The correlation of the serum level of L-carnitine with disease severity in patients with Amyotrophic lateral sclerosis.

Background: The relationship between reserve of L-carnitine and severity in patients with Amyotrophic lateral sclerosis (ALS) is not studied sufficiently. We decided to measure the serum levels of L-carnitine in patients and the relationship with ALS severity.

Method: This cross-sectional study evaluated the serum levels of L-carnitine in 30 patients with ALS (total-case) divided into two groups included 15 patients in the Oral-Fed (OF) group and 15 patients in the Enteral-Fed (EF) group, compared with 15 healthy people matched in age and sex in the control group.

Surgical Decompression for Space-Occupying Hemispheric Infarction: A Systematic Review and Individual Patient Meta-analysis of Randomized Clinical Trials.

Importance: In patients with space-occupying hemispheric infarction, surgical decompression reduces the risk of death and increases the chance of a favorable outcome. Uncertainties, however, still remain about the benefit of this treatment for specific patient groups.

Objective: To assess whether surgical decompression for space-occupying hemispheric infarction is associated with a reduced risk of death and an increased chance of favorable outcomes, as well as whether this association is modified by patient characteristics.

No benefits of hypothermia in patients treated with hemicraniectomy for large ischemic stroke.

Background Space-occupying middle cerebral artery brain infarcts are associated with the development of brain edema, which may lead to cerebral herniation and death despite early hemicraniectomy. Aims To evaluate the benefit of therapeutic hypothermia in patients with space-occupying cerebral infarction treated with hemicraniectomy within 48 h of stroke onset. Methods Patients aged 18-60 years with space-occupying cerebral infarction treated with hemicraniectomy within 48 h and hypothermia (33-34°C) were selected from a single university hospital between 2001 and 2010 (n = 53).

Long-term follow-up of patients undergoing capsule and double-balloon enteroscopy for identification and treatment of small-bowel vascular lesions: a prospective, multicenter study.

Background And Study Aims: Few data are available concerning the long-term outcome of patients treated endoscopically for bleeding small-bowel vascular lesions (SBVL). The aim of this study was to evaluate the risk of rebleeding after endoscopic therapy for SBVLs detected by video capsule enteroscopy (VCE). The secondary aim was to assess risk factors for rebleeding.

A case of homocystinuria due to CBS gene mutations revealed by cerebral venous thrombosis.

Background: Homocystinuria caused by cystathionine beta synthase (CBS) deficiency is most often diagnosed in childhood and has a variable expressivity. The most frequent abnormalities include intellectual disability, ectopia lentis, myopia, skeletal abnormalities or thromboembolism.

Objective: To report a case of homocystinuria unraveled by cerebral venous thrombosis (CVT).

Multicenter comparison of double-balloon enteroscopy and spiral enteroscopy.

Background And Aim: Spiral enteroscopy is a novel technique for small bowel exploration. The aim of this study is to compare double-balloon and spiral enteroscopy in patients with suspected small bowel lesions.

Methods: Patients with suspected small bowel lesion diagnosed by capsule endoscopy were prospectively included between September 2009 and December 2010 in five tertiary-care academic medical centers.

[Experts' recommendations for stroke management in intensive care: intracranial hypertension].

This article aims to describe the arguments underlying the experts' recommendations for management of stroke patients in the intensive unit, focusing on intracranial hypertension. This article describes the pathophysiology, diagnostic methods and therapeutic options for intracranial hypertension after stroke, including medical and surgical management.

Safety and efficacy of extractible self-expandable metal stents in the treatment of Crohn's disease intestinal strictures: a prospective pilot study.

Background: Endoscopic management of Crohn's disease (CD) intestinal strictures with balloon dilation is effective; however, recurrences are frequent and require further dilations or surgery. The use of extractible metallic stents may be as effective as balloon dilation with fewer recurrences. The aim was to investigate in a prospective pilot study the feasibility and clinical effectiveness of the use of extractible stents in the treatment of CD intestinal strictures.

