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• W2069471056 abstract "A 71-year-old Hispanic woman presented to our medical center with a general sense of feeling ‘unwell.’ She suffered from multiple medical conditions, including long-standing history of diabetes, hypertension, hypercholesterolemia, depression, and end-stage renal disease (ESRD) for which she has been on dialysis for the last 3 years. Seven years prior to her current admission, the patient presented with an acute onset of left seventh nerve palsy associated with hypercalcemia and submandibular and perihilar lymphadenopathy. Submandibular lymph node biopsy showed infiltration with a polymorphous population of lymphocytes and granulomas, findings consistent with sarcoidosis. She was treated with a course of corticosteroids and methotrexate, with complete resolution of symptoms within 2 years of the diagnosis. Steroids were discontinued 6 years and methotrexate 4 years before her current admission. Her sarcoidosis remained in complete remission. Her past medical history was also significant for primary hyperparathyroidism; she underwent partial parathyroidectomy in the early 1980s. The patient's kidney disease had been diagnosed 4 years prior, when she presented with a nephrotic syndrome and creatinine (Cr) of 1.5 mg per 100 ml (ref. 0.5–0.9 mg per 100 ml). The kidney biopsy performed at that time revealed advanced focal segmental glomerulosclerosis and she progressed to ESRD within 1 year of the diagnosis. Over the last 3 years, the patient has been maintained on regular thrice-weekly hemodialysis through a radial arteriovenous fistula with adequate clearance (urea reduction ratio ranging from 77 to 84%) and no major complications. The patient was brought to the emergency room by her family for unstable gait with ‘dance-like’ quality. The symptoms developed gradually over the period of 2 days. The patient neither missed dialysis nor had been ill, and had no sick contacts. Her medication regimen included aspirin, diltiazem, paroxetine, statin, glipizide, sevelamer, multivitamins, and stool softeners. She denied any exposure to antipsychotics or antiemetics. There was no family history of movement disorders, psychiatric disease, or renal disease. The patient used to work as a seamstress and retired several years ago. She did not smoke or drink and denied any illicit substance use. The patient's pulse was 82–88 per min and the blood pressure ranged from 130/70 to 180/80 mm Hg in the emergency room. The head and neck, cardiopulmonary, and abdominal examinations were normal. There was no extremity edema, and pulses were intact. She was alert and oriented to person, place, date, and current events. Her speech was fluent. Naming, repetition, and reading were intact. She was able to follow three-step commands and her memory was not affected. Her cranial nerves were also intact. Muscular examination revealed normal tone, full-strength, and intact deep tendon reflexes. She exhibited no asterixis or myoclonus. Her gait was unstable with wide stance and exaggerated steps with a dance-like quality. While at rest, she exhibited slow involuntary choreoathetotic movements. The movements ceased during sleep or after treatment with intravenous diphenhydramine in the emergency room, but reappeared upon awakening. Her initial laboratory evaluation revealed normal blood counts and serum electrolytes, Cr 5.8 mg per 100 ml (ref. 0.5–0.9 mg per 100 ml), BUN 34 mg per 100 ml (ref. 7–20 mg per 100 ml), and glucose 110 mg per 100 ml (ref. 70–105 mg per 100 ml). She had normal liver and thyroid function tests and negative urine toxicology screen. Other studies, including