Posterior Reversible Encephalopathy (PRES)

toilet two-sided psyche disorder (PRES)Posterior Reversible Encephalopathy (PRES) A R be Presenting Feature of phaeochromocytomaAbstract high riptide pressure in young is mostly callable to secondary causes and one of them is phaeochromocytoma. These be catecholamine secreting enterochromaffin tumors causing paroxysmal hypertension. Ad nephritic phaeochromocytoma presenting as posterior two-sided brain disease syndrome (PRES) is very r atomic number 18 and has not been exposit in writings so far. Here, we report a case of previously healthy stripling boy, who presented with shrewd flak severe headache, blurring of vision, generalized tonic clonic force back seizure and altered sensorium. His blood pressure was 234/126 mm Hg. The charismatic vibrancy imaging (magnetic resonance imaging) of mind-set showed hyperintense signal on T2- burden and silver r arfy everting recovery (FLAIR) images in bilateral parietal and occipital regions. High blood pressure and classica l MRI findings were consistent with the diagnosis of PRES. Abdominal echography (USG) revealed a objurgate supr atomic number 18nal gland intensity. A diagnosis of pheochromocytoma was substantiate by abdominal triple phase strain- computed imagery (CT) and 24-hour urinary metanephrine assay. later on the blood pressure was stabilized with alpha and beta blockade, adrenal tumor was surgically excised. Histopathologic examination of tissue confirmed the diagnosis of pheochromocytoma. The MRI brain showed complete effect of hyperintense signals (T2-weighted and FLAIR images) on two-months action. He was symptom at large(p) at six months and one year follow-up.Key words Posterior reversible encephalopathy syndrome (PRES) Reversible posterior leukoencephalopathy (RPLE) Pheochromocytoma Hypertensive Encephalopathy HypertensionAbbreviations ADC apparent public exposure coefficient CT computed tomography MRI magnetic resonance imaging DWI diffusion-weighted imaging PRES posterio r reversible encephalopathy syndrome submissionHypertension in young is mostly due to secondary causes which acknowledge renal diseases (chronic renal failure, renal artery stenosis, polycystic kidney disease), coarctation of the aorta, ashesic lupus erythematosus (SLE) and endocrinopathies. Pheochromocytomas are rare catecholamine secreting enterochromaffin tumors. The perseverings of pheochromocytoma usually present with spells of headache, sweating and palpitations due to uppity catecholamines. The rational manifestation of pheochromocytoma are uncommon. Posterior reversible encephalopathy syndrome (PRES) as a presenting feature of pheochromocytoma is rare.Case ReportA 14-year-old previously healthy boy presented in emergency department with subtile onset severe holocranial headache, blurring of vision, generalized tonic clonic motor seizure followed by altered sensorium. There was no previous history of nausea, vomiting, diarrhea, flushing, riotous sweating, migraine, aut oimmune and connective tissue disorders, drug abuse, toxin exposure, hypertension or diabetes mellitus. He had no similar illness in past and family history was negative. His cadence rate and blood pressure were 130 beats per minute and 234/126 mm Hg, respectively. He was confused but followed simple commands. The pupillary size and wanton reaction were normal on both sides. Fundus examination showed bilateral papilledema. He was moving all the four limbs equally without asymmetry on galling stimulus. Plantars were bilaterally extensor. Signs of meningeal irritation (neck rigidity and Kernigs signs) were negative.Complete hemogram, serum electrolyte, renal function tests and other biochemistry including thyroid function tests were normal. Serum anti-nuclear antibodies (ANA), anti-double-stranded DNA antibody (anti-dsDNA) and enzyme-linked-immunosorbent serologic assay test for human immunodeficiency virus (HIV) were negative. X-ray chest was normal. Electrocardiography (ECG) show ed tachycardia. charismatic resonance imaging (MRI) of brain showed hyperintense signal changes on T2-weighted and fluid attenuated inversion recovery (FLAIR) images in bilateral occipito-parietal regions. No restriction was seen on diffusion-weighted images (DWI) Figure 1. CT angiography of brain vessels was normal. High blood pressure and classical MRI findings were consistent with the diagnosis of PRES. On further evaluation, abdominal ultrasonography (USG) showed right adrenal mass. An abdominal triple phase contrast-enhanced CT scan revealed heterogeneous, contrast enhancing adrenal gland mass lesion measuring 32 x 26 mm suggestive of pheochromocytoma Figure 2. The diagnosis was confirmed by 24-hour urinary metanephrine assay. The plasma aldosterone concentration (PAC) and plasma renin activity (PRA) ratio (PAC/PRA) was 10.4. The 24- hour urinary normetanephrine was 31,572 g/24 hour (normal 63-402 g/24 hour), urinary metanephrine was 1,524 g/24 hour (normal 32-167 g/24 hour) an d plasma noradrenaline take was 18,635 pg/mL (normal 0-400 pg/mL).Patient was managed intensively with nitroprusside infusion to reduce blood pressure. injectable phenytoin was administered according to body weight to control seizures. in one case patient was stabilized, he was started on oral alpha-blocker prazosin (20 mg/day) followed by beta-blocker propranolol (40 mg/day). After adequate alpha and beta blockade, patient was think for surgery and resection of adrenal mass was done. Histopathologic examination confirmed the diagnosis of pheochromocytoma without invasion of the adrenal capsule Figure-3. The MRI brain showed complete resolution of hyperintense signals (T2-weighted and FLAIR images) and 24-hour urine metanephrines were normal on two-months follow-up. He was asymptomatic at six months and one year follow-up.DiscussionPheochromocytomas are neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The classical features bid holocranial headache, palpitations, hypertension, hyperhidrosis, hyperglycemia and hypermetabolism are due to excessive catecholamines production in these tumors. The cardiac complications such(prenominal) as arrhythmias, myocardial infarction and sudden deaths are associated with cardiotoxic set up of high blood catecholamines levels. Pheochromocytoma is a rare cause of secondary hypertension and accounts for 0.5% to 2.0% of all causes of hypertension in children. The neurological complications (ischemic or hemorrhagic stroke) are related to change magnitude platelet aggregation, hypertension and vasospasm due to high catecholamines levels. The various potential triggers of pheochromocytoma crisis leading to hemodynamic instability are stress, blood loss, surgery and anesthesia. The diagnosis of pheochromocytoma is confirmed by 24-hour urinary metanephrine and normetanephrine levels.Clinical features of reversible posterior leukoencephalopathy syndrome (PRES) are acute onset headaches, vision loss, seizures and altered sensorium. It is mostly due to hypertension, except other common causes are chronic renal disease, uremic encephalopathy, ergot alkaloids, steroids, chemotherapy, vasculitis and tumors. Pheochromocytoma is one of the rare cause of PRES due to secondary hypertension. However, to the outstrip of our knowledge, acute, stark initial presentation of pheochromocytoma as PRES has not been described in literature. Magnetic resonance images (MRI) of brain exemplaryly shows hyperintense signal changes on T2-weighted and fluid attenuated inversion recovery (FLAIR) images due to vasogenic edema. It mostly involves the cortical and subcortical white matter of the parietooccipital, frontal and temporal regions. Cerebral hemorrhage, cytotoxic edema and contrast enhancement are atypical imaging findings of PRES. The parietooccipital pallium and subcortical white matter were affected in our case. Classically, these signal abnormalities are reversible on antihypertensive therapy.The pathophysiology of PRES is still poorly understood, however, various shot have been proposed. Severe hypertension causes deranged autoregulation of sympathetically mediated cerebral arterioles. It leads to increased permeability in the blood-brain barrier and causes vasogenic edema. Sympathetic innervation of the vertebrobasilar system is not as extensive or as complete as that of the anterior circulation. Therefore, PRES predominantly affects parietooccipital cortex and subcortical white matter. Other affirmable mechanism may be endothelial dysfunction due to go toxins or chemotherapy agents. There may be cerebral infarction or hemorrhage due to compromise of the microcirculation by pressure from surrounding vasogenic edema. The drive etiology of the seizure remains unknown, but may result from effects of the pheochromocytoma on reducing seizure threshold via its actions on metabolic or hypertensive parameters. Our patient had high levels of cir culating catecholamines, produced autonomously by tumor. Once vasogenic edema subsided with antihypertensive therapy, all the abnormal MRI findings vanished.Clinical topography (acute onset headache, visual blurring, seizure and altered sensorium), high blood pressure and typical MRI findings suggested diagnosis of PRES. In our patient, other differential diagnosis such as thrombocytopenic thrombotic purpura (TTP), hemolytic uremic syndrome (HUS), encephalitis, systemic lupus erythematosus (SLE), brain mass lesions and drug toxicity were ruled out by appropriate clinical and laboratory investigations. In our patient, hypertension was detected for the first time on hospital admission and before that he never had any symptoms like headache, palpitations, perspiration or diarrhea. Our patient is of clinical interest as pheochromocytoma presented with life-threatening hypertensive encephalopathy. Management of pheochromocytoma requires aggressive approach including fluid resuscitation a nd antihypertensive therapy (both alpha and beta blockade) followed by surgical resection of tumor.ConclusionPheochromocytomas are catecholamine secreting enterochromaffin tumors causing paroxysmal hypertension. Our patient is of clinical interest as an acute, life-threatening hypertensive encephalopathy (PRES) as a presenting feature of adrenal pheochromocytoma has not been described in literature so far. Pheochromocytoma should be ruled out in every young patient with acute hypertensive encephalopathy.Figure LegendsFigure 1. Magnetic resonance imaging (MRI) of brain showing hyperintense signals on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in parieto-occipital region. No diffusion restriction is seen. T1-weighted axial (A), T2-weighted axial and sagittal (B,C), FLAIR (D), diffusion weighted (DWI) (E) and corresponding apparent diffusion coefficient (ADC) (F) images. These abnormal signals completely vanished on follow-up MRI after six weeks.Figure 2. Tripl e phase contrast-enhanced computed tomography (CT) of abdomen showing heterogenous enhancing mass lesion measuring 32 x 26 mm in right adrenal gland. Axial CT arterial phase (A,B), Venous phase (C) and Delayed phase (D).Figure 3. Hematoxylin and bromeosin (HE) stained microphotographs showing large pleomorphic nuclei, abundant basophilic cytoplasm and cell-nesting pattern (zellballen pattern). HE stain 40x view (A), 100x view (B,C).1