Neurological Manifestations of Uremic Encephalopathy Clinical Presentation

  • Author: Gabriel Bucurescu, MD, MS; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA   more...
 
Updated: Aug 17, 2010
 

History

  • Uremic encephalopathy (UE) is a consequence of renal failure (RF).
  • Symptoms begin insidiously and are often noticed not by the patients but by their family members or caregivers.
  • In many cases, impairment of the nervous system provides the first indication of metabolic derangements.
  • Symptoms may progress slowly or rapidly.
  • Changes in sensorium include loss of memory, impaired concentration, depression, delusions, lethargy, irritability, fatigue, insomnia, psychosis, stupor, catatonia, and coma.
  • Patients may complain of slurred speech, pruritus, muscle twitches, or restless legs.
Next

Physical

Physical findings are variable and depend on the severity of the encephalopathy. Neurologic findings range from normal to a comatose state. Cases of Wernicke syndrome associated with UE have been described in the literature, and Wernicke syndrome has been observed in patients with UE, dialysis dementia, or dialysis disequilibrium syndrome.

  • Findings include the following:
    • Myoclonic jerks, twitches, or fasciculations (ie, uremic twitch-convulsive syndrome postulated by Adams et al in 1997)
    • Asterixis
    • Dysarthria
    • Agitation
    • Tetany
    • Seizures, usually generalized tonic-clonic
    • Confusion, stupor, and other alterations in mental status
    • Coma
    • Sleep disorders
  • Some patients undergoing long-term dialysis acquire dialysis encephalopathy (or dialysis dementia), which is a subacute, progressive, and often fatal disease.[4] Aluminum toxicity either from aluminum phosphate salts or from aluminum in the dialysate were linked to the pathogenesis of dialysis dementia. Starting in the early and mid 1980s, aluminum was actively removed from the dialysate with a large reduction in the incidence of dialysis dementia.
    • Dialysis encephalopathy is believed to be part of a multisystem disease that includes encephalopathy, osteomalacic bone disease, proximal myopathy, and anemia.
    • Symptoms include dysarthria, apraxia, personality changes, psychosis, myoclonus, seizures and, finally, dementia.
    • In most cases, the condition progresses to death in 6 months.
  • Dialysis disequilibrium syndrome occurs in patients receiving hemodialysis.
    • Symptoms include headache, nausea, emesis, blurred vision, muscular twitching, disorientation, delirium, hypertension, tremors, and seizures.
    • The condition tends to be self-limited and subsides over several hours.
    • Dialysis disequilibrium syndrome is attributed to a reverse urea effect. Urea is cleared more slowly from the brain than from the blood, an effect that causes an osmotic gradient leading to the net flow of water into the brain and to transient cerebral edema.
  • Complications of renal transplantation can lead to UE. This occurrence has become more common as more patients are receiving renal transplants.
    • This condition is characterized by edema of the white matter.
    • Patients are at risk for primary brain lymphoma and opportunistic infections because of long-term immunosuppression.
  • Rejection encephalopathy has been observed in patients undergoing transplantation. They have systemic features of acute graft rejection, more than 80% of whom have symptoms in the first 3 months after transplantation. Overall prognosis is good, with rapid recovery after treatment of the rejection episode. The presumed pathology is cytokine production secondary to the rejection process.
  • Uremic polyneuropathy is the most common neurologic complication of RF.
Previous
Next

Causes

  • The exact cause of UE is unknown.
  • Accumulation of metabolites and, perhaps, imbalance in excitatory and inhibitory neurotransmitters are possible etiologies.
  • PTH and abnormal calcium control have also been identified as possible important contributing factors.
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Gabriel Bucurescu, MD, MS  Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center

Gabriel Bucurescu, MD, MS is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, and American Epilepsy Society

Disclosure: Nothing to disclose.

Specialty Editor Board

J Stephen Huff, MD  Associate Professor, Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health Sciences Center

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Richard J Caselli, MD  Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale

Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA  Associate Dean, College of Medicine, Professor of Neurology, Neurosurgery and Psychiatry, Director of Cognitive Neurology, Department of Neurology, University of South Florida College of Medicine; Director of Stroke Service, Tampa General Hospital; Director of Stroke Program, James A Haley Veterans Affairs Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, American Heart Association, and American Society of Neuroimaging

Disclosure: Nothing to disclose.

References

  1. De Deyn PP, D'Hooge R, Van Bogaert PP, Marescau B. Endogenous guanidino compounds as uremic neurotoxins. Kidney Int Suppl. Feb 2001;78:S77-83. [Medline].