the electrocardiogram and chest and abdominal radiographs, were all within normal limits. A computed tomography scan of the head without contrast was performed and, upon comparison to a prior scan 4 years earlier, was suggestive of new poorly defined lucencies in the basal ganglia region (Figure 1a). This study was followed by a magnetic resonance imaging (MRI), which revealed symmetric bilateral hyperintensity of putamen and globus pallidus on FLAIR and T2 sequences (Figure 1b). These changes corresponded to the hypodensities seen on the computed tomography scan and were not present on an MRI scan done 1 year before. There was no evidence of contrast uptake on T1 gadolinium-enhanced images. Magnetic resonance angiography was unremarkable. Chorea is characterized by involuntary, nonrepetitive, adventitious muscular contractions. It originates from a dysfunction of neuronal networks interconnecting basal ganglia and frontal motor cortex. A wide range of conditions have been associated with sporadic chorea.1.Cardoso F. Seppi K. Mair K.J. et al.Seminar on choreas.Lancet Neurol. 2006; 5: 589-602Abstract Full Text Full Text PDF PubMed Scopus (189) Google Scholar The conditions that are relevant to the presentation of our patient, an elderly diabetic with ESRD, include acute basal ganglia ischemia (although large vessel cerebrovascular disease was excluded by imaging in this case), exposure to toxins and pharmacologic agents affecting dopaminergic neuronal pathways (none detected by history in this case), hereditary neurodegenerative disease (such as late-onset Huntington's disease and related hereditary disorders), as well as various metabolic, autoimmune, inflammatory, infectious, and neoplastic conditions (as summarized in Table 1). In addition, several electrolyte abnormalities associated with kidney disease, renal replacement therapy, and diabetes may be involved in basal ganglia toxicity. Specifically, these include severe uremia, rapid correction of dysnatremia, acidosis, and severe hypo- or hyperglycemia. None of the above problems are present in this patient, who had been on adequate dialysis treatments without any evidence of serious metabolic abnormalities. A neurological consultation and hospital admission for a complete workup of possible etiologies are warranted in this case.Table 1Differential diagnosis for basal ganglia lesions in a hemodialysis patientCauseMechanism/CommentsWorkup in this caseHereditary Late-onset Wilson's diseaseAutosomal recessive defect in cellular copper transportNo hepatic disease, no cognitive changes, no corneal rings, spontaneous resolution Late-onset Huntington's disease and related genetic disorders2.Cupidi C. Piccoli F. La Bella V. Acute reversible parkinsonism in a diabetic–uremic patient.Clin Neurol Neurosurg. 2006; 108: 601-603Abstract Full Text Full Text PDF PubMed Scopus (25) Google ScholarProgressive neurodegenerative disorders characterized by chorea, cognitive decline, and behavioral changesNegative family history, no behavioral or cognitive impairment, spontanous resolutionEndocrine HyperthyroidismUnknown mechanismNormal thyroid function tests Fahr's syndromeCalcification of the basal gangliaNo calcifications in the basal ganglia by imagingElectrolyte/metabolic disorders Hypo/hypernatremia3.Dickoff D.J. Raps M. Yahr M.D. Striatal syndrome following hyponatremia and its rapid correction. A manifestation of extrapontine myelinolysis confirmed by magnetic resonance imaging.Arch Neurol. 1988; 45: 112-114Crossref PubMed Scopus (41) Google Scholar,4.Hadfield M.G. Kubal W.S. Extrapontine myelinolysis of the basal ganglia without central pontine myelinolysis.Clin Neuropathol. 