Intracranial arterial and arteriovenous malformations presenting with infarction. Lausanne Stroke Registry study.

Cerebral aneurysms and arteriovenous malformations (AVMs) are well-known sources of intracranial hemorrhage, but can also manifest as other clinical symptoms or remain clinically asymptomatic. The aim was to document and analyze cases of aneurysm or AVM with brain infarction. Survey on 4804 stroke patients treated at the Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland between 1978 and 2000 using the Lausanne Stroke Registry. Twenty patients presented with cerebral aneurysm and 21 with cerebral AVM. Hemorrhage was present in 100% of the AVM and in 75% of the aneurysm patients; in one (5%) of the remaining aneurysm patients, aneurysm and infarction were located in different territories. Infarction associated with Sylvian artery aneurysm was found in three (15%), vertebrobasilar ischemia because of fusiform left vertebral artery aneurysm in one (5%), and dural fistula draining to the distal transversal and left sigmoid sinus associated with a stroke in the territory of the left anterior inferior cerebellar artery in one patient. Ischemic stroke is infrequent, but important, complication in unruptured intracranial aneurysms and AVMs. The early recognition and therapy of these vascular malformations in selected patients can avoid a major neurological deficit or death caused by their rupture.

Myéloradiculopathie chronique progressive chez un homme de 61 ans [Chronic progressive myeloradiculopathy in a 61-year-old man]

A painful worsening of known difficulties in walking led us to investigate a man who presented a spastic paraparesis. Radiological investigations had to be repeated three times before making a diagnosis of a right C6 spinal dural arteriovenous fistula after a 22-month follow-up. Knowing the mechanisms leading to spinal venous hypertension may explain the low yield of the early radiological investigations that should be repeated. The efficiency of the treatment depends on the severity of the presurgical neurologic manifestations.

Anomalies vasculaires: exemple du syndrome de Klippel-Trenaunay [Vascular anomalies].

Les anomalies vasculaires constituent des affections rares présentes dès la naissance. Elles sont classées selon leur histologie en tumeurs ou malformations vasculaires. La connaissance de ces anomalies par le médecin de famille est importante, permettant ainsi leur diagnostic, qui repose essentiellement sur la clinique. Les caractéristiques anatomiques et hémodynamiques de l'anomalie sont précisées par l'angiologue grâce à l'écho-Doppler. Cet examen permet un suivi de la lésion et le diagnostic d'éventuelles complications. Un bilan radiologique peut compléter cette évaluation avant discussion multidisciplinaire en vue d'un traitement parfois difficile. Dans cet article, un bref aperçu des malformations vasculaires et de leur prise en charge multidisciplinaire est discuté, en particulier dans le cas du syndrome de Klippel-Trenaunay. Vascular anomalies are rare conditions that could be observed at all ages. They are classified, according to their histology, in vascular tumors or vascular malformations. The general practitioner plays a significant role in diagnosis and patient management, diagnosis being suspected on clinical history. In case of vascular anomaly, ultrasound-Doppler assessment is helpful to characterize morphologic and hemodynamic changes of the lesion and permits to monitor the evolution and to detect complications. Further investigations are often necessary prior to multidisciplinary management. In this article, a brief overview of vascular anomalies, their multidisciplinary management and the exemple of Klippel-Trenaunay syndrome are presented.

Rendu-Osler disease: treatment with oestrogen/progestagen versus octreotide.

In Rendu-Osler disease, haemorrhages due to gastrointestinal vascular malformations are common. Surgical and endoscopic treatments for haemorrhage due to gastrointestinal vascular malformations are compromised when lesions are diffuse, escape identification or are inaccessible to treatment. Hormonal treatment with oestrogen and progestagens is still controversial based on contradictory results from two randomised clinical trials. Although somatostatin and its long-acting analogue, octreotide, have been reported to be beneficial in preventing rebleeding, there is no consensus on this type of treatment. This case report shows how the combination of ethinyloestradiol and norethisterone markedly reduced the need for blood transfusions with few side effects in one patient; in comparison, octreotide seems less effective but this could be related to a worsening of the disease.

La thrombose veineuse profonde chez l'adolescent [Venous thromboembolic disease in adolescents]

Deep vein thrombosis in children and adolescents is a quite rare event. Risk factors most often associated with DVT in this particular population are: central vein catheters, neoplasia, vascular malformations and oral contraception. Diagnosis and management of DVT in adolescents does not differ greatly from that of adults. Compression ultrasound is the initial exam of choice. Hospitalization is often not necessary and treatment can be started by using low molecular weight heparin followed by oral anti-vitamin K antagonists. Thrombophilia screening is not routinely recommended and should be reserved for those patients for whom results would change therapeutical management.

Reemergence of dormant Coats disease after 30 years.

