Acta Neurol Belg DOI 10.1007/s13760-014-0370-3
NEURO-IMAGES
Cerebellar and brainstem variant of posterior reversible encephalopathy syndrome R. Ba˘las¸ a • S. Maier • E. Gliga Baubec Z. Bajko´ • A. Ba˘las¸ a
•
Received: 19 August 2014 / Accepted: 24 September 2014 Ó Belgian Neurological Society 2014
Keywords Posterior reversible encephalopathy syndrome Neuroimaging Cerebellar and brainstem variant of PRES Chronic renal disease Posterior reversible encephalopathy syndrome (PRES) represents a clinical and radiographic finding that comprises many aetiologies. Clinically, it is characterized by headache, impaired variable mental status, visual disturbances and seizures with a reversible course as the main cause is solved. The underlying pathophysiology seems to be a breakdown in cerebral autoregulation and endothelial dysfunction. Neuroimaging is essential for the diagnosis and typically consists of symmetrical white-matter oedema in the posterior regions of the cerebral hemispheres. The diffusion-weighted images (DWI) sequences play an important role in differential diagnosis and prognosis evaluation. Differential diagnosis must be made with illdefined lesions situated in the white matter of the posterior fossa structures of the brain. Isolated posterior fossa lesions are uncommon and represent a variant of PRES. McKinney
R. Ba˘las¸ a S. Maier Z. Bajko´ Department of Neurology, University of Medicine and Pharmacy Taˆrgu Mures¸ , Taˆrgu Mures¸ , Romania R. Ba˘las¸ a (&) Targu Mures¸ County Clinical Emergency Hospital, Neurology Clinic I, Gh. Marinescu 50, 540136 Taˆrgu Mures¸ , Mures¸ , Romania e-mail: [email protected] E. G. Baubec Transilvania Imagistica, Radiology, Taˆrgu Mures¸ , Romania A. Ba˘las¸ a Targu Mures¸ County Clinical Emergency Hospital, Neurosurgery, Taˆrgu Mures¸ , Romania
et al. [1], evaluated fluid attenuated inversion recovery (FLAIR) images of 124 patients with PRES to determine the incidence of this uncommon variant, which they refer to as the ‘‘central variant’’; the authors found that 4 % of patients with PRES had magnetic resonance imaging (MRI) findings consistent with the central variant that is, either brainstem, cerebellum or basal ganglia involvement and a lack of cortical or subcortical oedema of the cerebrum. The imagistic hallmark of PRES is reversibility once aetiological factors have been controlled [1–5]. We present a rare case of cerebellar and brainstem variant of PRES as the inaugural manifestation of an end-stage chronic renal disease. A 42-year-old male patient with no significant past medical history was admitted to the emergency department with complains of occipital headache, nausea, vomiting and dysequilibrium. The clinical examination was relevant only for elevated blood pressure values (190/110 mmHg). The neurological examination revealed globally brisk deep-tendon reflexes, ataxic gait and mild bilateral dysmetria. Upon admission, laboratory analysis revealed elevated blood urea nitrogen (194.7 mg/dl) and elevated serum creatinine 9.68 mg/dl. Autoimmune and infectious markers were negative. Cerebral computer tomography (CT) showed multiple white-matter hypodensities in both cerebellar hemispheres and the brainstem (Fig. 1). MRI showed confluent areas of hypersignal on T2 and FLAIR-weighted images situated in the cerebellar white matter as well as in the pons and midbrain (Fig. 2). Supratentorially, MRI revealed lacunar infarctions and leukoaraiosis (Fig. 2). No contrast was administered due to renal involvement. DWI showed slight hypointensity corresponding to the hyperintense lesions seen on T2 and FLAIR sequences.
