bluuebunny/crossref_metadata_2025 huggingface dataset:
A One-Year Prospective Study on Back Pain Among Novice Golfers
Authors: Burdorf Alex , Van Der Steenhoven George A. , Tromp-Klaren Eveline G. M.
Published: June 2007

We conducted a 1-year follow-up study on back pain among 196 men taking up golf. A questionnaire on individual characteristics, occupation, sports, and back pain was distributed at the start of the study and was followed by another questionnaire after 12 months. Eleven percent of the original subjects (25 of 211) were lost to followup, but withdrawal from the survey was not associated with health status. In the baseline survey ( N = 221), the self-reported lifetime cumulative inci dence of back pain was 63%; 28% reported back pain during the month before answering the questionnaire, and 13% reported current back pain. Athletes had an increased odds ratio of 2.1 (95% confidence interval, 1.1 to 4.0) for previous back pain. During the 12 months between the surveys, the incidence of first-time back pain was 8% and the incidence of recurrent back pain was about 45%. Men involved in golf and another sport demonstrated a risk of 1.4 for recurrence of back pain, compared with men playing golf only. Six sub jects attributed the recurrent back pain to playing golf. Given the high proportion of athletes in this study (68%), the risk factor of playing golf failed to demon strate an additional significant effect on the general relationship between sport and back pain.
Comparison of an Optical Catheter Office Arthroscope with a Standard Rigid Rod-Lens Arthroscope in the Evaluation of the Knee
Authors: Meister Keith , Harris N. Lindsay , Indelicato Peter A. , Miller Gary
Published: June 2007

In a prospective, randomized study, 47 patients under went arthroscopic evaluation of the knee in an operat ing room setting with both a standard rod-lens arthro scope and a newer flexible optical catheter fiberoptic system. The goal of the study was to assess the diag nostic accuracy of the newer catheter system, which is recommended for use in the office setting. Forty-four patients were included in the data analysis, three were eliminated because we were unable to perform an adequate examination with the catheter scope second ary to intraarticular adhesions or excessive bleeding. A comparison of the two systems revealed an overall underestimation and underrecognition of intraarticular knee pathologic changes. Anterior cruciate ligament tears were missed in 3 of 21 knees; no posterior cru ciate ligament tears were detected by the rod-lens arthroscope in 44 knees, but one false-positive result was "seen" using the optical catheter device. Similar trends in diagnosis (sensitivity, 25% to 67%; specificity, 96% to 99%) were seen for tears of the medial and lateral menisci, chondral lesions, and the identification of loose bodies. We conclude from the results of this study that the use of the optical catheter system for arthroscopic evaluation and treatment of the knee in the office setting may result in a significant compro mise in visual acuity, resulting in missed and incorrect diagnoses.
Upper Extremity Blood Flow in Collegiate and High School Baseball Pitchers
Authors: Bast Steven C. , Perry John R. , Poppiti Roberta , Vangsness C. Thomas , Weaver Fred A.
Published: June 2007

The arterial and venous volume blood flow in the dom inant and nondominant upper extremities of five male pitchers, ages 16 to 21, was measured using color flow duplex ultrasound. Blood-flow measurements were ob tained at baseline, after warmup, and after each se quence of 20 pitches until 100 pitches were thrown. Blood flow was additionally determined 1 hour after the last pitch. The velocity of each pitch was recorded with a speed gun. Anthropomorphic measurements of the upper extremity were obtained at baseline and imme diately after Pitch 100 using a standard measuring tape. The highest average arterial volume flow in the pitching arm occurred after 40 pitches, reaching a peak of 549 ml/min (56% increase from baseline). Thereaf ter, the average arterial blood flow steadily declined, reaching an average of 402 ml/min after the 100th pitch (14% increase from baseline). In contrast, the arterial blood flow in the nonpitching arm increased only slightly from baseline, reaching a maximal volume flow of 448 ml/min immediately after the warm-up period (10% increase from baseline). The volume flow then persistently fell to a level 30% below baseline after the 100th pitch. Although this small pilot study does not demonstrate causation between a decline in pitching performance and arterial blood flow, it suggests arterial flow in the dominant extremity falls as the pitch count increases.
Long-Term Functional Outcome After Primary Repair of the Lateral Ligaments of the Ankle
Authors: Kaikkonen Auvo , Hyppänen Ere , Kannus Pekka , Järvinen Markku
Published: June 2007

We retrospectively reexamined a total of 100 patients 6 to 8 years after primary repair of ruptured lateral liga ments of the ankle. Fifty-nine percent of the injuries had occurred in sports; the other main causes of the injuries were accidents at work (12%), in traffic (10%), and at home (4%). Almost two-thirds of the sports injuries were sustained in volleyball. The most com mon type of injury was a combined rupture of the anterior talofibular and the calcaneofibular ligaments (66 patients). According to subjective assessment, 74 patients had excellent or good results. For the anterior drawer sign test, 75 patients showed no sign of insta bility in their injured ankles. The scores in our perfor mance test protocol of ankle injuries were classified as excellent or good in 65 patients, fair in 27 patients, and poor in 8 patients. The overall long-term results were acceptable in the majority of the patients. Prospective, randomized studies are needed to clarify if nonopera tive treatment (i.e., early controlled mobilization) would give similar long-term results.
Patellofemoral Pain Syndrome
Authors: Arroll Bruce , Ellis-Pegler Elaine , Edwards A. , Sutcliffe Grant
Published: June 2007

