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Case report

Title: An uncommon cause of posterior reversible encephalopathy

syndromeAbstract: We report a case of 14 years female presented to us with new onset severe

hypertension and recurrent seizure episode. MRI brain was consistent with changes of

posterior reversible encephalopathy syndrome (PRES). She was treated with

antihypertensive drugs and responded well to treatment. On evaluation, she was diagnosed

to have ACTH dependent Cushing syndrome (CS). MRI pituitary did not show any evidence

of microadenomasbut there was complete resolution of changes of PRES on MRI brain. CT

chest showed a nodule in the middle lobe of left lung. CT guided biopsy was suggestive of

neuroendocrine tumour. This is the first case described in the literature of a patient with

ectopic ACTH syndrome (EAS).

Introduction:

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Case report:

A 14 year old female presented to emergency department with history of repeated episode of

generalised convulsion since last one day. She also gave history moderate to severe headache

and decreased vision since last four days. On examination she was found to have blood

pressure of 220 /110 mm Hg. She was subjected to MRI brain which showed changes

consistent with posterior reversible encephalopathy syndrome (PRES). She was started on

multiple antihypertensive medications for control of blood pressure. After adequate control of

blood pressure, headache decreased and she did not have any further episodes of seizure.

Vision improved completely after three days.

After stabilisation from acute illness, she was evaluated for cause of young onset

hypertension. She gave history of progressive weight gain and growth failure since last one

and half year. She also gave history suggestive proximal muscle weakness since last three

months. On examination, she had mooning, facial plethora, increased acne, hypertrichosis,dorsoscapular fat deposition, central obesity and knuckle and nail hyperpigmentation. But she

did not have catabolic signs like striae, easy brusability and proximal muscle weakness.

During hospital stay, she developed abdominal distension and severe constipation. She was

diagnosed to have paralytic ileus with severe hypokalaemia and metabolic alkalosis with

potassium of 1.4 meq/l and bicarbonate of 34meq/l. Her biochemical evaluation was

suggestive of ACTH dependent endogenous hypercortisolism with basal cortisol of 27 g/dl,

B.ACTH of 222 pg/ml, MN cortisol of 22.4 g/dl and LDDS of 8.6 g/dl. MRI pituitary and

brain showed complete resolution of changes of PRES but did not reveal any evidence of

pituitary microadenoma. EAS was suspected due to prominence of metabolic complications

and normal MRI pituitary. Corticotropin stimulated bilateral inferior petrosal sinus sampling

(BIPSS) was suggestive of peripheral source of ACTH secretion. Computer tomography

(CT) of chest showed a suspicious nodule in the right middle lobe of lung. CT guided biopsy

of this suspicious nodule revealed neuroendocrine tumour strongly positive for syneptophysin

and chromogranin. It also stained strongly for ACTH. She was finally diagnosed to have

bronchial carcinoid with EAS.

Discussion:

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Figrue 1: (A) Moon face with severe acne (B) Axial fluid-attenuated inversion recovery magnetic resonance

images showed moderate vasogenic edema (C) restricted diffusion on DWI mages (E) CT chest showed a

nodule in right middle lobe of lung (E) CT guided biopsy of suspected nodule

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