bluuebunny/crossref_metadata_2025 huggingface dataset:
Augmented Repair of Achilles Tendon Ruptures
Authors: Fernández-Fairén Mariano , Gimeno Carlos
Published: June 2007

We performed a series of repairs of Achilles tendon ruptures in athletes using suture augmented with a polyethylene terephthalate mesh synthetic graft. No postoperative immobilization was used. The 29 pa tients were aged 25 to 54 years and all practiced sports a minimum of 4 hours weekly. At the followup, ranging from 6 months to 5 years, ankle mobility was normal in all but one patient, who had lost 15° of extension. All the patients were able to do a series of 10 single-limb heel raises without any impairment when compared with the uninjured side. Average ankle flexion strength was 96% that of the uninjured side (range, 87% to 110%). There were no reruptures, although in two instances problems with scarring occurred. All patients but one were able to resume their sports activities at the same level of intensity as they had before their injuries. These results testify to the advantage of not using postoperative immobilization and beginning functional rehabilitation immediately in patients with such lesions.
Magnetic Resonance Imaging, Scintigraphy, and Arthroscopic Evaluation of Traumatic Hemarthrosis of the Knee
Authors: Adalberth Torsten , Roos Harald , Laurén Marten , Åkeson Per , Sloth Maja , Jonsson Kjell , Lindstrand Anders , Lohmander L. Stefan
Published: June 2007

Forty patients with traumatic knee hemarthrosis were examined within 1 week after injury and observations made with magnetic resonance imaging, scintigraphy, arthroscopic evaluation, radiography, and physical ex amination were compared. Thirty-four patients (85%) had anterior cruciate ligament injuries according to the arthroscopic findings and 28 (83%) of these had asso ciated meniscal tears. Magnetic resonance imaging confirmed the arthroscopic findings, especially if only meniscal tears that required surgery were taken into account (sensitivity, 94% for the lateral and 83% for the medial meniscus). However, the specificity of magnetic resonance imaging was only 29% and 27% for the lateral and medial menisci, respectively, and the accu racy was 28% and 50%, respectively. Marrow edemas, or bone bruises, were seen on magnetic resonance imaging in 80% of the patients and were mainly seen in the lateral compartment. Bone scans correlated well with magnetic resonance imaging findings of marrow edemas. Plain radiographs were normal in all but one case. We show that magnetic resonance imaging does not add information on the status of the anterior cruci ate ligament compared with the clinical examination, and that it may be as good as arthroscopic evaluation for the diagnosis of meniscal tears that require surgery.
Arthroscopic Treatment of Partial Rotator Cuff Tears in Young Athletes
Authors: Payne Loel Z. , Altchek David W. , Craig Edward V. , Warren Russell F.
Published: June 2007

Forty-three athletes under age 40, more than half of which were collegiate or professional, with partial rota tor cuff tears were treated arthroscopically and ob served for a minimum of 24 months. By history and mechanism of injury, two main groups were identified. Group A had 14 patients with acute, traumatic injuries. All 14 had inflamed subacromial bursas, but increased glenohumeral translation and labral lesions were un common. Twelve patients (86%) had satisfactory post operative results and nine (64%) returned to preinjury sports after arthroscopic subacromial decompression and tear debridement. Group B had 29 overhead ath letes with insidious, atraumatic shoulder pain. They were not as successful with debridement (19 [66%] satisfactory and 13 [45%] return to preinjury sports). Within Group B, three subgroups were identified based on the examination under anesthesia and subacromial inflammation. Group B1 (8 patients) had normal-ap pearing subacromial spaces and often increased ante rior glenohumeral translation with posterior labral tears. These patients did poorly after arthroscopic tear debridement (3 [38%] satisfactory and 2 [25%] return to sports). Group B2 (12 patients) had inflamed sub acromial bursas and increased glenohumeral transla tion. This group had marginal results with debridement (7 [58%] satisfactory and 6 [50%] return to sports). Group B3 (9 patients) with subacromial inflammation, yet without increased glenohumeral translation, had excellent pain relief (100%) but less than half (4) re turned to preinjury sports.
Intratester and Intertester Reliability of the KT-1000 Arthrometer in the Assessment of Posterior Laxity of the Knee
Authors: Huber Frances E. , Irrgang James J. , Harner Christopher , Lephart Scott
Published: June 2007

We measured tibial translation in the knees of 22 sub jects with posterior cruciate ligament tears or recon structions by using the KT-1000 arthrometer. To as sess the reliability of the device, two testers made measurements. One tester was a novice and the sec ond tester had substantial clinical experience with the KT-1000 arthrometer. The quadriceps neutral angle was found for the uninvolved knee. Anterior and pos terior translation and total anterior-posterior excursion were then measured. The quadriceps neutral angle was then reproduced in the involved knee and the same measurements were taken. Each subject was tested twice by each tester. The intraclass correlation coefficient values for the novice, experienced, and in tertester reliability were 0.67, 0.79, and 0.62, respec tively, for corrected posterior translation; 0.59, 0.68, and 0.64, respectively, for corrected anterior transla tion; 0.70, 0.74, and 0.29, respectively, for quadriceps neutral angle; and 0.84, 0.83, and 0.62, respectively, for total anterior-posterior excursion. Ninety-five per cent confidence intervals for the novice, experienced, and intertester reliability were ±2.95, ±2.53, and ±3.27 mm, respectively, for corrected posterior trans lation; ±3.99, ±3.89, and ±3.74 mm, respectively, for corrected anterior translation; and ± 10.70°, ± 11.73°, and ± 16.25°, respectively, for quadriceps neutral an gle. The KT-1000 arthrometer was found to be a mod erately reliable tool for the measurement of tibial trans lation in patients with posterior cruciate ligament tears and reconstructions.
A Rigorous Comparison Between the Sexes of Results and Complications After Anterior Cruciate Ligament Reconstruction
Authors: Barber-Westin Sue D. , Noyes Frank R. , Andrews Michelle
Published: June 2007

