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עמוד בית
Mon, 21.09.26

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June 2012
May 2012
March 2012
O.S. Cohen, I. Prohovnik, A. D. Korczyn, R. Inzelberg, Z. Nitsan, S. Appel, E. Kahana, H. Rosenmann and J. Chapman

Background: While myoclonus and ataxia are considered common in patients with familial Creutzfeld-Jakob disease (fCJD), other movement disorders are less prevalent.

Objectives: To systemically evaluate the frequency of extrapyramidal signs and movement disorders in patients with fCJD.

Methods: A detailed neurological examination, with special emphasis on movement disorders and extrpyramidal signs, was conducted in 43 consecutive symptomatic CJD patients (26 males and 17 females mean age 58.7 ± 8.9 yrs, range 43–77 years) carrying the E200K mutation in the PRNP gene.

Results: Limb or gait ataxia was noted in 38 patients (88%) (37 patients, 86%, had ataxia at presentation). Myoclonus was evident in 25/43 patients (58%) (21 patients, 49%, at presentation). In 95% of the patients (41/43) (37/43, 86% at presentation) at least one extrapyramidal sign throughout the disease course was noted, the most prevalent being rigidity (28/43, 65% of the patients and 22/43, 51% at presentation), followed by the glabellar sign (24/43, 56% of the patients and 22/43, 51% at presentation), bradykinesia (19/43, 44% and 15/43, 35% at presentation), dystonia (15/43, 35% 12/43, 28% at presentation) and tremor (13/43, 30% 12/43, 28% at presentation).

Conclusions: In this unique population of fCJD patients, myoclonus was less prevalent than previously reported while other extrpyramidal signs were common and occurred at a relatively early stage of the disease. The high prevalence of movement disorders can be added to other phenomena characteristic of this familial disorder among Libyan Jews. Whether this is attributable to the E200K mutation itself or to some other mechanism has still to be elucidated.

M. Schwartz and S. Sabetay

Dopaminergic neurons in the basal ganglia normally fire in a continuous manner, maintaining the striatal dopamine concentration at a relatively constant level. In Parkinson’s disease, dopaminergic treatment produces a discontinuous stimulation, inducing an intermittent pulsatile activation of the striatal receptors. Probably the oscillations in the dopamine level in the striatum contribute to the development of motor complications. Treatment with long-acting dopaminergic agents, or providing a more continuous dopaminergic effect in the striatum, has been associated with fewer clinical motor complications. This review describes the state of the art in the clinical approach to achieve the desired continuous dopaminergic stimulation, providing patients with the best clinical effect and probably minimal motor complications.

February 2012
L.V. Lage, J.F. de Carvalho, M.T.C. Caleiro, N.H. Yoshinari, L.M.H. da Mota, M.A Khamashta and W. Cossermelli

Background: Antibodies directed against endothelial cell surface antigens have been described in many disorders and have been associated with disease activity. Since the most prominent histopathologic feature in mixed connective tissue disease (MCTD) is the widespread and unique proliferative vascular lesion, our aim was to evaluate the frequency of anti-endothelial cell antibodies (AECA) in this condition.

Objectives: To evaluate the frequency of AECA in this disease and assess its clinical and laboratory associations.

Methods: Seventy-three sera from 35 patients with MCTD (Kasukawa’s criteria), collected during a 7 year period, were tested for immunoglobulins G and M (IgG and IgM) AECA by cellular ELISA, using HUVEC (human umbilical vein endothelial cells). Sera from 37 patients with systemic lupus erythematosus (SLE), 22 with systemic sclerosis (SSc) and 36 sera from normal healthy individuals were used as controls. A cellular ELISA using HeLa cells was also performed as a laboratory control method.

Results: IgG-AECA was detected in 77% of MCTD patients, 54% of SLE patients, 36% of SSc patients and 6% of normal controls. In MCTD, IgG-AECA was associated with vasculitic manifestations, disease activity and lymphopenia, and was also a predictor of constant disease activity. Immunosuppressive drugs were shown to reduce IgG-AECA titers. Since antibodies directed to HeLa cell surface were negative, AECA was apparently unrelated to common epitopes present on epithelial cell lines.

Conclusions: AECA are present in a large proportion of patients with MCTD and these antibodies decrease after immunosuppressive treatment.


 
November 2011
G. Vashitz, J. Meyer, Y. Parmet, Y. Henkin, R. Peleg, N. Liebermann and H. Gilutz

Background: There is a wide treatment gap between evidence-based guidelines and their implementation in primary care.

Objective: To evaluate the extent to which physicians "literally" follow guidelines for secondary prevention of dyslipidemia and the extent to which they practice "substitute" therapeutic measures.

Methods: We performed a post hoc analysis of data collected in a prospective cluster randomized trial. The participants were 130 primary care physicians treating 7745 patients requiring secondary prevention of dyslipidemia. The outcome measure was physician "literal" adherence or "substitute" adherence. We used logistic regressions to evaluate the effect of various clinical situations on “literal” and “substitute” adherence.

