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עמוד בית
Thu, 18.07.24

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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.

February 2012
L. Nesher, K. Riesenberg, L. Saidel-Odes, F. Schlaeffer and R. Smolyakov
Background: The southern region of Israel has recently experienced an influx of African refugees from the Eastern Sub-Sahara desert area. These influxes led to a significant increase in incidence of tuberculosis (TB) in that region.

Objectives: To review the data of African refugees diagnosed with TB between January 2008 and August 2010 at a tertiary care regional hospital.

Results: Twenty-five TB cases were diagnosed, 22 of which presented with pulmonary TB, 3 with  extra-pulmonary TB (EPTB), and 7 with combined pulmonary and EPTB. Only one case had concomitant human immunodeficiency virus (HIV) infection and multidrug-resistant TB. Fifteen patients underwent extensive radiological investigations including chest, abdominal and spine computed tomography, 1 was reviewed by magnetic resonance imaging, and 9 underwent tissue biopsy. Eighteen patients were admitted as suspected TB and 4 as suspected pneumonia or pulmonary infiltrates that could have been defined as suspected TB. All 24 HIV-negative cases were sensitive to first-line drugs for TB except one case that was resistant to streptomycin and one to rifampicin. All patients responded well to first-line therapy. The average duration of hospitalization was 8.7 days (range 1–36). Following diagnosis 23 patients were transferred to a quarantine facility.

Conclusions: We identified overutilization of medical resources and invasive procedures. For African refugees from the eastern Sub-Sahara who were HIV-negative and suspected of having TB, a sputum acid-fast smear and culture should have been the primary investigative tools before initiating treatment with four drugs (first-line), and further investigations should have been postponed and reserved for non-responders or for patients for whom the culture was negative. Physicians should maintain a high index of suspicion for EPTB in this population.
N. Shoenfeld, O. Rosenberg, M. Kotler and P.N. Dannon

The phenomenon of hair pulling has been recognized for centuries, yet the true prevalence of trichotillomania (TTM) is largely unknown and the topic has been sparsely studied. TTM is classified as an impulse-control disorder despite much debate about its etiology. In this review we summarize the different hypotheses, including impulse-control disorders, obsessive-compulsive disorders, behavioral problems and addiction, and the appropriate treatment methods. The combination of selective serotonin reuptake inhibitors and antipsychotic medications are shown to be most effective. Treatment with anti-addiction drugs seems relevant. Further research is needed to increase our knowledge regarding the etiology of TTM.

N. Moustafa-Hawash, T. Smolkin, A. Ilivitzki, A. Zimberg-Bossira, A. Gildish, R. Gershoni-Baruch and I.R. Makhoul
January 2012
Ronit Lubetzky, MD, Galit Zaidenberg-Israeli, MD, Francis B. Mimouni, MD, Shaul Dollberg, MD, Eyal Shimoni, PhD, Yael Ungar, PhD and Dror Mandel, MD

Background: Human milk produced during prolonged lactation (> 1 year) is extraordinarily rich in fat and has a higher energy content than human milk produced during short lactation.

Objectives: To estimate the fatty acid (FA) profile of human milk and to test the hypothesis that the proportion of C12 and C14 (two dietary saturated FA known to most promote hypercholesterolemia) in human milk during prolonged lactation is similar to that in short lactation.

Methods: We conducted a cross-sectional study of 30 mothers of term infants lactating for more than 1 year as compared with 25 mothers of full-term infants who lactated for 2–6 months. Milk was collected by manual expression in mid-breastfeeding.

Results: The two groups did not differ in maternal height, weight, body mass index, diet, infant birth weight and gestational age, but mothers in the prolonged lactation group were significantly older. There was a significant correlation between lactation duration and C12 or C14. The percentage of all FA combined (except for C12 and C14) decreased significantly over time. In contrast, C12:0 and C14:0 combined increased significantly during lactation (R2 = 10.0%, P < 0.03).

Conclusions: Women who lactated for more than 1 year had higher C12 and C14 FA percentages in their milk than women who lactated for 2–6 months.

Michael B. Levy, MD, Michael R. Goldberg, MD, PhD, Liat Nachshon, MD, Elvan Tabachnik, MD and Yitzhak Katz, MD

Background: Most reports in the medical literature on food allergy mortality are related to peanuts and tree nuts. There is limited knowledge regarding these reactions and often only a partial medical history is described.