Although there is a higher relative incidence of anterior cruciate ligament injuries in female than in comparable male athletes according to the literature, the majority of populations studied after reconstruction are male-dom inated. We wished to determine whether a selection bias for reconstruction based on sex is warranted ac cording to complications and outcome. Ninety-four pa tients (47 of each sex) were matched for chronicity of injury, age, preoperative sports activity levels, articular cartilage condition, and months of followup. All had patellar tendon autogenous reconstruction and a sim ilar program of immediate knee motion and early return to function. The results were rated with the Cincinnati Knee Rating System. At a mean of 26 months postop eratively, there were no significant differences for com plications or outcome between men and women. Women required an average of six more rehabilitation visits than men; however, none required additional surgery for knee motion complications and the rate of patellofemoral crepitus conversion was only 7%, lower than that found for men (15%). The overall failure rate was low, only 6% for women and 4% for men. We concluded that the functional rehabilitation program was effective, postoperative complications were few, and no scientific basis exists to use sex alone as a selection criteria for anterior cruciate ligament reconstruction.
Ankle Disk Training Influences Reaction Times of Selected Muscles in a Simulated Ankle Sprain
Authors: Sheth Parag , Bing Yu  , Laskowski Edward R. , An Kai-Nan
Published: June 2007

Ankle disk training has been used as an exercise in sports medicine clinics to help protect against ankle sprains. This study investigated the effects of ankle disk training on the contraction pattern of the anterior tibialis, posterior tibialis, peroneus longus, and flexor digitorum longus muscles in a simulated ankle sprain. Twenty adults were recruited and divided into a control group and an experimental group. A platform with a trapdoor was used to simulate a lateral ankle sprain. Electromyographic data were recorded from each sub ject in pretraining and posttraining tests. The experi mental group underwent ankle disk training for 8 weeks between the pretraining and posttraining tests. In the pretraining test, the four muscles started to contract simultaneously; in the posttraining test, the contrac tions of the anterior and posterior tibialis muscles were delayed. This delay favors the correction of excessive ankle inversion. This study examined the effects of one form of proprioceptive training on muscle reaction times, and its results may explain why such training can help protect against ankle sprains.
The Effect of Allograft Meniscal Replacement on Intraarticular Contact Area and Pressures in the Human Knee
Authors: Paletta George A. , Manning Tim , Snell Edward , Parker Richard , Bergfeld John
Published: June 2007

To define the biomechanical effects of total lateral meniscectomy and of subsequent lateral meniscal al lograft replacement on load transmission and distribu tion across the human knee, we mounted 10 fresh- frozen, young human cadaveric knees on a mechanical testing system. Peak pressure and contact area profiles were determined at 0°, 30°, and 60° of knee flexion using pressure-sensitive film and a den sitometer. Load transmission profiles were determined for each knee in a sequential test order: 1)intact knee, 2) after lateral meniscectomy, 3) after implantation of size-matched meniscal allograft fixed with bone plugs, and 4) after release of the anterior and posterior horn attachments of the allograft. Total lateral meniscec tomy resulted in a 45% to 50% decrease in total con tact area. Allograft replacement increased total contact area by 42% to 65% as compared with total meniscec tomy at all flexion angles. After release of the anterior and posterior horn attachments, contact area was identical to that after total meniscectomy. Total lateral meniscectomy resulted in a 235% to 335% increase in peak local contact pressure. Allograft replacement de creased these pressures by 55% to 65% at all flexion angles, but they remained significantly greater than those in the intact state. After release of the anterior and posterior horn attachments, contact pressures were identical to those after total meniscectomy. Com pared with total meniscectomy, meniscal allograft transplantation significantly increases contact area and decreases peak local contact pressures, but any bio mechanical advantages are lost without bone plug fix ation of the anterior and posterior horns.
Estrogen Affects the Cellular Metabolism of the Anterior Cruciate Ligament
Authors: Liu Stephen H. , Al-Shaikh Raad A. , Panossian Vahé , Finerman Gerald A. M. , Lane Joseph M.
Published: June 2007

