Cerebral Palsy

BACKGROUND: Post-extubation stridor may prolong length of stay in the intensive care unit, particularly if airway obstruction is severe and re-intubation proves necessary. Some clinicians use corticosteroids to prevent or treat post-extubation stridor, but corticosteroids may be associated with adverse effects ranging from hypertension to hyperglycaemia, so a systematic assessment of the efficacy of this therapy is indicated. OBJECTIVES: To determine whether corticosteroids are effective in preventing or treating post-extubation stridor in critically ill infants, children, or adults. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, CINAHL and reference lists of articles. The most recent searches were conducted in January 2009. SELECTION CRITERIA: Randomized controlled trials comparing administration of corticosteroids by any route with placebo in infants, children, or adults receiving mechanical ventilation via an endotracheal tube in an intensive care unit. DATA COLLECTION AND ANALYSIS: Three review authors independently assessed trial quality and extracted data. MAIN RESULTS: Eleven trials involving 2301 people were included: six in adults, two in neonates, three in children. All but one examined use of steroids for the prevention of post-extubation stridor; the remaining one concerned treatment of existing post-extubation stridor in children. Patients were drawn from heterogeneous medical/surgical populations. Dexamethasone given intravenously at least once prior to extubation was the most common steroid regimen utilized (uniformly in neonates and children). In neonates the two studies found heterogeneous results, with no overall statistically significant reduction in post extubation stridor (RR 0.42; 95% CI 0.07 to 2.32). One of these studies was on high-risk patients treated with multiple doses of steroids around the time of extubation, and this study showed a significant reduction in stridor. In children, the two studies were clinically heterogeneous. One study included children with underlying airway abnormalities and the other excluded this group. Prophylactic corticosteroids tended to reduce reintubation and significantly reduced post-extubation stridor in the study that included children with underlying airway abnormalities (N = 62) but not in the study that excluded these children (N = 153). In six adult studies (total N = 1953), the use of prophylactic corticosteroid administration did not significantly reduce the risk of re-intubation (RR 0.48; 95% CI 0.19 to 1.22). While there was a significant reduction in the incidence of post extubation stridor (RR 0.47; 95% CI 0.22 to 0.99), there was significant heterogeneity (I(2)=81%, X(2)=26.36, df=5, p<0.0001). Subgroup analysis revealed that post extubation stridor could be reduced in adults with a high likelihood of post extubation stridor when corticosteroids were administered as multiple doses begun 12-24 hours prior to extubation compared to single doses closer to extubation; the test for interaction for multiple versus single doses indicated RRR 0.22 (95% CI 0.10 to 0.47) for stridor with multiple doses. Side effects were uncommon and could not be aggregated. AUTHORS’ CONCLUSIONS: Using corticosteroids to prevent (or treat) stridor after extubation has not proven effective for neonates or children. However, given the consistent trends towards benefit, this intervention does merit further study, particularly for high risk children or neonates. In adults, multiple doses of corticosteroids begun 12-24 hours prior to extubation do appear beneficial for patients with a high likelihood of post extubation stridor.

Chronic active Epstein-Barr virus infection (CAEBV) is a unique and fatal lymphoproliferative disease (LPD), which often shows high serum IgG and/or IgE. The significance of such immunoglobulin abnormalities in CAEBV has not been fully evaluated and discussed. In addition, such clinical features mimic HIV-1 infection. We report here a case of CAEBV with M-protein detected which may shed a new light on the pathogenesis of this disease.

Paraneoplastic neuropathies have rarely been reported in patients with hematological malignancies. We report herein the case of a 65-year-old Japanese woman with chronic myelomonocytic leukemia (CMML) accompanying chronic inflammatory demyelinating polyneuropathy (CIDP). She had been diagnosed with refractory anemia and subsequently developed CMML with cytogenetic abnormalities including t(3;8)(q26;q24). While regenerating bone marrow following induction chemotherapy, she complained of numbness in the lower legs and then became unable to walk. Clinical and electrophysiological features were consistent with CIDP. Intravenous immunoglobulin treatment was insufficient, although corticosteroids reduced neurological symptoms. This case suggests CIDP as one of the autoimmune phenomena associated with myelodysplastic syndrome and immunosuppressive treatment represents an effective therapy.