1996; 15: 96-100PubMed Google ScholarBasal ganglia edema/extrapontine myelinolysisNormal serum Na levels, no history of dysnatremia Hypo/hyperglycemia5.Lai S.L. Tseng Y.L. Hsu M.C. Chen S.S. Magnetic resonance imaging and single-photon emission computed tomography changes in hypoglycemia-induced chorea.Mov Disord. 2004; 19: 475-478Crossref PubMed Scopus (27) Google Scholar, 6.Newman R.P. Kinkel W.R. Paroxysmal choreoathetosis due to hypoglycemia.Arch Neurol. 1984; 41: 341-342Crossref PubMed Scopus (58) Google Scholar, 7.Hsu J.L. Wang H.C. Hsu W.C. Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study.J Neurol. 2004; 251: 1486-1490Crossref PubMed Scopus (75) Google Scholar, 8.Branca D. Gervasio O. Le Piane E. et al.Chorea induced by non-ketotic hyperglycaemia: a case report.Neurol Sci. 2005; 26: 275-277Crossref PubMed Scopus (28) Google ScholarVery rare cause of isolated choreaNormal serum glucose levels, HgbA1C 7.8% Organic acidosis9.Erickson J.C. Jabbari B. Difazio M.P. Basal ganglia injury as a complication of the ketogenic diet.Mov Disord. 2003; 18: 448-451Crossref PubMed Scopus (18) Google Scholar,10.Brismar J. Ozand P.T. CT and MR of the brain in the diagnosis of organic acidemias. Experiences from 107 patients.Brain Dev. 1994; 16: 104-124Abstract Full Text PDF PubMed Scopus (74) Google ScholarUnclear mechanism, rareNormal bicarbonate, no anion gap Uremia/hemodialysis1.Cardoso F. Seppi K. Mair K.J. et al.Seminar on choreas.Lancet Neurol. 2006; 5: 589-602Abstract Full Text Full Text PDF PubMed Scopus (189) Google Scholar, 11.Wang H.C. Hsu J.L. Shen Y.Y. Acute bilateral basal ganglia lesions in patients with diabetic uremia: an FDG-PET study.Clin Nucl Med. 2004; 29: 475-478Crossref PubMed Scopus (35) Google Scholar, 12.Wang H.C. Brown P. Lees A.J. Acute movement disorders with bilateral basal ganglia lesions in uremia.Mov Disord. 1998; 13: 952-957Crossref PubMed Scopus (54) Google Scholar, 13.Wang H.C. Cheng S.J. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.J Neurol. 2003; 250: 948-955Crossref PubMed Scopus (73) Google Scholar, 14.Lee P.H. Shin D.H. Kim J.W. et al.Parkinsonism with basal ganglia lesions in a patient with uremia: evidence of vasogenic edema.Parkinsonism Relat Disord. 2006; 12: 93-96Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar, 15.Kim T.K. Seo S.I. Kim J.H. et al.Diffusion-weighted magnetic resonance imaging in the syndrome of acute bilateral basal ganglia lesions in diabetic uremia.Mov Disord. 2006; 21: 1267-1270Crossref PubMed Scopus (39) Google Scholar, 16.Okada J. Yoshikawa K. Matsuo H. et al.Reversible MRI and CT findings in uremic encephalopathy.Neuroradiology. 1991; 33: 524-526Crossref PubMed Scopus (60) Google ScholarChronic kidney disease, underdialysisStable and adequate dialysis treatments Thiamine deficiency17.Hung S.C. Hung S.H. Tarng D.C. et al.Chorea induced by thiamine deficiency in hemodialysis patients.Am J Kidney Dis. 2001; 37: 427-430Abstract Full Text PDF PubMed Scopus (37) Google ScholarMalnutritionNormal serum thiamine levelsDrugs and toxins Pharmacologic agents2.Cupidi C. Piccoli F. La Bella V. Acute reversible parkinsonism in a diabetic–uremic patient.Clin Neurol Neurosurg. 2006; 108: 601-603Abstract Full Text Full Text PDF PubMed Scopus (25) Google ScholarAntipsychotic, anti-Parkinsonian, and antiepileptic agents, verapamil, baclofen, tricyclics, lithium, theophylline, digoxin, cyclosporine, steroids, OCPsNot taking PsychostimulantsCocaine, amphetaminesNegative toxicology screen AlcoholsEthanol, methanol,18.Comoglu S. Ozen B. Ozbakir S. Methanol intoxication with bilateral basal ganglia infarct.Australas Radiol. 