Many therapies have been advocated for treating patellofemoral pain, which suggests little consensus on optimal treatment. We reviewed the high-quality evi dence for successful treatment of patellofemoral syn drome based on successful outcome information. To achieve this goal, we undertook a systematic search and critical appraisal of the literature on patellofemoral pain syndrome. Our definition of patellofemoral pain syndrome was broad and included patients with carti lage damage. We found five randomized controlled trials and some follow-up studies. The prognoses for most new cases of patellofemoral pain syndrome are good, although a proportion of patients with this syn drome will have persistent symptoms. Quadriceps muscle exercises were effective in treating this condi tion, and knee braces were not. Both prostheses and intramuscular glycosaminoglycan polysulfate had en couraging results for patients; however, these results need confirmation. There were many studies of biome chanics, which indicates that there is an assumption that an alteration of abnormal biomechanics would result in clinical benefit. Studies are needed that place more emphasis on the therapeutic benefit. There is limited evidence on which to base therapy, and there needs to be more high-quality research. Studies need to be longer, account for factors that predispose the patients, and have a more standardized means of assessing outcomes.
Epidemiology of Anterior Cruciate Ligament Injuries in Soccer
Authors: Bjordal Jan M. , Arnøy Frode , Hannestad Birte , Strand Torbjørn
Published: June 2007

We did a retrospective study of all anterior cruciate ligament injuries (972) verified by arthroscopic evalu ation at hospitals in the Hordaland region of Norway from 1982 to 1991. Our final study group comprised 176 patients who had participated in organized soccer and answered a questionnaire. The overall incidence rate was 0.063 injuries per 1000 game hours. Men incurred 75.6% (133) of the injuries. Women had an incidence rate of 0.10 injuries per 1000 game hours, significantly higher than that for men (0.057). The inci dence rate was higher (0.41) for men in the top three divisions. Most of the injuries (124) occurred during games. Contact injuries from tackling was the injury mechanism in 46.0% of the cases. Players on the offensive team incurred 122 (69.3%) of the injuries. Reconstructive surgery was performed on 131 (74.4%) of the injured players and was found necessary for return to a high level of play. Half of the players (87) returned to soccer; men at high levels of play had the highest return rate (88.9%), and men over age 34 had the poorest return rate (22.9%). Nearly one-third of the injured athletes gave up soccer because of poor knee function or fear of new injury.
Lumbar Mobility and Low Back Pain During Adolescence
Authors: Kujala Urho M. , Taimela Simo , Oksanen Airi , Salminen Jouko J.
Published: June 2007

In this 3-year longitudinal study, we studied lumbar mobility and the occurrence of low back pain among 98 adolescents who were free of previous severe low back pain: 33 nonathletes (16 boys, 17 girls), 34 boy athletes (ice hockey and soccer players) and 31 girl athletes (figure skaters and gymnasts). During the fol lowup, low back pain lasting longer than 1 week was reported by 29 athletes (15 boys and 14 girls) and by 6 nonathletes (3 boys and 3 girls). In multivariate analy ses, participation in sports and low maximal lumbar flexion at the baseline predicted low back pain during the followup among boys; however, these factors ac counted for only 16% of the variability between the groups with and without low back pain. Among girls, decreased range of motion in the lower lumbar seg ments, low maximal lumbar extension, and high body weight at the baseline were predictive of low back pain during the followup, accounting for 31 % of the variabil ity between the groups. The girls in the lowest tertile of maximal lumbar extension at baseline had a relative risk of 3.4 to have future low back pain compared with those in the highest tertile. We conclude that the low individual physiologic maximum of lower segment lum bar extension mobility may cause overloading of the low back among athletes involved in sports with fre quent maximal lumbar extension and that it predicts future low back pain.
The Effect of a Wrist Brace on Injury Patterns in Experimentally Produced Distal Radial Fractures in a Cadaveric Model
Authors: Moore Milan S. , Popovic Neven A. , Daniel Joseph N. , Boyea Steven R. , Polly David W.
Published: June 2007

We compared patterns of bony and ligamentous injury with distal radial fractures in braced and unbraced wrists using 20 paired fresh cadaveric upper extremi ties. A commercially available wrist brace was placed on one wrist in each pair. Specimens were then placed in a fast-loading gravity-driven device and subjected to loads averaging 16 kg from an average height of 78 cm. Postfracture radiographs were obtained, the spec imens were dissected, and fracture patterns and liga mentous integrity were assessed. The following frac ture types were produced: distal radial fractures (eight unbraced, seven braced) and intraarticular (seven un braced, four braced). Radiographically, seven un braced wrists demonstrated carpal bone fracture and one braced wrist demonstrated carpal fractures. Eight unbraced and three braced wrists sustained carpal intrinsic ligament injuries, four unbraced and one braced wrists demonstrated extrinsic ligament injuries. More capsular tears occurred in the unbraced group ( N = 8) than in the braced group ( N = 1). This model demonstrated a difference in the patterns of injury in unbraced and braced wrists subjected to the same mechanical conditions, which suggests that use of a wrist brace may alter patterns of wrist injury.
A Comparison of Results in Acute and Chronic Anterior Cruciate Ligament Ruptures of Arthroscopically Assisted Autogenous Patellar Tendon Reconstruction
Authors: Noyes Frank R. , Barber-Westin Sue D.
Published: June 2007

