- Email: [email protected]
Spinal cord involvement in posterior reversible encephalopathy syndrome (PRES)
56
Correspondences
B. Gory a,b,c,∗ P.-E. Labeyrie a,d R. Riva a R. Sivan-Hoffmann a L. Derex e J.-J. Lehot f F. Philippeau g F. Turjman a,b,c a DHU IRIS, service de neuroradiologie interventionnelle, hôpital neurologique Pierre-Wertheimer, hospices civils de lyon, 59, boulevard Pinel, 69677 Bron cedex, France b Université Claude-Bernard — Lyon 1, 69609 Lyon, France c Centre de neuroscience cognitive, UMR 5229, CNRS, 69675 Bron, France d Unité INSERM UMR-S 919, sérine, protéases et physiopathologie de l’unité neurovasculaire, GIP Cyceron, université Caen — Basse-Normandie, 14000 Caen, France e DHU IRIS, service d’urgences cérébrovasculaires, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69677 Bron cedex, France f Fédération hospitalière d’anesthésie-réanimation, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69677 Bron cedex, France g Service de neurologie, unité neurovasculaire, hôpital de Bourg-en-Bresse, 01012 Bourg-en-Bresse, France ∗
Corresponding author. DHU IRIS, Service de neuroradiologie interventionnelle hôpital neurologique Pierre-Wertheimer, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron, France. Tel.: +33 4 72 68 12 63; fax: +33 4 72 11 89 13. E-mail address: [email protected] (B. Gory) Available online 23 October 2015 http://dx.doi.org/10.1016/j.neurad.2015.07.002
Spinal cord involvement in posterior reversible encephalopathy syndrome (PRES)
cortical lesions in the frontal, insular and temporal lobes and basal ganglia, with brain stem and cervical spinal cord involvement. The lesions were hyperintense on fluidattenuated inversion recovery (FLAIR) and hypointense on T1 images, associated with a high apparent diffusion coefficient (ADC) and few microbleeds on susceptibility-weighted imaging. No gadolinium enhancement was observed. MR spectroscopy did not detect an abnormal lactate or myoinositol peak. Complementary cervical spinal cord imaging showed hypertrophy with expansile central spinal cord T2 hyperintensity involving four spinal segments (Fig. 2). The lesion was located essentially in grey matter. Her blood pressure had risen to 204/160 mmHg and the diagnosis of PRES with grade IV hypertensive retinopathy was confirmed. The patient received antihypertensive treatment. The onemonth follow-up MRI showed disappearance of the signal abnormalities (Fig. 3).
Discussion PRES is a complex syndrome typically characterised by an acute or subacute neurological syndrome with headache, altered mental function, visual disturbance and/or seizure. The most common inciting factors are arterial hypertension, eclampsia, chronic renal insufficiency, autoimmune diseases and chemotherapy. Its aetiopathogenesis is still controversial but two main theories have been suggested. These are hypertension-induced autoregulatory failure with brain hyperperfusion, and endothelial abnormality with vasoconstriction and hypoperfusion [1]. The MRI characteristics of PRES are vasogenic oedema, typically symmetrically involving the cortical and subcortical parietal or occipital lobes in 98% of cases. Other usual lesion locations are the frontal lobes (68%), temporal lobes (40%), and cerebellum (30%). Involvement of the basal ganglia (14%), brain stem (13%) [2] and deep white matter (18%) is less common [1]. Only a few cases report spinal cord involvement [3,4]. Spinal cord involvement includes
Neuroimaging features in posterior reversible encephalopathy syndrome (PRES) are characterised by vasogenic oedema symmetrically involving the cortical and subcortical parietal and occipital lobes and typically resolving within a few weeks of aetiological treatment. We report unusual neuroimaging findings of extensive lesions of PRES, including involvement of the cervical cord in a 17-year-old woman.
Case report A 17-year-old woman with a history of mental disorder consulted an ophthalmologist for visual disturbance with unusual headaches and nausea experienced for one month. The patient had no focal neurological symptoms, fever or history of recent infection or vaccination. The ophthalmological examination confirmed a major visual acuity loss (6/60 right and left eye) with bilateral papillary oedema (Fig. 1). A 3.0 T brain MRI was performed. The images revealed symmetrical bilateral cortical and sub-
Figure 1 Left fundus centered on the optic nerve. The ophtalmogical examination shows a grade IV hypertensive retinopathy associating a papilledema (thick arrow), a peripapillary hard exudates complicating chronic oedema and affecting macula (thick dotted arrow), an arteriolar narrowing (narrow dotted arrow), a cotton wool spot (narrow arrow) and a retinal hemorrhage (arrowhead).
