Objective: To describe the unusual clinical manifestations in a patient with posterior reversible encephalopathy syndrome.

Background: Posterior reversible encephalopathy syndrome (PRES) is a transient clinical and neuroradiologic syndrome caused by cerebral vasogenic edema. The major symptoms are headache, seizures, visual disturbance and encephalopathy. Movement disorders associated with PRES are exceptionally rare.

Method: Clinical observation and image examination in a patient with posterior reversible encephalopathy syndrome.

Results: The 84-year-old female suffered from acute onset consciousness change associated with high blood pressure, left upper limb involuntary grasping following by right hemiplegia and seizure attack after receiving vertebroplasty for T9 fracture. Brain magnetic resonance imaging (MRI) revealed left frontal subdural hemorrhage and multiple T2-weighted image/fluid attenuated inversion recovery high signal intensities in bilateral cerebral and cerbellar hemispheres, right side basal ganglia to corona radiata and brain stem, with gyral swelling in bilateral parieto-occipital lobes without obvious restricted diffusion [figure1]. Electroencephalography showed diffuse background slowing without epileptiform discharges. Her blood pressure fluctuated during hospitalization and hypomagnesemia was found. Autoimmune markers revealed low C3 and high anti-SSA, so hydroxychloroquine sulfate was prescribed. With strict blood pressure control and correction of hypomagnesemia, she gradually regained consciousness. She could say simple sentences and obey simple orders, but right dense hemiplegia persisted. Follow-up MRI on the 11th day after symptom onset showed new left frontal, cerebellum and bilateral parieto-occipital lobes ischemic lesions [figure2, 3]. Left upper limb involuntary grasping spontaneously improved and right basal ganglionic lesion resolved [figure3].

Conclusion: To our knowledge, acute unilateral involuntary movement as the presenting symptom of PRES has not been reported so far. Hypertensive crisis, hypomagnesia and autoimmune disorder could be the etiologies in this patient. Fluctuation of blood pressure may predispose to cerebral ischemia. Strict and careful cerebrovascular autoregulation monitoring may aid in the optimal hemodynamic management.

References: [1]. A reversible posterior leukoencephalopathy syndrome. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin MS, Lamy C, Mas JL, Caplan LR. N Engl J Med. 1996 Feb 22;334(8):494-500. [2]. Posterior Reversible Encephalopathy Syndrome Presenting as Opsoclonus-Myoclonus. Boland T, Strause J, Hu M, Santamaria D, Liang TW, Kremens D, Sergott R, Moussouttas M. Neuroophthalmology. 2012 Oct;36(4):149-152. [3]. Acute transient freezing of gait in a patient with posterior reversible encephalopathy syndrome. Nakajima A, Ueno Y, Shimura H, Kambe T, Nishioka K, Hattori N, Urabe T. BMC Neurology 2013, 13:79 [4]. Posterior reversible encephalopathy syndrome. Fischer M, Schmutzhard E. J Neurol. 2017 Aug;264(8):1608-1616.

« Back to 2019 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/acute-unilateral-involuntary-movement-in-a-patient-with-posterior-reversible-encephalopathy-syndrome/