Investigations from this laboratory have established the presence of estrogen receptors in the human an terior cruciate ligament. This study further investigates the effects of 17β-estradiol on the cellular proliferation and collagen synthesis of fibroblasts derived from the rabbit anterior cruciate ligament. Fibroblast prolifera tion and collagen synthesis in response to near log concentrations of 17β-estradiol (at 0.0029, 0.025, 0.25, 2.5, and 25 ng/ml) were assessed by measuring [3H]thymidine and [14 C]hydroxyproline incorporation, respectively. Collagen synthesis was significantly re duced with increasing local estradiol concentration (P < 0.001). Declining collagen synthesis was first noted at a 17β-estradiol concentration of 0.025 ng/ml. Within normal physiologic levels of estrogen (0.025 to 0.25 ng/ml), collagen synthesis was reduced by more than 40% of control, and at pharmacologic levels of 2.5 and 25 ng/ml, by more than 50% of control. A signifi cant reduction of fibroblast proliferation was also ob served with increasing estradiol concentrations (P = 0.023). Clinically, alterations in anterior cruciate liga ment cellular metabolism caused by estrogen fluctua tions may change the composition of the ligament, rendering it more susceptible to injury.
A Prospective Study of Prognostic Factors Concerning the Outcome of Arthroscopic Surgery for Anterior Ankle Impingement
Authors: van Dijk C. Niek , Tol Johannes L. , Verheyen Cees C. P. M.
Published: June 2007

Sixty-two consecutive patients with painful limited dor siflexion of the ankle not responding to nonoperative treatment participated in a prospective study. All 42 men and 20 women (average age, 31 years) under went arthroscopic surgery. Preoperative radiographs were graded according to an osteoarthritic and an impingement classification. Standardized followup took place at 4 months and 1 and 2 years after surgery. Results showed that the degree of osteoarthritic changes is a better prognostic factor for the outcome of arthroscopic surgery for anterior ankle impingement than size and location of the spurs. The hypothesis is that osteophytes without joint space narrowing are not a manifestation of osteoarthritic changes but rather the result of local (micro)trauma. After 2 years, 73% of the patients experienced overall excellent or good results; 90% of those without joint space narrowing had good or excellent results, and 50% of those with joint space narrowing had good or excellent results. At the 2-year followup, the group without joint space narrowing showed significantly better scores in pain, swelling, ability to work, and engagement in sports. This study also revealed that patients with less than 2 years of ankle pain before surgery and spurs located anterome dially were more satisfied with the outcome than when longer periods of preoperative pain were involved and when spurs were located anterolaterally.
Reconstruction of the Anterior and Posterior Cruciate Ligaments After Knee Dislocation
Authors: Noyes Frank R. , Barber-Westin Sue D.
Published: June 2007

We report a critical rating of results for 11 patients with bicruciate ligament reconstructions and immediate pro tected knee motion after knee dislocations (seven acute and four chronic). Six patients had concurrent repair or reconstruction of medial ligamentous struc tures, and six had reconstruction of the lateral and posterolateral ligaments. All patients returned for fol lowup at a mean of 4.8 years postoperatively. Fol low-up arthrometric testing at 20° of flexion showed 10 knees had less than 3 mm of increased total anterior- posterior displacement and 1 knee had 7 mm of in crease. At 70° of flexion, 9 knees had less than 3 mm of increased displacement and 2 knees had more than 6 mm of increase. The failure rates were as follows: 18% of posterior cruciate ligament reconstructions (2 of 11), 9% of anterior cruciate ligament reconstructions (1 of 11), 17% of lateral and posterolateral procedures, and 0% of medial collateral ligament procedures. At followup, five of the seven patients with acute injuries had no limitations with daily or sports activities. Three of the four patients with chronic ruptures were asymp tomatic with daily activities, but only one was asymp tomatic with light sports. Five patients (all acute inju ries) required treatment for knee motion limitations. Nine patients had full range of motion at followup. We concluded that simultaneous bicruciate ligament re constructions, performed with associated medial or lat eral procedures, are warranted to restore function to all ligament structures. Even though immediate motion was used, several patients required early manipulation or arthroscopic debridement, which restored full motion and prevented permanent arthrofibrosis.
The Reharvested Central Third of the Patellar Tendon
Authors: LaPrade Robert F. , Hamilton Christopher D. , Montgomery Ronald D. , Wentorf Fred , Hawkins Hal D.
Published: June 2007

We assessed the histologic, mechanical, and structural properties of the reharvested central-third patellar ten don in greyhounds. Twelve dogs had the central third of the patellar tendon (5 mm) removed with corre sponding bone blocks from the patella and tibia; the remaining tendon defect was loosely closed. Six dogs were sacrificed at 6 months and six at 12 months, and the central third of the patellar tendon was harvested from both the operative and the contralateral control knees. Analysis of the structural changes in the ten dons revealed a significant increase in thickness for reharvested tendons at both 6 and 12 months when compared with controls. The entire residual tendons were narrower at 6 months and were shorter at 12 months compared with controls. Mechanical testing showed that the average failure load, ultimate tensile strength, strain at failure, and average modulus for the reharvested central third of the patellar tendon were significantly less than that of controls at both 6 and 12 months. Analysis of collagen fiber size by electron microscopy revealed a significant increase in collagen fiber diameter at 6 months (135 ± 41 nm versus 49 ± 4 nm) but no difference between the operative limbs and controls at 12 months. The reharvested bone- patellar tendon-bone complex does not have the same properties as the primary patellar tendon graft up to 1 year after harvest in a canine model, and its use for revision cruciate ligament reconstruction must be care fully reexamined.
Telling other stories: heterodox critiques of supply in micro principles texts
Authors: Cohn Steve
Published: June 2007

