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| Year : 2001 | Volume
: 49
| Issue : 3 | Page : 311-3 |
Reversible posterior leukoencephalopathy syndrome : a report of 2 cases.
Arora A, Chowdhury D, Daga MK, Arora N, Gaiha M
Department of Neurology, G.B. Pant Hospital, New Delhi-110019, India.
Correspondence Address:
Department of Neurology, G.B. Pant Hospital, New Delhi-110019, India.
Reversible posterior leukoencephalopathy syndrome (RPLE) is an increasingly recognised disorder, most commonly associated with malignant hypertension, toxaemia of pregnancy or the use of immunosuppressive agents. Two cases of RPLE syndrome occurring in the setting of accelerated hypertension and eclampsia are described. Both patients had seizures, altered sensorium and typical findings on neuroimaging. They had complete clinical and radiological recovery. The clinical course, pathophysiology and neuroimaging features of RPLE syndrome are discussed.
How to cite this article:
Arora A, Chowdhury D, Daga M K, Arora N, Gaiha M. Reversible posterior leukoencephalopathy syndrome : a report of 2 cases. Neurol India 2001;49:311 |
Hinchey et al in a retrospective study, noted white matter oedema on neuroimaging in the posterior temporo-parieto-occipital regions in a variety of conditions including severe hypertension.[1] They proposed the name 'reversible posterior leuko encephalopathy' (RPLE), emphasizing its location and relatively reversible nature. Only a few cases of RPLE syndrome have since been reported in English literature. We report two cases of RPLE syndrome which has not been reported from India, till date, to the best of our knowledge.
Case 1 : An 18 year old female, deaf and mute since birth, presented with anasarca and oliguria. Physical examination revealed a normotensive conscious patient with pallor, pedal oedema and free fluid in the abdomen. There was no hepatosplenomegaly. Central nervous system (CNS) examination was normal. Investigations revealed nephrotic range proteinuria (5gm per day) with granular casts in the urine and normal serum biochemistry including haemogram, blood sugar, serum electrolytes, blood urea and serum creatinine. Antinuclear factor was negative. Ultrasound examination revealed free fluid in the abdomen with normal kidney size and echo texture. Subsequently, a kidney biopsy was done. Following the procedure, patient developed accelerated hypertension (BP-200/140 mm Hg) that was followed by status epilepticus. She was managed by injection diazepam and phenytoin for status epilepticus and nitroglycerine infusion for accelerated hypertension. She remained confused after the ictus for next 48 hours. During this period, there was no focal neurological deficit and fundus examination was normal. She remained hypertensive (BP-190/120 mmHg) for the next 72 hours and continued to receive nitroglycerine infusion. CT scan done 2 days after the ictus showed bilateral non-enhancing white matter hypodensities, predominantly in occipital and posterior parietal areas [Figure 1a]. Patient became normotensive after 3 days. By this time, she had become fully conscious and neurological examination did not reveal any fresh deficit. MRI done after 3 weeks showed significant resolution of white matter lesions. A repeat CT scan 6 weeks later showed almost complete resolution [Figure 1b]. Kidney biopsy revealed membrano-proliferative glomerulone-phritis. She was discharged after 8 weeks, on steroids and diuretics. On follow up, her nephrotic syndrome had partially responded (proteinuria of 1 gm per day). There had been no recurrence of hypertension and seizures.
Case 2 : A 28 year old female, primigravida, presented at full term with eclampsia (BP 160/100 mmHg, proteinuria and seizures, which were followed by unconsciousness). The labour was induced and she delivered a stillborn baby. She did not recover consciousness for 48 hours after the ictus, although her BP became normal after delivery. Examination at that time revealed an unconscious patient, responding to painful stimuli, with a pulse rate of 90/minute, BP of 130/80 mmHg and no meningeal signs or focal neurological deficit on CNS examination. She was given injection phenytoin, mannitol and lasix during this period. CT scan (head) 2 days after the ictus revealed bilateral white matter hypodensities in fronto-temporo-parietal regions. Patient regained consciousness after 48 hours and subsequent recovery was uneventful. A repeat CT scan done after 4 weeks showed significant resolution of white matter lesions.