We conducted a prospective study of 94 consecutive patients who received a patellar tendon autograft for anterior cruciate ligament rupture. Eighty-seven pa tients (93%) returned for followup a mean of 28 months postoperatively; 57 had chronic and 30 had acute or subacute ruptures. There were no significant differ ences between the subgroups for age, sex, articular cartilage lesions, or months of followup. Forty-six me niscal tears were repaired; 27 of these extended into the central avascular region. Rehabilitation empha sized immediate knee motion, but strenuous activity was delayed for at least 4 months. Only one patient had a knee motion complication, and stability (<3 mm, KT-2000 arthrometer, 134 N) was restored in 85% of knees with chronic ruptures and 92% of knees with acute ruptures. Earlier reconstruction should be con sidered in active persons as symptoms and limitations continued postoperatively in knees with chronic rup tures, leading to overall less satisfactory results. In patient rating of the overall knee condition, 69% of knees with chronic ruptures and 100% of knees with acute ruptures scored in the normal or very good range. Repair of meniscal tears that extend into the central avascular region should be considered, as 24 of the 27 (89%) menisci repaired showed clinical evi dence of healing and did not require reoperation.
Lower Extremity Arterial Disease in Sports
Authors: Abraham Pierre , Chevalier Jean-Michel , Leftheriotis Georges , Saumet Jean-Louis
Published: June 2007

The recent description of exercise-induced intimal fi brosis affecting mainly the iliac artery (and therefore usually described as external iliac artery endofibrosis) has dramatically changed the diagnostic approach of unexplained recurrent lower limb exercise pain, espe cially in cyclists. Because arterial disease is often as sociated with the aftereffect of various concomitant musculotendinous lesions, several months may pass before an arterial origin is suspected. The arterial origin of the pain must not be eliminated on normal ankle-to- arm index or normal Doppler velocity profiles at rest. Ultrasound examinations taken at rest may show the lesions in 80% of endofibrotic patients and allow for the diagnosis of popliteal entrapment syndrome during dorsiflexion of the foot. However, the hemodynamic consequences of a stenosis on the aortoiliofemoral axis can only be proved by measurement of the ankle- to-arm index after exercise. A cutoff of this index <0.5 provides an 85% sensitivity in the detection of endofi brosis. Invasive investigations (arteriography or an gioscopy) will confirm the diagnosis before surgery is discussed. Although long-term results in endofibrosis are unknown, most of the surgically treated patients return to competition.
Anterior Cruciate Ligament Reconstruction with Autogenous Patellar Tendon Graft in Patients with Articular Cartilage Damage
Authors: Noyes Frank R. , Barber-Westin Sue D.
Published: June 2007

To test if anterior cruciate ligament reconstruction with autogenous patellar tendon can alleviate symptoms and functional limitations and increase activity levels in patients with advanced articular cartilage damage, we looked at 53 patients with arthroscopically documented cartilage damage. In this group, a mean of 7.5 years had elapsed between the original injury and the recon struction, and 90 prior operative procedures had been done. Postoperatively, all patients had immediate mo tion and early functional rehabilitation. The results were assessed with the Cincinnati Knee Rating Sys tem. At followup (mean, 27 months), significant im provements were found for pain, swelling, giving way, functional limitations with daily and sports activities, and the overall rating score. Forty-two patients (79%) had returned to some type of athletic activity. Only three patients (6%) had failed results. In the patients' own ratings of the overall knee condition, 8 of 51 (16%) rated their knees as normal, 28 (55%) as very good, 7 (14%) as good, 5 (10%) as fair, and 3 (6%) as poor. We concluded that the majority of patients benefited from the arthroscopically assisted anterior cruciate ligament reconstruction because it decreased episodes of giving way with daily activities and increased activity without aggravating the preexisting arthrosis.
Delayed Diagnosis of Fatigue Fractures in the Elderly
Authors: Carpintero Pedro , Berral Francisco J. , Baena Paulino , Garcia-Frasquet Angeles , Lancho Jose L.
Published: June 2007

A retrospective study was made of 30 stress fractures in elderly patients engaged in noncompetitive sporting activities, mostly marching and running. Fractures were located exclusively in the lower limbs. In many cases the diagnosis was delayed and in several cases was incorrect because the stress fracture was mis taken for other conditions. Given that the number of senior citizens engaged in active life and sporting ac tivities is constantly growing, physicians should be aware of this type of fracture to ensure early diagnosis and appropriate treatment.
The Strain Behavior of the Anterior Cruciate Ligament During Squatting and Active Flexion-Extension
Authors: Beynnon Bruce D. , Johnson Robert J. , Fleming Braden C. , Stankewich Charles J. , Renström Per A. , Nichols Claude E.
Published: June 2007