Results: "Literal" adherence was modest for ordering a lipoprotein profile (35.1%) and for pharmacotherapy initiations (26.0%), but rather poor for drug up-titrations (16.1%) and for referrals for specialist consultation (3.8%). In contrast, many physicians opted for "substitute" adherence for up-titrations (75.9%) and referrals for consultation (78.7%). Physicians tended to follow the guidelines “literally” in simple clinical situations (such as the need for lipid screening) but to use "substitute" measures in more complex cases (when dose up-titration or metabolic consultation was required). Most substitute actions were less intense than the actions recommended by the guidelines.

Conclusions: Physicians often do not blindly follow guidelines, but rather evaluate their adequacy for a particular patient and adjust the treatment according to their assessment. We suggest that clinical management be evaluated in a broader sense than strict guideline adherence, which may underestimate physicians' efforts.
 

A. Blum, C. Simsolo, R. Sirchan and S. Haiek

Background: The "obesity paradox" is defined as an inverse association of good health, survival and obesity. Usually in healthy persons the more obese you are the more metabolic complications you have; however, thin patients with chronic obstructive pulmonary disease (COPD) have more cardiovascular complications and a higher mortality rate.

Objectives: To explore whether atherosclerosis and peripheral artery disease (PAD) contribute to the higher morbidity and mortality of patients with COPD.

Methods: This prospective study included 87 patients with chronic COPD who were treated in the pulmonary outpatient clinic; all signed a consent form before enrollment. We documented their lung function (FEV1%), body mass index (BMI) and ankle brachial index (ABI). The primary endpoints were to find an association between atherosclerosis and BMI in patients with COPD, and between atherosclerosis and severity of lung disease.

Results: Average ABI[1] was 1.01 ± 0.20, BMI[2] was 29.33 ± 7.48 kg/m2, and the abdominal circumference was 107.34 ± 18.87 cm. A positive correlation was found between BMI and ABI (P = 0.001) and between abdominal circumference and ABI (P = 0.000). Patients with peripheral artery disease were older (73.6 ± 11.5 vs. 68.1 ± 11.6 years old, P = 0.04), were thinner (average BMI 25.5 ± 6.2 vs. 31.06 ± 7.3, P = 0.001), and had a lower abdominal circumference (97.7 ± 18.3 vs. 111.7 ± 17.5 cm, P = 0.001). No such difference was observed for years of smoking. Male PAD patients with COPD had a lower BMI (25.2 ± 5.6 vs. 29.9 ± 7.4, P = 0.016), and their abdominal circumference was smaller (96.1 ± 18.0 vs. 110.2 ± 16.5 cm, P = 0.004). Female PAD patients with COPD had a lower BMI (26.3 ± 8.2 vs. 33.1 ± 7.0, P = 0.045), but their abdominal circumference was not different from females without PAD (102.0 ± 19.7 vs. 114.0 ± 19.4 cm, P = 0.162). Patients with PAD had a worse lung disease (FEV1% 34 ± 8% vs. 45 ± 16%, P = 0.01). During the 1 year of follow-up five patients died: two PAD patients due to acute myocardial infarction and three non-PAD patients died from pulmonary insufficiency (two patients) and pulmonary emboli (one patient).

Discussion: We found that COPD patients with PAD were older and thinner and had a lower abdominal circumference and a more progressive lung disease. Extensive atherosclerosis in patients with COPD may partly explain the “obesity paradox” observed in patients with COPD.






[1] ABI = ankle brachial index



[2] BMI = body mass index


D. Rosengarten, M.R. Kramer, G. Amir, L. Fuks and N. Berkman

Pulmonary epithelioid hemangioendothelioma (PEH), previously known as "intravascular bronchoalveolar tumor," is a rare vascular malignancy with an unpredictable prognosis. Treatment can vary from observation in asymptomatic patients to surgery in patients with resectable disease or chemotherapy in patients with disseminated disease. This report describes the clinical, radiological and pathological features of three cases of PEH and a review of the current literature.
 

October 2011
R. Farah and N. Makhoul

Background: Exacerbations of chronic obstructive pulmonary disease (COPD) are a major problem worldwide and are usually the main indication for mechanical ventilation (MV), especially in the intensive care unit (ICU). The rate of weaning failure is also high and prolonged MV leads to complications of intubation. The goal is to wean these patients as soon as possible.

Objective: To determine the optimal time necessary to start the weaning process.

Methods: In an attempt to determine the length of MV and stay in the ICU, we compared the length of MV, weaning, re-intubations and discharge during a 10 month period. This study included 122 patients on MV due to severe exacerbation of COPD who were not suitable for non-invasive ventilation. For each patient serial arterial blood gases were measured at admission and during hospitalization. PeCO2 (mixed expired CO2) was tested using a Datex S/5 instrument at follow-up.

Results: The study population comprised all patients who required MV; of these 122, 108 were ventilated from 6 to 140 hours (average 48 ± 42), 9 needed more than 168 hours, and 5 died due to severe ventilation-associated pneumonia. No correlation was found between pH, PCO2 and length of MV; these findings did not contribute to evaluation of the patient’s condition nor did they enable us to predict the length of treatment necessary.

Conclusion: Most of the patients (93%) ventilated for acute respiratory failure due to COPD required MV for only 6–90 hours.

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