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Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 321-324
Posterior reversible encephalopathy syndrome in a child with pheochromocytoma


Dept. of Radiology and Pediatrics, Amrita Institute of Medical Sciences, Amrita Lane, Elamakkara, Cochin-682026, India

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Keywords: Reversible Posterior Encephalopathy, Pheochromocytoma

How to cite this article:
Moorthy S, Subramaniam T S, Prabhu N K, Sree KK, Nair R G. Posterior reversible encephalopathy syndrome in a child with pheochromocytoma. Indian J Radiol Imaging 2002;12:321-4

How to cite this URL:
Moorthy S, Subramaniam T S, Prabhu N K, Sree KK, Nair R G. Posterior reversible encephalopathy syndrome in a child with pheochromocytoma. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 8];12:321-4. Available from: http://www.ijri.org/text.asp?2002/12/3/321/28470

   Introduction Top


Pheochromocytoma is a rare pediatric tumor. Posterior reversible encephalopathy syndrome (PRES) is a recently recognized syndrome characterised, clinically, by headache, confusion, seizure and visual loss associated with imaging findings of bilateral cortical and subcortical edema. First described in 1996, PRES has been reported to occur in a number of clinical conditions characterised by either an acute elevation of blood pressure or treatment with certain drugs [1],[2],[3]. We report a case of a 9-year-old boy with adrenal pheochromocytoma presenting with PRES. The association has not been reported before, to the best of our knowledge.


   Case report Top


A 9 year old boy born of non-consanguineous marriage was admitted with left sided focal seizures in status epilepticus. There was a past history of recurrent headache with projectile vomiting, excessive sweating and short-tempered behavior of 2 years duration, aggravated since 6 months. The child had been treated for migraine. On clinical examination, the patient was drowsy, incoherent, febrile, tachycardic with elevated blood pressure (146/102 mm Hg). The systemic examination showed no evidence of papilledema, subcutaneous nodules or any neurocutaneous markers. After initial stabilization in the ICU, the child became better oriented but continued to have slurring of speech, blurring of vision and photophobia. The blood pressure continued to be high (178/142 mm Hg). In view of the focal seizures a primary intracranial pathology was suspected and MR imaging was done. The FLAIR images showed focal hyperintense lesions involving the cortex and subcortical white matter in the bilateral parieto-occipital regions. There was also a left frontal lesion which appeared to be limited to the cortex. In addition, FLAIR images showed scattered hyperintense lesions in the bilateral cerebellar hemispheres, which were better delineated on spin echo T2 weighted images. Irregular cortical and subcortical enhancement was noted in the bilateral parieto occipital lesions [Figure - 1]. Infarcts due to vasculitis or encephalitis were considered most likely based on these MR findings.

In view of the elevated blood pressure an ultrasound of the abdomen was done which revealed a right adrenal mass. The 24 hour urinary VMA was 30.4 mg/24 hrs (normal- 1-8mg/24 hrs), the serum noradrenaline- 6611.8 pg/ml (normal -<600pg/ml) and serum adrenaline -72 pg/ml (normal-<100 pg/ml).

Subsequently helical CT of the abdomen showed a 4x5 cm inhomogenously enhancing right adrenal mass with no local infiltration and a normal contralateral adrenal [Figure - 2]. A 131-I metaiodobenzoguanidine (MIBG) scintigraphy showed intense uptake in the right adrenal gland. There was no other site of increased MIBG uptake in the abdomen or thorax. The hypertension was managed with nifedipine, labetolol and phentolamine. After 48 hours he became conscious, cooperative, the speech and vision became normal and the blood pressure settled. He had no obvious neurological deficit and was less diaphoretic with significant improvement of his headache. The MR images were reviewed and a diagnosis of PRES was considered. The patient was subjected to radical right adrenalectomy. The tumor was completely excised. The histopathology of the excised mass showed a well-encapsulated pheochromocytoma with extensive infarction. A repeat urinary VMA estimation on the 7th post-op day showed a fall to normal levels (3.6-mg/24 hrs). The patient was discharged with persistent low-grade hypertension on medication

A plain MRI done 16 weeks after the initial scan documented complete resolution of the supratentorial and infratentorial lesions, diagnostic of PRES [Figure - 3]. The child continued to be asymptomatic.