The effects of weightbearing (closed kinetic chain) and nonweightbearing (open kinetic chain) exercises on the biomechanical behavior of an injured anterior cru ciate ligament or a healing anterior cruciate ligament graft are unknown. To understand the effects of these exercises on the healing graft, we measured the strain behavior of the normal anterior cruciate ligament in human subjects while they performed squatting, a closed kinetic chain exercise, and active flexion-exten sion of the leg, an open kinetic chain exercise. The maximum anterior cruciate ligament strain values ob tained during squatting did not differ from those ob tained during active flexion-extension. Also, anterior cruciate ligament strain values obtained during squat ting were unaffected by the application of elastic resis tance intended to increase muscle activity. These find ings indicate that squatting, which produces a substantial compressive joint force, does not necessar ily protect the anterior cruciate ligament more than active flexion-extension of the leg, which is character ized primarily by contraction of the dominant quadri ceps muscle. These findings also demonstrate that increasing resistance during the squat exercise does not produce a significant increase in anterior cruciate ligament strain values, unlike increased resistance dur ing active flexion-extension exercise.
Environmental Implications of International Trade and Uneven Development: Toward a Critique of Environmental Economics
Authors: Liodakis George
Published: June 2007

The main premises and the environmental implications of neoclassical trade theory and environmental economics are put under Marxist scrutiny. An attempt is then made to develop an alternative Marxist approach in investigating the specific significance of the capitalist mode of production and, more specifically, the environmental implications of international trade and uneven development. As argued, environmental degradation is largely the result of capitalist development and not of "underdevelopment" or poverty in Southern countries. It is further argued that international trade is ruled by absolute and not comparative advantage. This has important environmental implications insofar as the non-optimal effects of specialization, in terms of resource use, and the imbalancing effects of free trade tend to exacerbate the environmental problem.
Veblen's Economic Theory: A Radical Analysis
Authors: Mouhammed Adil H.
Published: June 2007

Veblen is a systematic economic theorist whose economic views run in parallel with those of his contemporaries. Also, his economic analysis is important for Marx's system. Veblen's economic theory as analyzed here consists of four basic components: reserve capacity, markup pricing, productivity, and aggregate demand; these elements constitute a coherent structure of economic analysis containing both microeconomics and macroeconomics used not only to analyze capitalism but to replace it. It is also argued that Veblen's economic theory is radical, because it utilizes social conflict as the driving force for social evolution.
Neoliberalism and International Financial Instability
Authors: Wolfson Martin H.
Published: June 2007

This paper attempts to expand upon a Minskian model of domestic financial crises to take account of the following international developments: capital inflows that increase financial fragility, foreign financing of deregulated domestic speculative markets, overcapacity in export markets, capital outflows leading to collapsing currencies, restrictive macroeconomic policy oriented toward boosting international investor confidence, and International Monetary Fund bailout packages in the wake of financial crises.

The paper concludes that the institutional changes in the international financial system responsible for the developments listed above came about as a result of the policies and strategies of neoliberalism.
Going global: differential accumulation and the great U-turn in South Africa and Israel
Authors: Nitzan Jonathan , Bichler Shimshon
Published: June 2007

This paper offers a new theoretical approach for comparing the current political-economic U-turns in South Africa and Israel. Our principal focus is on a revised notion of capital, emphasizing the central role of differential accumulation by dominant capital groups. We further distinguish between an antagonistic "depth" regime in which differential accumulation is achieved via stagflation, and a less conflictual "breadth" regime where redistribution occurs through growth. Within this framework, we argue that both the recent transition in the two countries, as well as their former regimes, were greatly affected by global developments. Until the 1980s, accumulation in both countries depended largely on depth, characterized by a marked disparity between deepening crisis on the one hand, and rapid differential accumulation on the other. In South Africa, the large companies benefited disproportionately from the impact on gold profit of global inflation, and were therefore reluctant to abandon apartheid. Similarly, Israel's leading firms recorded spectacular gains riding the global arms race and regional conflict, and hence voiced little opposition to the continuation of a war economy at home. Recently, however, these global forces went into reverse, triggering in both countries a shift from depth to breadth. The disinflation associated with rapid globalization undermined gold profit in South Africa, while the end of the Cold War pulled the rug from under the global arms race, drying up the flow of war profit in Israel. In these new conditions, dominant capital groups in the two countries can sustain their differential accumulation only by investing outside their own borders. Capital mobility, though, requires political-economic stability, hence the support of these groups for democracy in South Africa and to regional reconciliation in Israel.
"...As broad as our life experience": visions of feminist political economy, 1972-1991
Authors: Mutari Ellen
Published: June 2007

The six previous special issues of the Review are analyzed both as texts that illuminate the concerns of specific historical moments and as part of the ongoing development of feminist political economy. Over the course of these issues, the diversity of women's experiences forms a basis for critiques of existing theory and the development of feminist analytical constructs.
A Few Observations on Financial Liberalization and Financial Instability
Authors: Weller Christian E.
Published: June 2007

Recent studies have conjectured that there may be a link between financial liberalization (FL) and financial instability in emerging economies. Most of these studies, however, do not investigate whether emerging economies are, in fact, becoming structurally more vulnerable to currency and banking crises. In this paper, I argue that emerging economies are becoming more susceptible to both currency and banking crises after FL. Using data for 27 emerging economies-excluding transition economies—from 1973 to the present, a univariate analysis indicates that the likelihood of currency crises may increase with stronger reactions to financial variables than to real or external trade variables. Similarly, for banking crises, interest rate, exchange rate, maturity, and default may increase, while simultaneously the support structure of the government seems to decline.
National Health Data and Older American Indians and Alaska Natives
Authors: Rhoades Dorothy A.
Published: June 2007

