Posterior Reversible Encephalopathy Syndrome an Early Sign of Systemic Sclerosis, According to Case Report

A recent Case Report entitled “Posterior Reversible Encephalopathy Syndrome as Presenting Form of Very Early Systemic Sclerosis,” describes a rare case of a patient with Posterior Reversible Encephalopathy Syndrome (PRES) caused by arterial hypertension and very early systemic sclerosis (SSc). The report is published in the journal Case Reports in Neurological Medicine.

Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological condition with a wide spectrum of symptoms. Typical neuroimaging consists of leukoencephalopathy in posterior regions. Pathogenesis of this disorder includes failure of the blood-brain barrier due to high systemic blood pressure (BP) as well as loss of integrity of vascular endothelium related with different triggers.

In the report, Ángel Luis Guerrero and colleagues from the Neurology Department at the Hospital Clínico Universitario in Valladolid, Spain describes the case of a female patient aged 64 years who was admitted to the hospital with a complaint of a pulsating headache for one week. The headache was more obvious in her frontal scalp, and was also accompanied by photophobia, phonophobia, and facial flushing.

After the neurological exam, the clinicians reported that the patient had brisk, deep tendon reflex. Results of magnetic resonance imaging (MRI) of the brain revealed subcortical lesions predominantly located in posterior regions. After monitoring her blood pressure, the clinicians were able to detect episodic arterial hypertension.

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The results of the blood tests showed that the patient was positive for anti-topoisomerase I antibodies. The clinicians then gave angiotensin-converting-enzyme inhibitors to control the blood pressure, and the patient reported an improvement in her headaches. After one month, the clinicians conducted a new MRI and the results showed an improvement in white matter lesions. However, Capillaroscopy revealed an interesting result confirming very early systemic sclerosis.

Based on this case report, the clinicians indicate that in SSc there is an impairment of the anti-endothelial cell antibodies, which causes vascular endothelium, and liberation of vasoconstrictors leading to high blood pressure and also to a disruption of blood-brain barrier auto regulation mechanisms.

In the report, they highlight that PRES can be considered an early manifestation of SSc. They also believe that even in the absence of skin lesions or Raynaud’s phenomenon, clinicians should consider PRES.