Purpose. We describe an atypical case of a patient with Coats disease that re-emerged after 30 years, illustrating a previously poorly understood long-term evolution of the disease. Methods. A 20-year-old man consulted for visual acuity (VA) decrease in the left eye (LE) to 0.3. Fundus examination revealed an exudative lesion with telangiectasias in the superior peripheral retina compatible with the diagnosis of Coats disease. Results. The patient was treated with cryotherapy and argon laser. Visual acuity improved to 0.5 and remained stable during a 1-year follow-up. The patient did not seek further clinical follow-up. Thirty years later, he returned complaining of a progressive VA decrease in the LE. Snellen VA was measured to counting fingers. Fundus examination revealed stage 3A Coats disease with macular exudation and a serous retinal detachment in the inferior quadrants requiring the placement of an encircling band, external drainage, and cryotherapy of the vascular lesions. After 10 additional sessions of argon laser on the vascular malformations, exudation regressed further and best-corrected VA increased to 0.1 at the end of the follow-up period. Conclusions. Coats disease must be considered as a chronic disease, which necessitates a very long-term follow-up even in the absence of subjective visual loss. The disease can reawaken and recur with force in previously unaffected areas of the retina several decades later. The gold standard treatment consists of cryotherapy and argon laser. However, in cases of very important retinal exudation, surgical management with subretinal drainage may be necessary.

An unusual cause of hemoptysis--aberrant origin of the left pulmonary artery from the ascending aorta in the adult.

Aberrant origin of a pulmonary artery from the ascending aorta is an uncommon congenital vascular malformation with poor survival without surgery. In this case report, we describe the unusual late diagnosis of this congenital malformation in an otherwise asymptomatic young man presenting with mild hemoptysis. We review the natural and modified history of this defect and the relevant aspects of follow-up in adult life.

Preoperative assessment of neurovesical function in children with anorectal malformation: association with vertebral and spinal malformations.

PURPOSE: We preoperatively assessed neurovesical function and spinal cord function in children with anorectal malformations. In cases of neurovesical dysfunction we looked for an association with vertebral malformation or myelodysplasia. MATERIALS AND METHODS: We prospectively evaluated 80 children with anorectal malformations via preoperative urodynamics and magnetic resonance imaging of the spine. Bladder compliance and volume, detrusor activity and vesicosphincteric synergy during voiding allowed urodynamic evaluation. Results were reported according to Wingspread and Krickenbeck classifications of anorectal malformations. RESULTS: Urodynamic findings were pathological in 14 children (18%). Pathological evaluations did not seem related to type of fistula or level of anorectal malformation. Vertebral anomalies were seen in 34 patients (43%) and myelodysplasia in 16 (20%). Neither vertebral anomaly nor myelodysplasia seemed associated with type of fistula or severity of anorectal malformation. Of 14 children with pathological urodynamics no vertebral anomaly or myelodysplasia was found in 7. Of 66 children with normal urodynamics 40 presented with vertebral or spinal malformation. CONCLUSIONS: Lower urinary tract dysfunction is common in patients with anorectal malformations. Normal spine or spinal cord does not exclude neurovesical dysfunction. Myelodysplasia or vertebral anomaly does not determine lower urinary tract dysfunction. Thus, we recommend preoperative urodynamic assessment of the bladder and magnetic resonance imaging of the spine in children with anorectal malformations.

Late outcome of spinal cord stimulation for unreconstructable and limb-threatening lower limb ischemia.

OBJECTIVES: To determine whether the initial benefits of spinal cord stimulation (SCS) treatment for critical limb ischemia (CLI) persist over years. DESIGN: Analysis of data prospectively collected for every CLI patient receiving permanent SCS. Follow-up range 12 to 98 months (mean 46+/-23, median 50 months). POPULATION: 87 patients (28% stage III, 72%stage IV) with unreconstructable CLI due (83%) or not (17%) to atherosclerosis and with an initial sitting/supine transcutaneous pO2 gradient >15 mmHg. METHODS: Assessment of actuarial patient survival (PS), limb salvage (LS) and amputation-free patient survival (AFPS). Analysis of the impact of 15 risk factors on long-term outcomes using the Fischer's exact test for categorical variables and the t test for continuous variables. RESULTS: Follow-up was complete for patient and limb survival. A single non-atherosclerotic patient died during follow-up. Among atherosclerotic patients PS decreased from 88% at 1y, to 76% at 3y, 64% at 5y and 57% at 7y. LS reached 84% at 1y, 78% at 2y, 75% at 3y and remained stable thereafter. Diabetes was found to affect LS (p<0.05) and heart disease to reduce PS (p<0.01). AFPS was reduced in heart patients (p<0.01), diabetics (p<0.05) and in patients with previous stroke (p<0.05). CONCLUSIONS: In CLI patients the beneficial effects of SCS persist far beyond the first year of treatment and major amputation becomes infrequent after the second year.

Spinal cord stimulation treatment for angina pectoris: more than a placebo?