Cerebral venous thrombosis in inflammatory bowel diseases: eight cases and literature review.

Background: While the association between inflammatory bowel diseases and thromboembolic events has long been evident, cerebral venous thrombosis in this context remains rare and underreported.

Methods: Among 351 consecutive patients with cerebral venous thrombosis collected in two neurology departments between 1997 and 2009, an analysis of patients with inflammatory bowel disease and a review of literature were performed.

Results: Eight patients had inflammatory bowel disease (6/287, 2/64), Crohn's disease in two, and ulcerative colitis in two.

[Hereditary episodic ataxia].

Introduction: Episodic ataxia (EA) designates a group of autosomal dominant channelopathies that manifest as paroxysmal attacks of imbalance and incoordination. EA conditions are clinically and genetically heterogeneous. Seven types of EA have been reported so far but the majority of clinical cases result from two recognized entities.

Accuracy of computed tomographic colonography in a nationwide multicentre trial, and its relation to radiologist expertise.

Objective: Reports on the accuracy of computed tomographic colonography (CTC) mainly involve series from expert institutions. The aims of this study were to assess CTC accuracy in a nationwide population and to relate it to radiologist performance in their initial training.

Design: Nationwide multicentre trial.

Multiple intra-abdominal venous thrombosis in ulcerative colitis: role of MDCT for detection.

Patients with ulcerative colitis are at increased risk for venous thrombosis. We report herein the case of a 28-year-old woman who developed multiple intraabdominal venous thrombosis, including partial Budd-Chiari syndrome in association with intracranial venous thrombosis and pulmonary embolism during the relapse of a known ulcerative colitis. Multidetector-row computed tomography (MDCT) allowed depiction of multiple intraabdominal sites of thrombosis including right and medial hepatic veins, left portal vein, splenic vein and left ovarian vein and demonstrated complete resolution of the multiple thrombi after anticoagulant therapy.

Carotid atherosclerotic markers in CADASIL.

Purpose: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a cerebral small vessel disease caused by mutations of the NOTCH3 gene. Marked variations in disease severity have raised the hypothesis that non-genetic factors may modulate the expressivity of the phenotype. The aim of the current study was to evaluate whether atherosclerosis, assessed by carotid duplex ultrasonography, is associated with variations in the clinical and MRI phenotype of CADASIL.

Clinical spectrum of type IV collagen (COL4A1) mutations: a novel genetic multisystem disease.

Purpose Of Review: This review dsecribes the clinical spectrum of a newly identified disorder related to COL4A1 gene mutations. COL4A1 encodes type IV collagen α1 chain, a crucial component of nearly all basement membrane including vasculature, renal glomerule and ocular structures.

Recent Findings: The human phenotypes are extremely variable between patients and between families, with disease onset as early as in the fetal period.

Obscure gastrointestinal bleeding: preliminary comparison of 64-section CT enteroclysis with video capsule endoscopy.

Objective: To retrospectively compare the diagnostic capabilities of 64-section CT enteroclysis with those of video capsule endoscopy (VCE) to elucidate the cause of obscure gastrointestinal bleeding.

Methods: Thirty-two patients who had 64-section CT enteroclysis and VCE because of obscure gastrointestinal bleeding were included. Imaging findings were compared with those obtained at double balloon endoscopy, surgery and histopathological analysis, which were used as a standard of reference.

Should decompressive surgery be performed in malignant cerebral venous thrombosis?: a series of 12 patients.

Background And Purpose: In malignant cerebral venous thrombosis (CVT) patients, emergency decompressive surgery has been suggested as a life-saving procedure. We report 12 patients with malignant CVT, among whom 8 underwent operation.

Methods: Retrospective study of 12 patients from 3 stroke units who had a malignant CVT as defined: (1) supratentorial cortical lesions attributable to superficial venous system thrombosis with or without sinus involvement; (2) with clinical (decreased consciousness and dilated pupils) or radiological signs of transtentorial herniation; (3) either at onset or after worsening despite heparin therapy.

