Markedly asymmetric presentation in multiple system atrophy

BackgroundMultiple system atrophy (MSA) presents with fairly symmetrical, levodopa unresponsive parkinsonism and additional features like autonomic dysfunction, cerebellar and corticospinal tract involvement. Marked asymmetry in atypical parkinsonism suggests alternative diagnosis like Corticobasal syndrome (CBS).MethodsWe describe five unusual cases, who presented initially with markedly asymmetric parkinsonism, rigid dystonic abnormal limb posturing and subsequently developed clinical and/or radiological features consistent with probable MSA-P.ResultsUsing the internationally accepted diagnostic criteria, the patients fulfilled the diagnostic criteria for probable MSA-P after 5 years from disease onset. Case 4 and 5 had characteristic MRI features and Case 2 was pathologically confirmed.ConclusionsWe use these cases to highlight that MSA-P MSA-P can present rarely with very marked asymmetry, dystonic limb and myoclonic jerks leading to a diagnosis of CBS at onset.     

Systems Biology and Biomechanical Model of Heart Failure

Heart failure is seen as a complex disease caused by a combination of a mechanical disorder, cardiac remodeling and neurohormonal activation. To define heart failure the systems biology approach integrates genes and molecules, interprets the relationship of the molecular networks with modular functional units, and explains the interaction between mechanical dysfunction and cardiac remodeling. The biomechanical model of heart failure explains satisfactorily the progression of myocardial dysfunction and the development of clinical phenotypes. The earliest mechanical changes and stresses applied in myocardial cells and/or myocardial loss or dysfunction activate left ventricular cavity remodeling and other neurohormonal regulatory mechanisms such as early release of natriuretic peptides followed by SAS and RAAS mobilization. Eventually the neurohormonal activation and the left ventricular remodeling process are leading to clinical deterioration of heart failure towards a multi-organic damage. It is hypothesized that approaching heart failure with the methodology of systems biology we promote the elucidation of its complex pathophysiology and most probably we can invent new therapeutic strategies.     

Role of orthoptics and scoring system for orbital floor blowout fracture: surgical or conservative treatment

AIM: To assess the role of orthoptics in referring patients with orbital floor blowout fracture (OFBF) for conservative or surgical treatment and based on the results, to propose a scoring system for such decision making. METHODS: A retrospective analysis of 69 patients with OFBF was performed (35 treated conservatively, 34 surgically). The role of orthoptics in referring to surgery or conservative treatment was retrospectively evaluated, the factors with the highest significance for decision making were identified, and a scoring system proposed using Logistic regression. RESULTS: According to defined criteria, the treatment was unsuccessful in 2 (6%) surgically treated and only in one (3%) conservatively treated patient. The proposed scoring system includes the defect size and several values resulting from the orthoptic examination, the elevation of the eyebulb measured on Lancaster screen being the most significant. CONCLUSION: The study demonstrates the benefits of orthoptic examination when making decisions on conservative or surgical treatment and for diagnosing ocular motility disorder (with or without binocular diplopia) in OFBF patients. The proposed scoring system could, following verification in a prospective study, become a valuable adjunctive tool.     

Long-term survival of the uncemented Balgrist total hip replacement cup

Purpose

The purpose of this study was to evaluate the long-term clinical and radiographic outcomes of the Balgrist total hip replacement cup.

Methods

We report the results of a retrospective review of 195 total hip prostheses with Balgrist cup implanted in 178 patients. There were 59 men and 119 women with a mean age at surgery of 52.3 years (range, 19–72). Cox regression was used to determine the influence of independent variables on the survivorship of the prosthesis.

Results

There were 117 (66 %) of 178 patients, and 131 (67 %) of 195 hips available for assessment at a mean follow-up of 17.4 years. A total of 21 patients (22 hips) were lost from follow-up (12 %). A total of 25 patients (27 hips) required some type of re-operation during the follow-up period. Eight of 27 re-operations (30 %) were directly caused by the Balgrist cup failure due to fatigue fracture of the metallic shell (n = 5; 19 %), polyethylene wear (n = 2; 7 %) and aseptic loosening (n = 1; 4 %). The mean polyethylene wear rate was 0.068 mm/year (range, 0.008–0.230; SD = 0.043). The risk of re-operation for any reason was decreased with dysplastic hip as a primary diagnosis (hazard ratio; HR = 0.250; 95 % CI 0.086–0.725) and with greater age at the time of surgery (HR = 0.923; 95 % CI 0.880–0.969). The 19-year survivorship with re-operation for any reason was 75.6 % (95 % CI 67.0–84.2). The 19-year survivorship with re-operation for the Balgrist cup failure was 90.5 % (95 % CI 83.4–97.6).

Conclusions

The Balgrist cup continues to provide excellent clinical and radiological outcomes. This is associated at least in part with a low polyethylene wear rate. The main reason for the Balgrist cup failure is fatigue fracture of the metallic shell.     

Discriminating value of total minor physical anomaly score on the Waldrop scale between patients with bipolar I disorder and normal controls

Minor physical anomalies (MPAs) are slight structural aberrations indicative of abnormal neurodevelopment. Most studies of MPAs in bipolar disorder have yielded limited results. We attempted to assess the potential value of MPAs as a classifying test in the status bipolar I patients vs. normal controls. Sixty one bipolar I patients and 103 controls were evaluated for MPAs using a slightly modified version of the Waldrop scale. The specificity, sensitivity and predictive value of different total MPA (MPA-T) scores were determined. The cut-off MPA-T scores that optimally discriminated patients from controls (exhibiting the most balanced sets of sensitivity, specificity, positive and negative predictive values) were MPA-T≥4 and MPA-T≥5. These values set a “border zone” in which bipolar I patients began to prevail significantly over controls. The latter presented most frequently with MPA-T ≤3 and rarely with MPA-T ≥6. Bipolar I patients prevailed among outliers (subjects with significantly higher MPA-T scores). Our data establish MPA-T score as a reliable index in distinguishing between bipolar I patients and normal controls and are consistent with the hypothesis of abnormal neurodevelopment in bipolar disorder.     

Neonatal lipopolysaccharide treatment has long‐term effects on monoaminergic and cannabinoid receptors in the rat

Brain inflammation in early life has been proposed to play important roles in the development of anxiety and psychosis‐related behaviors in adulthood, behaviors that rely on the integrity of dopamine and/or serotonin systems. Moreover recent behavioral and anatomical evidence suggests involvement of CB1 receptors in the control of emotion and mood. In this study, we determined the effects of neonatal LPS treatment on dopamine, serotonin, and cannabinoid receptor binding in adulthood. Rats were treated with the bacterial endotoxin lipopolysaccharide (LPS) on postnatal day (PND) 3 and 5. Dopamine D1, D2, serotonin 5HT1A, 5HT2A, and serotonin transporter and cannabinoid CB1 receptor binding across several brain regions were measured autoradiographically in adulthood (PND 85). Neonatal LPS treatment caused a significant increase in dopamine D2 in the nucleus accumbens and olfactory tubercle, a decrease in 5HT1A receptor binding in the hippocampus CA1 and ventromedial hypothalamus. A decrease in CB1 receptor binding after neonatal LPS was observed in the amygdala. Neonatal LPS had no significant impact on dopamine D1, serotonin 5HT2A or serotonin transporter binding in any of the brain regions examined. Our results suggest long lasting, region specific effects and differential impact on dopamine, serotonin and cannabinoid receptor systems following neonatal inflammation, that may form the basis for compromised anxiety and psychosis related behaviors. Synapse, 2013. © 2013 Wiley Periodicals, Inc.