Correspondences
57
Figure 2 Axial (a) and sagittal (b) fluid-attenuated inversion recovery (FLAIR) MR images show hyperintensity in the frontal lobes, basal ganglia, pons, medulla and cervical spinal cord; associated with diffuse microhaemorrhages (white arrow) on SWI (c). Sagittal (d) and axial (e) T2 cervical MR images show expansile central spinal cord T2 hyperintensity spanning 4 spinal segments.
extreme high blood pressure and/or grade IV hypertensive retinopathy. As in our case, MRI lesions are expansile with central spinal cord T2 hyperintensity spanning at least 4 spinal segments and originating at the cervicomedullary junction [3,4].
Brain microhaemorrhage was reported as an uncommon imaging feature of PRES, and better visualised with the new susceptibility-weighted images. The presence of microhaemorrhage on SWI does not appear to correlate with the MR imaging severity/extent of oedema or any other
Figure 3 a: initial sagittal FLAIR MR images shows hyperintensity in pons, medulla and cervical spinal cord (white arrow); b: one-month follow-up MRI after antihypertensive treatment shows disappearance of FLAIR signal abnormalities.
58
Correspondences
particular imaging findings. Moreover, microhaemorrhages probably developed before PRES and were possibly related to an underlying vasculopathy, but new lesions do develop in many patients with PRES and persist for a long time thereafter [5]. In this case, the differential diagnosis of PRES was excluded based on the clinical history, radiological and complementary examination findings. The absence of recent vaccination, fever, and gadolinium enhancement tended to rule out acute disseminated encephalomyelitis (ADEM). Urine toxicity tests were negative. MR spectroscopy did not show a lactate peak suggestive of metabolic disease. The final diagnosis of PRES was actually confirmed at the onemonth follow-up which subsequently showed disappearance of signal abnormalities after high blood pressure treatment.
Conclusion We report an unusual case of PRES with extensive cervical spinal cord involvement revealed by bilateral papillary oedema. The presence of brain microhaemorrhage on SWI suggested a vascular origin. The search for high blood pressure was subsequently a key feature in the diagnosis of PRES.
Disclosure of interest The authors declare that they have no competing interest.
References [1] Bartynski WS, Boardman JF. Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2007;28:1320—7, http://dx.doi.org/ 10.3174/ajnr.A0549. [2] Barnaure I, Horvath J, Lovblad K-O, Vargas M-I. Atypical brainstem presentation of posterior reversible encephalopathy syndrome (PRES). J Neuroradiol 2014;41:143—4, http://dx.doi.org/ 10.1016/j.neurad.2013.05.002. [3] De Havenon A, Joos Z, Longenecker L, Shah L, Ansari S, Digre K. Posterior reversible encephalopathy syndrome with spinal cord involvement. Neurology 2014;83:2002—6, http://dx.doi.org/ 10.1212/WNL.0000000000001026. [4] Hou X, Xu J, Chen Z, Li G, Jiang H. Posterior reversible encephalopathy syndrome with involvement of the cervical cord and medulla: a case report. J Clin Diagn Res 2015;9:CD01—2, http://dx.doi.org/10.7860/JCDR/2015/10756.5376. [5] McKinney AM, Sarikaya B, Gustafson C, Truwit CL. Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. Am J Neuroradiol 2012;33:896—903, http://dx.doi.org/10.3174/ajnr.A2886.
