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Uremic Encephalopathy

Uremic Encephalopathy Syndrome

Uremic encephalopathy is a complication of renal failure leading to cerebral dysfunction. It is characterized by nausea, vomiting, lethargy and disorientation in the initial stages progressing to delirium, seizures and later coma, if untreated. Diagnosis is based on clinical signs, cognitive tests, renal function tests, serum biochemistry, and imaging studies of the brain.


Presentation

Uremic encephalopathy (UE) is an acquired metabolic complication of renal failure with accumulation of toxic metabolites which cross the blood brain barrier to cause cerebral dysfunction. It manifests in the form of acute or subacute neurological features which are usually reversible [1] [2] [3] [4]. Initial symptoms of UE are nausea, vomiting, loss of appetite, drowsiness, inability to concentrate emotional lability, irritability, confusion progressing, if untreated, to seizures, delirium, myoclonus, asterixis and coma [5].

Once treatment of renal dysfunction is instituted patients may present with impaired cognition, generalized asthenia, and peripheral neuropathy [6]. Renal dialysis in chronic renal failure or end-stage renal disease is associated with dysequilibrium and dementia. Dialysis-associated disequilibrium is characterized by nausea, muscle cramps, altered sensorium, and seizures while dialysis-associated dementia is a progressive condition with increased incidence of mortality seen in patients on long-term hemodialysis. Dialysis encephalopathy can be either sporadic, epidemic or associated with pediatric renal disease [6].

Stupor
  • Drowsiness Diminished ability to concentrate Slowed cognitive functions More severe signs and symptoms of uremic encephalopathy include the following: Vomiting Emotional volatility Decreased cognitive function Disorientation Confusion Bizarre behavior Stupor[emedicine.medscape.com]
  • Cerebral Dysfunctions* Changes in sensorium : loss of memory, impaired concentration, depression, delusions, lethargy, irritability, fatigue, insomnia, psychosis, stupor, catatonia, and coma.[prezi.com]
  • These symptoms are followed by shortened attention span and muscular incoordination, asterixis, and lethargy, progressing to stupor and coma. Multiple episodes of PSE are not uncommon.[alzheimer-europe.org]
  • Clinical Indicators: Showing some of the following signs and symptoms: Altered Mental Status from baseline Coma Confusion Delirium Disorientation Lethargic Psychosis Restlessness Stupor Patient may become more alert when underlying condition is treated[forums.acdis.org]
  • Further reading [ edit ] The Diagnosis of Stupor and Coma by Plum and Posner, ISBN 0-19-513898-8 , remains one of the best detailed observational references to the condition.[en.wikipedia.org]
Altered Mental Status
  • mental status (confusion) Cranial nerve signs (nystagmus) Papilledema Hyperreflexia, clonus, asterixis Altered gait Stupor Coma (occurs only if uremia remains untreated and progresses) Author Specialty Editor Board Francisco Talavera, PharmD, PhD Adjunct[emedicine.medscape.com]
  • 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.[ruralneuropractice.com]
  • Clinical Indicators: Showing some of the following signs and symptoms: Altered Mental Status from baseline Coma Confusion Delirium Disorientation Lethargic Psychosis Restlessness Stupor Patient may become more alert when underlying condition is treated[forums.acdis.org]
  • Symptoms and course Fulminant hepatic failure results from severe inflammatory or necrotic liver disease of rapid onset and progressive neurological signs from altered mental status, stupor and coma, often within hours or days.[alzheimer-europe.org]
  • Hypotension ( low blood pressure ) due to many factors (for example, bleeding , major infection, or blood pressure medications) may result in encephalopathy with symptoms of fainting , weakness, and altered mental status.[emedicinehealth.com]
Tremulousness
  • Within 30 minutes of the end of the dexmedetomidine infusion, symptoms of the twitch-convulsive syndrome returned, manifesting as acute tremulousness.[ncbi.nlm.nih.gov]

Workup

Workup in a patient presenting with confusion, nystagmus, hyperreflexia, clonus, asterixis and coma should include a thorough history and neurological examination. An ophthalmic examination may reveal papilledema. UE can be evaluated with many cognitive tests to measure psychomotor speed, short-term recognition and ability to make simple decisions. Laboratory tests are likely to show anemia (due to chronic renal disease), leukocytosis (if there is an underlying infection), with renal function tests [7] showing severely elevated blood urea nitrogen and serum creatinine levels. Serum electrolyte and glucose levels should be ordered to evaluate sodium and blood glucose levels to exclude electrolyte abnormalities, hyperglycemia, and hyperosmolar syndromes. Serum calcium, magnesium, phosphate and parathyroid hormone levels should be checked to exclude other causes of encephalopathy. A toxicology screen should also be performed. An electroencephalogram (EEG) obtained in UE is likely to show a disorganized pattern with absent alpha waves and occasional theta and delta waves.

