Home About us Current issue Back issues Submission Instructions Advertise Contact Login   

Search Article 
  
Advanced search 
 
Saudi Journal of Kidney Diseases and Transplantation
Users online: 2131 Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size 
 


 
Table of Contents   
CASE REPORT  
Year : 2014  |  Volume : 25  |  Issue : 3  |  Page : 625-629
Posterior reversible encephalopathy syndrome with tactile hallucinations secondary to dialysis disequilibrium syndrome


1 Department of Medicine, King Abdulaziz Medical City, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
2 Department of Anesthesia, King Abdulaziz Medical City, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
3 Department of Medicine, King Saud University, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia

Click here for correspondence address and email

Date of Web Publication9-May-2014
 

   Abstract 

We report what we believe is the first case of posterior reversible encephalopathy syndrome (PRES) secondary to dialysis disequilibrium syndrome (DDS) in patients in whom all other possible causes of PRES were excluded and in whom a transient episode of tactile hallucination also occurred. We believe that this case of DDS was particularly severe, leading to PRES because of the late institution of dialysis therapy and the concomitant severe degree of metabolic acidosis on presentation.

How to cite this article:
Soomro A, Al Bahri R, Alhassan N, Hejaili FF, Al Sayyari AA. Posterior reversible encephalopathy syndrome with tactile hallucinations secondary to dialysis disequilibrium syndrome. Saudi J Kidney Dis Transpl 2014;25:625-9

How to cite this URL:
Soomro A, Al Bahri R, Alhassan N, Hejaili FF, Al Sayyari AA. Posterior reversible encephalopathy syndrome with tactile hallucinations secondary to dialysis disequilibrium syndrome. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2019 Sep 20];25:625-9. Available from: http://www.sjkdt.org/text.asp?2014/25/3/625/132216

   Introduction Top


Dialysis disequilibrium syndrome (DDS) is mostly seen in patients undergoing rapid hemodialysis (HD) during one of the initial treatment sessions and typically presents with headache, nausea, blurry vision, seizures and coma. [1],[2] Posterior reversible encephalopathy syndrome (PRES) is a diagnosis that can often be missed [3],[4] and manifests clinically with headache, altered mental status, disorientation, seizures and focal neurological signs and radiologically characterized by focal reversible vasogenic edema most commonly involving the parieto-occipital region. [5],[6] Involvement of the anterior cerebral regions, deep white matter and the brain stem are also frequently seen in the majority of patients. [7] In the majority of patients, full clinical recovery with radiological resolution occur within days to a few weeks if prompt diagnosis is made and treatment is given early. [7],[8]

We report here a patient with PRES secondary to dialysis DDS in whom blood pressure was not elevated and in whom all other possible etiologies were excluded.


   Case Report Top


The patient is a 16-year-old girl diagnosed with chronic kidney disease eight months prior to this admission. At that time, her renal biopsy showed severe pauci-immune crescentic glomerulonephritis with 11 sclerosed glomeruli of the 18 examined in the biopsy with a moderate degree of tubulointerstitial fibrosis. Her c-ANCA was 1.5 u/mL (normal range <10 u/mL) and p-ANCA was 1.55 umol/L (normal value <6 U/mL) and anti-DNA was 6.51 IU/ mL (normal range <20 IU/mL).

A diagnosis of ANCA-negative pauci-immune crescentic glomerulonephritis was made and she received methylprednisolone and four cycles of IV cyclophosphamide and maintained on a regimen of prednisolone, mychophenolate mofetil, amlodipine, labetalol and cotrimoxazole. At her initial presentation, her serum creatinine (SCr) was 642 umol/L, which did not improve with the medications given. Dialysis was recommended but the family refused, and continued to refuse dialysis that was offered during her follow-up outpatient clinic visits. Throughout the follow-up period, the ANCA level remained negative.

She now presented in the emergency room with uremic symptoms (tiredness, lethargy, loss of appetite, nausea and vomiting). She gave no history of headache, loss of consciousness, confusion, seizures or signs of fluid overload. Her blood pressure was 126/85, pulse 95, temperature 36.9°C and O 2 Sat 99%. The respiratory rate was 20/min and her weight was 39.2 kg. Labs revealed SCr level of 634 umol/L (eGFR 7 mL/min/1.73 m 2 ), serum urea of >44 mmol/L, serum CO 2 of 5 mmol/L, serum sodium of 131 mmol/L and serum calcium of 1.99 mmol/L. Her C and P ACNA levels were unremarkable. CMV IgM antibody negative, anti-DNA 1.25 IU/mL, lactic acid 1.3 mmol/L (normal range 0.5-2.2 mmol/L), TSH, free T3 and free T4 were all within the normal range.