2001; 45: 357-358Crossref PubMed Scopus (24) Google Scholar ethylene glycol19.Reddy N.J. Lewis L.D. Gardner T.B. et al.Two cases of rapid onset Parkinson's syndrome following toxic ingestion of ethylene glycol and methanol.Clin Pharmacol Ther. 2007; 81: 114-121Crossref PubMed Scopus (28) Google ScholarNegative toxicology screen Carbon monoxide20.Park S. Choi I.S. Chorea following acute carbon monoxide poisoning.Yonsei Med J. 2004; 45: 363-366Crossref PubMed Scopus (14) Google ScholarHypoxic basal ganglia injuryNormal carboxyhemoglobin levels Manganese,21.Ohtake T. Negishi K. Okamoto K. et al.Manganese-induced Parkinsonism in a patient undergoing maintenance hemodialysis.Am J Kidney Dis. 2005; 46: 749-753Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar Aluminum,22.Arieff A.I. Aluminum and the pathogenesis of dialysis encephalopathy.Am J Kidney Dis. 1985; 6: 317-321Abstract Full Text PDF PubMed Scopus (47) Google Scholar LeadDirect basal ganglia toxicityNormal serum levelsVascular Cerebrovascular diseaseThrombosis/infarctionNormal MRA, symmetric bilateral lesions Polycythaemia veraHyperviscosity/infarctionNormal hematocrit HUS/TTPMicrothrombosis/infarctionNo hemolysis, normal platelet count Moyamoya diseaseProgressive arterial occlusive disease, likely hereditaryNormal MRA, no family history Post-pump chorea23.Medlock M.D. Cruse R.S. Winek S.J. et al.A 10-year experience with postpump chorea.Ann Neurol. 1993; 34: 820-826Crossref PubMed Scopus (57) Google ScholarComplication of extracorporeal circulationNo documented intradialytic hypotensionAutoimmune/inflammatory SLEVasculitis/inflammationNegative autoimmune serologies, normal complement levels, no systemic symptoms. APSThrombosis/infarctionNegative APL/ACL serologies Small vessel vasculitisVasculitis/inflammationNegative ANCA, normal CRP, no systemic symptoms. Multiple sclerosis plaquesDemyelination/inflammationNo evidence of plaques on imaging, CSF fluid negative for oligoclonal bands Sydenham's choreaRheumatic fever, typically in childhood, antibody-mediated (molecular mimicry between streptococcal and CNS antigens)Negative ASO titers, advanced age, no evidence of rheumatic fever Postinfectious or postviccinal encephalitisAutoimmune reaction following exposure to foreign antigens (molecular mimicry)No history of recent infections or vaccinations Neurosarcoidosis24.Gizzi M.S. Lidov M. Rosenbaum D. Neurosarcoidosis presenting as a tumour of the basal ganglia and brainstem: sequential MRI.Neurol Res. 1993; 15: 93-96PubMed Google ScholarGranulomatous inflammation may rarely involve basal gangliaNo evidence of sarcoidosis on MRI, lack of systemic symptoms, normal ACE levelsInfections HIV/AIDSPossible direct HIV infection of subcortical structuresNegative HIV test NeurosyphilisDirect infection in the basal gangliaNegative RPR and CSF VDRL Bacterial endocarditisTypically unilateral septic embolic diseaseBilateral lesions, no evidence of endocarditis Parasitic infections and TBToxoplasmosis, cysticercosis, tuberculous granulomasNo evidence by imaging, negative PPD Creutzfeldt–Jakob DiseaseSporadic human prion diseaseNegative CSF 14-3-3 protein, spontaneous resolutionTumors Mass lesionsCNS lymphoma or metastatic lesionsNo evidence of tumor on imaging Paraneoplastic syndromesAnti-Hu and anti-CRMP5 antibodiesNegative paraneoplastic antibody panelACE, angiotensin converting enzyme; ACL, anti-cardiolipin; ANCA, anti-neurtophilic cytoplasmic antibodies; APL, anti-phospholipid; APS, anti-phospholipid syndrome; ASO, anti-streptolysin O; CNS, central nervous system; CRP, C-reactive protein; CSF, cerebrospinal fluid; Hgb, hemoglobin; HUS, hemolytic uremic syndrome; MRA, magnetic resonance