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Acta Neurol Belg
Fig. 1 Axial cerebral CT scan revealing diffuse hypointense lesions in both cerebellar hemispheres (a–c) and brainstem (d–f)
Fig. 2 Axial T2 FLAIR-weighted cerebral MRI showing multiple hyperintense lesions in both cerebellar hemispheres and brainstem (a–e) and multiple microvascular lesions (lacunar infarctions and leukoaraiosis) in both cerebral hemispheres (f)
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Fig. 3 Control Axial T2 FLAIR-weighted cerebral MRI images, 3 weeks after the first examination showing the disappearance of the hyperintense lesions from the cerebellar hemispheres and brainstem (a–e) and the persistence of the cerebral microvascular lesions (f)
The nephrological consultation established the diagnosis of end-stage renal disease. During the 14 days hospitalization, the patient underwent nine haemodialysis sessions, in the course of which renal tests improved and subsequently the patient presented a favourable neurological evolution. The repeated MRI showed a normalization of the brainstem and cerebellar signals on T2 and FLAIR sequences (Fig. 3). As a result, this patient was diagnosed as PRES because of rapid clinical and radiological recovery. Neurologists should be aware that the atypical imaging findings in PRES might mimic a vast range of pathologic processes. The recognition of atypical variants of PRES is important for appropriate management of these patients in a timely manner, avoiding other unnecessary invasive tests like brain biopsy, preventing severe complications like obstructive hydrocephalus. Conflict of interest
References 1. McKinney AM, Jagadeesan B, Truwit CL (2013) Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema. Am J Roentgenol 201:631–638 2. Kitaguchi H, Tomimoto H, Miki Y et al (2005) A brainstem variant of reversible posterior leukoencephalopathy syndrome. Neuroradiology 47:652–656 3. Karakis I, MacDonald JA, Stefanidou M et al (2009) Clinical and radiological features of brainstem variant of hypertensive encephalopathy. J Vasc Interv Neurol 2:172–176 4. Fugate JE, Claassen DO, Cloft HJ et al (2010) Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc 85:427–432 5. Kute VB, Trivedi HL, Shah PR et al (2013) Posterior reversible encephalopathy syndrome—an under recognized manifestation of chronic kidney disease. Indian J Crit. Med 17:318–320
Authors have nothing to declare.
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NEURO-IMAGES
Cerebellar and brainstem variant of posterior reversible encephalopathy syndrome R. Ba˘las¸ a • S. Maier • E. Gliga Baubec Z. Bajko´ • A. Ba˘las¸ a
•
Received: 19 August 2014 / Accepted: 24 September 2014 Ó Belgian Neurological Society 2014
Keywords Posterior reversible encephalopathy syndrome Neuroimaging Cerebellar and brainstem variant of PRES Chronic renal disease Posterior reversible encephalopathy syndrome (PRES) represents a clinical and radiographic finding that comprises many aetiologies. Clinically, it is characterized by headache, impaired variable mental status, visual disturbances and seizures with a reversible course as the main cause is solved. The underlying pathophysiology seems to be a breakdown in cerebral autoregulation and endothelial dysfunction. Neuroimaging is essential for the diagnosis and typically consists of symmetrical white-matter oedema in the posterior regions of the cerebral hemispheres. The diffusion-weighted images (DWI) sequences play an important role in differential diagnosis and prognosis evaluation. Differential diagnosis must be made with illdefined lesions situated in the white matter of the posterior fossa structures of the brain. Isolated posterior fossa lesions are uncommon and represent a variant of PRES. McKinney
R. Ba˘las¸ a S. Maier Z. Bajko´ Department of Neurology, University of Medicine and Pharmacy Taˆrgu Mures¸ , Taˆrgu Mures¸ , Romania R. Ba˘las¸ a (&) Targu Mures¸ County Clinical Emergency Hospital, Neurology Clinic I, Gh. Marinescu 50, 540136 Taˆrgu Mures¸ , Mures¸ , Romania e-mail: [email protected] E. G. Baubec Transilvania Imagistica, Radiology, Taˆrgu Mures¸ , Romania A. Ba˘las¸ a Targu Mures¸ County Clinical Emergency Hospital, Neurosurgery, Taˆrgu Mures¸ , Romania