In view of the severe uremic symptoms and metabolic acidosis, dialysis was commenced. One hour after the end of the first session of HD, which lasted for 2.5 h, with an arterial blood flow of 250 mL/min, she started to develop new-onset headaches, confusion and disorientation to person and time. Following the dialysis session, SCr dropped to 364 μmol/ L and BUN to 25.9 mmol/L, and serum CO 2 increased to 20 mmol/L, and serum sodium was 133 mol/L [Table 1].
Table 1: Pre- and post-dialysis biochemical findings.

Click here to view


At the time of these new symptoms, her BP was 132/76, temperature 36.7°C and O 2 saturation 98%. There were no other neurological symptoms or localizing signs.

A diagnosis of acute disequilibrium syndrome was made. The neurological symptoms worsened after the third daily session of HD, with increasing restlessness and insomnia, and a brain computed tomography (CT) was performed primarily to exclude other brain pathologies, including the possibility of vasculitis. It showed extensive hypodensity involving the periventricular deep and subcortical white matter of the frontal, parietal and occipital lobes in both cerebral hemispheres with cortical involvement in the right occipital lobe - highly suggestive of PRES [Figure 1].
Figure 1: Brain computed tomography at presentation (left) and 3 weeks later showing (right) complete resolution.

Click here to view


It was not possible to perform magnetic resonance imaging (MRI) of the brain at the same time due to the patient's confusion and uncooperativeness. However, it was possible to do it 1 week after the initial brain CT scan. The MRI was reported as follows: "compared to the CT brain of a week earlier a reduction in the bilateral multifocal brain white matter changes are noted supporting the clinical diagnosis of PRES" [Figure 2].
Figure 2: Brain magnetic resolution imaging 1 week after presentation (left) and 3 weeks after presentation (right) showing complete resolution).

Click here to view


The patient's hospital course

The patient was prescribed daily 1-h dialysis sessions along with D50 mL after each dialysis. No significant improvement was observed in her condition over the next five days and she started complaining of visual hallucinations with the perception of "worms creeping under her skin." She was seen by the psychiatrist who diagnosed her clinically as a case of acute psychosis and prescribed risperidone for the same.

She then made progressive recovery starting by the end of the first week with complete resolution of her symptoms by four weeks after the beginning of symptoms. Repeated CT and MRI imaging of the brain three weeks after the initial presentation showed complete resolution of the previous findings [Figure 1] and [Figure 2].

She continues to receive regular thrice weekly dialysis as an outpatient, and risperidone was discontinued before discharge.


   Discussion Top


The pathogenesis of DDS is not fully understood, but it has been explained by many theories in the literature. [2],[9],[10],[11] One of these is the so-called "reverse urea hypothesis" that suggests that the acute removal of urea from the brain through the blood-brain barrier is much slower than from the plasma, which creates an osmotic gradient that results in water shifting into the brain cells with subsequent cerebral edema. [2],[12]

The diagnosis of PRES is often missed, particularly in nephrology. [3],[4],[7] It is a clinico-radiological diagnosis that manifests clinically as headache, altered mental status, disorientation, seizures and focal neurological signs, together with radiological findings characterized by focal reversible vasogenic edema most commonly involving the parieto-occipital region. [5],[6],[7] Often, however, the anterior cerebral regions, deep white matter and the brain stem are also involved in many patients. [7] This condition was found to be mostly associated with malignant hypertension, pre-eclampsia and immunosuppressive therapy. [6],[8] It has never been reported before to be linked to DDS, except once. [13] However, it is quite likely that PRES in their reported case might be as a result of the severe hypertension at the time of presentation (BP reached up to 223/123) in addition to the use of rapamycin, which has been reported to cause PRES. [14],[15]

In our case, DDS was considered to be the most likely clinical diagnosis for many reasons. Firstly, the onset of the neurological symptoms was immediately after the initiation of HD in the absence of previous history of any neurological or psychiatric problems. Secondly, there was a delay of initiation of dialysis and the severe uremic symptoms as well as the presence of severe metabolic acidosis at presentation. Thirdly, there was rapid reduction of plasma urea after the first dialysis, which must have generated an osmotic gradient that supports the theory of "reverse urea hypothesis." [12] Fourthly, although PRES has been reported to occur with the use of some immunosuppressant, [6] there was no previous report of mychophenolate mofetil - which our patient was on - causing PRES. Additionally, this drug was held on admission prior to the development of her neurological symptoms. Lastly, the patient has recovered completely - clinically and radiologically - in a few weeks, which goes well with the known clinical behavior and reversibility of the condition.

On a related note, it is worth mentioning that one of the symptoms that were experienced by our patient was tactile hallucination-associated acute psychosis. She described it clearly as "worms crawling underneath the skin of her legs." This type of hallucination is rather rare, and is more commonly encountered in elderly females. [16] It is often associated with substance abuse, mainly cocaine and alcohol withdrawal, as part of delirium tremens. [17] It is also seen as a manifestation of schizophrenia or affective psychosis. [18] Our patient had no history of psychiatric illness, and, therefore, we believe that this unique type of hallucination can be added to the wide spectrum of the neuropsychiatric manifestations of DDS/PRES.