This paper develops a common ground for heterodox critiques of the treatment of supply in neoclassical micro principles texts. The paper critiques both the textbooks' formal analytic propositions and their rhetorical complement in metaphors and stories.
Obstacles facing women's grassroots development strategies in Mexico
Authors: Eber Christine E. , Tanski Janet M.
Published: June 2007

Women in the indigenous township of San Pedro Chenalho, Chiapas have responded to economic crisis and structural adjustment in Mexico with cooperative survival strategies. While women have obtained a certain level of empowerment through these strategies, they have also faced severe obstacles to the development and success of their grassroots initiatives. This article examines the transformative potential of the women's collective projects, analyzes the obstacles they face, and considers the implications of the case of Chenalho for other marginalized communities facing similar impediments to grassroots development.
Manufacturing and global turbulence: Brenner' s misinterpretation of profit rate differentials
Authors: Duménil Gérard , Lévy Dominique
Published: June 2007

Robert Brenner's analysis of the structural crisis of the late 20th century, in his Economics of Global Turbulence, is based on the observation of the decline of the profit rate, as is common among Marxist economists. Instead of the usual reference to the features of technical change, Brenner explains the decline of the profit rate in the whole economy by its fall within manufacturing industries, resulting from excess international competition. Brenner's basic insight is based on the observation that, after World War II, in the United States, the profit rate of manufacturing industries was larger than in other U.S. industries, and declined considerably more. This observation follows from a misinterpretation of profit rate differentials. Actually, the profit rate of manufacturing industries was not significantly different from that of most other industries, with the exception of a group of very capital intensive industries such as railroads, which accounts for only 13 percent of total output. This difference, which remains to be explained, has nothing to do with international competition.
The Costs of U.S. Hegemony: Military Power, Military Spending, and U.S. Trade Performance
Authors: Oden Michael
Published: June 2007

For David Gordon and his collaborators in the social structures of accumulation (SSA) tradition, the willingness to threaten potential adversaries with military forces capable of acting on a global scale was seen to positively affect the U.S. terms of trade. U.S. hegemony yielded specific supply side benefits as favorable terms of trade reduced relative input prices and boosted profitability. Although Gordon and his co-authors recognize the potential costs of sustaining a large, globally active military apparatus, these costs are not incorporated in the econometric models supporting the SSA theory. This paper attempts to extend the SSA analysis by empirically measuring the effects of both military spending and military power on U.S. trade performance over the 1951-1987 period. It is shown that while military power and spending commitments may have positive effects on the terms of trade, these military variables had direct and indirect negative impacts on the U.S. net export balance and therefore domestic aggregate demand. In the context of a model of U.S. growth performance open to international transactions, the overall effect of the postwar military system on U.S. economic growth was likely negative in the cold-war era.
The Existence, Endogeneity, and Synchronization of Long Waves: Structural Time Series Model Estimates
Authors: Goldstein Jonathan P.
Published: June 2007

In the spirit of David Gordon's application of advanced econometric techniques to problems in political economy and his interest in long waves, this paper develops a structural time series model for testing the existence, endogeneity, and synchronization of long waves in the output series for 11 countries. The results show a strong long wave pattern with a high degree of endogenous behavior in 8 of 11 GDP series. Strong evidence for the synchronization of long waves based on international transmission mechanisms is found, while weaker evidence exists for synchronization based on similar institutional structures. Other long wave hypotheses are also addressed.
Determinants of Health-Related Quality of Life Among Older American Indians and Alaska Natives
Authors: Goins R. Turner , John Robert , Hennessy Catherine Hagan , Denny Clark H. , Buchwald Dedra
Published: June 2007

During the past decade, health-related quality of life (HRQoL) has been recognized in both clinical and community health research as an important health outcome and a needed supplement to conventional health outcomes. The authors provide a profile of HRQoL and examine its determinants among American Indians and Alaska Natives aged 50 or older. Multivariate analyses of cross-sectional survey data from the 1996-1998 Centers for Disease Control Behavioral Risk Factor Surveillance System were conducted. Thirty-four percent of the sample reported fair or poor self-rated health. The mean number of poor health days in the past month ranged from 4 to 6 on different measures. Age, sex, education, annual household income, employment status, hypertension, and obesity were associated with aspects of HRQoL. Further research aimed at eliminating health disparities among this population should focus on identifying additional indicators of poor HRQoL and on understanding variables that mediate the relationship between disease and HRQoL.
Latino Disaster Vulnerability
Authors: Peguero Anthony A.
Published: June 2007

When a natural or manmade disaster strikes, there is usually little time for citizens to prepare and protect themselves. In September 2003, the U.S. Department of Homeland Defense implemented a National Response Plan dealing with many forms of disaster. However, when a disaster hits, not all citizens are equally prepared or protected. A sample of 165 Latino and 1,069 Non-Latino Florida single-family homeowners is drawn from the 1999 Florida Statewide Mitigation Survey (SMS) to investigate the problems associated with the dissemination of hurricane mitigation information among Florida’s Latino homeowners. Results from logistic regression analyses reveal that Latino homeowners prefer to utilize friends and family as sources of disaster preparation information. The significance of research findings and policy implications are discussed.
Unmet Needs in Pediatric Epilepsy
Authors: Glauser Tracy A. , Pellock John M.
Published: June 2007