The population of older American Indians and Alaska Natives (AI/ANs) is growing rapidly, but few population-based reports of the health of older AI/ANs exist. The objective was to assess the representation of older AI/ANs in population based health data sets. Health- and aging-related population-based data sets were reviewed for inclusion of AI/AN adults. Data sets were identified using online searches of the Inter-University Consortium for Political and Social Research and the National Archive of Computerized Data on Aging, and federal health care and research agencies. Unweighted sample sizes of AI/ANs were collected for each study by age group. Of 190 data sets reviewed, only 25 (13%) contained at least 100 AI/ANs ages 65 years or older, and less than 5% contained 500 or more. Older AI/ANs are underrepresented in national data sets. Concerted efforts to improve data collection regarding the health and health care needs of this population are needed.
The Native Investigator Development Program: Increasing the Presence of American Indian and Alaska Native Scientists in Aging-Related Research
Authors: Manson Spero M. , Goins R. Turner , Buchwald Dedra S.
Published: June 2007

The 2005 National Academy of Sciences’ report, Assessment of NIH Minority Research Training Programs: Phase 3, underscored the importance of minority representation in the scientific workforce for acquiring the knowledge required to address our nation’s racial and ethnic health disparities. This conclusion stands in sharp contrast to the finding that, in 1999, the National Institutes of Health supported35,000grants; among the principal investigators, only 9 were designated American Indian or Alaska Native (AI/AN). How can we increase the presence of AI/ANs in leadership roles? The authors describe a highly successful postdoctoral training program that prepares young AI/AN investigators for careers as independent, externally funded scientists who work at the nexus of research on culture, aging, and health. The program employs a structured, skill-driven approach that intensively mentors Native trainees without requiring relocation from their home institutions. This model holds promise for training similar populations in this and other fields.
English Proficiency and Access to Health Insurance in Hispanics Who Are Elderly
Authors: Caesar Lena G.
Published: June 2007

Medicare, as a publicly funded insurance program, has produced significant improvement in the overall health of America’s elderly populations. However, health disparities still persist between Hispanic and non-Hispanic White populations in terms of overall access to health services. This study utilized data from the Hispanic Established Population for Epidemiological Studies of the Elderly (H-EPESE) survey (1993 to 1994), to compare the relationship between levels of English proficiency and access to Medicare and other health insurance among 3,050 Hispanics who were elderly. Results of chi-square tests of independence indicated a significant relationship ( p = .001) between respondents’ levels of English proficiency and access to Medicare and other health insurance. Implications regarding the impact of limited English proficiency on access to adequate health care for Hispanics who are elderly are discussed.
Genetics of Epilepsy
Authors: Willmore L. James , Ueda Yuto
Published: June 2007

Understanding the molecular biology of epilepsy is a challenge for modern science. Epilepsy results from alternations in fundamental mechanisms of brain and membrane function. Although an understanding of the mode of inheritance and the etiology of genetic epilepsy syndromes forms the basis for genetic counseling, the development of specific therapies will come from knowing the basic mechanisms of epilepsy. Defining the genes causing epilepsy requires an unambiguous definition of seizure phenotype, along with the stability of that trait, an unremitting clinical course, and an abundance of clinical material. This article reviews the task of defining the genetics of epilepsy and discusses genetic methodology, idiopathic generalized and localization-related partial epilepsies, neuronal migration disorders, progressive myoclonus epilepsies, molecular biology of epileptogenesis, and future research. (J Child Neurol 2002;17:S18—S27).
Epilepsy, Intelligence, and Psychiatric Disorders in Patients With Cerebellar Hypoplasia
Authors: Parmeggiani Antonia , Posar Annio , Scaduto Maria Cristina , Chiodo Simona , Giovanardi-Rossi Paola
Published: June 2007

The cerebellum is involved in motor and cognitive functions and behavior. Its role in controlling epileptic seizures has been demonstrated in the literature. Genetic factors can enhance epilepsy susceptibility when the cerebellum is damaged. We examined the occurrence and features of epilepsy, intelligence, and psychiatric disorders in 28 patients with cerebellar hypoplasia. We compared patients with (10; 35.7%) and without (18; 64.3%) epilepsy. The statistical evaluation showed a significant prevalence of familial antecedents for seizures in patients with epilepsy ( P < .01); cerebral associated lesions and type of cerebellar hypoplasia did not influence the occurrence of epilepsy, which was partial in 80% of cases. Profound mental retardation prevailed in patients with epilepsy ( P < .05). Both mental retardation (75%) and pervasive developmental disorders (17.8%) prevailed in our cases with respect to the general population ( P < .000). Cerebellar hypoplasia in our sample seems to be an important risk factor for the occurrence of epilepsy, mental retardation, and psychiatric disorders. ( J Child Neurol 2003; 18: 1—4).
Building a Division of Child Neurology: A Tribute to Michael J. Painter, MD
Authors: Schor Nina F. , Crumrine Patricia K.
Published: June 2007

