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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2016  |  Volume : 27  |  Issue : 2  |  Page : 395-397
Asymptomatic pontine and extra-pontine lesions in a patient with end-stage renal disease


1 Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiology, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication11-Mar-2016
 

   Abstract 

Osmotic demyelination syndrome leading to central pontine/extra-pontine myelinolysis (CPM/EPM) occurs mainly in patients with history of alcohol abuse, malnourishment, following liver transplantation and less commonly, in association with other systemic diseases. Asymptomatic CPM/EPM is rare. Patients with end-stage renal disease (ESRD) who develop CPM/EPM are usually symptomatic with florid neurologic manifestations. Herein, we present a patient with ESRD on maintenance hemodialysis who was incidentally detected to have pontine and extra-pontine lesions suggestive of myelinolysis without any neurologic signs or symptoms.

How to cite this article:
Yadav RK, Das CJ, Bagchi S, Agarwal S. Asymptomatic pontine and extra-pontine lesions in a patient with end-stage renal disease. Saudi J Kidney Dis Transpl 2016;27:395-7

How to cite this URL:
Yadav RK, Das CJ, Bagchi S, Agarwal S. Asymptomatic pontine and extra-pontine lesions in a patient with end-stage renal disease. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2019 May 21];27:395-7. Available from: http://www.sjkdt.org/text.asp?2016/27/2/395/178578

   Introduction Top


Osmotic demyelination syndrome (ODS) leading to central pontine/extra-pontine myelinolysis (CPM/EPM) occurs mainly in patients with history of alcohol abuse, malnourishment, liver transplant, and less commonly, in association with other systemic diseases. [1]

Asymptomatic CPM/EPM is rare. [2],[3],[4],[5] Patients with end-stage renal disease (ESRD) who develop CPM/EPM are usually symptomatic with florid neurologic manifestations. [6],[7] Neurologic complications are known to occur in patients with chronic kidney disease (CKD), especially when they progress to ESRD. [6] ODS has been reported during or following hemodialysis with significant neurological manifestations. [6],[7] Asymptomatic CPM/EPM is very rare with only around seven case reports in patients with history of alcohol abuse, malnutrition, human immunodeficiency virus infection, and liver disease. [2],[3],[4],[5] Herein, we report pontine and extrapontine lesions suggestive of myelinolysis in an asymptomatic patient with ESRD on maintenance hemodialysis with no other evident precipitating factors.


   Case Report Top


A 35-year-old male with adult polycystic kidney disease (ADPKD)-ESRD on maintenance hemodialysis for the past one year presented to us in August 2013 for a living donor renal transplantation. His wife was the prospective donor. He gave no family history of ADPKD; however, his mother had died following a cerebrovascular accident (CVA) in 1990, the details of which were unavailable.

On examination, he was fully conscious, oriented with no neurological deficit. He gave no past history of altered sensorium, convulsions, or focal weakness. Since he had ADPKD and a family history of CVA, a screening magnetic resonance imaging (MRI) of the brain with angiography (nongadolinium) was performed to rule out cerebral aneurysm before transplant.

MRI showed bilateral white matter signal abnormalities involving the pons, basal ganglia, and peri-ventricular deep white matter suggestive of pontine and extra-pontine myelinolysis [Figure 1]. There was no cerebral aneurysm.
Figure 1: Magnetic resonance imaging shows T1 hypointense (A), T2 (B and D) and fluid-attenuated inversion recovery (C) hyper-intensity involving the pons with sparing of dorsal tegmentum and ventrolateral peripheral rim. The lesion does not show diffusion restriction (E and F) and no elevation of choline is seen within the lesion. Similar lesions are also seen in bilateral putamen, caudate nuclei, periventricular white matter, corona radiata, and centrum semiovale. These lesions are suggestive of pontine and extra-pontine myelinolysis.

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He was receiving twice weekly hemodialysis for more than one year preceding the imaging. However, he had no symptomatic or documented episodes of hyponatremia. He had no history of dialysis disequilibrium following the initiation of dialysis.