Hinchey et al while drawing attention to a reversible posterior leukoencephalopathy syndrome, stressed hypertensive encephalopathy, eclampsia and immunosuppressive drugs such as cyclosporine as major aetiologies.[1] Most of their patients (n = 11/15) had focal or generalised seizures. Visual abnormalities present in 10 patients, consisted of cortical blindness, homonymous hemianopia and blurred vision. Focal neurological deficits were uncommon. In our patients, we did not find any focal deficits or visual abnormalities, although both the patients had generalised seizures. The first patient had a transient accelerated hypertension following kidney biopsy, which precipitated RPLE. Abrupt hypertension (present in 12/15 cases of Hinchey et al) has been well documented as one of the causes of RPLE syndrome, which from pathophysiological standpoint, can be viewed as a capillary leak syndrome.[1],[2] The second case reported here developed RPLE following eclampsia, which is a well-known and important aetiological factor for this syndrome.[3]
The findings on neuroimaging in RPLE syndrome include non-enhancing white matter abnormalities, that appear as areas of low attenuation on CT scan, and appear hypointense on T1WI, MRI and hyperintense on T2WI. The lesions are seen mainly in the posterior regions of the cerebral hemispheres.[4],[5] These abnormalities partially or completely resolve on follow up scanning, thereby suggesting subcortical oedema without infarction. Both the patients reported here had white matter abnormalities as described above, either on CT scan or MRI.
It is important to note that neuroimaging usually reveals sparing of the calcarine and paramedian occipital lobe structures, a fact that distinguishes RPLE from bilateral infarction of the posterior cerebral artery territory.[1] Although MRI yields higher resolution and may show small, focal abnormalities beyond resolution of CT, it is not mandatory for diagnosis of RPLE.[1] Essentially, the diagnosis of RPLE syndrome is retrospective. Significant reversal of neuroradiological abnormalities coupled with complete clinical recovery suggests the diagnosis.
Sudden elevation of blood pressure disrupts the autoregulatory mechanisms of CNS vasculature, leading to development of areas of vasoconstriction and vasodilatation, breakdown of blood brain barrier and focal transudation of fluid and petechial haemorrhages.[6] Hypertensive encephalopathy and eclampsia share similar pathophysiology.[7] Fluid accumulation during pregnancy or puerperium may accentuate the tendency for brain oedema to occur.[7] The susceptibility of the posterior portion of the brain to lesions seen in RPLE is recognized, but poorly understood.[8] Altered vascular reactivity may result from an increased sensitivity to circulating vasopressors, a deficiency of vasodilating prostaglandins and endothelial cell dysfunction, which causes vasospasm and organ hypoperfusion, activation of the coagulation cascade and fluid exudation.[2] Immunosuppressive agents may lead to RPLE by direct toxic effects on endothelium, vasoconstriction by elaboration of endothelin or production of microthrombi.[9],[10] Subsequently, other investigators have also reported cases of RPLE occurring in conjunction with renal failure, cisplatin, erythropoietin toxicity and polyarteritis nodosa.[11],[12],[13],[14]
Intensive investigations and long term antiepileptic therapy is not warranted as RPLE is essentially reversible.[1] It should also be emphasised that RPLE syndrome may occur in normotensive patients and the oedema may extend to other areas of the brain like
basal ganglia, frontal lobes, cerebellum and brainstem.[3],[4],[15] Occasionally, gray matter may also be involved.[4] Furthermore, the syndrome may not always be truly reversible.[15] As this syndrome occurs in heterogenous settings, all aspects of its aetiopathogenesis have not been elucidated. Newer insight into the pathogenesis of this entity may be forthcoming with increased awareness.
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| 11. | Bakshi R, Bates VE, Mechtlor LL et al : Occipital lobe seizures as the major clinical manifestation of reversible posterior leukoencephalopathy syndrome: magnetic resonance imaging findings. Epilepsia1998; 39 : 295-299. |
| 12. | Ito Y, Arahata Y, Goto Y et al : Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome. Am J Neuroradiol 1998; 19 : 415-417. |
| 13. | Delanty N, Vaughan C, Frucht S et al : Erythropoietin associated hypertensive posterior leukoencephalopathy. Neurology1997; 49 : 686-689. |
| 14. | Arai M, Shigeno K, Wada M : A reversible posterior leukoencephalopathy syndrome in a patient with classical polyarteritis nodosa. Rinsho Shinkeigaku 1997; 37 : 64-66. |
| 15. | Schwartz RB. A RPLE syndrome. N Engl J Med1996; 334 : 1743. |
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