Objective: To record and characterize all known cases of mortality due to food allergy in Israel occurring during the period 2004–2011.

Methods: All cases of food allergy-related mortality that were known to medical personnel or were published in the Israeli national communications media were investigated. We interviewed the parents and, when feasible, physicians who treated the final event.

Results: Four cases of food-related mortality were identified: three cases were due to cow’s milk and one to hazelnut. All were exposed to a hidden/non-obvious allergen. All four had a history of asthma but were not on controller medications, and all had experienced previous non-life threatening accidental reactions. Three of the four patients had not been evaluated by an allergist, nor were they prescribed injectable epinephrine. The one patient who had been prescribed injectable epinephrine did not use it during her fatal anaphylactic attack.

Conclusions: Fatal reactions to cow’s milk and hazelnut but not to peanut are the only reported food mortality cases in Israel. Although these patients had previous reactions following accidental exposures, none had experienced a life-threatening reaction. Patients at risk are not adequately evaluated by allergists, nor are they prescribed and instructed on the proper use of injectable epinephrine. Cow’s milk should be considered a potentially fatal allergen.




 



 
December 2011
R. Dabby, M. Sadeh, O. Herman, L. Leibou, E. Kremer, S. Mordechai, N. Watemberg and J. Frand

Background: Myotonic dystrophy type 2 (DM2) is an autosomal dominant, multisystem disorder caused by a CCTG tetranucleotide repeat expansion located in intron 1 of the zinc finger protein 9 gene (ZNF9 gene) on chromosome 3q 21.3.

Objectives: To describe the clinical, electrophysiologic and pathologic findings in patients with myotonic dystrophy 2.

Methods: We evaluated 10 patients genetically, clinically and electrophysiologically during the years 2007 to 2008.

Results: All patients were of Jewish European ancestry. Among affected individuals, eight patients had symptoms of proximal muscle weakness, two had muscle pain, and two exhibited myotonia. On physical examination six patients had severe weakness of hip flexor muscles. Seven individuals underwent cataract surgery, and cardiac involvement was seen in one case. On the initial electromyographic (EMG) examination five patients demonstrated myotonic discharges; repeated studies showed these discharges in nine cases. Six muscle biopsies showed non-specific pathological changes. Seven patients had an affected first-degree relative with either a diagnosed or an undiagnosed muscular disorder, consistent with an autosomal dominant trait.

Conclusions: DM2 may often present with proximal muscle weakness without myotonia. EMG may initially fail to show myotonic discharges, but these discharges may eventually show in most cases on repeated EMG. Thus, DM2 may be underdiagnosed and should be included in the differential diagnosis of adult patients of Jewish European ancestry presenting with proximal lower limb weakness.
 

November 2011
E. Greenberg, I. Treger and J. Schwarz

Background: Little is known of the risk factor disparities in first stroke among Jewish and Arab patients undergoing rehabilitation in Israel.

Objectives: To investigate the age, gender and risk factor disparities in first stroke among Jewish (immigrant and non-immigrant) and Arab patients undergoing rehabilitation and to compare the prevalence and odds ratio of stroke risk factors in these patients.

Methods: The database of the Department of Neurological Rehabilitation C at Loewenstein Rehabilitation Center was used to investigate first ischemic and hemorrhagic stroke patients admitted for hospital rehabilitation over a 15 year period, January 1993 to December 2008. Particular attention was paid to age, gender and risk factor disparities.

Results: The 2000 patients with first stroke who were included in the study were grouped as Jewish (immigrant and non-immigrant) and Arab (237 Arabs, 370 non-immigrant Jews and 1393 immigrant Jews). A high percentage of Arab patients were found to have hypertension and diabetes mellitus, while a high percentage of Jewish immigrants had stenosis of the internal carotid artery.

Conclusions: The study demonstrated some differences in the effect of risk factors between the groups. It may be important to address such differences when developing stroke preventative strategies in this population of Jewish and Arab stroke survivors in Israel.
 

October 2011
T. Wolak, A. Belkin, V. Ginsburg, G. Greenberg, O. Mayzler, A. Bolotin, E. Paran and G. Szendro

Background: Percutaneous angioplasty (PTA) and stenting is an established procedure for the treatment of hypertension caused by atherosclerotic renal artery stenosis. However recently, the decision whether or not to perform this procedure has raised considerable debate.

