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Posterior reversible encephalopathy syndrome as presenting form of very early systemic sclerosis.

Pedraza MI, Barbado J, Ruiz M, Guerrero ÁL - Case Rep Neurol Med (2015)

Bottom Line: Methods.Conclusion.PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

View Article: PubMed Central - PubMed

Affiliation: Neurology Department, Hospital Clínico Universitario, Avenida Ramón y Cajal 3, 47005 Valladolid, Spain.

ABSTRACT
Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers. Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known. Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed "active pattern," considered typical of SSc. Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

No MeSH data available.


Related in: MedlinePlus

Capillaroscopy. (a) Moderate loss of capillaries. (b) Giant capillaries. (c) Capillary microhemorrhages. (d) Granular flow.
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fig2: Capillaroscopy. (a) Moderate loss of capillaries. (b) Giant capillaries. (c) Capillary microhemorrhages. (d) Granular flow.

Mentions: A 64-year-old woman with a history of occasional arterial hypertension with no medical treatment and episodic headache with nauseas and vomiting was admitted due to one-week history of pulsating headache, especially prominent on frontal scalp and accompanied by phonophobia, photophobia, and facial flushing with blood pressure of 175/76 mmHg. Pain worsened with cephalic movement and improved in the darkness. Physical examination on admission including lung, heart, skin, and joint did not show any alteration. No Raynaud phenomenon was described and observed. Neurological examination revealed brisk deep tendon reflex with increased reflexogenic area in four limbs. Fundoscopy was normal, as well as cranial computed tomography (CT). The laboratory examination performed in the emergency department showed normal hemogram with hemoglobin level of 13.5 gr/dL and normal biochemistry with creatinine level of 0.71 mg/dL. Brain magnetic resonance imaging (MRI) revealed subcortical hyperintensities in FLAIR and diffusion-weighted sequences, mainly located in posterior regions (Figure 1(a)). Carotid ultrasound imaging and transcranial duplex showed a generalized increase in flow velocity. BP was monitored and elevated BP episodes were detected. BP was controlled with enalapril and headache improved. Laboratory tests showed the following data: creatinine, 0.8 mg/dL, LDH, 275 U/L, plaque count, 153000, ESR, 10 mm, and CPR, 2.37 mg/L. The immunology test only revealed the presence of antinuclear antibodies 1/80 with positive anti-topoisomerase I (scl-70) antibodies. In a new brain MRI a month later an improvement of white matter lesions in FLAIR and no abnormalities in diffusion-weighted imaging (Figure 1(b)) were observed. Finally capillaroscopy (Figure 2) showed “active pattern” with frequent giant capillaries, moderate loss of capillaries, mild disorganization of the capillary architecture, frequent capillary microhemorrhages, and background edema; all of these findings are considered typical of SSc.


Posterior reversible encephalopathy syndrome as presenting form of very early systemic sclerosis.

Pedraza MI, Barbado J, Ruiz M, Guerrero ÁL - Case Rep Neurol Med (2015)

Capillaroscopy. (a) Moderate loss of capillaries. (b) Giant capillaries. (c) Capillary microhemorrhages. (d) Granular flow.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4352928&req=5

fig2: Capillaroscopy. (a) Moderate loss of capillaries. (b) Giant capillaries. (c) Capillary microhemorrhages. (d) Granular flow.
Mentions: A 64-year-old woman with a history of occasional arterial hypertension with no medical treatment and episodic headache with nauseas and vomiting was admitted due to one-week history of pulsating headache, especially prominent on frontal scalp and accompanied by phonophobia, photophobia, and facial flushing with blood pressure of 175/76 mmHg. Pain worsened with cephalic movement and improved in the darkness. Physical examination on admission including lung, heart, skin, and joint did not show any alteration. No Raynaud phenomenon was described and observed. Neurological examination revealed brisk deep tendon reflex with increased reflexogenic area in four limbs. Fundoscopy was normal, as well as cranial computed tomography (CT). The laboratory examination performed in the emergency department showed normal hemogram with hemoglobin level of 13.5 gr/dL and normal biochemistry with creatinine level of 0.71 mg/dL. Brain magnetic resonance imaging (MRI) revealed subcortical hyperintensities in FLAIR and diffusion-weighted sequences, mainly located in posterior regions (Figure 1(a)). Carotid ultrasound imaging and transcranial duplex showed a generalized increase in flow velocity. BP was monitored and elevated BP episodes were detected. BP was controlled with enalapril and headache improved. Laboratory tests showed the following data: creatinine, 0.8 mg/dL, LDH, 275 U/L, plaque count, 153000, ESR, 10 mm, and CPR, 2.37 mg/L. The immunology test only revealed the presence of antinuclear antibodies 1/80 with positive anti-topoisomerase I (scl-70) antibodies. In a new brain MRI a month later an improvement of white matter lesions in FLAIR and no abnormalities in diffusion-weighted imaging (Figure 1(b)) were observed. Finally capillaroscopy (Figure 2) showed “active pattern” with frequent giant capillaries, moderate loss of capillaries, mild disorganization of the capillary architecture, frequent capillary microhemorrhages, and background edema; all of these findings are considered typical of SSc.

Bottom Line: Methods.Conclusion.PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

View Article: PubMed Central - PubMed

Affiliation: Neurology Department, Hospital Clínico Universitario, Avenida Ramón y Cajal 3, 47005 Valladolid, Spain.

ABSTRACT
Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers. Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known. Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed "active pattern," considered typical of SSc. Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

No MeSH data available.


Related in: MedlinePlus