During the past 25 years, much new information about the epidemiology, pathophysiology, and prognosis of various forms of childhood epilepsy has been compiled. This has resulted in improved treatments that have helped an increasing number of children with epilepsy overcome unchecked seizures and the accompanying social stigma, enabling them to lead full and rewarding lives. The purpose of this supplement is to present a scientific and clinically relevant examination of some of the major breakthroughs in pediatric epilepsy, including a review of the latest advances in our understanding of the fundamental basis of childhood epilepsy. Additionally, this supplement will discuss persisting diagnostic issues, explore the role and use of new therapeutic options, and contemplate future treatment approaches. The articles are the result of a closed symposium held in January 2001. (J Child Neurol 2002;17:S1—S3).
Using the New Antiepilepsy Drugs in Children
Authors: Wheless James W.
Published: June 2007

New epilepsy treatment options are becoming available to physicians and may help provide safe and effective seizure management in patients for whom traditional drug therapies have been unsuccessful. Presented here is a brief discussion on the screening models used to test drugs for efficacy against various seizure types and the mechanisms of action and pharmacology of antiepilepsy drugs. This is followed by a more detailed review of the clinical pharmacology of the latest three antiepilepsy drugs introduced to the United States: levetiracetam, oxcarbazepine, and zonisamide. All three appear to have advantages over some traditional antiepilepsy drugs and with more clinical experience may replace them in certain seizure types or epilepsy syndromes. (J Child Neurol 2002;17:S58—S64).
Lennox-Gastaut Syndrome
Authors: Crumrine Patricia K.
Published: June 2007

Lennox-Gastaut syndrome is a type of childhood epilepsy that has enormous detrimental effects on the patient's physical and developmental health and can also take a dramatic toll on the well-being of the patient's family. Lennox-Gastaut syndrome is characterized by variable etiology, multiple types of intractable seizures, and cognitive impairment in most patients. It is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment with standard antiepilepsy drugs. This article reviews the etiology of Lennox-Gastaut syndrome, characteristics of predominant seizure types, methods of evaluating patients for Lennox-Gastaut syndrome, and available treatments including antiepilepsy drug therapy, ketogenic diet, and surgical options. (J Child Neurol 2002;17:S70—S75).
Lamotrigine Versus Valproate Monotherapy—Associated Weight Change in Adolescents With Epilepsy: Results From a Post Hoc Analysis of a Randomized, Double-Blind Clinical Trial
Authors: Biton Victor , Levisohn Paul , Hoyler Sheri , Vuong Alain , Hammer Anne E.
Published: June 2007

Recent trends toward obesity and associated health risks in children highlight the significance of weight gain as a side effect with certain antiepilepsy drugs. No previous study has prospectively compared, in adolescents, weight effects for two commonly used antiepilepsy drugs. We report results from a post hoc subanalysis of adolescent data from a randomized, double-blind study comparing weight effects of lamotrigine and valproate. Patients were

12 years of age with new-onset partial or generalized seizures who were randomized 1:1 to lamotrigine or valproate. Patients were escalated to a dose range of 100 to 500 mg/day for lamotrigine and 10 to 60 mg/kg/day for valproate based on clinical response, with target doses maintained for 24 weeks. Results are reported for adolescents aged 12 to 20 years. Weight changes during maintenance were higher ( P < .05) in valproate ( n= 20) patients than in lamotrigine ( n= 18) patients, and change in body mass index was higher ( P < .05) in valproate patients at the end of the study. At week 32, mean body mass index in the valproate group was above the 85th percentile representing “at risk for overweight.” Whereas weight remained stable in adolescents treated with lamotrigine, weight increased in those treated with valproate by week 10 of this study and continued to increase at the end of the study. ( J Child Neurol 2003; 18: 133—139).
Unfolding Story of Inclusion-Body Myositis and Myopathies: Role of Misfolded Proteins, Amyloid-β, Cholesterol, and Aging
Authors: Askanas Valerie , King Engel W.
Published: June 2007

Sporadic inclusion-body myositis and hereditary inclusion-body myopathies are progressive muscle diseases leading to severe disability. We briefly summarize their clinical pictures and pathologic diagnostic criteria and discuss the latest advances in illuminating their pathogenic mechanism(s). We emphasize how different etiologies might lead to the strikingly similar pathology and possibly similar pathogenic cascade. On the basis of our research, several processes seem to be important in relation to the still speculative pathogenesis, including (a) increased transcription and accumulation of amyloid-β precursor protein and accumulation of its proteolytic fragment amyloid-β; (b) abnormal accumulation of components related to lipid metabolism, for example, cholesterol, accumulation of which is possibly owing to its abnormal trafficking; (c) oxidative stress; (d) accumulations of other Alzheimer's disease—related proteins; and (e) a milieu of muscle cellular aging in which these changes occur. We discuss a potentially very important role of unfolded and/or misfolded proteins as a possible mechanism in the formations of the inclusion bodies and other abnormalities. ( J Child Neurol 2003;18:185-190) .
Left-Sided Facial Nevus With Contralateral Leptomeningeal Angiomatosis in a Child With Sturge-Weber Syndrome: Case Report
Authors: Widdess-Walsh Peter , Friedman Neil Roy
Published: June 2007

