Severe and isolated headache associated with hypertension as unique clinical presentation of posterior reversible encephalopathy syndrome
© Milani et al.; licensee BioMed Central Ltd. 2014
Received: 31 October 2013
Accepted: 9 July 2014
Published: 25 July 2014
Posterior reversible encephalopathy syndrome is a potentially reversible clinicoradiologic syndrome characterized by headache, mental confusion, visual disturbances and seizures associated with posterior cerebral lesions on radiological imaging. Prompt treatment of this condition is mandatory to avoid severe irreversible complications.
We report a 9-year-old boy with arterial hypertension and headache as unique clinical presentation of posterior reversible encephalopathy syndrome.
Severe and isolated headache associated with arterial hypertension can be the unique clinical presentation of posterior reversible encephalopathy syndrome. This syndrome must be considered even in absence of all typical symptoms to prevent the progression of a potentially life threatening condition.
KeywordsPosterior reversible encephalopathy syndrome Arterial hypertension Headache
Posterior reversible encephalopathy syndrome (PRES), first described by Hinchey et al. in 1996 , is a clinicoradiological condition presenting with headache, seizures, alterations of conscious level and loss of vision, accompanied by characteristic Magnetic Resonance Imaging (MRI) findings. Both the neurological and radiological features are potentially reversible . Nevertheless several complications as status epilepticus, intracranial hemorrhage, and massive ischemic infarction have been reported in association with this syndrome [3, 4]. The removal of the underlying causes is essential to prevent long term sequelae [3, 4].
We report a child with PRES presenting as hypertension and severe headache, with rapid clinical and neuroimaging normalization after a prompt treatment.
A previously normotensive 9-year-old boy was admitted to the Pediatric Emergency Department with bilateral periorbital edema and gross hematuria for 24 hours.
20 days before, he had experienced an episode of mild sore throat spontaneously recovered.
Blood and urine exams on admission
Red blood cells
4.2 x 106/mmc
3.9 – 5.2 x 106/mmc
34.5% - 42.5%
10.5 - 14.5 g/dL
< 0.5 mg/dL
3.5 – 5.0 mE/L
1.12 – 1.32 mmol/L
< 0.7 mg/dL
15 – 40 mg/dL
3.5 – 5.0 g/dL
86 – 184 mg/dL
0 – 200 U/ml
< 0.4 mg/mg
Red cells number
55 per high-power field
< 5 per high-power field
Hyaline casts on sediment
Since the 2nd day of hospitalization, the patient was treated with one bolus of methylprednisolone (15 mg/Kg) per day associated with strict blood pressure monitoring.
Methylprednisolone was discontinued. Candesartan was initiated and on 6th day of hospitalization, blood pressure normalized and both headache and periorbital edemas resolved. On 9th day of hospitalization, a control MRI showed complete regression of all the abnormalities (Figures 1B, D and 2B, D). Arterial hypertension, MRI findings and clinical outcome were consistent with the diagnosis of PRES.
The child was discharged in good clinical condition without any further treatment.
3 weeks later, clinical examination was unremarkable and blood pressure 105/70 mmHg. Complement C3 and serum creatinine normalized and urinalysis revealed only persistent isolated microscopic hematuria (7 cells per high-power field).
The literature describes posterior regions of the brain being frequently involved, but concomitant anterior lesions are often detected. Brain stem, basal ganglia, deep white matter, or splenium of the corpus callosum lesions are also described in less than one third of the patients [1, 8]. The pathophysiology of this syndrome is not fully understood, but appears to be multifactorial. The underlying mechanism could be a brain capillary leak syndrome following hypertension, fluid retention or cytotoxic effects of immunosuppressive agents on the vascular endothelium. A sudden rise in blood pressure is probably the most common cause. It can induce disruption of cerebral vascular auto-regulation, mostly in the posterior cerebral vasculature, causing leakage of fluid into the brain parenchyma [6–10]. Indeed a gradual blood pressure control is recommended to avoid cerebral hypoperfusion and increased morbidity .
Moreover this syndrome can be irreversible or even fatal if an appropriate treatment is not started promptly .
To the best of our knowledge, severe and isolated headache and hypertension have never been reported as unique clinical presentation of PRES. We speculate that the prompt recognition and treatment of this syndrome prevented its progression to the full blown syndrome.
The parents signed a case report consent for publication.
Magnetic resonance imaging
Posterior reversible encephalopathy syndrome.
Authors have no financial relationship relevant to this article to disclose.
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