In conclusion, we believe that we are reporting the first case of DDS-related PRES in whom other possible causes were excluded and in whom a transient episode of tactile hallucination also occurred. We believe that this case of DDS was particularly severe thus leading to PRES because of the late commencement of dialysis and the concomitant severe degree of metabolic acidosis on presentation.


   Conflict of Interest Top


The authors have no conflict of interest to declare and no grants or any other form of funding was received by any of the authors.

 
   References Top

1.Krishnan AV, Kiernan MC. Neurological complications of chronic kidney disease. Nat Rev Neurol 2009;5:542-51.  Back to cited text no. 1
    
2.Rizzo MA, Frediani F, Granata A, Ravasi B, Cusi D, Gallieni M. Neurological complications of hemodialysis: State of the art. J Nephrol 2012;25:170-82.  Back to cited text no. 2
    
3.El Karoui K, Le Quintrec M, Dekeyser E, et al. Posterior reversible encephalopathy syndrome in systemic lupus erythematosus. Nephrol Dial Transplant 2008;23:757-63.  Back to cited text no. 3
    
4.Hagemann G, Ugur T, Witte OW, Fitzek C. Recurrent posterior reversible encephalopathy syndrome (PRES). J Hum Hypertens 2004;18: 287-9.  Back to cited text no. 4
    
5.Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior Reversible Encephalopathy Syndrome: Associated Clinical and Radiologic Findings. Mayo Clin Proc 2010;85:427-32.  Back to cited text no. 5
    
6.Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500.  Back to cited text no. 6
    
7.Staykov D, Schwab S. Posterior reversible encephalopathy syndrome. J Intensive Care Med 2012;27:11-24.  Back to cited text no. 7
    
8.Giriºgen I, Tosun A, Sönmez F, Ozsunar Y. Recurrent and atypical posterior reversible encephalopathy syndrome in a child with peritoneal dialysis. Turk J Pediatr 2010;52: 416-9.  Back to cited text no. 8
    
9.Silver SM, Sterns RH, Halperin ML. Brain swelling after dialysis: Old urea or new osmoles? Am J Kidney Dis 1996;28:1-13.  Back to cited text no. 9
    
10.Trinh-Trang-Tan MM, Cartron JP, Bankir L. Molecular basis for dialysis disequilibrium syndrome: Altered aquaporin and urea transporter expression in brain. Nephrol Dial Transplant 2005;20:1984-8.  Back to cited text no. 10
    
11.Silver SM. Cerebral edema after rapid dialysis is not caused by an increase in brain organic osmolytes. J Am Soc Nephrol 1995;6:1600-6.  Back to cited text no. 11
    
12.Patel N, Dalal P, Panesar M. Dialysis dis-equilibrium syndrome: A narrative review. Semin Dial 2008;21:493-8.  Back to cited text no. 12
    
13.Sheth KN, Wu GF, Messé SR, Wolf RL, Kasner SE. Dialysis disequilibrium: Another reversible posterior leukoencephalopathy syndrome? Clin Neurol Neurosurg 2003;105: 249-52.  Back to cited text no. 13
    
14.Wu Q, Marescaux C, Wolff V, et al. Tacro-limus-associated posterior reversible encephalopathy syndrome after solid organ transplantation. Eur Neurol 2010;64:169-77.  Back to cited text no. 14
    
15.Qin W, Tan CY, Huang X, Huang Z, Tao Y, Fu P. Rapamycin-induced posterior reversible encephalopathy in a kidney transplantation patient. Int Urol Nephrol 2011;43:913-6.  Back to cited text no. 15
    
16.Viejo J, Sánchez C. A fictitious insect infestation. Description of a case of Ekbom's Syndrome. Boletín de la Real Sociedad Española de Historia Natural. Sección biológica 2010; 104:47-49.  Back to cited text no. 16
    
17.Berrios GE. Tactile hallucinations: Conceptual and historical aspects. J Neurol Neurosurg Psychiatry 1982;45:285-93.  Back to cited text no. 17
    
18.Shahid A, Patel M, Avenido J, Jabeen S, Riley W. Hallucinations: Common features and causes. Curr Psychiatry 2011;10:22-9.  Back to cited text no. 18
    

Top
Correspondence Address:
Professor Abdulla A Al Sayyari
Professor of Medicine, King Saud Bin Abdulaziz University for Health Sciences, P. O. Box 22490, Riyadh 11426
Saudi Arabia
Login to access the Email id


DOI: 10.4103/1319-2442.132216

PMID: 24821163

Rights and Permissions


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

Top
   
 
 
    Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  
 


 
    Abstract
   Introduction
   Case Report
   Discussion
   Conflict of Interest
    References
    Article Figures
    Article Tables
 

 Article Access Statistics
    Viewed2380    
    Printed29    
    Emailed0    
    PDF Downloaded429    
    Comments [Add]    

Recommend this journal