Imaging studies like magnetic resonance imaging (MRI) or computed tomography (CT) scan of the head are only indicated to exclude intracranial tumors and hematoma or hemorrhage. CT scan findings are not specific for UE but MRI will reveal an increase in signal intensity either in the cerebral cortex or in the basal ganglia. [8] [9]. This is also called the lentiform fork sign. Based on the MRI findings, UE has been classified into two varieties [10]:

  • Cortical: involvement of the cortex is more common, develops in any patient with UE, is associated with posterior reversible encephalopathy syndrome (PRES) and not affected by diabetes mellitus [1] [3] [4]. MRI shows mainly vasogenic edema [11].
  • Bilateral basal ganglia involvement: this is rare and usually seen in Asian patients with diabetes mellitus [1] [2] [3] [12] [13]. MRI shows vasogenic as well as cytotoxic edema [11].
Triphasic Waves
  • The EEG background in uremic encephalopathy demonstrates bilateral, frontal-predominant slow activity, which may include diphasic and triphasic waves. 3 Photomyoclonic and photoconvulsive responses to photic stimulation may occur. 3 When seizures occur[epilepsy.com]
  • Furthermore, in these cases, other epileptic patterns in the form of spikes or sharp waves can also be detected. [39] However, triphasic waves are not specific only for hepatic encephalopathy.[neurologyindia.com]
Absent A-Waves
  • An electroencephalogram (EEG) obtained in UE is likely to show a disorganized pattern with absent alpha waves and occasional theta and delta waves.[symptoma.com]

Treatment

  • We reported a rare case of a patient with UE that had unusual imaging manifestations for whom timely diagnosis and treatment assured recovery.[ncbi.nlm.nih.gov]
  • This case report a 59 years-old male in regular dialysis treatment with neurologic emergency characterized by neurologic signs as deep sopor the cause of which was uremic encephalopathy.[moh-it.pure.elsevier.com]
  • Uremic encephalopathy treatment options: 1. dialysis treatment: adequate dialysis can reduce the occurrence of uremic encephalopathy. 2. renal transplantation: can fundamentally eradicate the treatment of uremia 3. reduce drug damage 4. symptomatic treatment[120kidney.com]
  • The treatment for uremic encephalopathy is dialysis of some sort - peritoneal, etc. - or a kidney transplant, of course. But the latter is not the immediate treatment of choice.[health.stackexchange.com]
  • After several dialysis treatments, his BUN decreased and the dexmedetomidine was weaned, without return of the symptoms of twitch-convulsive syndrome.[ncbi.nlm.nih.gov]

Prognosis

  • Seizures indicated a grave prognosis. Five of six patients with seizures died. In some patients, progressive uremic encephalopathy may develop without hemodialysis.[ncbi.nlm.nih.gov]
  • Consequently, the prognosis varies from patient to patient and ranges from complete recovery to a poor prognosis that often leads to permanent brain damage or death. Thank You Very Much![prezi.com]
  • Seizures indicated a grave prognosis. Five of six patients with seizures died. In some patients, progressive wemic encephalopathy may develop without hemodialysis.[neurology.org]
  • The combination of AKI and neurological disturbances signals a poor prognosis with RRT having little effect on neurological improvement or mortality.[icm-experimental.springeropen.com]
  • Consequently, the prognosis varies from patient to patient and ranges from complete recovery to a poor prognosis that often leads to permanent brain damage or death.[medicinenet.com]

Etiology

  • The etiology of uremic encephalo pathy is especially complicated. If the patient is not rescued in time, it may endanger the patient's life.[120kidney.com]
  • Uremic encephalopathy may occur in a patient with acute kidney injury or chronic kidney failure of any etiology. One contributing factor to uremic encephalopathy may involve imbalances of neurotransmitter amino acids within the brain.[emedicine.medscape.com]
  • Another theory about the etiology of uremic encephalopathy suggests imbalances of neurotransmitter amino acids within the brain.[thehealthscience.com]
  • […] of Altered Mental Status such as: Alzheimer’s disease Chemical dependencies CVA or TIA Hx Medications Parkinson’s disease Lewy body dementia Mood disorders Schizophrenia Seizure Hx Tumors Tests to determine etiology may include but are not limited to[forums.acdis.org]
  • Definition, etiologies and mortalities. JAMA 1996;275:470-3. 49. Garcıa-Martınez R, Simon-Talero M, Cordoba J. Prognostic assessment in patients with hepatic encephalopathy. Dis Markers 2011;31:171-9. 50. Young B.[neurologyindia.com]

