a pilot study

Background Utilising fluorescence optical imaging (FOI), the distribution of an intravenously applied colouring agent indocyanine green (ICG) can be analysed with the potential to identify malperfusion by little to no tissue enhancement. Systemic sclerosis (SSc) is characterised by the presence of digital ulcers reflecting progressive vasculopathy. The objective was to investigate the potential of FOI in the detection of disturbed microcirculation in the hands and fingers of patients with SSc and to link FOI findings to clinical signs of ischemia such as digital ulcers and pitting scars. Methods In this cross-sectional study, 63 patients with SSc and 26 healthy subjects were examined. FOI was performed in all 89 individuals and compared to clinical data and capillaroscopic findings assembled for the SSc cohort. Results Healthy subjects showed initial ICG signals in their fingertips in 93.6%, SSc patients in 78.5% (limited SSc) and 43.2% (diffuse SSc). Moreover, in SSc patients, FOI findings were significantly associated with a late capillaroscopic pattern, disseminated SSc features, a diffuse SSc subtype, and the presence of digital ulcers or pitting scars. Intra- and inter-reader reliability for FOI amounted to κ = 0.786 and κ = 0.834, respectively. Conclusions FOI is able to detect areas of reduced microcirculation in patients with SSc with high association to capillaroscopic findings. The results pave the way for future FOI investigations into its role in the prediction of complications due to an impaired acral perfusion

Detection of severe digital vasculopathy in systemic sclerosis by colour Doppler sonography is associated with digital ulcers

Einleitung: Die systemische Sklerose (SSc) gehört zur Erkrankungsgruppe der Kollagenosen und zeichnet sich durch eine progressive Fibrosierung des Haut- und Bindegewebes sowie durch eine Mikro- und Makroangiopathie aus. Zunehmende Umbauprozesse an den Gefäßen führen zu einer permanenten akralen Minderperfusion, die das Risiko digitaler Ulzerationen erhöht. Obwohl die Duplexsonographie einen wesentlichen Bestandteil der Gefäßdiagnostik darstellt, kommt sie bei der SSc bisher nicht regulär zum Einsatz. Ziel dieser Arbeit ist ein Vergleich der durch Duplexsonographie darstellbaren Gefäßveränderungen mit anderen bildgebenden Verfahren (Kapillarmikroskopie) und mit klinischen Zeichen einer akralen Minderperfusion. Methodik: Insgesamt wurden 32 Gefäße (16 pro Hand) der Finger, Handflächen und Handgelenke von 79 Patienten mit SSc mittels Duplexsonographie untersucht. Jedes untersuchte Gefäß wurde als normal, verengt oder verschlossen klassifiziert, wobei die beiden letztgenannten morphologischen Kategorien als pathologisch gewertet wurden. Ergebnisse: 40% der darstellbaren Gefäße (n = 2488) wiesen Verengungen oder Verschlüsse auf. Dabei war der Anteil pathologischer Gefäße bei den Fingerarterien (Aa. digitales palmares propriae) mit 49% höher als bei den weiter proximalen Gefäßen (16%; p < 0,0001). An Fingern mit digitalen Ulzerationen oder Narbengrübchen („pitting scars“) zum Erhebungszeitpunkt zeigte sich häufiger zumindest eines der beiden untersuchten Gefäße pathologisch verändert (p = 0,0009). Ein höherer Anteil pathologischer Gefäße fand sich bei Patienten mit in der Raucheranamnese mehr als 20 Packungsjahren, einem erhöhten C-reaktiven Protein (CRP)Wert, bestehender Raynaud-Symptomatik in der letzten Woche und aus der Vorgeschichte bekannten bzw. aktuell bestehenden digitalen Ulzerationen oder „pitting scars“. Durch eine ROC- Analyse (AUC = 0,727) wurde ein Trennwert von ≥ 20% pathologischen Gefäßen für das Vorhandensein von digitalen Ulzeration oder „pitting scars“ mit einer Sensitivität von 90,7% und einer Spezifität von 47,8% festgelegt. Vergleichbare Ergebnisse erbrachte ein verkürztes Untersuchungsprotokoll, in dem nur die acht Gefäße des zweiten bis fünften Fingers der rechten Hand erfasst wurden (AUC = 0,751; Sensitivität: 93,0%; Spezifizität: 43,5%). Schlussfolgerung: Pathologische Veränderungen in der Duplexsonographie der Handarterien bei Patienten mit systemischer Sklerose sind mit klinischen Zeichen einer chronischen Minderdurchblutung assoziiert, zum Beispiel dem Raynaud-Phänomen und bestehenden digitalen Ulzerationen. Die Ergebnisse unterstützen die Hypothese, dass durch die Duplexsonographie der Handarterien das Ausmaß der Angiopathie erfasst werden kann. Ein auf acht zu untersuchende Gefäße verkürztes Protokoll könnte in der klinischen Routine die Gefäßdiagnostik bei Patienten mit SSc ergänzen.Introduction: Systemic Sclerosis (SSc) is a connective tissue disease characterised by progressive tissue fibrosis and angiopathy. The continuous remodelling of the vessels leads to permanent malperfusion of the acres and increases the risk for digital ulcers. Despite being highly important in general vascular diagnostic procedures, Colour Doppler ultrasonography (CDUS) is rarely applied in SSc. In this study, the morphologic changes of digital arteries as seen in CDUS are compared to capillaroscopic patterns as well as the presence of clinical signs of acral malperfusion. Methods: Alongside the gathering of nailfold capillaroscopy, clinical, and laboratory data, 32 arteries of the fingers, palms and wrists from each of 79 SSc-patients were examined with CDUS. Each vessel was classified as either normal, narrowed, or occluded with the last two morphologic manifestations being considered pathologic. Results: 40% of all assessable arteries (n = 2488) showed narrowed or occluded lumens. The percentage of pathologic vessels was higher in finger arteries (proper palmar digital arteries) than in the more proximal vessels, with 49% and 16% of narrowed or occluded vessels, respectively (p < 0.0001). Fingers with digital ulcers or pitting scars were more likely to have at least one pathologic artery (p = 0.0009). The mean percentage of narrowed or occluded vessels was higher in patients with at least 20 pack years, an elevated C-reactive protein level, episodes of Raynaud’s phenomenon in the past week, and present or past digital ulcers or pitting scars (DU/PS). With ROC-analysis (AUC = 0.727), a cut-off value of ≥ 20% pathologic vessels (sensitivity: 91%; specificity: 48%) for the presence of DU/PS was determined. Similar results could be obtained when focusing on the eight arteries of the right hand’s digits II-V (AUC = 0.751; sensitivity: 93%; specificity: 44%). Conclusion: The extent of pathologic vessels in CDUS of hand and finger arteries are associated with clinical signs of chronic malperfusion, e.g. Raynaud’s phenomenon and digital ulcers. A shortened examination protocol of CDUS (proper palmar arteries of right hand’s digits II-V) could amplify diagnostics procedures to assess vasculopathy in systemic sclerosis