Isabelle Ract a,∗ Adrien Poujade b Béatrice Carsin-Nicol a Frédéric Mouriaux b Jean-Christophe Ferré a a Department of neuroradiology, hôpital Pontchaillou, CHU de Rennes, 2, rue Henri-Le-Guilloux, 35000 Rennes, France b Department of ophtalmology, hôpital Pontchaillou, CHU de Rennes, 2, rue Henri-Le-Guilloux, 35000 Rennes, France
∗
Corresponding author. E-mail address: [email protected] (I. Ract) Available online 12 November 2015 http://dx.doi.org/10.1016/j.neurad.2015.09.004
Did an eighteen-century anatomist provide the first known description of a spinal cord vascular malformation? Antoine Portal (1742—1832) is essentially remembered for the role he played in the creation of the Royal Academy of Medicine by Louis XVIII (Fig. 1). Yet, in 1803, Portal had published a monumental treatise of anatomy in five volumes, while he was in charge of the chair of human anatomy at the Museum of Natural History in Paris, a position he owed to Buffon himself. The third volume of this treatise is dedicated to the heart and blood vessels. The chapter discussing the disposition of the posterior spinal arteries (p. 218—219) is complemented by the following footnote: ‘‘I found these arteries blown up and filled with blood, as if they had been strongly injected, in the portion of the medullary cylinder [spinal cord] corresponding to the dorsal vertebrae in a man who suffered, at the time of a peripneumonia, a very significant weakness of the inferior extremities with decreased sensitivity’’. (author’s transl.) This brief comment may represent the first reference to a spinal cord vascular malformation known so far. The clinical information, while limited, is compatible with a myelopathic syndrome. Morphologically, Portal mentioned dilated arteries, but he more likely observed the engorged dorsal perimedullary veins that are typically associated with various types of spinal arteriovenous fistulas. In fact, the distinction between arterialized veins and true arteries would remain virtually impossible until the introduction of selective spinal angiography in the late nineteen-sixties. For this reason, the early literature on spinal vascular malformations is difficult to analyze. Gaupp in 1885 [1] and Brasch in 1900 [2] can likely be credited with the first descriptions of perimedullary and dural arteriovenous fistulas, respectively, while a few earlier reports remain too vague to be categorized with certainty. These observations include for example, a possible case of epidural arteriovenous fistula by Levy in 1854 [3], another of diffuse congestion of the epidural venous system by Ollivier d’Angers in 1827 [4], and several instances of intramedullary aneurysms reported by Liouville in 1871 [5] and by Hebold in 1885 [6]. We would like to suggest that Portal’s extremely brief anatomopathological observation represents the earliest known account of a spinal cord vascular malformation.
Disclosure of interest The author declares that he has no competing interest.
Correspondences
B. Gory a,b,c,∗ P.-E. Labeyrie a,d R. Riva a R. Sivan-Hoffmann a L. Derex e J.-J. Lehot f F. Philippeau g F. Turjman a,b,c a DHU IRIS, service de neuroradiologie interventionnelle, hôpital neurologique Pierre-Wertheimer, hospices civils de lyon, 59, boulevard Pinel, 69677 Bron cedex, France b Université Claude-Bernard — Lyon 1, 69609 Lyon, France c Centre de neuroscience cognitive, UMR 5229, CNRS, 69675 Bron, France d Unité INSERM UMR-S 919, sérine, protéases et physiopathologie de l’unité neurovasculaire, GIP Cyceron, université Caen — Basse-Normandie, 14000 Caen, France e DHU IRIS, service d’urgences cérébrovasculaires, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69677 Bron cedex, France f Fédération hospitalière d’anesthésie-réanimation, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69677 Bron cedex, France g Service de neurologie, unité neurovasculaire, hôpital de Bourg-en-Bresse, 01012 Bourg-en-Bresse, France ∗
Corresponding author. DHU IRIS, Service de neuroradiologie interventionnelle hôpital neurologique Pierre-Wertheimer, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron, France. Tel.: +33 4 72 68 12 63; fax: +33 4 72 11 89 13. E-mail address: [email protected] (B. Gory) Available online 23 October 2015 http://dx.doi.org/10.1016/j.neurad.2015.07.002
Spinal cord involvement in posterior reversible encephalopathy syndrome (PRES)
cortical lesions in the frontal, insular and temporal lobes and basal ganglia, with brain stem and cervical spinal cord involvement. The lesions were hyperintense on fluidattenuated inversion recovery (FLAIR) and hypointense on T1 images, associated with a high apparent diffusion coefficient (ADC) and few microbleeds on susceptibility-weighted imaging. No gadolinium enhancement was observed. MR spectroscopy did not detect an abnormal lactate or myoinositol peak. Complementary cervical spinal cord imaging showed hypertrophy with expansile central spinal cord T2 hyperintensity involving four spinal segments (Fig. 2). The lesion was located essentially in grey matter. Her blood pressure had risen to 204/160 mmHg and the diagnosis of PRES with grade IV hypertensive retinopathy was confirmed. The patient received antihypertensive treatment. The onemonth follow-up MRI showed disappearance of the signal abnormalities (Fig. 3).
