Reversible posterior leukoencephalopathy syndrome due to adrenal pheochromocytoma

Abstract

Rationale: Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare neuropathic syndrome with typical clinical and radiological features. There are large amounts of risk factors resulting in RPLS, those including hypertension, eclampsia, neoplasia treatment, renal failure, systemic infections, chemotherapy, and immunosuppressive therapy after organ transplantation. Patient Concerns: A 27-year-old male patient was admitted for a 2-week history of paroxysmal tic of limbs along with consciousness disorder. Blood pressure elevation was discovered for the first time on admission, and the highest record was 210/150 mmHg during hospitalization. Neurological examinations were positive among mental state, speech, reaction and pathological reflex. The computed tomography scan of the abdomen demonstrated a mass derived from right adrenal gland. The magnetic resonance imaging of the brain showed reversible lesions in the centrum ovale, paraventricular, area and corpus callosum. Diagnoses: After control of blood pressure and rationally preoperative preparation, the mass was radically resected and verified to be pheochromocytoma by postoperative pathologic findings. He was diagnosed as having RPLS due to adrenal pheochromocytoma. Interventions: The right adrenal gland mass was completely removed after 2 weeks of α-blockers and β-blockers to treat hypertension. Outcomes: One week after surgery, the cerebral lesions of RPLS gradually faded and the blood pressure was easy to control well. Lessons: A few case reports of RPLS related to pheochromocytomas had been documented in the literature. Therefore, we believe that pheochromocytomas may be a potential risk factor of RPLS. If patients receive timely diagnosis and treatment, it can often lead to a favorable prognosis.

Pheochromocytoma manifesting as cortical blindness secondary to PRES with associated TMA: a case report and literature review

“…Han et al presented a case of pheochromocytoma complicated by PRES in a 27-year-old male with seizures and consciousness disorder. They also noted two additional cases, one of which occurred in a pediatric patient whose initial symptoms included blurred vision and right sided paresthesias [ 29 ]. Although these cases vary slightly in presentation, especially on neurologic examination, the common link between them is the emergence of PRES preceded by an abrupt change in blood pressure to significant levels.…”

“…Although these cases vary slightly in presentation, especially on neurologic examination, the common link between them is the emergence of PRES preceded by an abrupt change in blood pressure to significant levels. The clinical presentation of PRES includes palpitations, anxiety, headaches, fatigue, seizures, encephalopathy, and visual disturbances [ 29 , 30 ]. Characteristic radiographic findings depict cerebral vasogenic edema in the subcortical white and gray matter of the bilateral posterior parieto-occipital regions [ 29 , 31 ].…”

“…The clinical presentation of PRES includes palpitations, anxiety, headaches, fatigue, seizures, encephalopathy, and visual disturbances [ 29 , 30 ]. Characteristic radiographic findings depict cerebral vasogenic edema in the subcortical white and gray matter of the bilateral posterior parieto-occipital regions [ 29 , 31 ]. MRI typically demonstrates T1 hypo- or iso- intensities, T2 & FLAIR hyperintensities in the parietal and occipital lobes, and infrequently in the cerebellum, basal ganglia, spinal cord, brain stem, centrum ovale, corpus callosum, and paraventricular area [ 29 ].…”

“…Two hypotheses have been suggested regarding the pathogenesis of PRES. One theory, “cerebral hyperperfusion,” proposes a perturbation of cerebral autoregulation triggered by severe blood pressure elevation leading to breakdown of the blood–brain-barrier (BBB) and increased permeability, the infiltration of plasma and macromolecules into brain cells, and subsequent vasogenic edema [ 29 , 31 ]. The second theory, “the toxic/immunogenic theory,” suggests that exogenous (ex.…”