  2. Liu M, Liang Y, Chigurupati S, Lathia JD, Pletnikov M, Sun Z, et al. Acute kidney injury leads to inflammation and functional changes in the brain. J Am Soc Nephrol. Jul 2008;19(7):1360-70. [Medline]. [Full Text].

  3. Lacerda G, Krummel T, Hirsch E. Neurologic presentations of renal diseases. Neurol Clin. Feb 2010;28(1):45-59. [Medline].

  4. Brouns R, De Deyn PP. Neurological complications in renal failure: a review. Clin Neurol Neurosurg. Dec 2004;107(1):1-16. [Medline].

  5. Yoon CH, Seok JI, Lee DK, An GS. Bilateral basal ganglia and unilateral cortical involvement in a diabetic uremic patient. Clin Neurol Neurosurg. Jun 2009;111(5):477-9. [Medline].

  6. Röhl JE, Harms L, Pommer W. Quantitative EEG findings in patients with chronic renal failure. Eur J Med Res. Apr 26 2007;12(4):173-8. [Medline].

  7. Singh NP, Sahni V, Wadhwa A, Garg S, Bajaj SK, Kohli R. Effect of improvement in anemia on electroneurophysiological markers (P300) of cognitive dysfunction in chronic kidney disease. Hemodial Int. Jul 2006;10(3):267-73. [Medline].

  8. Cheng BC, Chang WN, Lu CH, Chen JB, Chang CS, Lee CH. Bacterial meningitis in hemodialyzed patients. J Nephrol. Mar-Apr 2004;17(2):236-41. [Medline].

  9. Arieff AI, Mahoney CA. Pathogenesis of dialysis encephalopathy. Neurobehav Toxicol Teratol. Nov-Dec 1983;5(6):641-4. [Medline].

  10. Balzer S, Kuttner K. [Early auditory evoked potential. A diagnostic parameter in uremic encephalopathy]. HNO. Oct 1996;44(10):559-66. [Medline].

  11. Biasioli S, D'Andrea G, Chiaramonte S, Fabris A, Feriani M, Ronco C, et al. The role of neurotransmitters in the genesis of uremic encephalopathy. Int J Artif Organs. Mar 1984;7(2):101-6. [Medline].

  12. Biasioli S, D'Andrea G, Fabris A, Feriani M, La Greca G. The pathogenesis of uremic encephalopathy (UE). Int J Artif Organs. Jan 1985;8(1):59-60. [Medline].

  13. Bolton CF, Young GB. Uremic encephalopathy. In: Neurological Complications of Renal Disease. Boston, MA: Butterworths; 1990:43-74.

  14. Bourne JR, Teschan PE. Computer methods, uremic encephalopathy, and adequacy of dialysis. Kidney Int. Oct 1983;24(4):496-506. [Medline].

  15. Brouns R, De Deyn PP. Neurological complications in renal failure: a review. Clin Neurol Neurosurg. Dec 2004;107(1):1-16. [Medline].

  16. Chiappa KH, Hill RA. Evoked Potentials in Clinical Medicine. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1997.

  17. Chow KM, Wang AY, Hui AC, Wong TY, Szeto CC, Li PK. Nonconvulsive status epilepticus in peritoneal dialysis patients. Am J Kidney Dis. Aug 2001;38(2):400-5. [Medline].

  18. De Deyn PP, Saxena VK, Abts H, et al. Clinical and pathophysiological aspects of neurologic complications in renal failure. Acta Neurol Belg. 1992;92:191-206. [Medline].

  19. De Deyn PP, Vanholder R, D'Hooge R. Nitric oxide in uremia: effects of several potentially toxic guanidino compounds. Kidney Int Suppl. May 2003;S25-8. [Medline].

  20. Ganesh SK, Hulbert-Shearon T, Port FK, Eagle K, Stack AG. Mortality differences by dialysis modality among incident ESRD patients with and without coronary artery disease. J Am Soc Nephrol. Feb 2003;14(2):415-24. [Medline].

  21. Habach G, Bloembergen WE, Mauger EA, Wolfe RA, Port FK. Hospitalization among United States dialysis patients: hemodialysis versus peritoneal dialysis. J Am Soc Nephrol. May 1995;5(11):1940-8. [Medline].

  22. Hung SC, Hung SH, Tarng DC, Yang WC, Chen TW, Huang TP. Thiamine deficiency and unexplained encephalopathy in hemodialysis and peritoneal dialysis patients. Am J Kidney Dis. Nov 2001;38(5):941-7. [Medline].

