Posterior reversible encephalopathy syndrome with essential thrombocythemia

Abstract

Rationale: Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disease of the posterior subcortical white matter that manifests as headache, seizures, visual impairment, disturbance of consciousness, and changes in mental state. While PRES is associated with specific imaging findings involving the posterior circulation area of the brain. In the present study, we report the first case of PRES associated with essential thrombocythemia (ET). Patient concerns: A 49-year-old man suddenly experienced headache, followed by the gradual appearance of consciousness disorders and mental behavior abnormalities. Neurological tests showed that the patient had a Glasgow Coma Scale score of 12, normal muscle strength and tension of the limbs, and was negative for meningeal irritation. Diagnosis: Magnetic resonance imaging of the brain showed extensive vasogenic edema in the deep white matter of the right cerebellum and the left occipital and temporal lobes and a diagnosis of PRES was considered. Routine blood test showed that his platelet count was markedly increased, and the JAK2 V617F mutation analysis with allele-specific real-time polymerase chain reaction was positive. The bone marrow biopsy indicated an increasing number of megakaryocytes. These findings indicated ET. Interventions: PRES was treated with a dehydrating agent and supportive and symptomatic treatments. Aspirin tablets were prescribed to address the patient's ET. Outcome: After treatment, the abnormal findings on head imaging were completely reversed. His neurological symptoms were completely relieved. Lessons: PRES may be correlated with ET; specifically, ET may trigger PRES and be a risk factor for the acute onset of neurological deficits.

Case Report: PRES-Like Syndrome With Reversible Cortical Blindness Associated With Essential Thrombocythemia

“…To our knowledge, this report is the second to document a case of PRES and ET (5). Brain MRI in our patient showed restricted diffusion in DWI and reduced ADC indicating cytotoxic edema, which is consistent with ischemic infarction and different from vasogenic edema of typical PRES.…”

“…There are few reports of PRES combined with ET, so the relationship between PRES and ET is not well-understood. Previous studies suggested that endothelial dysfunction may be involved in the mechanism of PRES in ET patients (3,5). In this patient, the residual contrast agent was still found in the head CT after 3 h of CTA and MRV excluded venous sinus thrombosis, which suggested that there might be venous stasis caused by ET.…”

Erythropoietin-Associated Posterior Reversible Encephalopathy Syndrome

“…While prior reports have discussed the development of PRES in patients with end-stage renal failure receiving ESAs for chronic anemia,12 our patient is unusual in that he had anemia secondary to a viral etiology. PRES is usually associated with malignant hypertension, eclampsia, severe kidney disease, chemotherapy, autoimmune disorders, and immunosuppressive therapy, but has also been reported with other blood cell disorders and treatments, such as essential thrombocythemia, acute intermittent porphyria, and blood transfusion 13–17. PRES, previously known as posterior leukoencephalopathy syndrome, was first described in 1996 by Hinchey et al16 and typically presents with headache, altered mental status, seizures, and visual loss 17–19.…”

“…PRES is usually associated with malignant hypertension, eclampsia, severe kidney disease, chemotherapy, autoimmune disorders, and immunosuppressive therapy, but has also been reported with other blood cell disorders and treatments, such as essential thrombocythemia, acute intermittent porphyria, and blood transfusion. [13][14][15][16][17] PRES, previously known as posterior leukoencephalopathy syndrome, was first described in 1996 by Hinchey et al 16 and typically presents with headache, altered mental status, seizures, and visual loss. [17][18][19] Characteristic neuroimaging findings include bilateral, symmetric edema most notable in the white matter of the parieto-occipital regions with sparing of the calcarine and paramedian occipital-lobe structures, differentiating PRES from bilateral infarction of the posterior cerebral arteries.…”

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

“…201 Hematologic malignancies 18,25,39,[202][203][204][205] which may cause PRES in pediatric patients include acute lymphoblastic leukemia (ALL), 206 acute myeloblastic leukemia, 18 non-Hodgkin's lymphoma, 65 diffuse large B-cell lymphoma, 207 Burkitt's lymphoma, 39 anaplastic large cell lymphoma, 205 and hemophagocytic lymphohistiocytosis. 202 Other causes of PRES include, though not limited to, nonmalignant hematologic disease, 22,40 sickle cell anemia, 33 thalassemia, 22,208,209 essential thrombocythemia, 210 thrombotic thrombocytopenic purpura, 40 typical 211 and atypical 212 hemolytic uremic syndrome, blood transfusions, 32 infection, 38 connective tissue disorders and autoimmune disease, 32 pancreatitis, 213 electrolyte disturbances (e.g., hypomagnesemia, hypocalcemia, and hypercalcemia), licorice, 214 head trauma, 215 and eclamptic cerebrovascular dysautoregulation. 4,5, 22,34,49,52,54,58,113,165,195,[216][217][218][219][220]…”