et al. [1], evaluated fluid attenuated inversion recovery (FLAIR) images of 124 patients with PRES to determine the incidence of this uncommon variant, which they refer to as the ‘‘central variant’’; the authors found that 4 % of patients with PRES had magnetic resonance imaging (MRI) findings consistent with the central variant that is, either brainstem, cerebellum or basal ganglia involvement and a lack of cortical or subcortical oedema of the cerebrum. The imagistic hallmark of PRES is reversibility once aetiological factors have been controlled [1–5]. We present a rare case of cerebellar and brainstem variant of PRES as the inaugural manifestation of an end-stage chronic renal disease. A 42-year-old male patient with no significant past medical history was admitted to the emergency department with complains of occipital headache, nausea, vomiting and dysequilibrium. The clinical examination was relevant only for elevated blood pressure values (190/110 mmHg). The neurological examination revealed globally brisk deep-tendon reflexes, ataxic gait and mild bilateral dysmetria. Upon admission, laboratory analysis revealed elevated blood urea nitrogen (194.7 mg/dl) and elevated serum creatinine 9.68 mg/dl. Autoimmune and infectious markers were negative. Cerebral computer tomography (CT) showed multiple white-matter hypodensities in both cerebellar hemispheres and the brainstem (Fig. 1). MRI showed confluent areas of hypersignal on T2 and FLAIR-weighted images situated in the cerebellar white matter as well as in the pons and midbrain (Fig. 2). Supratentorially, MRI revealed lacunar infarctions and leukoaraiosis (Fig. 2). No contrast was administered due to renal involvement. DWI showed slight hypointensity corresponding to the hyperintense lesions seen on T2 and FLAIR sequences.
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Acta Neurol Belg
Fig. 1 Axial cerebral CT scan revealing diffuse hypointense lesions in both cerebellar hemispheres (a–c) and brainstem (d–f)
Fig. 2 Axial T2 FLAIR-weighted cerebral MRI showing multiple hyperintense lesions in both cerebellar hemispheres and brainstem (a–e) and multiple microvascular lesions (lacunar infarctions and leukoaraiosis) in both cerebral hemispheres (f)
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Acta Neurol Belg
Fig. 3 Control Axial T2 FLAIR-weighted cerebral MRI images, 3 weeks after the first examination showing the disappearance of the hyperintense lesions from the cerebellar hemispheres and brainstem (a–e) and the persistence of the cerebral microvascular lesions (f)
The nephrological consultation established the diagnosis of end-stage renal disease. During the 14 days hospitalization, the patient underwent nine haemodialysis sessions, in the course of which renal tests improved and subsequently the patient presented a favourable neurological evolution. The repeated MRI showed a normalization of the brainstem and cerebellar signals on T2 and FLAIR sequences (Fig. 3). As a result, this patient was diagnosed as PRES because of rapid clinical and radiological recovery. Neurologists should be aware that the atypical imaging findings in PRES might mimic a vast range of pathologic processes. The recognition of atypical variants of PRES is important for appropriate management of these patients in a timely manner, avoiding other unnecessary invasive tests like brain biopsy, preventing severe complications like obstructive hydrocephalus. Conflict of interest
References 1. McKinney AM, Jagadeesan B, Truwit CL (2013) Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema. Am J Roentgenol 201:631–638 2. Kitaguchi H, Tomimoto H, Miki Y et al (2005) A brainstem variant of reversible posterior leukoencephalopathy syndrome. Neuroradiology 47:652–656 3. Karakis I, MacDonald JA, Stefanidou M et al (2009) Clinical and radiological features of brainstem variant of hypertensive encephalopathy. J Vasc Interv Neurol 2:172–176 4. Fugate JE, Claassen DO, Cloft HJ et al (2010) Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc 85:427–432 5. Kute VB, Trivedi HL, Shah PR et al (2013) Posterior reversible encephalopathy syndrome—an under recognized manifestation of chronic kidney disease. Indian J Crit. Med 17:318–320
Authors have nothing to declare.
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