Hypertension-Induced Cerebellar Encephalopathy and Hydrocephalus in a Male

doi:10.1016/j.pediatrneurol.2005.07.005

Pediatric Neurology

Volume 34, Issue 1, January 2006, Pages 72-75

https://doi.org/10.1016/j.pediatrneurol.2005.07.005 Get rights and content

Hypertensive encephalopathy is believed to be caused by an abrupt elevation in systemic blood pressure. It rarely occurs in children and can be neurologically devastating if it is not recognized and treated immediately. This report describes an 11-year-old male who presented with edema and a cerebellar lesion, with acute obstructive hydrocephalus resulting from hypertensive encephalopathy. A shunt was inserted to relieve pressure in the acute stage. The patient’s hydrocephalus and cerebellar swelling subsided when his blood pressure was controlled. The cerebellar lesion had been initially diagnosed as a glioma. In children, a cerebellar lesion occurring with acute obstructive hydrocephalus and hypertensive encephalopathy is rare but reversible. Clinicians should be aware of this condition because it might be misdiagnosed as a tumor of the posterior fossa.

Introduction

Hypertensive encephalopathy is a condition triggered by an abrupt elevation of systemic arterial blood pressure [1], [2], [3]. Its heralding features are failure of autoregulation, breakdown of the blood-brain barrier, and extravasation of fluid and protein into the brain parenchyma [4]. Hypertensive encephalopathy can be neurologically devastating, as it can lead to rapidly progressive headache, nausea, vomiting, seizure, and disturbed consciousness. The characteristic pattern of edema affects children and adults and most commonly involves the subcortical white matter of the occipital lobes [1], [2], [3], [4]. Immunosuppressive drugs, renovascular disease, eclampsia, and idiopathic essential hypertension can induce hypertensive encephalopathy [1], [2], [3].

This report describes an 11-year-old male with acutely elevated arterial blood pressure and subsequent encephalopathy. To our knowledge, acute obstructive hydrocephalus with cerebellar encephalopathy has not been reported in children. We emphasize that this condition might be misdiagnosed as tumor-associated hydrocephalus if it is not recognized early.

Section snippets

Case Report

An 11-year-old male presented to our outpatient clinic with a 6-month history of chronic headache characterized by dull bilateral temporal pain. One week before his presentation, the headaches had increased in severity. He had no vomiting or limb weakness.

Findings on physical and neurologic examination were normal. Transcranial color Doppler sonography revealed mild ventriculomegaly and possibly abnormal hyperechogenicity in the parenchyma of the posterior fossa. One week later, the patient

Discussion

Hypertensive encephalopathy, also known as reversible occipitoparietal encephalopathy or reversible posterior leukoencephalopathy, is induced by an abrupt elevation in arterial blood pressure and clinically characterized by headache, blurred vision, seizure, or disturbed consciousness [1]. In patients with hypertensive encephalopathy, abnormally high blood pressure and increased permeability of the vascular walls overpower their autoregulation of cerebral blood flow. Underlying etiologies of

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He has been in satisfactory and stable condition for more than one year. Eight reports of nine patients met the inclusion criteria [6–13]. These included our two case studies, resulting in a total of 11 cases for this descriptive analysis.
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.
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.
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.
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Posterior reversible encephalopathy syndrome (PRES) is a recently proposed cliniconeuroradiologic entity with several well-known causes, such as hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressive drugs, as well as some causes more recently described. PRES is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction. The pathogenesis is incompletely understood. Two opposing hypotheses are commonly cited, but the issue is controversial: (1) the current more popular theory suggests that severe hypertension exceeds the limits of autoregulation, leading to breakthrough brain edema; (2) the earlier original theory suggests that hypertension leads to cerebral autoregulatory vasoconstriction, ischemia, and subsequent brain edema.
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Case reportHypertension-Induced Cerebellar Encephalopathy and Hydrocephalus in a Male

Introduction

Section snippets

Case Report

Discussion

A reversible posterior leukoencephalopathy syndrome

N Engl J Med

Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathyThree names for an old syndrome

J Child Neurol

Posterior leukoencephalopathy syndrome

Postgrad Med J

Primary hypertension-induced cerebellar encephalopathy causing obstructive hydrocephalus

J Neurosurg

Hypertensive encephalopathyFindings on CT, MR imaging, and SPECT imaging in 14 cases

Am J Roentgenol

Hypertensive encephalopathy in children

Am J Neuroradiol

Acute hypertensive encephalopathyFindings on spin-echo and gradient echo MR imaging

Am J Roentgenol

Case report
Hypertension-Induced Cerebellar Encephalopathy and Hydrocephalus in a Male