Sturge-Weber syndrome is characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma. Variants of this classical presentation have been described in the literature, some of which have prognostic significance. We report a magnetic resonance imaging (MRI)-confirmed variant of a leptomeningeal angioma contralateral to the facial nevus. We describe one patient with Sturge-Weber syndrome who presented with a left-sided facial nevus, left eye glaucoma, episodes of left-sided weak-ness, and right-sided leptomeningeal angiomatosis by gadolinium-enhanced brain MRI. The literature regarding variants of Sturge-Weber syndrome and their prognosis is reviewed. The prognosis for this variant is likely similar to Sturge-Weber syndrome with an ipsilateral leptomeningeal angioma. ( J Child Neurol 2003;18:304—305).
Neurocysticercosis Masquerading as a Cerebral Infarct
Authors: Wraige Elizabeth , Graham John , Robb Stephanie A. , Jan Wajanat
Published: June 2007

The differential diagnosis of acute focal neurologic deficit in childhood is diverse. We report the case of a child presenting with an acute hemiparesis persisting for longer than 24 hours following a focal seizure. The clinical history, examination findings, and results of cranial magnetic resonance imaging (MRI) were initially interpreted as consistent with an arterial ischemic cerebral infarction. Follow-up cranial MRI performed 9 months later revealed changes indicative of neurocysticercosis. Review of original neuroimaging resulted in a revision of the diagnosis to neurocysticercosis. The clinical history, together with neuroimaging findings, is highly compatible with a diagnosis of neurocysticercosis but unusual because it occurred in a child resident in a nonendemic area who had never traveled to an endemic area and whose diet excluded pork. The case reported raises two important issues. The first is the need to carefully consider the differential diagnosis of acute hemiparesis, including unusual causes. Second, it raises awareness of the potential for neurocysticercosis to occur in low-risk patients in nonendemic areas. ( J Child Neurol 2003;18:298—300).
Oligohydrosis and Hyperthermia: Pilot Study of a Novel Topiramate Adverse Effect
Authors: Ben-Zeev Bruria , Watemberg Nathan , Augarten Arie , Brand Nathan , Yahav Yaakov , Efrati Ori , Topper Leon , Blatt Ilan
Published: June 2007

A 6-year-old boy with partial complex seizures developed recurrent episodes of hyperthermia 2 months after topiramate was introduced into his antiepilepsy drug regimen. Further investigation revealed that the febrile episodes were related to environmental temperature and physical activity. A pilocarpine iontophoresis sweat test showed that the amount of sweat produced by the child was 5% that of age-matched controls. Topiramate discontinuation resulted in the disappearance of febrile episodes and normalization of sweat quantity in repeat sweat testing. Based on this observation and the previous data on zonisamide and isolated case reports on topiramate-related hyperthermia and the effect on sweat production, topiramate was suspected of causing oligohydrosis. A pilot study was carried out involving 13 additional children and young adults (age range 1—37 years) receiving topiramate. All patients were directly questioned regarding symptoms of decreased sweating and heat intolerance, went through a pilocarpine iontophoresis sweat test, and were compared with 14 age-matched controls who went through the sweat test for unrelated reasons. Nine of the patients were found to have reduced sweat quantity on the pilocarpine iontophoresis sweat test (including index case) (mean 0.089 g/30 minutes, SD 0.082; age-matched control: mean 0.21 g/30 minutes, SD 0.06). Eight of them were children (below 16 years). However, only three patients revealed symptoms related to heat intolerance. Topiramate is most likely responsible for decreased sweat production as detected by a pilocarpine iontophoresis sweat test. The effect seems to be more significant in children than in adults. There is a discrepancy between test results and clinical symptoms. Interestingly, oligohydrosis was found to be a relatively common side effect of zonisamide. Both zonisamide and topiramate share a carbonic anhydrase inhibitor activity. The significance of oligohydrosis in hot climates should not be underestimated. Its extent, the role of sweat test prediction, and clinical significance during topiramate treatment should be further estimated. ( J Child Neurol 2003;18:254—257).
Subacute Sclerosing Panencephalitis Presenting With Hemiparesis in Childhood: Case Report
Authors: Özyürek Hamit , Değerliyurt Aydan , Turanli Guzide
Published: June 2007

Subacute sclerosing panencephalitis is a chronic and fatal disease of the central nervous system. Most patients present with progressive psychointellectual disturbances. A 14-month-old girl was admitted to our hospital because of left-sided hemiparesis. During hospitalization, focal and generalized seizures occurred. The electroencephalogram (EEG) revealed that periodic lateralized discharges consisted of polyspike and high-voltage slow waves in the left hemisphere. The clinical and EEG findings and positive serology of measles in the cerebrospinal fluid were consistent with subacute sclerosing panencephalitis. In conclusion, we suggest that subacute sclerosing panencephalitis be considered in the differential diagnosis of focal neurologic signs in infants. ( J Child Neurol 2003;18:363—365).
Diffuse Neuroaxonal Involvement in Mucolipidosis IV as Assessed by Proton Magnetic Resonance Spectroscopic Imaging
Authors: Bonavita Simona , Virta Anette , Jeffries Neal , Goldin Ehud , Tedeschi Gioacchino , Schiffmann Raphael
Published: June 2007

