Osteoid osteoma of the hip stimulating an early onset of osteoarthritis

Thirty-six patients with osteoid osteomas in the hip were retrospectively studied, including 30 with intraarticular nidi and six with extracapsular nidi. Osteoarthritis developed in 50% of those patients with intraarticular lesions; none of the patients with extracapsular lesions had joint changes. Rheumatologic studies were conducted with eight of the patients with osteoarthritis. The cause of the degenerative joint disease is open to speculation. However, as five of the eight patients had major histocompatibility (HLA) markers for rheumatoid arthritis, the presence of this HLA factor may indicate the patients at risk for inflammatory changes in the joint.     

Autism and epilepsy: cause, consequence, comorbidity, or coincidence?

Autism is associated with epilepsy in early childhood, with evidence suggesting that individuals with both autism and more severe cognitive impairment are at higher risk. However, the incidence of an abnormal electroencephalogram and/or epilepsy in the full range of pervasive developmental disorders (PDDs) is not well defined. This naturalistic study addresses the incidence of epilepsy and electroencephalographic abnormalities in children with PDDs. The clinical history and electroencephalograms of 56 children diagnosed with PDD-not otherwise specified, autism, or Asperger syndrome were retrospectively reviewed. Forty percent of children with autism were diagnosed with epilepsy. Abnormal electroencephalograms and epilepsy occurred at significantly higher rates in children in the more impaired range of the autism spectrum (P<0.05). These findings suggest that the use of neurological investigative techniques such as electroencephalography should be a consequence of careful clinical evaluation and should be considered routinely during evaluation of more impaired individuals.     

Spiral exchange cannula for the occluded drainage catheter

A technique for exchange of occluded drainage catheters or placement of two guide wires into a target using a spiral exchange cannula is described. A spiral exchange cannula with a preloaded sheath and threads at the distal end is "screwed" into the drainage catheter, and the sheath is advanced over the catheter. This method prevents dislodgment of the drainage catheter and also permits easy catheter exchange when the lumen of the drainage catheter is occluded.     

Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila

Spinal and bulbar muscular atrophy (SBMA) is a heritable neurodegenerative disease caused by the expansion of a polyglutamine [poly(Q)] repeat within the androgen receptor (AR) protein. We studied SBMA in Drosophila using an N-terminal fragment of the human AR protein. Expression of a pathogenic AR protein with an expanded poly(Q) repeat in Drosophila results in nuclear and cytoplasmic inclusion formation, and cellular degeneration, preferentially in neuronal tissues. We have studied the influence of ubiquitin-dependent modification and the proteasome pathway on neural degeneration and AR protein fragment solubility. Compromising the ubiquitin/proteasome pathway enhances degeneration and decreases poly(Q) protein solubility. Our data further suggest that Hsp70 and the proteasome act in an additive manner to modulate neurodegeneration. Through the over-expression of a mutant of the SUMO-1 activating enzyme Uba2, we further show that poly(Q)-induced degeneration is intensified when the cellular SUMO-1 protein conjugation pathway is altered. These data suggest that post-translational protein modification, including the ubiquitin/proteasome and the SUMO-1 pathways, modulate poly(Q) pathogenesis.     

Acute flaccid paralysis surveillance in Southern Italy

The purpose of this study was to report the results of the first two years' surveillance programme of acute flaccid paralysis (AFP) in Southern Italy (Calabria). All paediatric, neurology and infectious diseases wards from 23 hospitals were selected. Stool and serum samples to determine the presence of poliovirus were collected. Throat swabs were taken within 10 days of onset of illness. During the period March 1997-April 1999 eight cases of AFP were reported and four of them, three females and one male, occurred in children younger than 15 y of age, although none was confirmed as poliomyelitis. The rate of non-polio AFP in 1997 and 1998 was, respectively, 0.24 and 0.73 per 100 000 persons under 15 y of age. Our results demonstrated that an active surveillance has permitted us to immediately detect AFP cases and to exclude those due to wild polio virus and to vaccine-associated cases and indicated that our area seems to be 'polio-free'.     

Two Cases of Nonconvulsive Status Epilepticus in Association with Tiagabine Therapy

We report two patients with intractable partial seizures who developed generalized nonconvulsive status epilepticus (NCSE) after receiving tiagabine (TGB). Neither had a history of absence seizures or generalized epileptic discharges on prior EEG monitoring. Clinicians need to be aware of a possible association between TGB and NCSE.     

How to improve the physical health of people with severe mental illness? A multicentric randomized controlled trial on the efficacy of a lifestyle group intervention

Background People with severe mental illnesses (SMI) have a mortality rate two times higher compared to the general population, with a decade of years of life lost. In this randomized controlled trial (RCT), we assessed in a sample of people with bipolar disorder, major depressive disorder, and schizophrenia spectrum disorder, the efficacy of an innovative psychosocial group intervention compared to a brief psychoeducational group intervention on patients’ body mass index (BMI), body weight, waist circumference, Framingham and HOMA-IR indexes. Methods This is a multicentric RCT with blinded outcome assessments carried out in six Italian university centers. After recruitment patients were randomized to receive a 6-month psychosocial intervention to improve patients’ physical health or a brief psychoeducational intervention. All recruited patients were assessed with standardized assessment instruments at baseline and after 6 months. Anthropometric parameters and blood samples have also been collected. Results Four-hundred and two patients with a diagnosis of bipolar disorder (43.3%), schizophrenia or other psychotic disorder (29.9%), or major depression (26.9%) were randomly allocated to the experimental (N = 206) or the control group (N = 195). After 6 months, patients from the experimental group reported a significant reduction in BMI (odds ratio [OR]: 1.93, 95% confidence intervals [CI]: 1.31–2.84; p < 0.001), body weight (OR = 4.78, 95% CI: 0.80–28.27, p < 0.05), and waist circumference (OR = 5.43, 95% CI: 1.45–20.30, p < 0.05). Participants with impaired cognitive and psychosocial functioning had a worse response to the intervention. Conclusions The experimental group intervention was effective in improving the physical health in SMI patients. Further studies are needed to evaluate the feasibility of this intervention in real-world settings.     