Epidemiology

  • Written by internationally renowned experts, this clinical reference offers helpful advice with the most recent information on the definition, epidemiology, pathophysiology, and clinical causes of acute kidney failure as a fundamental prerequisite for[books.google.com]
  • Epidemiology Frequency United StatesMost patients with a CrCl level less than 10% of normal probably develop some degree of encephalopathy; however, they may not be clearly symptomatic.[thehealthscience.com]
  • Epidemiology and healthcare utilization of neurogenic bladder patients in a US claims database. Neurourol Urodyn. 2011;30:395–401. View Article PubMed Google Scholar Copyright[bmcneurol.biomedcentral.com]
  • Methods Statistics Trigonometry Medical & Nursing Anatomy Anesthesiology Audiology Bacteriology Biochemistry Bioethics Biomedical Science Cardiology Cardiovascular Childbirth Chiropractic Dentistry Dermatology Diagnostic Imaging Drugs Endocrinology Epidemiology[brainscape.com]
  • There are very few studies dealing with the epidemiology of the encephalopathic aspect of renal dysfunction.[radiopaedia.org]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of uremic encephalopathy is not well understood and multiple potential "uremic toxins" have been evaluated. Of these, parathyroid hormone is the only substance to be clearly linked to clinical findings.[ncbi.nlm.nih.gov]
  • The pathophysiology of uremic encephalopathy is not well understood and multiple potential 'uremic toxins' have been evaluated. Of these, parathyroid hormone is the only substance to be clearly linked to clinical findings.[indiana.pure.elsevier.com]
  • Prompt identification of uremia as the cause of encephalopathy is essential because symptoms are readily reversible following initiation of dialysis. [5, 6] Pathophysiology Uremic encephalopathy has a complex pathophysiology, and many toxins that accumulate[thehealthscience.com]
  • Written by internationally renowned experts, this clinical reference offers helpful advice with the most recent information on the definition, epidemiology, pathophysiology, and clinical causes of acute kidney failure as a fundamental prerequisite for[books.google.com]
  • Although the detailed pathophysiology of UE is still unknown, the MRI and clinical changes observed in this patient suggest that diffuse edematous brain damage is one of the characteristic features of UE.[jstage.jst.go.jp]

Prevention

  • The goal is to identify a dysfunctional AVF early enough to intervene in a timely manner, either to assist the maturation process or to prevent thrombosis.[moh-it.pure.elsevier.com]
  • The treatments and prevention of each are discussed.[ncbi.nlm.nih.gov]
  • Written by internationally renowned experts, this clinical reference offers helpful advice with the most recent information on the definition, epidemiology, pathophysiology, and clinical causes of acute kidney failure as a fundamental prerequisite for prevention[books.google.com]
  • Early recognition of encephalopathy in the setting of decreased renal function is crucial to prevent morbidity or mortality. Race No racial predilection exists. Sex No significant association between sex and incidence exists.[thehealthscience.com]
  • Prevention Do not skip or avoid scheduled dialysis. Take all medications as directed and have frequent assessments of mental status.[prezi.com]

References

Article

  1. Wang HC, Cheng SJ. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients. J Neurol. 2003;250:948–955.
  2. Kim TK, Seo SI, Kim JH, Lee NJ, Seol HY. Diffusion-weighted magnetic resonance imaging in the syndrome of acute bilateral basal ganglia lesions in diabetic uremia. Mov Disord. 2006;21:1267–1270.
  3. Wang HC, Brown P, Lees AJ. Acute movement disorders with bilateral basal ganglia lesions in uremia. Mov Disord. 1998;13:952–957.
  4. Prüss H, Siebert E, Masuhr F. Reversible cytotoxic brain edema and facial weakness in uremic encephalopathy. J Neurol. 2009;256:1372–1373.
  5. Chertow GM, Marsden PA et.al. Brenner and Rector's The Kidney. Saunders. ISBN:1416061932.
  6. Mahoney CA, Arieff AI. Uremic encephalopathies: clinical, biochemical and experimental features. Am J. Kidney Dis. 1982 Nov; 2(3):324 -36
  7. Yamamoto T, Satomura K, Okada S, et al. Risk factors for neurological complications in complete hemolytic uremic syndrome caused by Escherichia coli O157. Pediatr Int. 2009 Apr; 51(2):216-9.
  8. Schmidt M, Sitter T, Lederer SR, Held E, Schiffl H. Reversible MRI changes in a patient with uremic encephalopathy. J Nephrol. 2001 Sep-Oct;14(5):424-7.
  9. Kim DM, Lee IH, Song CJ. Uremic Encephalopathy: MR Imaging Findings and Clinical Correlation. AJNR Am J Neuroradiol. 2016 Sep; 37 (9):1604-9.
  10. Kang E, Jeon SJ, Choi S-S. Uremic encephalopathy with atypical magnetic resonance features on diffusion weighted images. Korean j Radiol. 2012 Nov-Dec; 13(6): 808-811
  11. Yoon CH, Seok JI, Lee DK, An GS. Bilateral basal ganglia and unilateral cortical involvement in a diabetic uremic patient. Clin Neurol Neurosurg. 2009;111:477–479.
  12. Raskin NH. Neurological complications of renal failure. In: Aminoff MJ, editor. Neurology and general medicine. New York: Churchill Livingstone; 1995. pp. 303–319.
  13. Raskin NH, Fishman RA. Neurologic disorders in renal failure (first of two parts) N Engl J Med. 1976;294:143–148.

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Last updated: 2018-06-22 02:19