Mucolipidosis IV is an autosomal recessive disorder caused by mutations in MCOLN1, which codes for mucolipin, a transient receptor potential protein. In order to investigate brain metabolic abnormalities in mucolipidosis IV, we studied 14 patients (11 children, 3 adults) by proton magnetic resonance spectroscopic imaging. The ratios of N-acetylaspartate/ creatine-phosphocreatine and N-acetylaspartate/choline-containing compounds in patients with mucolipidosis IV were significantly reduced in all regions of interest except the parietal gray matter and thalamus. The ratios of choline-containing compounds/creatine-phosphocreatine was not significantly reduced in patients compared with controls. The ratio of N-acetylaspartate/creatine-phosphocreatine were significantly lower ( P = .005) in the more neurologically impaired patients compared with the least impaired. For every region of interest, except for parietal gray matter, the ratio of N-acetylaspartate/creatine-phosphocreatine was lower in the more motorically impaired patient group. There was no difference for the ratio of N-acetylaspartate/creatine-phosphocreatine between younger and older patients. These findings suggest that mucolipidosis IV is largely a static developmental encephalopathy associated with diffuse neuronal and axonal damage or dysfunction. Mucolipin deficiency impairs motor more than sensory central nervous system pathways. ( J Child Neurol 2003;18:443—449).
Hyperkinetic Seizures in Children
Authors: Weinstock Arie , Giglio Pierre , Kerr Susan L. , Duffner Patricia K. , Cohen Michael E.
Published: June 2007

The objective of this study was to delineate the clinical and video-electroencephalographic (EEG) manifestations of children with complex partial seizures with a predominant “hyperkinetic” presentation. Certain types of partial seizures can be difficult to differentiate from nonepileptic seizures because of their intense motor presentation and, at times, lack of alteration of consciousness. Based on a published semiologic seizure classification, this type of seizures can be described as “hyperkinetic,” characterized by intense motor activity involving the extremities and trunk. We report five children diagnosed with hyperkinetic seizures by video-EEG monitoring. All patients were referred for video-EEG evaluation because of an initial suspicion of pseudoseizures. Presented in this study is a review of the patients' clinical data, including video-EEG evaluation. There were three boys and two girls; the mean age at presentation was 10 ± 3 years. In four patients, there was a history of behavioral disorder, with two patients carrying a diagnosis of attention-deficit hyperactivity disorder (ADHD). One girl had significant developmental delay and an abnormal neurologic examination. Brain magnetic resonance imaging was normal in three patients and abnormal in two. The semiology of the seizures consisted of stereotypic intense motor activity, mainly upper extremity flailing and kicking. Screaming and shouting were noted in three cases, and intense fear was present in two patients. The hyperkinetic ictal activity progressed to tonic-clonic seizures in two patients. Seizures occurred out of sleep or on awakening in four patients. The interictal EEG activity was normal in one patient and revealed a continuous generalized slowing and slowing of the posterior dominant rhythm in two patients. One of the latter patients had interictal epileptiform activity in the frontal and midline regions. An intermittent rhythmic slow activity of the left hemisphere with superimposed bifrontal sharp waves was noted in the fifth patient. The ictal EEG revealed profuse superimposed electromyographic (EMG) activity in all patients, making some of the EEG interpretation difficult to analyze, particularly a longitudinal bipolar montage. However, with digital manipulation of the ictal EEG data, such as changes in EEG sensitivity, application of fast frequency filters, and use of different EEG montages, it was possible to discern an ictal EEG pattern or postictal slowing following the diffuse EMG artifact in all patients. On clinical follow-up, adequate seizure control was achieved in three patients. Based on the clinical history, one patient was diagnosed with autosomal dominant nocturnal frontal lobe epilepsy. Diagnosis of hyperkinetic seizures can be difficult because of the similarity of the clinical manifestations with nonepileptic events such as certain parasomnias and pseudoseizures. Video-EEG is the most effective way of diagnosing this type of seizure. ( J Child Neurol 2003;18:517—524).
Juvenile-Onset Leigh Syndrome With an Acute Polyneuropathy at Presentation
Authors: Stickler David E. , Carney Paul R. , Valenstein Edward R.
Published: June 2007

Subacute necrotizing encephalomyelopathy or Leigh syndrome can be associated with a polyneuropathy. By far, the most commonly encountered neuropathy is a chronic neuropathy with features of demyelination, which can be the presenting manifestation of Leigh syndrome. On rare occasions, the neuropathy encountered can represent a more acute process. We describe a rare case of juvenile-onset Leigh syndrome presenting with an acute polyneuropathy. ( J Child Neurol 2003;18:574—576).
Trigeminal Myokymia in a Young Girl
Authors: Mancias Pedro , Butler Ian J.
Published: June 2007

Myokymia results from complex bursts of repetitive discharges of a motor unit typically attributable to a demyelinating condition. We report a 12-year-old girl with unilateral trigeminal myokymia who presented with involuntary jaw movements. Electromyography demonstrated unilateral rhythmic myokymic discharges in the left masseter and temporalis muscles at a rate of 3.5 Hz. Abnormal jaw movements spontaneously resolved over 8 months without treatment or residual deficits. The pathophysiology of myokymic discharges is discussed. ( J Child Neurol 2003;18:572—574).
A Child With Diamond-Blackfan Anemia, Methylenetetrahydrofolate Reductase Mutation, and Perinatal Stroke
Authors: Butrum Matthew W. , Williams Linda S. , Golomb Meredith R.
Published: June 2007

