Fatal atypical reversible posterior leukoencephalopathy syndrome: a case report

Journal of Medical Case Reports

Christoph Kleinschnitz²  Nico Melzer¹  Laszlo Solymosi⁴  Ansgar Schütz⁴  Camelia-Maria Monoranu³  Carsten Wessig²  Stefanie Kristin Golombeck¹ 

[1] Current address: Department of Neurology, University of Muenster, Albert-Schweitzer-Campus 1, Muenster, 48149, Germany;Department of Neurology, University of Wuerzburg, Josef-Schneider-Straße 11, Wuerzburg, 97080, Germany;Institute of Neuropathology, University of Wuerzburg, Josef-Schneider-Straße 2, Wuerzburg, 97080, Germany;Department of Neuroradiology, University of Wuerzburg, Josef-Schneider-Straße 11, Wuerzburg, 97080, Germany

关键词: Reversible posterior leukoencephalopathy syndrome;    Generalized cerebral edema;    Cerebral autoregulation;    Blood pressure;   

Others  :  1195139
DOI  :  10.1186/1752-1947-7-14

 received in 2012-07-17, accepted in 2012-11-08,  发布年份 2013

【 摘 要 】

Introduction

Reversible posterior leukoencephalopathy syndrome – a reversible subacute global encephalopathy clinically presenting with headache, altered mental status, visual symptoms such as hemianopsia or cortical blindness, motor symptoms, and focal or generalized seizures – is characterized by a subcortical vasogenic edema symmetrically affecting posterior brain regions. Complete reversibility of both clinical signs and magnetic resonance imaging lesions is regarded as a defining feature of reversible posterior leukoencephalopathy syndrome. Reversible posterior leukoencephalopathy syndrome is almost exclusively seen in the setting of a predisposing clinical condition, such as pre-eclampsia, systemic infections, sepsis and shock, certain autoimmune diseases, various malignancies and cytotoxic chemotherapy, transplantation and concomitant immunosuppression (especially with calcineurin inhibitors) as well as episodes of abrupt hypertension. We describe for the first time clinical, radiological and histological findings in a case of reversible posterior leukoencephalopathy syndrome with an irreversible and fatal outcome occurring in the absence of any of the known predisposing clinical conditions except for a hypertensive episode.

Case presentation

A 58-year-old Caucasian woman presented with a two-week history of subacute and progressive occipital headache, blurred vision and imbalance of gait and with no evidence for raised arterial blood pressure during the two weeks previous to admission. Her past medical history was unremarkable except for controlled arterial hypertension. Cerebral magnetic resonance imaging demonstrated cortical and subcortical lesions with combined vasogenic and cytotoxic edema atypical for both venous congestion and arterial infarction. Routine laboratory and cerebrospinal fluid parameters were normal. The diagnosis of reversible posterior leukoencephalopathy syndrome was established.

Within hours after admission the patient showed a rapidly decreasing level of consciousness, extension and flexion synergisms, bilaterally extensor plantar responses and rapid cardiopulmonary decompensation requiring ventilatory and cardiocirculatory support. Follow-up cerebral imaging demonstrated widespread and confluent cytotoxic edematous lesions in different arterial territories, global cerebral swelling, and subsequent upper and lower brainstem herniation. Four days after admission, the patient was declared dead because of brain death.

Conclusion

This case demonstrates that fulminant and fatal reversible posterior leukoencephalopathy syndrome may occur spontaneously, that is, in the absence of any of the known predisposing systemic conditions.

【 授权许可】

   
2013 Golombeck et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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