In this era of changing priorities, regulations, and resources, it is useful to look both back and forward at the building of a division of child neurology in the context of the emergence of child neurology as a nationally and internationally recognized distinct subspecialty of both pediatrics and neurology. Both Pittsburgh and national colleagues of Dr Michael J. Painter were fortunate to have such a glimpse at a Festschrift held in his honor in January 2002 and, more recently, at the October 2002 meeting of Professors of Child Neurology, where he presented his view of “Building a Child Neurology Division the Old-Fashioned Way.” The narrative that follows draws heavily from both of these events and from insights gained from Dr Painter's long and broad experience in the establishment and nurturing of both the specialty of child neurology and many of its current practitioners. ( J Child Neurol 2003; 18:127—130).
Epilepsy, Atherosclerosis, Myocardial Infarction, and Carbamazepine
Authors: de Chadarévian Jean-Pierre , Legido Agustin , Miles Daniel K. , Katsetos Christos D.
Published: June 2007

Epileptic patients receiving medications such as phenytoin or phenobarbital have been noted to have an exceedingly low incidence of myocardial infarction, but children treated with carbamazepine have shown alteration of their serum lipid profile that could predispose them to atherosclerosis. This report seems to corroborate this point and describes the findings in an 11-year-old boy who died following a major seizure, rhabdomyolysis, and renal failure. The autopsy demonstrated that he had marked coronary atherosclerosis and myocardial infarction. The child had been treated with carbamazepine for more than 10 years. ( J Child Neurol 2003;18: 150—151).
Prospective Preliminary Analysis of the Development of Autism and Epilepsy in Children With Infantile Spasms
Authors: Askalan Rand , Mackay Mark , Brian Jessica , Otsubo Hiroshi , McDermott Catherine , Bryson Susan , Boyd Jennifer , Snead Carter , Roberts Wendy , Weiss Shelly
Published: June 2007

The objective of this study was to compare the efficacy of corticotropin (ACTH) versus vigabatrin in treating infantile spasms and to determine which medication has a more favorable long-term outcome in terms of cognitive function, evolution of epilepsy, and incidence of autism. Patients with infantile spasms were included in the study if they were 3 to 16 months old, had hypsarrhythmia, and had no previous treatment with vigabatrin or corticosteroids. Patient evaluation included electroencephalographic and psychometric measures before and after treatment. Patients were stratified based on etiology (idiopathic or symptomatic) and sex and then randomized between the ACTH and vigabatrin treatment groups. Each of the treatment groups received either ACTH or vigabatrin for 2 weeks. At the end of 2 weeks of treatment, patients were considered responders if spasms and hypsarrhythmia resolved. Nonresponders were crossed over and treated with the alternate drug. Nine patients were included in the study. Three patients received ACTH, one of whom was a responder. Six patients received vigabatrin, three of whom were responders. The five nonresponders received both therapies. All patients had some degree of developmental plateau or regression before the initiation of treatment. Four patients with idiopathic infantile spasms showed improved cognitive function following treatment. The remaining five patients remained significantly delayed. Five patients with symptomatic infantile spasms had epilepsy following treatment; three of them were in the autistic spectrum. The small number of infants in this pilot study is insufficient to determine which of the two drugs is more effective. However, the following trends were identified: vigabatrin may be more effective for patients with symptomatic infantile spasms; patients with idiopathic infantile spasms tend to have a better cognitive outcome; and patients with symptomatic infantile spasms tend to develop both epilepsy and autism. ( J Child Neurol 2003; 18: 165—170).
Unilateral Schizencephaly and Contralateral Polymicrogyria Associated With Umbilical Cord Mass
Authors: Hahn Jin S. , Lewis Ann J.
Published: June 2007

We report a 6-month-old boy with diffuse hypertonia and developmental delay who had unilateral separated-lip schizencephaly and contralateral polymicrogyria. The contralateral polymicrogyria was associated with an incomplete clefting in that hemisphere. An umbilical cord hamartoma is presumed to have caused hypoperfusion to the early developing brain, resulting in bilateral lesions. ( J Child Neurol 2003; 18: 232—234).
Do Partial Seizures Predict an Increased Risk of Seizure Recurrence After Antiepilepsy Drugs Are Withdrawn?
Authors: Hawash Karameh Y. , Rosman N. Paul
Published: June 2007

Most children who are seizure free on antiepilepsy drugs for 2 or more years remain seizure free when taken off antiepilepsy drugs. We studied 27 children with well-controlled epilepsy in whom seizures unexpectedly recurred after antiepilepsy drug withdrawal. Seizures were focal in 20 of 27 cases (74%). In 11 of the 20 cases (55%), there was also a late onset of seizures (after 2 years) and an abnormal electroencephalogram (EEG) at antiepilepsy drug withdrawal. Of the remaining 9 patients with focal seizures, 3 (15%) had only a late seizure onset, 3 (15%) had only an abnormal EEG, and 3 (15%) had neither a late onset of seizures nor an abnormal EEG. In the 7 patients without focal seizures, 6 of 7 (86%) had a late seizure onset and/or an abnormal EEG. Our study suggests that partial seizures can be the most important predictor of unanticipated seizure recurrence when antiepilepsy drugs are withdrawn, particularly with late onset of seizures and an abnormal EEG at antiepilepsy drug withdrawal. A large, multicenter, prospective study looking at these and other potential risk factors for seizure recurrence is needed. ( J Child Neurol 2003;18:331—337).
Brain Single Photon Emission Computed Tomographic Evaluation of Patients With Childhood Absence Epilepsy
Authors: Kapucu L. Özlem , Serdaroğlu Ayşe , Okuyaz Çetin , Köse Gülşen , Gücüyener Kivilcim
Published: June 2007