Disturbed microcirculation in the hands of patients with systemic sclerosis detected by fluorescence optical imaging: a pilot study

Abstract Background Utilising fluorescence optical imaging (FOI), the distribution of an intravenously applied colouring agent indocyanine green (ICG) can be analysed with the potential to identify malperfusion by little to no tissue enhancement. Systemic sclerosis (SSc) is characterised by the presence of digital ulcers reflecting progressive vasculopathy. The objective was to investigate the potential of FOI in the detection of disturbed microcirculation in the hands and fingers of patients with SSc and to link FOI findings to clinical signs of ischemia such as digital ulcers and pitting scars. Methods In this cross-sectional study, 63 patients with SSc and 26 healthy subjects were examined. FOI was performed in all 89 individuals and compared to clinical data and capillaroscopic findings assembled for the SSc cohort. Results Healthy subjects showed initial ICG signals in their fingertips in 93.6%, SSc patients in 78.5% (limited SSc) and 43.2% (diffuse SSc). Moreover, in SSc patients, FOI findings were significantly associated with a late capillaroscopic pattern, disseminated SSc features, a diffuse SSc subtype, and the presence of digital ulcers or pitting scars. Intra- and inter-reader reliability for FOI amounted to κ = 0.786 and κ = 0.834, respectively. Conclusions FOI is able to detect areas of reduced microcirculation in patients with SSc with high association to capillaroscopic findings. The results pave the way for future FOI investigations into its role in the prediction of complications due to an impaired acral perfusion

Cavernoma-related epilepsy: Review and recommendations for management - Report of the Surgical Task Force of the ILAE Commission on Therapeutic Strategies

Cerebral cavernous malformations (CCMs) are well-defined, mostly singular lesions present in 0.4-0.9% of the population. Epileptic seizures are the most frequent symptom in patients with CCMs and have a great impact on social function and quality of life. However, patients with CCM-related epilepsy (CRE) who undergo surgical resection achieve postoperative seizure freedom in only about 75% of cases. This is frequently because insufficient efforts are made to adequately define and resect the epileptogenic zone. The Surgical Task Force of the Commission on Therapeutics of the International League Against Epilepsy (ILAE) and invited experts reviewed the pertinent literature on CRE. Definitions of definitive and probable CRE are suggested, and recommendations regarding the diagnostic evaluation and etiology-specific management of patients with CRE are made. Prospective trials are needed to determine when and how surgery should be done and to define the relations of the hemosiderin rim to the epileptogenic zone

Cavernoma-related epilepsy: review and recommendations for management--report of the Surgical Task Force of the ILAE Commission on Therapeutic Strategies.

Cerebral cavernous malformations (CCMs) are well-defined, mostly singular lesions present in 0.4-0.9% of the population. Epileptic seizures are the most frequent symptom in patients with CCMs and have a great impact on social function and quality of life. However, patients with CCM-related epilepsy (CRE) who undergo surgical resection achieve postoperative seizure freedom in only about 75% of cases. This is frequently because insufficient efforts are made to adequately define and resect the epileptogenic zone. The Surgical Task Force of the Commission on Therapeutics of the International League Against Epilepsy (ILAE) and invited experts reviewed the pertinent literature on CRE. Definitions of definitive and probable CRE are suggested, and recommendations regarding the diagnostic evaluation and etiology-specific management of patients with CRE are made. Prospective trials are needed to determine when and how surgery should be done and to define the relations of the hemosiderin rim to the epileptogenic zone

Critique of the 2017 epileptic seizure and epilepsy classifications

This article critiques the International League Against Epilepsy (ILAE) 2015-2017 classifications of epilepsy, epileptic seizures, and status epilepticus. It points out the following shortcomings of the ILAE classifications: (1) they mix semiological terms with epileptogenic zone terminology; (2) simple and widely accepted terminology has been replaced by complex terminology containing less information; (3) seizure evolution cannot be described in any detail; (4) in the four-level epilepsy classification, level two (epilepsy category) overlaps almost 100% with diagnostic level one (seizure type); and (5) the design of different classifications with distinct frameworks for newborns, adults, and patients in status epilepticus is confusing. The authors stress the importance of validating the new ILAE classifications and feel that the decision of Epilepsia to accept only manuscripts that use the ILAE classifications is premature and regrettable