|IMAGES IN NEPHROLOGY
|Year : 2007 | Volume
| Issue : 4 | Page : 185-186
Reversible cause of blindness in end stage renal disease on dialysis
M Singh Jhawar1, P Pawar1, U George2, P George1, B Pawar3, Y Singh4
1 Department of Internal Medicine, Christian Medical College and Hospital, Ludhiana - 141 008, India
2 Department of Radiology, Christian Medical College and Hospital, Ludhiana - 141 008, India
3 Department of Nephrology, Christian Medical College and Hospital, Ludhiana - 141 008, India
4 Department of Neurology, Christian Medical College and Hospital, Ludhiana - 141 008, India
Department of Internal Medicine, Christian Medical College and Hospital, Brown Road, Ludhiana - 141 008, Punjab
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jhawar M S, Pawar P, George U, George P, Pawar B, Singh Y. Reversible cause of blindness in end stage renal disease on dialysis. Indian J Nephrol 2007;17:185-6
A ten year-old child with end stage renal disease on maintenance hemodialysis was admitted with pneumonia and sepsis. He improved after treatment with antibiotics. A week later, he had generalized tonic clonic seizures and myoclonic jerks. Weakness (MRC grade 4/5) with decrease in tone in all muscle groups of upper and lower limb of the right side of the body was noticed. Deep tendon reflexes were absent and extensor plantar response was seen on the right side. He was slow, confused and complained of loss of vision in both eyes. Pupils were bilaterally equal and reacting to light. The blood pressure was 150/110 mm Hg. Electroencephalogram showed generalized epileptiform abnormalities. Cerebrospinal fluid examination was normal. A day after the onset of these symptoms, his blood pressure increased to 200/120 mm Hg. Intravenous glyceryl trinitrate infusion was initiated and antihypertensive medications optimized for adequate control. Daily hemodialysis was done for five days.
Magnetic resonance imaging of the brain showed bilateral cortical and subcortical hyperintensities involving the occipital and the parietal lobes on T2 and T2 flair images [Figure - 1] and corresponding hypointense foci on T1 weighted images [Figure - 2].
No contrast enhancement was seen. A diagnosis of reversible posterior leukoencephalopathy (RPLE) was made.
Over a period of 48 hours, his motor power improved to normal. His vision started improving first in the left and then six hours later, in the right eye. Within two weeks, his vision improved to finger counting at three meters.
Follow-up magnetic resonance imaging (MRI) six weeks later showed complete resolution with no residual signal abnormality [Figure - 3]. A concurrent neurological examination was also normal. The reversibility of neurological and radiological features confirmed RPLE.
| Discussion|| |
RPLE is a relatively recent clinical entity.  This clinical and radiological syndrome has been associated with hypertension, preeclampsia, renal failure, immunosupression, cytotoxic drugs, connective tissue disorders, thrombotic thrombocytopenic purpura, porphyria and organ transplantation.  Pathogenesis related to brain capillary leakage and disruption of the blood brain barrier has been considered to be a result of these factors. 
Seizures are amongst the first clinical manifestations. The patient may have headache, confusion and visual disturbance ranging from hemianopia, visual neglect, cortical blindness and Anton's syndrome.  Fundus and pupillary reflex are usually normal  and a few patients may have weakness and incoordination of limbs. 
A rapid increase of blood pressure from the baseline may be responsible for RPLE, especially in young and pregnant patients with normally low baseline recordings.  Autoregulation of the cerebral vasculature may be overwhelmed by the sudden rise in blood pressure resulting in vasodilatation and resultant vasogenic edema. 
Neuroimaging with computerized tomography (CT) scan and MRI may be used with demonstrable benefit of MRI in picking smaller lesions.  Images show predominant white matter abnormalities appearing as low attenuation areas on the CT scan and hypointense lesions on T1 and hyperintense lesions on T2 weighted images.  A similar pattern was demonstrated in our patient on MRI. RPLE predominantly affects cerebral white matter localized to posterior parietal and occipital lobes and may spread to the basal ganglia, brain stem and cerebellum.  The calcarine and paramedian occipital lobes are spared in RPLE differentiating it from bilateral posterior cerebral artery infarcts.  MRI images suggest vasogenic edema and pathological correlation with brain biopsy in a case report showed edematous white matter with no vessel wall injury. 
End stage renal disease with accelerated hypertension resulted in this syndrome of neurological and radiological findings, which was reversed within a week of aggressive blood pressure control and daily hemodialysis in this patient. RPLE is a retrospective diagnosis based on clinical and radiological findings that resolve completely.  However, recovery may not be complete if treatment is delayed. 
Effective treatment with blood pressure control and reduction/withdrawal of offending medications is crucial.  Long-term anti epileptic medication is not recommended as the condition is reversible in most patients. 
The initial case series of fifteen patients included four patients of chronic kidney disease and eight with renal impairment.  Early recognition and treatment of RPLE is associated with gratifying outcomes.
| Acknowledgment|| |
We acknowledge the role of Vinay Wilson for photography and technical assistance.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]