterized by altered mental state ranging from mild confusion to comatose state, tremor, asterixis, multifocal myoclonus, and seizures.[2] The involvement of basal ganglia is rare. Wang et al. reported a unique clinical condition in diabetic uremic patients characterized by acute movement disorder due to bilateral basal ganglia lesions. This was most commonly reported in Asian patients.[3] The movement disorder can be either acute parkinsonism with patients presenting with rigidity, bradykinesia, and postural instability or chorea as in our case. There are two cases of generalized chorea due to uremia reported in Indian patients so far.[4 5] There are various explanations reported for the involvement of basal ganglia. Wang et al. performed F-18 fluorodeoxyglucose positron emission tomography on two diabetic uremic patients with bilateral basal ganglia lesions and demonstrated glucose hypometabolism in putamen, frontal, and occipital cortices. This may be due to compromised cellular function in basal ganglia due to diabetic microangiopathy or failure of utilization of energy compounded by the negative effects of uremic toxins and acid–base abnormalities on the basal ganglia cells.[6] Basal ganglia lesions are due to cytotoxic edema surrounded by vasogenic edema (due to focal hyperemia secondary to abnormal dilatation of small vessels).[7] Other explanations include hypoglycemia which may act as one of the trigger factors causing this syndrome as reported by Jurynczyk et al.[8] Tajima et al. suggested that the basal ganglia lesions may be due to demyelination.[9] There is difference in the resolution of clinical and radiological abnormalities. The resolution of clinical abnormalities may be complete in one-fifth of the cases, partial in half of the cases, and no resolution in 30% of the cases. However, resolution of the radiological abnormalities is observed in almost 90% of the cases.[4] Our patient had complete clinical recovery within 1 week of initiation of hemodialysis. Other causes of bilateral basal ganglia lesions include hypoxia, hyperammonemia, hypoglycemia, hyperglycemia, osmotic demyelination syndrome, deep vein thrombosis, toxins such as carbon monoxide poisoning, viral encephalitis such as Japanese encephalitis, prionopathies such as Creutzfeldt-Jakob disease, and degenerative disease such as Wilson's disease. CONCLUSION Bilateral basal ganglia lesions in uremia are mainly due to the effects of metabolic abnormalities and uremic toxins on the compromised basal ganglionic cellular function causing cytotoxic and vasogenic edema. These abnormalities are potentially reversible if recognized promptly. This is the third reported Indian case of generalized chorea in uremia with bilateral basal ganglia lesions. The knowledge of this entity is necessary for the treating neurologists, nephrologists, and intensivist. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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          Most cited references 9

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          Neurological complications in renal failure: a review.

          Neurological complications whether due to the uremic state or its treatment, contribute largely to the morbidity and mortality in patients with renal failure. Despite continuous therapeutic advances, many neurological complications of uremia, like uremic encephalopathy, atherosclerosis, neuropathy and myopathy fail to fully respond to dialysis. Moreover, dialytic therapy or kidney transplantation may even induce neurological complications. Dialysis can directly or indirectly be associated with dialysis dementia, dysequilibrium syndrome, aggravation of atherosclerosis, cerebrovascular accidents due to ultrafiltration-related arterial hypotension, hypertensive encephalopathy, Wernicke's encephalopathy, hemorrhagic stroke, subdural hematoma, osmotic myelinolysis, opportunistic infections, intracranial hypertension and mononeuropathy. Renal transplantation itself can give rise to acute femoral neuropathy, rejection encephalopathy and neuropathy in graft versus host disease. The use of immunosuppressive drugs after renal transplantation can cause encephalopathy, movement disorders, opportunistic infections, neoplasms, myopathy and progression of atherosclerosis. We address the clinical, pathophysiological and therapeutical aspects of both central and peripheral nervous system complications in uremia.
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            The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.

            Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.
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              Acute movement disorders with bilateral basal ganglia lesions in uremia.

              Acute and subacute extrapyramidal movement disorders are rarely reported in uremic patients. We report three such cases with basal ganglia lesions. All three had advanced renal failure with high serum creatinine levels. One of the patients had a history of ischemic heart disease and acute pulmonary edema with hypoxemia. Another patient had experienced arterial hypotension during previous hemodialysis. The third had prominent metabolic acidosis. One of the patients developed generalized dyskinesias, whereas the other two developed gait disturbances. Neuroimaging studies in all three cases showed bilateral changes in the basal ganglia. The natural history was self-limiting with gradual improvement. Diminution of the basal ganglia lesions was demonstrated on follow-up imaging in two of the three cases. We conclude that acute or subacute movement disorders with bilateral basal ganglia lesions may occur in uremia. Hypoperfusion with global brain ischemia and selective vulnerability of the basal ganglia to uremic toxins may account for these lesions.
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                Author and article information

                Journal
                J Neurosci Rural Pract
                J Neurosci Rural Pract
                JNRP
                Journal of Neurosciences in Rural Practice
                Medknow Publications & Media Pvt Ltd (India )
                0976-3147
                0976-3155
                August 2017
                : 8
                : Suppl 1
                : S156-S158
                Affiliations
                Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru - 560 054, Karnataka, India
                [1 ]Department of Nephrology, MS Ramaiah Medical College and Hospital, Bengaluru - 560 054, Karnataka, India
                Author notes
                Address for correspondence: Dr. Rohan R. Mahale, Department of Neurology, M. S. Ramaiah Medical College and Hospital, Bengaluru - 560 054, Karnataka, India. E-mail: rohanmahale83@ 123456gmail.com
                Article
                JNRP-8-156
                10.4103/jnrp.jnrp_158_17
                5602251
                Copyright: © 2017 Journal of Neurosciences in Rural Practice

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