Diamond-Blackfan anemia is a congenital hypoproliferative anemia known to be associated with diverse physical anomalies affecting the thumb, craniofacial bones, urogenital system, and heart; prematurity; and fetal demise. We report the case of a 16-month-old boy with Diamond-Blackfan anemia noted to have decreased use of his right side since birth. Magnetic resonance imaging demonstrated a large area of encephalomalacia in the left middle cerebral artery territory. Magnetic resonance angiography showed marked attenuation of the left middle cerebral artery, suggesting a remote thromboembolic event. The laboratory results were remarkable for a decreased hemogolobin of 9.5 g/dL and increased platelets of 591,000/μL. He was heterozygous for the methylenetetrahydrofolate reductase gene C677T mutation. An echocardiogram demonstrated a patent foramen ovale versus an atrial septal defect with left to right shunting. Perinatal stroke may be a rare complication of Diamond-Blackfan anemia in the setting of other risk factors. ( J Child Neurol 2003;18:800—802).
Peripheral Leukocytosis in Children With Febrile Seizures
Authors: Mohebbi Mohammad R. , Holden Kenton R. , Mohammadi Mahmoud
Published: June 2007

Peripheral blood leukocytosis has been reported following febrile seizures as a result of infection, the seizure, or both. To examine this relationship, 238 consecutive children ≤ 5 years of age who experienced their first febrile seizure were evaluated. Lumbar punctures were electively done on 128 of the children to rule out central nervous system infection. Total leukocyte counts and duration of fever before the seizure were negatively correlated ( r = —.175, P < .05). In logistic regression analysis, the logarithm of fever duration before the seizure was negatively associated with leukocytosis (leukocyte count ≥ 15,000 cells/μL [odds ratio: 0.117, P < .05]). Cerebrospinal fluid glucose concentrations were significantly correlated with increased body temperature ( r =.230, P < .05) and increased leukocyte counts ( r = .255, P < .01). No significant association was found between leukocyte counts and the characteristics of the febrile seizure event. By all indications, changes in leukocyte counts appear more likely to be related to the length and underlying etiology of the fever than to the seizure itself. ( J Child Neurol 2004;19:47—50).
Behavioral Characteristics of Prader-Willi Syndrome in Korea: Comparison With Children With Mental Retardation and Normal Controls
Authors: Kim Jae-won , Yoo Hee-jeong , Cho Soo-churl , Hong Kang-E Michael , Kim Boong-nyun
Published: June 2007

The purpose of this study was to examine the psychiatric characteristics of children with Prader-Willi syndrome in Korea, focusing particularly on their behavioral problems and obsessive-compulsive spectrum symptoms. Fourteen patients with Prader-Willi syndrome, together with their parents, underwent a psychiatric interview and parent questionnaire consisting of a Child Behavior Checklist. Twenty-four patients with mental retardation and 45 normal students were selected as control groups. Compared with the normal control group, the rates of inclusion in the clinical range and the mean scores with regard to social problems, thought problems, attention problems, delinquent behavior, aggressive behavior, externalizing problems, and total problems profiles were significantly higher ( P < .01) in the Prader-Willi syndrome group according to the results of the Child Behavior Checklist. Compared with the mental retardation group, there was a statistically significant ( P < .05) difference in the delinquent behavior profile. Comparing selected Child Behavior Checklist items related to obsessive-compulsive spectrum symptoms, the Prader-Willi syndrome group showed significantly more ( P < .05) compulsion, skin picking, and stealing than the mental retardation or normal control groups. These findings suggest that children with Prader-Willi syndrome in Korea have many behavioral problems, including obsessive-compulsive spectrum symptoms, needing proper psychiatric attention and treatment. This is the first study in Korea to evaluate the psychiatric and behavioral characteristics of children with Prader-Willi syndrome. ( J Child Neurol 2005;20:134—138).
Long-Term Prognosis of Epilepsies and Related Seizure Disorders in Fukuyama-Type Congenital Muscular Dystrophy
Authors: Yoshioka Mieko , Higuchi Yoshihisa
Published: June 2007

Fukuyama-type congenital muscular dystrophy is an autosomal recessive disorder prevalent in Japan that is characterized by congenital muscular dystrophy, cobblestone lissencephaly, and eye anomalies. We examined 46 patients with Fukuyama-type congenital muscular dystrophy and followed their progress for more than 3 years, with special reference to long-term prognosis of seizure disorders and the relationship between seizures and neuropathologic abnormalities. Seizures were observed in 37 patients (80%). The average age at onset was 3 years, 1 month. Initial seizures usually occurred after a febrile episode, although one third of patients had afebrile seizures from the onset. All patients had generalized tonic-clonic convulsions at febrile disorders, and these were followed by complex partial seizures or secondary generalized seizures. Later these seizures developed into Lennox-Gastaut syndrome in three patients. Electroencephalography (EEG) showed paroxysmal discharges in 22 of 37 patients with seizures (59%). The main focus was in the frontal, temporal, or central region. Lesions with marked cortical dysplasia detected by computed tomography, magnetic resonance imaging, or autopsy showed focal paroxysmal discharges on EEG. ( J Child Neurol 2005;20:385—391).