angiogram; MRI, magnetic resonance imaging; OCPs, oral contraceptive pills; PPD, purified protein derivative; RPR, rapid plasma reagin; SLE, systemic lupus erythematosus; TB, tuberculosis; TTP, thrombotic thrombocytopenic purpura; URR, urea reduction ratio; VDRL, veneral disease research laboratory.Only selected relevant causes are referenced. Open table in a new tab ACE, angiotensin converting enzyme; ACL, anti-cardiolipin; ANCA, anti-neurtophilic cytoplasmic antibodies; APL, anti-phospholipid; APS, anti-phospholipid syndrome; ASO, anti-streptolysin O; CNS, central nervous system; CRP, C-reactive protein; CSF, cerebrospinal fluid; Hgb, hemoglobin; HUS, hemolytic uremic syndrome; MRA, magnetic resonance angiogram; MRI, magnetic resonance imaging; OCPs, oral contraceptive pills; PPD, purified protein derivative; RPR, rapid plasma reagin; SLE, systemic lupus erythematosus; TB, tuberculosis; TTP, thrombotic thrombocytopenic purpura; URR, urea reduction ratio; VDRL, veneral disease research laboratory. Only selected relevant causes are referenced. The patient was admitted to the hospital, neurological consultation was obtained, and a lumbar puncture was performed. The opening pressure was 22 cm H2O and cerebrospinal fluid was clear and contained 39 RBCs per mm3 with no visible WBCs. Cerebrospinal fluid glucose was 100 mg per 100 ml (ref. 40–70 mg per 100 ml) and protein was 47 mg per 100 ml (ref. 15–45 mg per 100 ml). Other negative cerebrospinal fluid studies included bacterial, fungal, and AFB cultures, VDRL, Lyme PCR, viral encephalitis panel, oligoclonal banding, paraneoplastic antibody panel, and 14-3-3 protein. Serum tests for aluminum, cyanide, manganese, lead, and carboxyhemoglobin were all within normal limits. Other negative tests included rheumatoid factor, C-reactive protein, RPR, PPD, Lyme antibody test, ANA, anti-dsDNA, ANCA, hepatitis panel, antibodies to SSA, SSB, U1RNP, Sm, Scl-70, and Jo-1 antigens. The results of additional workup are summarized in Table 2. While in the hospital, the patient was dialyzed three times a week, with adequate clearance and no episodes of intradialytic hypotension. Her neurologic symptoms improved gradually with conservative management. On the sixth day of her admission, she was transferred to an in-patient rehabilitation facility. Her symptoms resolved completely within 12 days, and she was discharged home in a stable condition. An MRI scan performed 6 weeks after the initial presentation demonstrated complete resolution of abnormal T2 signal intensities (Figure 1c) and there was no recurrence of symptoms after 1 year of follow-up.Table 2Results of additional laboratory testsTestResultReference rangeParathyroid hormone (pg ml−1)2498–51Calcium (mg per 100 ml)8.98.4–9.8Phosphorus (mg per 100 ml).3.72.5–4.3Serum ACE (IU l−1)369–67Ceruloplasmin (mg per 100 ml)1925–63Serum copper (μg per 100 ml)8580–155Thiamine (μg per 100 ml)3.21.6–4.0Vitamin B12 (pg ml−1)1292279–996Homocysteine (μmol l−1)12.84.4–10.8Folate (ng ml−1)10.55.4–18.0Iron (μg per 100 ml)4041–141TIBC (μg per 100 ml)208251–406Ferritin (ng ml−1)103510–150Hemoglobin A1C (%)7.8—ACE, angiotensin converting enzyme; TIBC, total iron-binding capacity. Open table in a new tab ACE, angiotensin converting enzyme; TIBC, total iron-binding capacity. There are only a handful of case reports of acute extrapyramidal movement disorders in patients with ESRD on dialysis. Most of the reports came from the Asian continent (12 cases from Taiwan,11.Wang H.C. Hsu J.L. Shen Y.Y. Acute bilateral basal ganglia lesions in patients with diabetic uremia: an FDG-PET study.Clin Nucl Med. 2004; 29: 475-478Crossref PubMed Scopus (35) Google Scholar, 12.Wang H.C. Brown P. Lees A.J. Acute movement disorders with bilateral basal ganglia lesions in uremia.Mov Disord. 