This study was performed to determine the utility of 99mTc-hexamethylpropylenamine oxime (HMPAO) brain single photon emission computed tomography (SPECT) in evaluating patients with childhood absence epilepsy. Twenty-three patients (13 female, 10 male), aged 7 to 15 years (mean age 10.3 ± 2.2), were studied. All patients underwent a detailed neurologic examination, interictal and ictal electroencephalography (EEG), computed tomography, and/or magnetic resonance imaging, and SPECT. The baseline study was performed during the interictal period and the activation study was performed on a separate day while the patients were having seizures provoked by hyperventilation. Their EEGs were monitored at the same time. Transaxial, sagittal, and coronal slices were obtained for both studies. The mean counts per pixel were calculated on 11 regions of interest on three representative transaxial slices. Count density was calculated for each region. Region-to-occipital cortex ratios were obtained. For each region, normalized ratios were used to obtain a side-to-side percent asymmetry index between baseline and activation studies. Visual interpretation of the baseline study showed that 10 of the 23 patients had a detectable abnormality in regional cerebral blood flow during the interictal period. These abnormalities consisted of relative hypoperfusion in the frontal lobes that could involve neighboring parietal and temporal regions. The activation study revealed that 13 of 23 patients had relative hyperperfusion in these brain regions that were relatively hypoperfused in the baseline study. These hyperperfused regions occupied larger areas than baseline hypoperfused regions. All patients had global increased perfusion in the ictal study. The side-to-side asymmetry indexes for these visually interpreted regional cerebral blood flow abnormalities ranged from 2 to 6%. The relatively consistent pattern of frontal regional cerebral blood flow alterations suggests that altered frontal lobe functions can be implicated in patients with childhood absence epilepsy. ( J Child Neurol 2003;18:542—548).
Globoid Cell Leukodystrophy (Krabbe's Disease): Update
Authors: Suzuki Kunihiko
Published: June 2007

The classic globoid cell leukodystrophy (Krabbe's disease) is caused by genetic defects in a lysosomal enzyme, galactosylceramidase. It is one of the two classic genetic leukodystrophies, together with metachromatic leukodystrophy. The mode of inheritance is autosomal recessive. Typically, the disease occurs among infants and takes a rapidly fatal course, but rarer late-onset forms also exist. Clinical manifestations are exclusively neurologic with prominent white-matter signs. The pathology is unique, consisting of a rapid and nearly complete disappearance of myelin and myelin-forming cells— the oligodendrocytes in the central nervous system and the Schwann cells in the peripheral nervous system, reactive astroytic gliosis, and infiltration of the unique and often multinucleated macrophages (“globoid cells”) that contain strongly periodic acid—Schiff (PAS)—positive materials. A normally insignificant but highly cytotoxic metabolite, galactosylsphingosine (psychosine), is also a substrate of galactosylceramidase and is considered to play a critical role in the pathogenesis. The galactosylceramidase gene has been cloned, and a large number of disease-causing mutations have been identified. Equivalent genetic galactosylceramidase deficiency occurs in several mammalian species, such as mouse, dog, and monkey. Recently, deficiency of one of the sphingolipid activator proteins, saposin A, was demonstrated to cause a late-onset, slowly progressive globoid cell leukodystrophy at least in the mouse, with all of the phenotypic consequences of impaired degradation of galactosylceramidase substrates. Human globoid cell leukodystrophy owing to saposin A deficiency might be anticipated and should be suspected in human patients with a late-onset leukodystrophy with normal galactosylceramidase activity when other possibilities are also excluded. The only serious attempt at treating human patients is bone marrow transplantation, which can provide significant alleviation of symptoms, particularly in those patients with later-onset, more slowly progressive globoid cell leukodystrophy. ( J Child Neurol 2003;18:595—603).
Metachromatic Leukodystrophy: Recent Research Developments
Authors: Gieselmann Volkmar
Published: June 2007

Metachromatic leukodystrophy is a lysosomal storage disorder caused by the deficiency of arylsulfatase A, causing the storage of the sphingolipid sulfatide. The disease is characterized by a progressive demyelination, which results in severe, finally lethal, neurologic symptoms. Genetically, the disease is heterogeneous, most mutant alleles are private, and only three have a frequency worth mentioning. An arylsulfatase A—deficient knockout mouse displays some of the disease features seen in patients but does not show the widespread demyelination characteristic of the disease. Nevertheless, this animal model was used to investigate the therapeutic potential of bone marrow stem cell—based gene therapy. Although treated animals show considerable arylsulfatase A activity in many tissues, including the brain, the effect on sulfatide storage was disappointing. Only in the kidney and liver of animals with very high enzyme levels was lipid storage positively affected. In particular, no effect was seen in the brain. These results suggest that bone marrow stem cell—based gene therapy could be of limited value in the treatment of this disease. The pathogenesis of the disease is poorly understood. It is not yet known how sulfatide storage negatively affects the metabolism of the oligodendrocyte. The amount and distribution of various proteins in the myelin of arylsulfatase A—deficient mice were investigated. It was shown that the myelin protein, myelin and lymphocyte protein (MAL), is reduced in deficient animals and that the myelin protein proteolipid displays an altered distribution within the myelin membranes. Possibly, these alterations contribute to the development of demyelination in this disease. ( J Child Neurol 2003;18:591—594).
MECP2 and Beyond: Phenotype—Genotype Correlations in Rett Syndrome
Authors: Christodoulou John , Weaving Linda S.
Published: June 2007