We also considered other causes of asymptomatic myelinolysis. He was not malnourished. He did not have any history of alcohol abuse, chronic liver disease, or any critical illness with prolonged hospitalization. [2],[3],[4],[5]

The MRI was repeated after four weeks and the lesions persisted. The patient continues to remain neurologically asymptomatic.


   Discussion Top


ODS leading to CPM and EPM is commonly seen in patients who are alcoholic, malnourished, with chronic liver disease, after liver transplantation, burns, pituitary and urologic surgery and, other systemic diseases. [1]

The exact etiology remains unclear. However, these patients are generally sick with electrolyte disturbances (most frequently hyponatremia) or fluid imbalance due to other comorbidities. Rapid correction of hyponatremia creates an osmotic gradient with a hypotonic extracellular fluid, which is believed to cause the cerebral edema and demyelination.

ODS in patients with ESRD is most likely caused by the rapid changes in plasma solute levels, resulting in the change of serum osmolality that occurs during or after hemodialysis. [1] However, these osmolality shifts which cause dialysis disequilibrium after hemodialysis initiation do not cause ODS as frequently as theoretically expected. Affected patients are usually sick with a biphasic clinical course: an initial encephalopathy or convulsions secondary to hyponatremia, an intervening period of stabilization when the hyponatremia is corrected, followed by rapid worsening with dysarthria, dysphagia, quadriparesis, and even a "locked in" state.

There have been few reports of incidentally detected asymptomatic CPM/EPM in literature mostly in patients with alcoholism, malnourishment, and after liver transplants. [2]3],[4],[5]

Tarhan et al [7] reviewed 17 patients with ESRD on hemodialysis who were diagnosed with ODS based on MRI findings. However, all these patients had neurologic signs and symptoms which had manifested during or within 24 h after hemodialysis. In addition, eight patients were hyponatremic and eight others had hyperglycemia at the time of the episode. Although all their patients were symptomatic, the clinical symptoms did not always match with the MRI findings.

Our patient had absolutely no neurologic signs or symptoms at presentation or in the past. He also had no documented hyponatremia or dialysis disequilibrium. However, as seen in [Table 1], his blood urea levels were high (probably due to inadequate dialysis in the past), which could have led to osmotic imbalance.
Table 1: Relevant laboratory investigations at the time of evaluation.

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There are very few case reports of asymptomatic CPM/EPM and to our knowledge, none in CKD-ESRD patients. Although our patient was asymptomatic, this case highlights the importance of slow correction of uremia as well as hyponatremia when we dialyze a patient with ESRD.

Conflict of interest: None declared.

 
   References Top

1.
Martin RJ. Central pontine and extra-pontine myelinolysis: The osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry 2004;75 Suppl 3:iii22-8.  Back to cited text no. 1
    
2.
Girmenia F, Colosimo C, Di Biasi C, Faroni J, Pozzilli C. Central pontine myelinolysis: Report of an asymptomatic case. Ann Ital Med Int 1995;10:53-4.  Back to cited text no. 2
    
3.
Strub MU, Steck AJ, Fuhr P. Asymptomatic central pontine myelinolysis. Neurology 1999; 53:914.  Back to cited text no. 3
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4.
Fryer JP, Fortier MV, Metrakos P, et al. Central pontine myelinolysis and cyclosporine neurotoxicity following liver transplantation. Transplantation 1996;61:658-61.  Back to cited text no. 4
    
5.
Lupato A, Fazio P, Fainardi E, Cesnik E, Casetta I, Granieri E. A case of asymptomatic pontine myelinolysis. Neurol Sci 2010;31:361-4.  Back to cited text no. 5
    
6.
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. 6
    
7.
Tarhan NC, Agildere AM, Benli US, Ozdemir FN, Aytekin C, Can U. Osmotic demyelination syndrome in end-stage renal disease after recent hemodialysis: MRI of the brain. Am J Roentgenol 2004;182:809-16.  Back to cited text no. 7
    

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Correspondence Address:
Soumita Bagchi
Department of Nephrology, All India Institute of Medical Sciences, New Delhi
India
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DOI: 10.4103/1319-2442.178578

PMID: 26997398

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