Leptomeningeal metastasis: MR imaging

Seven patients with central nervous system neoplasia and leptomeningeal metastases, proved either at initial diagnosis or on follow-up with contrast material-enhanced computed tomography (CT), were evaluated with magnetic resonance (MR) imaging. In two patients, diffuse sulcal enhancement on CT scans was inapparent on T1- or T2-weighted MR images. Likewise, in four patients diffuse cisternal enhancement on CT scans was not identifiable with MR. Nodular or focal cisternal masses were identified with both CT and MR imaging in three patients; in two, however, MR imaging provided less information. Ependymal and subependymal metastases identified with CT (two patients) were indistinguishable on MR images from periventricular abnormalities of radiation therapy and/or hydrocephalus. These findings suggest that leptomeningeal metastasis may be so subtle or inapparent as to be overlooked with MR imaging alone. Thus, CT and MR imaging should be considered complementary techniques for initial diagnosis and follow-up of tumors with a propensity for leptomeningeal metastasis.     

Postural assessment in class III patients before and after orthognathic surgery

Purpose

This study aimed to assess body posture before and after bimaxillary orthognathic surgery by photogrammetry in skeletal class III patients.

Methods

Thirty-one patients with skeletal class III dentofacial deformities (14 men, 17 women) who underwent orthodontic preparation for surgery were included in this non-randomized controlled trial. Of these, 15 who did not undergo orthognathic surgery during the period of this study served as controls. Postural assessment was performed by photogrammetry using SAPO® (Postural Assessment Software) based on anterior-, posterior-, and lateral-view images taken 1 month before and 4 months after bimaxillary orthognathic surgery with internal rigid fixation (or 4 months after the initial assessment, for the control group). The study was approved by PUCRS Research Ethics Committee, and written informed consent was obtained from all individual participants prior to their inclusion in the study.

Results

There was no significant difference between groups for age, gender, and GAP. In the intervention group, the right leg/hindfoot angle, which initially indicated a valgus deformity, normalized after intervention (P?<?0.048). Posterior displacement of the head (P?<?0.005) and trunk (P?<?0.004) were observed after intervention.

Conclusions

These results suggest that correction of class III dentofacial deformities by bimaxillary orthognathic surgery can produce systemic postural adjustments, especially posterior displacement of the head and trunk and knee and ankle valgus.

     

Presentation,management,and outcome of newly diagnosed glioblastoma in elderly patients

OBJECT: Optimum management for elderly patients with newly diagnosed glioblastoma(GBM) in the temozolomide(TMZ) era is not well defined.The object of this study was to clarify outcomes in this population.METHODS: The authors retrospectively reviewed 105 consecutive cases involving elderly patients(age ≥ 65 years) with newly diagnosed GBM who were treated at the Mayo Clinic between 2003 and 2008.RESULTS: The patients’ median age was 74 years(range 66-87 years),and the median Karnofsky Performance Status(KPS) score was 80(range 40-90).Half of the patients underwent biopsy and half underwent resection.Patients with deep-seated lesions(19 patients [18%]) or multifocal lesions(34 patients [32%]) were more likely to have biopsy than resection(p = 0.0001 and 0.0009,respectively).New persistent neurological deficits developed in 7 patients(6.7%).Postoperative hemorrhage occurred in 6 patients(5.7%),all of whom underwent biopsy.Complete follow-up data regarding adjuvant treatment was available in 84 patients.Forty-one(49%) were treated with chemotherapy(mostly TMZ) and radiation therapy(RT),and 23(27%) with RT alone.Nineteen(23%) received only palliative care after surgery(more common with biopsy,p = 0.03).Chemotherapy complications occurred in 28.6%(Grade 3 or 4 hematological complications in 11.9%).The median values for progression-free survival(PFS) and overall survival(OS) were 3.5 and 5.5 months.In a multivariate analysis,younger age(p = 0.03,risk ratio [RR] 0.34,95% CI 0.13-0.89),single lesion(p = 0.02,RR 0.51,95% CI 0.30-0.89),resection(p = 0.04,RR 0.54,95% CI 0.31-0.94),and adjuvant treatment(p = 0.0001,RR 0.24,95% CI 0.11-0.49) were associated with better OS.Only adjuvant treatment was significantly associated with prolonged PFS(p = 0.0007,RR 0.27,95% CI 0.13-0.57).With combined therapy with resection,RT,and chemotherapy,the median PFS and OS were 8 and 12.5 months,respectively.CONCLUSIONS: The prognosis for GBM worsens with increasing age in elderly patients.With important risks,resection and adjuvant treatment are associated with prolonged survival.Although selection bias cannot be excluded in this retrospective study,advanced age alone should not necessarily preclude optimal resection followed by adjuvant radiochemotherapy.