Highlights
- •We describe a cerebellar variant of posterior reversible encephalopathy syndrome.
- •We investigate the clinico-radiological features of such an entity.
- •Prompt recognition and aggressive treatment of the cause is critical.
- •Most patients have a benign course after fast control of blood pressure.
- •We should be aware of the diverse imaging patterns in PRES.
Abstract
Background
Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly
recognized disorder in humans. However, isolated cerebellar involvement in PRES is
extremely uncommon. In this study, we sought to investigate its clinical and radiological
features by describing a cohort of cases with PRES and isolated cerebellar involvement.
Methods
We report 2 patients with PRES with only cerebellar involvement and identified additional
9 cases using the PubMed database with the MeSH terms “posterior reversible encephalopathy
syndrome”, “hypertensive encephalopathy”, “hypertension”, “cerebellum”, “encephalopathy”,
and “magnetic resonance imaging”. We then collectively analyzed the clinical and imaging
characteristics of these 11 cases.
Results
The average age was 28 years, with 8 male and 3 female patients. All cases had severe acute hypertension
and T2 hyperintensity on MRI exclusively centered within the cerebellum. Of 11 patients,
7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive
treatment, and resolution of the cerebellar lesions on follow-up imaging. A total
of 5 of the 11 patients received external ventricular drainage due to obstructive
hydrocephalus and only 2 of the 11 had a seizure.
Conclusions
Isolated cerebellar involvement in PRES may be a unique variant that affects younger,
male cases with severe acute hypertension and hypertensive retinopathy, but not necessarily
seizure. Most patients have full recovery after fast control of blood pressure. Awareness
of atypical neuroimaging features in PRES is critical for appropriate treatment.
Keywords
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References
- A reversible posterior leukoencephalopathy syndrome.N. Engl. J. Med. 1996; 334: 494-500
- Clinical spectrum of reversible posterior leukoencephalopathy syndrome.Arch. Neurol. 2008; 65: 205-210
- Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings.Mayo Clin. Proc. 2010; 85: 427-432
- Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings.AJR Am. J. Roentgenol. 2007; 189: 904-912
- Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema.AJR Am. J. Roentgenol. 2013; 201: 631-638
- Isolated cerebellar involvement in posterior reversible encephalopathy syndrome in a child with acute lymphoblastic leukemia.Indian J. Med. Paediatr. Oncol. 2011; 32: 211-213
- Hypertension-induced cerebellar encephalopathy and hydrocephalus in a male.Pediatr. Neurol. 2006; 34: 72-75
- Isolated cerebellar involvement in a case of posterior reversible leukoencephalopathy.Clin. Radiol. 2006; 61: 983-986
- Reversible hypertensive cerebellar encephalopathy and hydrocephalus.J. Neurol. Neurosurg. Psychiatry. 2007; 78: 1008-1009
- Primary hypertension-induced cerebellar encephalopathy causing obstructive hydrocephalus. Case report.J. Neurosurg. 2003; 98: 1307-1311
- Pediatric posterior reversible encephalopathy syndrome presenting with isolated cerebellar edema and obstructive hydrocephalus.J. Neurosurg. Pediatr. 2014; 14: 344-347
- Isolated cerebellar reversible leukoencephalopathy syndrome in a patient with end stage renal disease.Intern. Med. 2008; 47: 43-45
- Upward transtentorial herniation, hydrocephalus, and cerebellar edema in hypertensive encephalopathy.Neurologist. 2005; 11: 171-175
- Posterior reversible encephalopathy syndrome: long-term follow-up.J. Neurol. Neurosurg. Psychiatry. 2010; 81: 773-777
- Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema.AJNR Am. J. Neuroradiol. 2008; 29: 1043-1049
- Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging.AJNR Am. J. Neuroradiol. 2012; 33: 896-903
- Posterior reversible encephalopathy syndrome in infection, sepsis, and shock.AJNR Am. J. Neuroradiol. 2006; 27: 2179-2190
- Brainstem involvement in hypertensive encephalopathy: clinical and radiological findings.Neurology. 2004; 62: 1417-1419
- Hypertensive encephalopathy extending into the whole brainstem and deep structures.Hypertens. Res. 2002; 25: 797-800
- Parietal occipital edema in hypertensive encephalopathy: a pathogenic mechanism.Eur. Neurol. 1996; 36: 25-28
- Regional cerebral blood flow autoregulation in normotensive and spontaneously hypertensive rats—effects of sympathetic denervation.Stroke. 1986; 17: 981-984
- Hypertensive brainstem encephalopathy: three cases presenting with severe brainstem edema.Neurology. 1999; 53: 652-654
- A brainstem variant of reversible posterior leukoencephalopathy syndrome.Neuroradiology. 2005; 47: 652-656
- Posterior reversible encephalopathy syndrome with spinal cord involvement.Neurology. 2014; 83: 2002-2006
Article info
Publication history
Published online: July 03, 2015
Accepted:
July 2,
2015
Received in revised form:
June 15,
2015
Received:
January 21,
2015
Identification
Copyright
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