1998; 13: 952-957Crossref PubMed Scopus (54) Google Scholar, 13.Wang H.C. Cheng S.J. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.J Neurol. 2003; 250: 948-955Crossref PubMed Scopus (73) Google Scholar 2 cases from Korea,14.Lee P.H. Shin D.H. Kim J.W. et al.Parkinsonism with basal ganglia lesions in a patient with uremia: evidence of vasogenic edema.Parkinsonism Relat Disord. 2006; 12: 93-96Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar,15.Kim T.K. Seo S.I. Kim J.H. et al.Diffusion-weighted magnetic resonance imaging in the syndrome of acute bilateral basal ganglia lesions in diabetic uremia.Mov Disord. 2006; 21: 1267-1270Crossref PubMed Scopus (39) Google Scholar and 1 case from Japan16.Okada J. Yoshikawa K. Matsuo H. et al.Reversible MRI and CT findings in uremic encephalopathy.Neuroradiology. 1991; 33: 524-526Crossref PubMed Scopus (60) Google Scholar). Only one European case has been reported to date in a diabetic patient with advanced renal disease.2.Cupidi C. Piccoli F. La Bella V. Acute reversible parkinsonism in a diabetic–uremic patient.Clin Neurol Neurosurg. 2006; 108: 601-603Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar This case illustrates diagnostic difficulties in evaluation of chorea in an elderly diabetic patient with ESRD. Typically, an extensive battery of tests is needed to exclude a long list of potential etiologies. Magnetic resonance imaging, lumbar puncture, and a serologic workup for potential autoimmune, inflammatory, infectious, and neoplastic causes are required. Some of the diagnoses can only be excluded retrospectively, after a longer period of clinical follow-up. For example, hereditary neurodegenerative diseases presenting with chorea were excluded in this case because of spontaneous resolution of symptoms and radiographic findings. In addition, a thorough history and investigation of medical records is needed to rule out exposure to common medications or toxins producing basal ganglia dysfunction. In this case, a complete medical history combined with an exhaustive neurological workup (summarized in Table 1) excluded the most common diagnoses. Dialysis patients are prone to a number of metabolic derangements that predispose to basal ganglia injury. Chronic uremia due to kidney disease2.Cupidi C. Piccoli F. La Bella V. Acute reversible parkinsonism in a diabetic–uremic patient.Clin Neurol Neurosurg. 2006; 108: 601-603Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar and inadequate dialysis12.Wang H.C. Brown P. Lees A.J. Acute movement disorders with bilateral basal ganglia lesions in uremia.Mov Disord. 1998; 13: 952-957Crossref PubMed Scopus (54) Google Scholar, 13.Wang H.C. Cheng S.J. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.J Neurol. 2003; 250: 948-955Crossref PubMed Scopus (73) Google Scholar, 16.Okada J. Yoshikawa K. Matsuo H. et al.Reversible MRI and CT findings in uremic encephalopathy.Neuroradiology. 1991; 33: 524-526Crossref PubMed Scopus (60) Google Scholar has been previously linked to basal ganglia injury, but exact causation has not been clearly established. Rapid correction of uremia may sometimes induce generalized encephalopathy attributed to cerebral edema (dialysis disequilibrium syndrome),25.Arieff A.I. Dialysis disequilibrium syndrome: current concepts on pathogenesis and prevention.Kidney Int. 1994; 45: 629-635Abstract Full Text PDF PubMed Scopus (134) Google Scholar but it has not been reported to cause isolated extrapyramidal symptoms. On the other hand, rapid correction of chronic hyponatremia, as may occur during dialysis treatment, has been reported to induce extrapontine myelinolysis involving basal ganglia and striatal pathways.3.Dickoff D.J. Raps M. Yahr M.D. Striatal syndrome following hyponatremia and its rapid correction. A manifestation of extrapontine myelinolysis confirmed by magnetic resonance imaging.Arch Neurol. 