Discussion PRES is a complex syndrome typically characterised by an acute or subacute neurological syndrome with headache, altered mental function, visual disturbance and/or seizure. The most common inciting factors are arterial hypertension, eclampsia, chronic renal insufficiency, autoimmune diseases and chemotherapy. Its aetiopathogenesis is still controversial but two main theories have been suggested. These are hypertension-induced autoregulatory failure with brain hyperperfusion, and endothelial abnormality with vasoconstriction and hypoperfusion [1]. The MRI characteristics of PRES are vasogenic oedema, typically symmetrically involving the cortical and subcortical parietal or occipital lobes in 98% of cases. Other usual lesion locations are the frontal lobes (68%), temporal lobes (40%), and cerebellum (30%). Involvement of the basal ganglia (14%), brain stem (13%) [2] and deep white matter (18%) is less common [1]. Only a few cases report spinal cord involvement [3,4]. Spinal cord involvement includes
Neuroimaging features in posterior reversible encephalopathy syndrome (PRES) are characterised by vasogenic oedema symmetrically involving the cortical and subcortical parietal and occipital lobes and typically resolving within a few weeks of aetiological treatment. We report unusual neuroimaging findings of extensive lesions of PRES, including involvement of the cervical cord in a 17-year-old woman.
Case report A 17-year-old woman with a history of mental disorder consulted an ophthalmologist for visual disturbance with unusual headaches and nausea experienced for one month. The patient had no focal neurological symptoms, fever or history of recent infection or vaccination. The ophthalmological examination confirmed a major visual acuity loss (6/60 right and left eye) with bilateral papillary oedema (Fig. 1). A 3.0 T brain MRI was performed. The images revealed symmetrical bilateral cortical and sub-
Figure 1 Left fundus centered on the optic nerve. The ophtalmogical examination shows a grade IV hypertensive retinopathy associating a papilledema (thick arrow), a peripapillary hard exudates complicating chronic oedema and affecting macula (thick dotted arrow), an arteriolar narrowing (narrow dotted arrow), a cotton wool spot (narrow arrow) and a retinal hemorrhage (arrowhead).
Correspondences
57
Figure 2 Axial (a) and sagittal (b) fluid-attenuated inversion recovery (FLAIR) MR images show hyperintensity in the frontal lobes, basal ganglia, pons, medulla and cervical spinal cord; associated with diffuse microhaemorrhages (white arrow) on SWI (c). Sagittal (d) and axial (e) T2 cervical MR images show expansile central spinal cord T2 hyperintensity spanning 4 spinal segments.
extreme high blood pressure and/or grade IV hypertensive retinopathy. As in our case, MRI lesions are expansile with central spinal cord T2 hyperintensity spanning at least 4 spinal segments and originating at the cervicomedullary junction [3,4].
Brain microhaemorrhage was reported as an uncommon imaging feature of PRES, and better visualised with the new susceptibility-weighted images. The presence of microhaemorrhage on SWI does not appear to correlate with the MR imaging severity/extent of oedema or any other
Figure 3 a: initial sagittal FLAIR MR images shows hyperintensity in pons, medulla and cervical spinal cord (white arrow); b: one-month follow-up MRI after antihypertensive treatment shows disappearance of FLAIR signal abnormalities.
58
Correspondences
particular imaging findings. Moreover, microhaemorrhages probably developed before PRES and were possibly related to an underlying vasculopathy, but new lesions do develop in many patients with PRES and persist for a long time thereafter [5]. In this case, the differential diagnosis of PRES was excluded based on the clinical history, radiological and complementary examination findings. The absence of recent vaccination, fever, and gadolinium enhancement tended to rule out acute disseminated encephalomyelitis (ADEM). Urine toxicity tests were negative. MR spectroscopy did not show a lactate peak suggestive of metabolic disease. The final diagnosis of PRES was actually confirmed at the onemonth follow-up which subsequently showed disappearance of signal abnormalities after high blood pressure treatment.
