Israel Medical Association Journal

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.

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.

Hip arthroscopy is one of the most rapidly evolving arthroscopy techniques. It combines the benefits of a minimally invasive procedure and a short rehabilitation period. Improved instrumentation and technical skills have advanced our ability to accurately diagnose and treat various conditions. The role of this procedure continues to evolve with new indications that might change the outcome of degenerative joint disease of the hip joint. Hip arthroscopy is indicated for both traumatic and atraumatic intra and extra-articular conditions. Femoroacetabular impingement (FAI) is increasingly recognized as a disorder that can lead to progressive articular chondral and labral injury. A variety of arthroscopic techniques allows the treatment of labral and acetabular rim pathology as well as peripheral compartment femoral head-neck abnormalities. It is now recognized that labral tears that were traditionally treated with simple debridement are often associated with underlying FAI. Hip arthroscopy is also being used for surgery to the structures surrounding the hip as snapping hip syndromes, greater trochanteric pain syndrome, and arthroscopic repair of abductor tendon tears. Better understanding of the arthroscopic anatomy, improved operative techniques, lowered complication rate and objective outcome measuring tools will further define the optimal role of hip arthroscopy and improve its outcome.

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.

Beta blockers are a fundamental treatment in chronic heart failure (HF), yet concern and disagreement regarding their role in the treatment of decompensated HF and during hospitalization are common in clinical practice. This review summarizes the literature on various aspects of beta blocker treatment during acute and chronic decompensated HF. In recent years evidence has accumulated concerning the efficacy and tolerability of beta blockers in decompensated HF. Clinical analyses show that withdrawal of chronic beta blockade should be avoided when possible during hospitalization and that beta blocker therapy be initiated as soon as hemodynamic stability and a euvolemic state are achieved. This strategy may increase adherence to beta blockers after discharge and lower rehospitalization and mortality rates. We also discuss the various positive inotrope regimens (phosphodiesterase inhibitors, levosimendan, dobutamine) and their interactions with beta blockers in decompensated HF.

Background: Vitamin D is increasingly associated with the pathology of cognition and mental illness. Vitamin D receptors have been detected on neurons that regulate behavior.

Objective: To assess vitamin D serum concentrations in patients with major depression and schizophrenia as compared to healthy controls and to determine if a correlation exists between serum levels of vitamin D and disease activity.  

Methods: We recruited 50 patients with schizophrenia and compared them to 33 patients with major depression and 50 controls with no major psychopathology. The Positive and Negative Syndrome Scale (PANSS) for schizophrenia and the Hamilton Depression scale for depression were administered on the same day the blood samples were drawn. We used LIAISON® 25-OH vitamin D (DiaSorin) immunoassay to measure serum concentrations of 25-OH vitamin D.

Results: Lower serum vitamin D concentrations were detected among patients with schizophrenia (15.0 ± 7.3 ng/ml) compared to patients with depression (19.6 ± 8.3 ng/ml) and to controls (20.2 ± 7.8 ng/ml, P < 0.05). We found no correlation between disease activity, measured by the PANSS score, and vitamin D levels.   

Conclusions: Serum vitamin D levels were lower in patients with schizophrenia as compared to patients with depression and to healthy controls. No correlation was found between serum concentration and disease activity. Additional studies are needed to elucidate the role of vitamin D in the autoimmune mechanism and in the pathogenesis of schizophrenia.

Background: Measurements of adolescents who at birth were large (long and/or heavy) for gestational age are scant.

Objectives: To determine the correlation between birth length and weight in female and male neonates born long and/or overweight for gestational age, with their height and weight at age 17.

Methods: We reviewed the records of the Rabin Medical Center for birth data of 96 full-term neonates born long and overweight for gestational age (FT-lo,ow), 33 full-term neonates born long but with normal weight for gestational age (FT-lo,nw), 148 full-term neonates born overweight but with normal length for gestational age (FT-nl,ow), and 401 full-term neonates born with normal birth length and weight (FT- nl,nw).

Results: Neonates of both genders born long and overweight at birth (FT-lo,ow) were taller and heavier at age 17 years than those born FT-nl,nw: females: 167.8 ± 5.1 cm and 64.6 ± 10.3 kg vs. 162.6 ± 5.5 cm and 59.3 ± 11.1 kg (P < 0.001 for height and P = 0.026 for weight) and males: 182.4 ± 8.1 cm and 80.6 ± 20.4 kg vs. 174.5 ± 6.2 cm and 67.4 ± 12.3 kg (P < 0.001). The correlations between birth length and height at age 17 for both genders were statistically significant (P < 0.001), as were those between birth weight and the weight and body mass index (BMI) at age 17 for both genders (P < 0.001). There was no correlation between birth length and weight or BMI at age 17.

Conclusions: Full-term neonates of both genders born large for gestational age become tall adolescents and weigh more at age 17 than children with a normal birth length and weight.

Background: The prevalence and severity of the most troublesome symptoms in terminally ill patients are well known and have been studied in many settings. However, these symptoms change during the course of advanced disease.

Objectives: To evaluate the range and trajectory of symptoms in the final stage of life as measured a month prior to death.

Methods: Patients with an expected prognosis of less than 6 months were recruited for the study. Excluded were non-Hebrew or Russian speakers, and patients with a diagnosis of brain tumor or with cognitive impairment. A structured questionnaire was used to interview patients and their caregivers at home every 2 weeks until death. We present a comparison analysis of 45 patients who completed both interviews 2 and 4 weeks before death.

Results: There were five symptoms (fatigue, pain, reduced well-being, lack of appetite, somnolence) that were reported most frequently, occurring in more than 70% of the patients. Most of the symptoms showed a worsening trend towards death.

Conclusions: Assessing the presence and severity of symptoms as a guide to start or modify treatment is recommended. Knowledge of how symptoms change in the final stage of life could better assist in the management of resources and could help patients and their families in their final preparations.