1988; 45: 112-114Crossref PubMed Scopus (41) Google Scholar,4.Hadfield M.G. Kubal W.S. Extrapontine myelinolysis of the basal ganglia without central pontine myelinolysis.Clin Neuropathol. 1996; 15: 96-100PubMed Google Scholar In addition, animal models suggest increased basal ganglia susceptibility to hypoxic injury in the presence of hyponatremia.26.Ayus J.C. Armstrong D. Arieff A.I. Hyponatremia with hypoxia: effects on brain adaptation, perfusion, and histology in rodents.Kidney Int. 2006; 69: 1319-1325Abstract Full Text Full Text PDF PubMed Scopus (64) Google Scholar Dialysis patients are also more prone to thiamine depletion17.Hung S.C. Hung S.H. Tarng D.C. et al.Chorea induced by thiamine deficiency in hemodialysis patients.Am J Kidney Dis. 2001; 37: 427-430Abstract Full Text PDF PubMed Scopus (37) Google Scholar as well as aluminum22.Arieff A.I. Aluminum and the pathogenesis of dialysis encephalopathy.Am J Kidney Dis. 1985; 6: 317-321Abstract Full Text PDF PubMed Scopus (47) Google Scholar and manganese toxicity.21.Ohtake T. Negishi K. Okamoto K. et al.Manganese-induced Parkinsonism in a patient undergoing maintenance hemodialysis.Am J Kidney Dis. 2005; 46: 749-753Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar All three have been observed to cause movement disorders. In this case, the patient had been on adequate dialysis since May 2003; thus direct effect of uremia is unlikely. She had no history of chronic hyponatremia, and both pre- and postdialysis serum sodium levels were in the normal range. Her thiamine, aluminum, and manganese levels were also within normal limits. In most,11.Wang H.C. Hsu J.L. Shen Y.Y. Acute bilateral basal ganglia lesions in patients with diabetic uremia: an FDG-PET study.Clin Nucl Med. 2004; 29: 475-478Crossref PubMed Scopus (35) Google Scholar, 12.Wang H.C. Brown P. Lees A.J. Acute movement disorders with bilateral basal ganglia lesions in uremia.Mov Disord. 1998; 13: 952-957Crossref PubMed Scopus (54) Google Scholar, 13.Wang H.C. Cheng S.J. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.J Neurol. 2003; 250: 948-955Crossref PubMed Scopus (73) Google Scholar but not all,14.Lee P.H. Shin D.H. Kim J.W. et al.Parkinsonism with basal ganglia lesions in a patient with uremia: evidence of vasogenic edema.Parkinsonism Relat Disord. 2006; 12: 93-96Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar prior cases of bilateral basal ganglia injury in patients with ESRD, affected patients were also diabetics. Both hypoglycemia5.Lai S.L. Tseng Y.L. Hsu M.C. Chen S.S. Magnetic resonance imaging and single-photon emission computed tomography changes in hypoglycemia-induced chorea.Mov Disord. 2004; 19: 475-478Crossref PubMed Scopus (27) Google Scholar,6.Newman R.P. Kinkel W.R. Paroxysmal choreoathetosis due to hypoglycemia.Arch Neurol. 1984; 41: 341-342Crossref PubMed Scopus (58) Google Scholar and hyperglycemia7.Hsu J.L. Wang H.C. Hsu W.C. Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea. A PET study.J Neurol. 2004; 251: 1486-1490Crossref PubMed Scopus (75) Google Scholar,8.Branca D. Gervasio O. Le Piane E. et al.Chorea induced by non-ketotic hyperglycaemia: a case report.Neurol Sci. 2005; 26: 275-277Crossref PubMed Scopus (28) Google Scholar may rarely induce chorea. Two cases of hypoglycemia-induced chorea in insulin-dependent diabetics on dialysis have been reported recently.5.Lai S.L. Tseng Y.L. Hsu M.C. Chen S.S. Magnetic resonance imaging and single-photon emission computed tomography changes in hypoglycemia-induced chorea.Mov Disord. 