Indian J Pediatr (March 2015) 82(3):294–295 DOI 10.1007/s12098-014-1516-2


Posterior Reversible Encephalopathy Syndrome due to Ectopic Cushing’s Syndrome: An Uncommon Presentation of a Rare Disease Rajeev Rajkumar Kasaliwal & Harshal Kakade & Sweta Budyal & Anurag Ranjan Lila & Tushar Bandgar & Nalini Shah

Received: 4 January 2014 / Accepted: 12 June 2014 / Published online: 3 July 2014 # Dr. K C Chaudhuri Foundation 2014

To the Editor: Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological entity characterised by headache, nausea, vomiting, seizures, visual disturbances and altered mental status [1, 2]. Only one case of PRES due to Cushing’s syndrome secondary to micronodular adrenocortical hyperplasiahas been reported in English literature [3]. A 13-y-old girl presented to the emergency department with multiple episodes of generalised tonic-clonic convulsions and altered sensorium for one day. She also complained of headache and decreased vision in both the eyes for last three days. Her blood pressure was 180/100 mmHg. Her clinical examination was consistent with Cushing’s syndrome (CS) including mooning of face, facial plethora, excessive acne, hypertrichosis, livid striae on the abdomen, thighs and axillary region. Magnetic resonance imaging (MRI) of brain showed hyper intensity in occipital-parietal region on T2W and FLAIR sequences with absence of restricted diffusion on diffusion weighted imaging (DWI). These changes were consistent with the radiological appearance of PRES. She was treated with three antihypertensive medications to normalise the blood pressure. Repeat MRI brain after 10 d showed complete resolution of changes of PRES. Biochemical evaluation revealed adrenocorticotropin

R. R. Kasaliwal (*) : H. Kakade : S. Budyal : A. R. Lila : T. Bandgar : N. Shah Department of Endocrinology, Seth G. S. Medical College & KEM Hospital, Mumbai 400012, Maharashtra, India e-mail: [email protected]

(ACTH) dependent endogenous hypercortisolism with 8 am serum cortisol of 27.6 μg/dl, basal ACTH of 82 pg/ml, mid night serum cortisol of 17 μg/dl and standard low dose dexamethasone suppression test (LDDST) of 8.4 μg/dl. MRI pituitary did not reveal any evidence of microadenoma. Corticotropin releasing hormone (CRH) stimulated bilateral inferior petrosal sinus sampling (b/l IPSS) was consistent with peripheral source of ACTH secretion. A suspicious nodule of 1 cm size was seen in right lung on computerised tomography (CT). CT guided biopsy of the lung lesion was suggestive of neuroendocrine tumor with strongly positive synaptophysin and chromogranin immunostaining. This led to the final diagnosis of ectopic ACTH syndrome due to bronchial carcinoid. Unfortunately, patient declined surgery and was subsequently lost to follow-up (Fig. 1). To best of our knowledge, this is the first case of PRES due to ectopic CS. Glucocorticoid mediated hypertension might have played a casual role in the occurrence of PRES in CS in our patient [4]. In conclusion, our case highlights the importance of PRES as a rare presenting feature of ectopic ACTH syndrome (EAS) probably caused by more rapidly progressive hypertension (HTN) in EAS.

Indian J Pediatr (March 2015) 82(3):294–295


Fig. 1 a, b MRI brain showing hyper intense lesion in occipitoparietal regionon T2W and FLAIR sequence c Moon face with facial plethora and increased acne d Normal MRI pituitary, e Resolution of edema on followup T2W MRI sequence f CT guided biopsy of suspicious lesion in right middle lobe of lung g, h, i Histopathology of the lesion showing neuroendocrine tumor with positive synaptophysin and chromogranine immunostaining

Conflict of Interest None. Source of Funding None.

References 1. Hinchey J, Chaves C, Appignani BA. Reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334:494–500.

2. Bartynski WS. Posterior reversible encephalopathy syndrome, Part 2: fundamental imaging and clinical features. Am J Neuroradiol. 2008;29:1036–42. 3. Lodish M, Patronas NJ, Stratakis CA. Reversible posterior encephalopathy syndrome associated with micronodular adrenocortical disease and Cushing syndrome. Eur J Pediatr. 2009;169:125–6. 4. Lodish MB, Sinaii N, Patronas N, Batista DL, Keil M, Samuel J, et al. Blood pressure in pediatric patients with Cushing syndrome. J Clin Endocrinol Metab. 2009;94:2002–8.

Posterior reversible encephalopathy syndrome due to ectopic Cushing's syndrome: an uncommon presentation of a rare disease.

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