Conclusion We report an unusual case of PRES with extensive cervical spinal cord involvement revealed by bilateral papillary oedema. The presence of brain microhaemorrhage on SWI suggested a vascular origin. The search for high blood pressure was subsequently a key feature in the diagnosis of PRES.
Disclosure of interest The authors declare that they have no competing interest.
References [1] Bartynski WS, Boardman JF. Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2007;28:1320—7, http://dx.doi.org/ 10.3174/ajnr.A0549. [2] Barnaure I, Horvath J, Lovblad K-O, Vargas M-I. Atypical brainstem presentation of posterior reversible encephalopathy syndrome (PRES). J Neuroradiol 2014;41:143—4, http://dx.doi.org/ 10.1016/j.neurad.2013.05.002. [3] De Havenon A, Joos Z, Longenecker L, Shah L, Ansari S, Digre K. Posterior reversible encephalopathy syndrome with spinal cord involvement. Neurology 2014;83:2002—6, http://dx.doi.org/ 10.1212/WNL.0000000000001026. [4] Hou X, Xu J, Chen Z, Li G, Jiang H. Posterior reversible encephalopathy syndrome with involvement of the cervical cord and medulla: a case report. J Clin Diagn Res 2015;9:CD01—2, http://dx.doi.org/10.7860/JCDR/2015/10756.5376. [5] McKinney AM, Sarikaya B, Gustafson C, Truwit CL. Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. Am J Neuroradiol 2012;33:896—903, http://dx.doi.org/10.3174/ajnr.A2886.
Isabelle Ract a,∗ Adrien Poujade b Béatrice Carsin-Nicol a Frédéric Mouriaux b Jean-Christophe Ferré a a Department of neuroradiology, hôpital Pontchaillou, CHU de Rennes, 2, rue Henri-Le-Guilloux, 35000 Rennes, France b Department of ophtalmology, hôpital Pontchaillou, CHU de Rennes, 2, rue Henri-Le-Guilloux, 35000 Rennes, France
∗
Corresponding author. E-mail address: [email protected] (I. Ract) Available online 12 November 2015 http://dx.doi.org/10.1016/j.neurad.2015.09.004
Did an eighteen-century anatomist provide the first known description of a spinal cord vascular malformation? Antoine Portal (1742—1832) is essentially remembered for the role he played in the creation of the Royal Academy of Medicine by Louis XVIII (Fig. 1). Yet, in 1803, Portal had published a monumental treatise of anatomy in five volumes, while he was in charge of the chair of human anatomy at the Museum of Natural History in Paris, a position he owed to Buffon himself. The third volume of this treatise is dedicated to the heart and blood vessels. The chapter discussing the disposition of the posterior spinal arteries (p. 218—219) is complemented by the following footnote: ‘‘I found these arteries blown up and filled with blood, as if they had been strongly injected, in the portion of the medullary cylinder [spinal cord] corresponding to the dorsal vertebrae in a man who suffered, at the time of a peripneumonia, a very significant weakness of the inferior extremities with decreased sensitivity’’. (author’s transl.) This brief comment may represent the first reference to a spinal cord vascular malformation known so far. The clinical information, while limited, is compatible with a myelopathic syndrome. Morphologically, Portal mentioned dilated arteries, but he more likely observed the engorged dorsal perimedullary veins that are typically associated with various types of spinal arteriovenous fistulas. In fact, the distinction between arterialized veins and true arteries would remain virtually impossible until the introduction of selective spinal angiography in the late nineteen-sixties. For this reason, the early literature on spinal vascular malformations is difficult to analyze. Gaupp in 1885 [1] and Brasch in 1900 [2] can likely be credited with the first descriptions of perimedullary and dural arteriovenous fistulas, respectively, while a few earlier reports remain too vague to be categorized with certainty. These observations include for example, a possible case of epidural arteriovenous fistula by Levy in 1854 [3], another of diffuse congestion of the epidural venous system by Ollivier d’Angers in 1827 [4], and several instances of intramedullary aneurysms reported by Liouville in 1871 [5] and by Hebold in 1885 [6]. We would like to suggest that Portal’s extremely brief anatomopathological observation represents the earliest known account of a spinal cord vascular malformation.
Disclosure of interest The author declares that he has no competing interest.