The association of Rett syndrome with pathogenic mutations of the methyl-CpG binding protein 2 ( MECP2) gene was first made in 1999. Since that time, it has been found that the clinical phenotype can, at least in part, be explained in terms of the type and location of the MECP2 mutation and epigenetic factors such as skewing of X-chromosome inactivation. In addition, MECP2 mutations may be associated with non—Rett syndrome clinical phenotypes, including nonsyndromic and syndromic X-linked mental retardation and Angelman-like phenotypes. Intense research efforts are currently focused on understanding the pathogenesis of Rett syndrome, using sophisticated techniques such as microarray analysis, and the development of mouse models, with an ultimate aim being the development of targeted therapies that could ameliorate or even prevent the devastating consequences of this enigmatic neurodevelopmental disorder. ( J Child Neurol 2003;18:669—674).
Silent Stroke in a Case of β-Thalassemia Major Associated With Chronic Renal Failure and Diabetes Mellitus
Authors: Çaksen Hüseyin , Odabaş Dursun , Akbayram Sinan , Öner Ahmet Faik , Arslan Şükrü , Cesur Yaşar , Üner Abdurrahman
Published: June 2007

Severe anemia, growth retardation, diabetes mellitus, cardiac disorders, and, infrequently, stroke are well-known complications of thalassemia major. We report a girl, age 7 years, 2 months, with βthalassemia major associated with chronic renal failure, diabetes mellitus, and cardiomyopathy in whom a silent stroke was noted during follow-up. She was diagnosed with thalassemia major at age 6 months, chronic renal failure at age 3 years, 3 months, and diabetes mellitus and cardiomyopathy at age 7 years. Although cranial computed tomography was found to be normal at the age of 3 years, 3 months, magnetic resonance imaging showed cerebral infarct in the right frontal region at 7 years, 2 months. A thrombophilic panel revealed increased factor VIII and decreased protein C concentrations. She died from disseminated intravascular coagulation at age 7 years, 9 months. We did not record any clinical findings of stroke during her follow-up. We think that diabetes mellitus, dilated cardiomyopathy, and increased factor VIII and decreased protein C concentrations led to the occurrence of cerebral infarct. In conclusion, we emphasize that children with thalassemia major should be monitored closely for stroke. We also suggest that stroke can show a silent progression in severely affected children, as in our case. ( J Child Neurol 2003;18:798—800).
Regulatory Disorders in Israeli Infants: Epidemiologic Perspective
Authors: Tirosh Emanuel , Bettesh Bendrian Sharon , Golan Galia , Tamir Adal , Cohen Dar Michael
Published: June 2007

The concept of regulatory disorders has been clinically defined. However, epidemiologic validity has not yet been investigated. Children between the ages of 6 and 36 months ( N = 450) were included in the study. Two age groups were analyzed separately: 6 to 17 months (53%) and 18 to 36 months (47%). A specially designed questionnaire in Arabic and Hebrew pertaining to the physiologic and behavioral aspects of regulatory disorders was administered at the mother and child health clinics in northern Israel. It was established in the present study that a minimum of four regulatory items should be reported for the diagnosis of a regulatory disorder, as perceived by parents. Factor analysis revealed 6 and 10 different aggregates of regulatory items in the young and older age groups, respectively. The general prevalence of regulatory disorder as defined in the present study was found to be 18%. Age, maternal education, and sex were found to be significantly related to the prevalence of regulatory disorders. No difference in the prevalence of regulatory disorders between Arab and Jewish infants was found. Further validations of the concept of regulatory disorder as well as devising appropriate measurements in well-designed studies are warranted. ( J Child Neurol 2003; 18: 748—754).
Extreme Microcephaly With Agyria-Pachygyria, Partial Agenesis of the Corpus Callosum, and Pontocerebellar Dysplasia
Authors: Sztriha László , Al-Gazali Lihadh I.
Published: June 2007

A 1-year-old boy with extreme microcephaly and a complex brain malformation is reported. Magnetic resonance imaging revealed an abnormal gyral pattern with features of the agyria-pachygyria spectrum, partial agenesis of the corpus callosum, severely hypoplastic posterior cerebellar vermis, and an abnormal foliation pattern of the cerebellar hemispheres associated with a flat and wide isthmus and pons. Although this phenotype shares some features with malformations classified as microcephaly with a simplified gyral pattern, microlissencephaly, or lissencephaly with cerebellar hypoplasia, none of the several subgroups of these categories are identical to the cerebral dysgenesis found in this patient. ( J Child Neurol 2005;20:170—172).