The objective of reporting this case was to introduce a unique cause of shoulder pain in a high-level Division I NCAA collegiate baseball player. Various neurovascular causes of shoulder pain have been described in the overhead athlete, including quadrilateral space syndrome, thoracic outlet syndrome, effort thrombosis, and suprascapular nerve entrapment. All of these syndromes are uncommon and frequently are missed as a result of their rarity and the need for specialized tests to confirm the diagnosis. This pitcher presented with nonspecific posterior shoulder pain that was so severe he could not throw more than 50 feet. Eventually, intermittent axillary artery compression with the arm in abduction resulting from hypertrophy of the pectoralis minor and scalene muscles was documented by performing arteriography with the arm in 120 degrees abduction. MRI-MR angiographic evaluation revealed no anatomic abnormalities. The patient was treated successfully with a nonoperative rehabilitation program and after 6 months was able to successfully compete at the same level without pain.

Acetabular retroversion (AR) alters load distribution across the hip and is more prevalent in pathologic conditions involving the hip. We hypothesized the abnormal orientation and mechanical changes may predispose certain individuals to stress injuries of the femoral neck. We retrospectively reviewed the anteroposterior (AP) pelvic radiographs of 54 patients (108 hips) treated for a femoral neck stress fracture (FNSF) and compared these radiographs with those for a control group of patients with normal pelvic radiographs. We determined presence of a crossover sign (COS), femoral neck abnormalities, and neck shaft angle. The prevalence of a positive COS was greater in patients with stress fractures than in the control subjects (31 of 54 [57%] versus 17 of 54 [31%], respectively) and higher than for control subjects reported in the literature. Thirteen patients had radiographic changes of the femoral neck consistent with femoroacetabular impingement (FAI). These radiographic abnormalities were seen more commonly in retroverted hips. A greater incidence of AR was noted in patients with FNSF. Potential implications include more aggressive screening of military recruits with AR and the new onset of hip pain. Finally, we present an algorithm we use to diagnose and treat these relatively rare FNSFs. Level of Evidence: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

The development of effective pharmacotherapy for major depression is important because it is such a widespread and debilitating mental disorder. Here, we have reviewed preclinical and clinical studies on tianeptine, an atypical antidepressant which ameliorates the adverse effects of stress on brain and memory. In animal studies, tianeptine has been shown to prevent stress-induced morphological sequelae in the hippocampus and amygdala, as well as to prevent stress from impairing synaptic plasticity in the prefrontal cortex and hippocampus. Tianeptine also has memory-protective characteristics, as it blocks the adverse effects of stress on hippocampus-dependent learning and memory. We have further extended the findings on stress, memory and tianeptine here with two novel observations: 1) stress impairs spatial memory in adrenalectomized (ADX), thereby corticosterone-depleted, rats; and 2) the stress-induced impairment of memory in ADX rats is blocked by tianeptine. These findings are consistent with previous research which indicates that tianeptine produces anti-stress and memory-protective properties without altering the response of the hypothalamic-pituitary-adrenal axis to stress. We conclude with a discussion of findings which indicate that tianeptine accomplishes its anti-stress effects by normalizing stress-induced increases in glutamate in the hippocampus and amygdala. This finding is potentially relevant to recent research which indicates that abnormalities in glutamatergic neurotransmission are involved in the pathogenesis of depression. Ultimately, tianeptine’s prevention of depression-induced sequelae in the brain is likely to be a primary factor in its effectiveness as a pharmacological treatment for depression.

BACKGROUND AND PURPOSE: Transient global amnesia (TGA) is characterized by sudden anterograde and retrograde amnesia lasting for up to 24 hours. Diffusion-weighted magnetic resonance imaging (DWI) in cases of TGA and ischemia demonstrates a high frequency of high signal intensities restricted to the hippocampus, and this has been proposed as an etiology of TGA. The aims of this study were to characterize the DWI and single-photon-emission computed tomography (SPECT) findings during the acute and recovered phases of TGA and to correlate the findings with oculomotor abnormalities. METHODS: Five consecutive patients with a clinical diagnosis of TGA underwent DWI and SPECT of the brain within 24 hours after symptom onset and again 3 days later. Eye movements were also recorded using three-dimensional video-oculography. RESULTS: In all patients, DWI disclosed small punctuate (1-3 mm), high-signal lesions in the lateral portion of the hippocampus. The initial SPECT also revealed hypoperfusion in the cerebellar vermis, which had recovered by the follow-up examination. Three patients showed saccadic hypermetria or impaired smooth pursuit only during the acute phase. CONCLUSIONS: Our patients with TGA showed cerebellar vermian hypoperfusion in addition to ischemic insults to the lateral hippocampus. The oculomotor abnormalities observed in our patients support the occurrence of cerebellar dysfunction during the TGA attack.