Suspected anastomotic recurrence of Crohn disease after ileocolic resection: evaluation with CT enteroclysis.

Purpose: To determine the utility of computed tomographic (CT) enteroclysis for characterization of the status of the anastomotic site in patients with Crohn disease who had previously undergone ileocolic resection.

Materials And Methods: Written informed consent was prospectively obtained from all patients, and the institutional review board approved the study protocol. CT enteroclysis findings in 40 patients with Crohn disease who had previously undergone ileocolic resection were evaluated independently by two readers.

Diagnostic accuracy of esophageal capsule endoscopy versus conventional upper digestive endoscopy for suspected esophageal squamous cell carcinoma.

Background And Study Aim: A video capsule similar to that used in small-bowel capsule endoscopy is now available for esophageal exploration. The aim of our study was to compare the accuracy of upper endoscopy (esophageal gastroduodenoscopy [EGE]) with esophageal capsule endoscopy (ECE) in patients at risk of esophageal squamous cell cancer (SCC).

Patients And Methods: 68 patients at risk of SCC secondary to a history of head and neck neoplasia were included in this comparison of techniques for detecting SCC and dysplasia.

Do patients have any special medical or rehabilitation difficulties after a craniectomy for malignant cerebral infarction during their hospitalization in a physical medicine and rehabilitation department?

Objectives: To observe whether medical complications, the evolution of neurological disorders and dependence and/or the discharge destinations are different for patients treated by craniectomy for malignant cerebral infarction in the middle cerebral artery compared to patients treated medically for severe or malignant cerebral infarction in the same cerebral territory, during their hospitalization in a physical medicine and rehabilitation department.

Patients And Methods: This retrospective study compared patients treated by craniectomy for malignant cerebral infarction in the middle cerebral artery and patients treated medically for severe or malignant cerebral infarction in the same cerebral territory. Patients were paired according to age, lesion side and hospitalization period.

Sinking skin flap syndrome and paradoxical herniation after hemicraniectomy for malignant hemispheric infarction.

Background And Purpose: "Sinking skin flap" (SSF) syndrome is a rare complication after large craniectomy that may progress to "paradoxical" herniation as a consequence of atmospheric pressure exceeding intracranial pressure. The prevalence and characteristics of SSF syndrome after hemicraniectomy for malignant infarction of the middle cerebral artery are not well known.

Methods: We analyzed a prospective cohort of 27 patients who underwent hemicraniectomy for malignant middle cerebral artery infarction.

Unilateral carotid granulomatous arteritis and Crohn's disease.

Chronic idiopathic granulomatous arteritis of the large vessels - and, specifically, "Takayasu's arteritis" and "giant cell arteritis" - is an unusual condition that rarely leads to stroke and is only occasionally associated with Crohn's disease. We report here on a unique case of a 56-year-old man with a 25-year history of Crohn's disease who also had a 4-year history of recurrent right-sided ischaemic strokes and partial seizures, and a unilateral progressive retrograde occlusion of the right internal and common carotid arteries. Biopsies of the temporal and carotid arteries showed large-vessel granulomatous arteritis, with features of both giant cell and Takayasu's arteritis.

CT enteroclysis features of uncomplicated celiac disease: retrospective analysis of 44 patients.

Purpose: To describe the computed tomographic (CT) enteroclysis features of uncomplicated celiac disease (CD) and to determine the most indicative appearance of this condition by using a retrospective case-control study.

Materials And Methods: This study had institutional review board approval. The CT enteroclysis examinations of 44 consecutive patients with proved uncomplicated CD (21 men, 23 women; mean age, 44.

[Management of ileal stenosis in patients with Crohn's disease].

Stenosis is the most frequent complication during Crohn's disease. The lesion can be inflammatory, or due to a fibrosing or neoplastic process. The medical treatment with anti-inflammatory drugs is usually sufficient as first line treatment; fibrous lesions require endoscopic or surgical procedures while neoplastic lesions require surgery.