2004; 19: 475-478Crossref PubMed Scopus (27) Google Scholar Diabetics with kidney disease may be more prone to hypoglycemia due to changes in insulin clearance and metabolism. In addition, insulin is not significantly removed by either peritoneal or hemodialysis due to its high molecular weight. This puts diabetic dialysis patients at higher risk of hypoglycemia, which then can mediate basal ganglia injury. Patients with diabetes are also prone to ketoacidosis. Ketotic hyperglycemia,27.Crausman R.S. Wen J. Al-Shalabi S. Choreoathetosis and diabetes.Diabetes Care. 1997; 20: 1209-1210Crossref PubMed Scopus (10) Google Scholar highly ketogenic diet,9.Erickson J.C. Jabbari B. Difazio M.P. Basal ganglia injury as a complication of the ketogenic diet.Mov Disord. 2003; 18: 448-451Crossref PubMed Scopus (18) Google Scholar as well as other organic acidemias10.Brismar J. Ozand P.T. CT and MR of the brain in the diagnosis of organic acidemias. Experiences from 107 patients.Brain Dev. 1994; 16: 104-124Abstract Full Text PDF PubMed Scopus (74) Google Scholar have all been reported to cause basal ganglia injury. In our patient, there was no evidence of markedly abnormal glucose values or anion gap acidosis on admission and during hospitalization. Her diabetes was adequately controlled with oral agents and she had not been receiving insulin. Finally, chorea has been described in children following an open cardiac surgery requiring cardiopulmonary bypass.23.Medlock M.D. Cruse R.S. Winek S.J. et al.A 10-year experience with postpump chorea.Ann Neurol. 1993; 34: 820-826Crossref PubMed Scopus (57) Google Scholar The mechanism is unclear, but children who developed this complication spent more time on pump, were more hypothermic, and more were likely to have had a circulatory arrest compared to age-matched controls. These data suggest that basal ganglia injury may be induced by hypoperfusion or, less likely, by hypothermia. Theoretically, intradialytic hypotension in a susceptible patient with cerebral vascular disease may lead to a similar type of ischemic injury. Patients with diabetes and ESRD are known to carry a higher burden of macro- and microvascular pathology. It is likely that a patient with advanced microvascular disease in the basal ganglia region is more susceptible to other toxic insults, hypo/hyperglycemia, acidosis, hypoxia, and/or hypoperfusion. There is a possibility that an undetected episode of intradialytic hypotension superimposed on underlying cerebral microvascular disease precipitated symptoms in this case. Our case contributes to a growing evidence for the risk of basal ganglia injury in dialysis patients. This risk may be greatest for diabetic patients and, as illustrated by our case, is not limited to patients of Asian ancestry. In this case, a comprehensive neurological workup failed to identify a specific cause of basal ganglia injury. Larger epidemiologic studies are needed to establish a prevalence of this disorder in ESRD patients and explore causative associations between basal ganglia injury, diabetes, ethnicity, and dialysis-related risk factors." @default.
• W2069471056 created "2016-06-24" @default.
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• W2069471056 date "2008-05-01" @default.
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• W2069471056 title "Acute chorea and bilateral basal ganglia lesions in a hemodialysis patient" @default.
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