  23. Jagadha V, Deck JH, Halliday WC, Smyth HS. Wernicke's encephalopathy in patients on peritoneal dialysis or hemodialysis. Ann Neurol. Jan 1987;21(1):78-84. [Medline].

  24. Jeppsson B, Freund HR, Gimmon Z, et al. Blood-brain barrier derangement in uremic encephalopathy. Surgery. Jul 1982;92(1):30-5. [Medline].

  25. Kiley JE, Pratt KL, Gisser DG, Schaffer CA. Techniques of EEG frequency analysis for evaluation of uremic encephalopathy. Clin Nephrol. Jun 1976;5(6):279-85. [Medline].

  26. Liu M, Liang Y, Chigurupati S, Lathia JD, Pletnikov M, Sun Z, et al. Acute kidney injury leads to inflammation and functional changes in the brain. J Am Soc Nephrol. Jul 2008;19(7):1360-70. [Medline].

  27. Lockwood AH. Neurologic complications of renal disease. Neurol Clin. Aug 1989;7(3):617-27. [Medline].

  28. Mahoney CA, Arieff AI. Uremic encephalopathies: clinical, biochemical, and experimental features. Am J Kidney Dis. Nov 1982;2(3):324-36. [Medline].

  29. Moe SM, Sprague SM. Uremic encephalopathy. Clin Nephrol. Oct 1994;42(4):251-6. [Medline].

  30. Murphy SW, Foley RN, Barrett BJ, Kent GM, Morgan J, Barré P, et al. Comparative hospitalization of hemodialysis and peritoneal dialysis patients in Canada. Kidney Int. Jun 2000;57(6):2557-63. [Medline].

  31. Niedermeyer E. Metabolic central nervous system disorders. In: Basic Principles, Clinical Applications, and Related Fields. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1993:405-18.

  32. Norenberg MD, Bruce-Gregorios J. Renal disease. In: Textbook of Neuropathology. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1997:575-9.

  33. Ropper AH, Brown RH. Uremic encephalopathy. In: Adams and Victor's Principles of Neurology. 8th ed. New York, NY: McGraw-Hill Professional; 2005:969-71.

  34. Schmidt M, Sitter T, Lederer SR, et al. Reversible MRI changes in a patient with uremic encephalopathy. J Nephrol. Sep-Oct 2001;14(5):424-7. [Medline].

  35. Souheaver GT, Ryan JJ, DeWolfe AS. Neuropsychological patterns in uremia. J Clin Psychol. Jul 1982;38(3):490-6. [Medline].

  36. Topczewska-Bruns J, Pawlak D, Chabielska E, et al. Increased levels of 3-hydroxykynurenine in different brain regions of rats with chronic renal insufficiency. Brain Res Bull. Aug 15 2002;58(4):423-8. [Medline].

  37. Wu AW, Fink NE, Marsh-Manzi JV, Meyer KB, Finkelstein FO, Chapman MM, et al. Changes in quality of life during hemodialysis and peritoneal dialysis treatment: generic and disease specific measures. J Am Soc Nephrol. Mar 2004;15(3):743-53. [Medline].

  38. Zucker I, Yosipovitch G, David M, et al. Prevalence and characterization of uremic pruritus in patients undergoing hemodialysis: uremic pruritus is still a major problem for patients with end-stage renal disease. J Am Acad Dermatol. Nov 2003;49(5):842-6. [Medline].

 
Previous
Next
 
EEG in a 56-year-old man with uremic encephalopathy. He became increasingly lethargic, requiring intubation. EEG shows absence of a posterior dominant alpha rhythm and diffuse bilateral slowing with mixed theta- and delta-frequency signal. A single sharp wave is present in the left occipital region, phase reversing at O1. From top to bottom: Fp1-F7, F7-T3, T3-T5, T5-O1, O1-O2, O2-T6, T6-T4, T4-F8, F8-Fp2, Fp2-Fp1, F3-C3, C3-P3, P3-O1, F4-C4, C4-P4, P4-O2, Fz-Cz, and ECG.
EEG in a 56-year-old man with uremic encephalopathy. From top to bottom: Fp1-F7, F7-T3, T3-T5, T5-O1, Fp2-F8, F8-T4, T4-T6, T6-O2, Fp1-F3, F3-C3, C3-P3, P3=O1, Fp2-F4, F4-C4, C4-P4, P4-O2, Fz-Cz, ECG.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.