Athetoid Movements as Initial Manifestation of Primary Sjögren Syndrome.

Background: Primary Sjögren syndrome (pSS) is an autoimmune disorder characterized by exocrine gland and extraglandular symptoms. We present a case report of pSS with an initial presentation of athetoid movements. Case Report: A 74-year-old female presented with a 2-month history of slow undulating movements in her trunk and thighs that eventually spread to her neck and lower extremities. She also reported dry eyes, dry mouth, as well as pain in her shoulders and thighs. Her proinflammatory markers and rheumatologic profile were positive. Her salivary gland biopsy revealed a Focus score > 2. Brain magnetic resonance imaging was normal. A diagnosis of pSS was made. The patient's symptoms improved with hydroxychloroquine, pilocarpine, gabapentin, and clonazepam. Discussion: Clinicians should consider and screen for primary autoimmune disorders as a cause of subacute athetoid movements in elderly patients. Although aggressive treatment has been recommended, treatment should be tailored to each patient's specific needs.

Altered CSF cytokine network in amyotrophic lateral sclerosis patients: A pathway-based statistical analysis.

INTRODUCTION: Increased cytokine expression is a prominent finding in amyotrophic lateral sclerosis (ALS). Due to their interdependence and pleiotropism, interpretation of CSF concentrations of a single cytokine is challenging. We describe a cytokine analysis in ALS patients using a pathway-based statistical method to identify changes in the whole cytokine network. METHODS: We analyzed 19 cytokines in CSF of ALS patients and controls. An equality of concentration matrices was conducted that allowed us to evaluate disturbances in the relationship of cytokines between controls and ALS patients with less and more than 12months of disease length. MANOVA assessed differences in cytokines and interdependence was compared by partial correlations among specific pairs of cytokines. RESULTS: Seventy-seven ALS patients and 13 control subjects were included. Significant differences were identified in the cytokine pathway between controls and ALS patients with less (p<0.0001) and more than 12months of disease length (p<0.0001), and between ALS patients with less than 12months and those with more than 12months (p=0.0058). In ALS patients with a shorter disease length, IL4 and IL6 were negatively correlated (-0.3571), whereas in ALS>12months, a positive correlation was detected (0.4080). CONCLUSIONS: The pathway-based statistical method revealed remarkable variations in the whole cytokine pathway in ALS patients and controls. Cytokine-network changes and the positive correlation between IL4 and IL6 were related to disease length; these variations might explain the deleterious immunological effects on motor neurons. A further analysis using this method is needed to confirm the exact interaction among cytokines in ALS.

Higher doses of CD34+ progenitors are associated with improved overall survival without increasing GVHD in reduced intensity conditioning allogeneic transplant recipients with clinically advanced disease.

OBJECTIVE: The influence of CD34+ cell dose on the outcome of allogeneic peripheral blood stem cell (PBSC) transplantation after reduced intensity conditioning (RIC) remains controversial. The impact of the number of CD34+ hematoprogenitors infused on transplant outcome and on the incidence of graft versus host disease (GVHD) was analyzed. MATERIALS AND METHODS: Data of 138 patients with advanced hematological diseases who received an allogeneic PBSC transplant after RIC were analyzed. Donors were mobilized with granulocyte colony-stimulating factor and underwent one to three apheresis procedures. Incidence of acute and chronic GVHD and overall and event-free survival (OS and EFS) was determined. RESULTS: The median number of CD34+ cells infused was 5.57 × 10(6) kg(-1) (range: 1.1-15.6). There was no relationship between CD34+ cell dose and neutrophil or platelet engraftment. Patients receiving ≥5 × 10(6) kg(-1) CD34+ cells had a 63.1% 5-year OS when compared with 48.2% for those receiving a lower number (P = 0.024). At 5-year follow-up, there was no significant difference in EFS between the groups (44% vs. 42.8%, P = 0.426). No relationship between CD34+ cell dose and acute GVHD was found (P = 0.1). Relapse rate was the same in patients with and without acute GVHD (P = 0.117). A nonsignificant improvement on OS and EFS in patients who developed chronic GVHD was found (P = 0.57 and 0.41). CONCLUSION: A CD34+ cell dose ≥5 × 10(6) kg(-1) was associated with a significantly higher OS, but no improved EFS in high-risk patients. The number of CD34+ progenitors infused had no influence on the incidence of acute or chronic GVHD.