   Discussion Top


Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumour with a reported incidence of one in 100,000 patient- years. About 10% of these tumours are seen in the pediatric age group. In children, 70% of pheochromocytomas arise in the adrenal gland and the right adrenal is more often involved. Headaches and diaphoresis are the most frequent presenting symptoms. In children, the hypertension is usually sustained. Hypertensive encephalopathy leading to seizure is well known, but uncommon [4],[5]. PRES as a presenting feature of pheochromocytoma has not been reported previously.

PRES is a recently recognized syndrome characterised, clinically, by headache, confusion, seizure and visual loss associated with imaging findings of bilateral cortical and subcortical edema. Both the clinical and imaging findings resolve after appropriate therapy [1]. Hypertensive encephalopathy, toxemia of pregnancy, cyclosporin- A toxicity and ureamic encephalopathy are the most common causes of PRES [6]. Though PRES is, by definition, reversible, failure to correct the underlying hypertension or other causative factor can lead to irreversible neuronal injury [3].

Loss of cerebral autoregulation is considered to be the pathologic mechanism causing PRES. Both hypertension induced uncontrolled vasospasm and autoregulatory failure leading to hyperperfusion are thought to play a role in the pathogenesis [6]. The cortical and subcortical involvement seen in our patient and its predominant parieto occipital distribution is typical of this syndrome. The FLAIR sequence, by suppressing CSF signal, is uniquely suited to demonstrate the characteristic cortical involvement of PRES [6]. Lesions in other parts of the supratentorial brain and infratentorial lesions are also common [6],[7]. While contrast enhancement is not a consistent finding in PRES lesions, it was a prominent feature in our patient. The transient breakdown of the blood brain barrier and leakage of protein, which forms the pathophysiological basis of PRES lesions, can explain contrast enhancement.

Based on our initial evaluation of the MRI, we had given a high probability for infarcts. The diagnosis of PRES became apparent to us only when the patient dramatically improved with anti-hypertensive therapy. Diffusion weighted imaging would mitigate against this error. Studies using diffusion weighted imaging show that the signal abnormality on T2 images reflects vasogenic rather than cytotoxic edema allowing PRES lesions to be differentiated from infarct [8].

PRES has been reported in three children receiving L-asparaginase for myeloproliferative disease. In all three patients, a clinical diagnosis of PRES was made only after the recognition of its typical MR findings [9]. Hypertensive encephalopathy is uncommon in children. The rarity of the condition often makes the recognition of the characteristic findings on MRI essential to differentiate PRES from other conditions like vasculitis and infective encephalitis, which require an entirely different line of management.

 
   References Top

1.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. 1    
2.Truwit CL, Denaro CP, Lake JR, DeMarco T. MR imaging of reversible cyclosporine A-induced neurotoxicity. AJNR AM J Neuroradiol 1991;12:651-659  Back to cited text no. 2    
3.Sengar AR, Gupta RK, Dhanuka AR, Roy R, Das K. MR imaging, MR angiography and MR spectroscopy of the brain in eclampsia. AJNR Am J Neuroradiol 1995;16:1344-1346   Back to cited text no. 3    
4.Chernausek SD. Pheochromocytoma and the multiple endocrine neoplasia syndromes. In: Sperling MA, ed. Pediatric endocrinology. Philadelphia: Saunders WB; 1996; 315-327   Back to cited text no. 4    
5.Lack EE. Tumours of the adrenal gland. In: Fletcher CDM ed. Diagnostic histopathology of tumours. 2nd ed. London: Churchill Livingstone; 2000; 1057-1082  Back to cited text no. 5    
6.Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000;21:1199-1206  Back to cited text no. 6    
7.Schwartz RB, Jones KM, Kalina P et al. Hypertensive encephalopathy: findings on CT, MR imaging and SPECT imaging in 14 cases. AJR Am J Roentgenol 1992;152: 379-383   Back to cited text no. 7    
8.Schaeffer PW, Buonanno FS, Gonzalez RG, Schwamm LH. Diffusion weighted imaging discriminates between cytotoxic and vasogenic edema in a patient with eclampsia. Stroke 1997; 28:1082-1085  Back to cited text no. 8    
9.Cooney JM, Bradley WG, Symko SC, Patel ST, Groncy PK. Hypertensive encephalopathy: Complications in children treated for myeloproliferative disorders-report of three cases. Radiology 2000; 214: 711-716  Back to cited text no. 9    

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Correspondence Address:
S Moorthy
Dept. of Radiology and Pediatrics, Amrita Institute of Medical Sciences, Amrita Lane, Elamakkara, Cochin-682026
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

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