The insulin receptor (IR) plays a crucial role in mediating the metabolic and proliferative functions triggered by the peptide hormone insulin. There is considerable evidence that abnormalities in both IR expression and function may account for malignant transformation and tumour progression in some human neoplasias, including breast cancer. PPARgamma is a ligand-activated, nuclear hormone receptor implicated in many pleiotropic biological functions related to cell survival and proliferation. In the last decade, PPARgamma agonists-besides their known action and clinical use as insulin sensitizers-have proved to display a wide range of antineoplastic effects in cells and tissues expressing PPARgamma, leading to intensive preclinical research in oncology. PPARgamma and activators affect tumours by different mechanisms, involving cell proliferation and differentiation, apoptosis, antiinflammatory, and antiangiogenic effects. We recently provided evidence that PPARgamma and agonists inhibit IR by non canonical, DNA-independent mechanisms affecting IR gene transcription. We conclude that IR may be considered a new PPARgamma “target” gene, supporting a potential use of PPARgamma agonists as antiproliferative agents in selected neoplastic tissues that overexpress the IR.

BACKGROUND:: The pathophysiology and management of epiphrenic diverticula remain controversial. This study investigated the underlying functional abnormalities and long-term results of surgical treatment. METHODS:: Patients with symptoms and epiphrenic diverticula who had undergone long myotomy and Belsey Mark IV fundoplication were reviewed retrospectively. They were assessed before and after surgery by radiology, functional testing and endoscopy, and compared with a group of 40 normal volunteers. RESULTS:: The study included 23 consecutive symptomatic patients who had surgery, 20 of whom had oesophageal spastic disorders. Lower oesophageal sphincter (LOS) incoordination was considered the most constant functional abnormality (P < 0.001). After operation oesophageal diameter increased, contraction pressures decreased and peristalsis was reduced. LOS resting and gradient pressures decreased (P = 0.001). Despite unchanged acid exposure values, endoscopy revealed increased mucosal damage after operation (P = 0.003). New columnar-lined metaplasia was documented in eight patients (P = 0.013). Symptoms had decreased after a median of 61 months (P = 0.001). CONCLUSION:: Epiphrenic diverticulum was associated with spastic dysfunction and LOS abnormalities. A long myotomy including the LOS relieved functional obstruction and symptoms, but partial fundoplication did not prevent reflux damage. Copyright (c) 2009 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.

BACKGROUND: Moderate caffeine intake during pregnancy is common, but little is known about its potential association with birth defects. METHODS: The National Birth Defects Prevention Study is a population-based, case-control study of major birth defects, excluding infants with single-gene disorders and chromosomal abnormalities. This analysis includes infants with cleft lip with or without cleft palate (CL/P) and cleft palate only (CPO), excluding infants whose cleft was secondary to holoprosencephaly or amniotic band sequence. Mothers reported dietary caffeine intake from coffee, tea, sodas, and chocolate in the year before pregnancy and reported intake of medications containing caffeine during pregnancy. We assessed the association between dietary caffeine intake, frequency of consuming each type of caffeinated beverage, medications containing caffeine, and CL/P or CPO among infants born from October 1997 through December 2004. RESULTS: This analysis included 1531 infants with CL/P, 813 infants with CPO, and 5711 infants with no major birth defects (controls). Examining dietary sources among control mothers, 11% reported consuming at least 300 mg of caffeine per day and 17% reported consuming less than 10 mg of caffeine per day; high consumption (>/=3 servings per day) was reported by 8% (coffee), 4% (tea), and 15% (sodas); medications containing at least 100 mg caffeine/dose were reported by less than 1%. Although some effect estimates were elevated for moderate caffeine intake from all beverages, estimates were closer to the null for high caffeine levels. Isolated CL/P was associated with use of medications containing at least 100 mg of caffeine per dose. CONCLUSIONS: Our data do not suggest an association between maternal dietary caffeine intake and orofacial clefts, but caffeine-containing medications merit further study. Birth Defects Research (Part A), 2009. (c) 2009 Wiley-Liss, Inc.