Objectives: To examine the association between the basic clinical and radiological characteristics of candidates for renal artery PTA and the clinical outcome of the procedure in terms of improvement of blood pressure control and renal function.

Methods: We conducted a retrospective cohort study of all patients who underwent percutaneous transluminal renal artery angioplasty (PTRA) and stent implantation in a tertiary medical center during the period 2000–2007. The clinical and radiological data were extracted from the medical file of each patient. Blood pressure measurements and creatinine level were recorded before the procedure and 1 month, 6 months, 12 months and 18 months after PTRA.

Results: Thirty-two patients were included in the final statistical analysis. The mean age of the study population was 66.6 ± 8.8 years old and 75% were men. There was a significant reduction in both systolic and diastolic blood pressure 1 month after the procedure: 160.5 ± 24.7 vs. 141.8 ± 23.6 mmHg and 83.8 ± 12.9 vs. 68.8 ± 11.8 mmHg respectively (P < 0.001). The reduction in blood pressure was constant throughout the follow-up period and was evident 18 months after the procedure: 160.5 ± 24.7 vs. 135.0 ± 35.1 mmHg and 83.8 ± 12.9 vs. 71.3 ± 16.5 mmHg respectively (P < 0.001). However, no improvement in renal function was observed at any time during the follow-up period. We could not demonstrate an association between clinical or radiological features and the clinical outcome after PTRA.

Conclusions: Our findings show that PTRA[1] can be considered an effective procedure for improving blood pressure control in patients with atherosclerotic renal artery stenosis (ARAS) and resistant hypertension. This research, together with previous studies, strengthens the knowledge that the decline in glomerular filtration rate seen in many patients with ARAS is non-reversible and is not improved by PTRA.






[1] PTRA = percutaneous transluminal renal artery angioplasty


September 2011
I. Berger

Attention deficit hyperactivity disorder (ADHD) is among the most prevalent chronic health disorders affecting school-age children. The disorder is the subject of much debate for several reasons, the major one being the diagnostic process, which in some aspects is unstructured and can be relatively easily biased. The impact of undiagnosed or misdiagnosed ADHD on the lives of many children can be severe. Therefore, it is important to understand the complexities of the diagnostic procedure in ADHD, including the cultural bias effect, the limitations of the DSM-IV-TR definitions, the effect of comorbid conditions on the diagnostic process, the gene-environment interactions, and the need to compose an objective, more accurate, and generally accepted diagnostic battery of tests. This review addresses the diagnostic difficulties of ADHD and considers some steps that would make ADHD a more easily identifiable disorder.
 

E. Avitan-Hersh, G. Berger and R. Bergman
August 2011
B. Knyazer, J. Levy, E. Rosenberg, T. Lifshitz and I. Lazar
June 2011
J. Bishara, E. Goldberg, L. Madar-Shapiro, J. Behor and Z. Samra

Background: The rate of infection with Clostridium difficile colitis and its associated mortality have been increasing in the last decade. The molecular epidemiology of C. difficile in Israel has as yet not been studied.

Objectives: To screen for the existence of the 027 and 078 ribotypes and determine the longitudinal molecular epidemiology of the circulating clinical C. difficile isolates in a large hospital in central Israel.

Methods: Polymerase chain reaction (PCR) ribotyping was performed on C. difficile isolates obtained from hospitalized patients from November 2003 to May 2004 (first study period) and September 2009 (second study period). Isolates with PCR[1] ribotype patterns, unlike those of the available reference strains (078 and 027), were labeled with letters. Forty-six isolates from the first study period and 20 from the second were analyzed.

Results: PCR strain typing of C. difficile isolates yielded approximately 26 unique ribotypes. During the first study period, ribotype A and B accounted for 30% and 28%, respectively, whereas ribotype E and K accounted for 6.5% for each. During the second study period, ribotypes A, E and K disappeared, and the incidence of ribotype B decreased from 28% to 15%. One isolate (1/20, 5%) emerged during the second period and was identified as ribotype 027. Moxifloxacin resistance was found in 93% of ribotype A isolates, 81% of the ribotype B group, and in 44% of other ribotypes.

Conclusions: The predominant ribotypes circulating in our institution were diverse and changing. This is the first report on the emergence of the 027 ribotype in Israel.






[1] PCR = polymerase chain reaction


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