BACKGROUND: The effects of thoracolumbal spinal cord stimulation (SCS) are confined to restricted microcirculatory areas. This limitation is generally attributed to a predominantly segmental mode of action on the autonomic nervous system. The goal of this study was to determine whether SCS applied close to supraspinal autonomic centers would induce generalized hemodynamic changes that could explain its alleged antianginal properties. METHODS: Invasive hemodynamic tests were performed in 15 anesthetized Göttingen minipigs submitted to iterative cervical SCS of various duration and intensity. RESULTS: Hemodynamic changes exceeding 10% were observed in 59 of 68 SCS sessions (87%). Their extent and time to peak varied with SCS intensity. At 2, 5, and 10 V, significant (t test p < 0.05) peak changes occurred in cardiac output (+34%, +29%, and +28%, respectively), stroke volume (+19%, +16%, +15%), mean pressure (+9%, +27%, +40%), heart rate (+14%, +23%, +14%), systemic (-17%, NS, NS), and pulmonary vascular (25%, NS, NS) resistances. Strikingly, at 2 V, the increase in cardiac output (+34%) was higher than the synchronous rise in rate pressure product (+22%), indicating efficient cardiac work. At 10 V, however, the cardiac work was inefficient (rate pressure product + 53%/cardiac output + 28%). CONCLUSIONS: Low-voltage cervical neuromodulation reduces the postcharge and improves cardiac work efficiency. The resulting reduction in oxygen myocardial demand may account for decreased anginal pain.

Specific inhibition of the JNK pathway promotes locomotor recovery and neuroprotection after mouse spinal cord injury.

Limiting the development of secondary damage represents one of the major goals of neuroprotective therapies after spinal cord injury. Here, we demonstrate that specific JNK inhibition via a single intraperitoneal injection of the cell permeable peptide D-JNKI1 6h after lesion improves locomotor recovery assessed by both the footprint and the BMS tests up to 4 months post-injury in mice. JNK inhibition prevents c-jun phosphorylation and caspase-3 cleavage, has neuroprotective effects and results in an increased sparing of white matter at the lesion site. Lastly, D-JNKI1 treated animals show a lower increase of erythrocyte extravasation and blood brain barrier permeability, thus indicating protection of the vascular system. In total, these results clearly point out JNK inhibition as a promising neuroprotective strategy for preventing the evolution of secondary damage after spinal cord injury.

Percutaneous radiofrequency ablation of primary intraosseous spinal glomus tumor.

The glomus tumor is a rare, benign, but painful vascular neoplasm arising from the neuromyoarterial glomus. Primary intraosseous glomus tumor is even rarer, with only about 20 cases reported in the literature so far, 5 of which involved the spine. Surgical resection is currently considered the treatment of choice. We herewith present an uncommon case of primary intraosseous spinal glomus tumor involving the right pedicle of the eleventh thoracic vertebra (T11). To our knowledge, this is the first case of primary intraosseous spinal glomus tumor successfully treated by percutaneous CT-guided radiofrequency ablation (RFA).

Mechanisms of Symptomatic Spinal Cord Ischemia After TEVAR: Insights From the European Registry of Endovascular Aortic Repair Complications (EuREC).

Abstract Purpose: To test the hypothesis that simultaneous closure of at least 2 independent vascular territories supplying the spinal cord and/or prolonged hypotension may be associated with symptomatic spinal cord ischemia (SCI) after thoracic endovascular aortic repair (TEVAR). Methods: A pattern matching algorithm was used to develop a risk model for symptomatic SCI using a prospective 63-patient single-center cohort to test the positive predictive value (PPV) of prolonged intraoperative hypotension and/or simultaneous closure of at least 2 of 4 the vascular territories supplying the spinal cord (left subclavian, intercostal, lumbar, and hypogastric arteries). This risk model was then applied to data extracted from the multicenter European Registry on Endovascular Aortic Repair Complications (EuREC). Between 2002 and 2010, the 19 centers participating in EuREC reported 38 (1.7%) cases of symptomatic spinal cord ischemia among the 2235 patients in the database. Results: In the single-center cohort, direct correlations were seen between the occurrence of symptomatic SCI and both prolonged intraoperative hypotension (PPV 1.00, 95% CI 0.22 to 1.00, p = 0.04) and simultaneous closure of at least 2 independent spinal cord vascular territories (PPV 0.67, 95% CI 0.24 to 0.91, p = 0.005). Previous closure of a single vascular territory was not associated with an increased risk of symptomatic spinal cord ischemia (PPV 0.07, 95% CI 0.01 to 0.16, p = 0.56). The combination of prolonged hypotension and simultaneous closure of at least 2 territories exhibited the strongest association (PPV 0.75, 95% CI 0.38 to 0.75, p<0.0001). Applying the model to the entire EuREC cohort found an almost perfect agreement between the predicted and observed risk factors (kappa 0.77, 95% CI 0.65 to 0.90). Conclusion: Extensive coverage of intercostal arteries alone by a thoracic stent-graft is not associated with symptomatic SCI; however, simultaneous closure of at least 2 vascular territories supplying the spinal cord is highly relevant, especially in combination with prolonged intraoperative hypotension. As such, these results further emphasize the need to preserve the left subclavian artery during TEVAR.