Late-onset metachromatic leukodystrophy: genotype strongly influences phenotype.

Background: P426L and I179S are the two most frequent mutations in juvenile and adult metachromatic leukodystrophy (late-onset MLD), which, in contrast to infantile MLD, show marked phenotypic heterogeneity.

Objective: To search for genotype-phenotype correlations in late-onset MLD.

Methods: The authors reviewed the clinical course of 22 patients homozygous for mutation P426L vs 20 patients heterozygous for mutation I179S, in which the second arylsulfatase A (ASA) mutation had also been determined.

Safety and immunogenicity of the modified adult tick-borne encephalitis vaccine FSME-IMMUN: results of two large phase 3 clinical studies.

A prospective, randomised, multicentre, single-blind phase 3 study was performed to assess the safety of a vaccination schedule consisting of two vaccinations (21-35 days apart) with the tick-borne encephalitis (TBE) vaccine FSME-IMMUN "adults" (five consecutive lots) in comparison to another licensed TBE vaccine (Encepur), with polygeline) (two lots) in healthy volunteers (n=3966) aged 16-65 years. The safety of the third vaccination with FSME-IMMUN "adults" (6 months after the first vaccination) was investigated in a follow-up study on the same population (n=3705) and TBE antibody titres were analysed pre- and post-vaccination in a subgroup of volunteers (n=564). Following the first vaccination, the overall incidence of fever (> or =38.

[Central bone transport system optimizes reconstruction of bone defects. Results of 40 treatments].

Background: This article describes the results, experiences, and advantages of a bone transport system with one single rope or a fully implantable distraction nail for the treatment of bone defects that totally avoids soft tissue cross traction.

Methods: 40 patients with bone defects were treated by central bone transport systems. 30 post-traumatic defects (12 aseptic, 18 post-septic) and 10 defects after resection of bone tumors (2 benign, 8 malignant).

The bioexpandable prosthesis: a new perspective after resection of malignant bone tumors in children.

Bone reconstruction after resection of malignant bone tumors of the lower extremity in children and adolescents remains a challenge in limb-sparing surgery. Equal limb length at maturity and good functional outcome are the main goals of these surgeries but often are difficult to achieve. Expandable endoprostheses are available but usually require repeated operative interventions for lengthening.

Reduction of high dislocation of the hip using a distraction nail before arthroplasty.

We report the case of a 22-year-old woman who underwent plate and screw fixation for a traumatic left acetabular fracture and fixation with cancellous screws for an associated femoral neck fracture. Two months later, the internal fixation became infected and was removed. This resulted in a painful high dislocation of the hip.

[Tractive force measurement in bone transport--an in vivo investigation in humans].

Bone transport applying the principle of distraction osteogenesis makes it possible to reconstruct long bone defects caused by trauma or resection of bone tumors. The method employing a central cable, developed in Munich, is especially suitable for such applications. The main bone fragments are stabilized by an external fixateur, and bone transport is effected with a single central cable fixed to the tip of the segment, and driven by an external, programmable motor.

[Central cable system--fully automatic, continuous distraction osteogenesis for the lengthening treatment of large bone defects].

Distraction osteogenesis involving bone transport enables the reconstruction of large bone defects. The main bone fragments are usually stablilised externally, an intermediate bone segment is separated and moved through the defect at a rate of about 1 mm/day. New high-quality bone is built up in the constantly enlarging osteotomy gap.

Cartilage atrophy in the knees of patients after seven weeks of partial load bearing.

Objective: It is currently unknown whether human cartilage properties change during short periods of partial load bearing. We used a post-ankle fracture model to explore whether changes in cartilage morphology occur in the knee under conditions of partial load bearing.

Methods: The knees of 20 patients with Weber type B and type C fractures were examined using magnetic resonance imaging.

Lipofundin-induced intracranial pressure rise after severe traumatic brain injury--a case report.

Early nutrition is a recognized component of neurosurgical intensive care treatment. The authors present the case of a patient suffering from severe traumatic brain injury who responded with reproducible intracranial pressure (ICP) crisis to infusion of Lipofundin, a fatty soybean oil-based emulsion for parenteral nutrition. During the described ICP rise, the patient remained hemodynamically stable, therefore an anaphylactic reaction seems to be unlikely.

Neuronavigation combined with electrophysiological monitoring for surgery of lesions in eloquent brain areas in 42 cases: a retrospective comparison of the neurological outcome and the quality of resection with a control group with similar lesions.

The purpose of this study was to achieve a more radical resection of tumors in the area of the motor cortex via minimal craniotomy using a combination of neuronavigation and neurophysiological monitoring with direct electrical cortical stimulation and to compare retrospectively the clinical outcome and postoperative magnetic resonance imaging with a control group that was operated on in our service when the combination of these monitoring techniques was not available. A total of 42 patients with tumors in or near the central region underwent surgery with neuronavigation guidance and neurophysiological monitoring. The primary motor cortex was identified intraoperatively by the somatosensory evoked phase reversal method and direct cortical stimulation.

[Measuring ligament elasticity of the knee joint--elasticity measuring strip and its alternatives].

Those techniques for measuring ligament tension at the knee joint that are most commonly cited and easiest to carry out are discussed. These include four techniques based on the use of strain gauges. Apart from the Omega transducer and the buckle transducer, there is also the tendon force transducer, and the application of strain gauges to the bony ligament insertion sites.

[Dislocation tendency, stabilizing effect and sintering tendency of different lumbar vertebrae cages in an in vitro experiment].

For biomechanical purposes, interbody fusion cages should not dislocate, should provide high stability, and should have a low subsidence risk. Zientek (Marquardt Medzintechnik), Stryker (Stryker Implants), and Ray lumbar interbody fusion cages (Surgical Dynamics) were tested in this study. They were implanted by pairs from a posterior approach without further stabilization.

Pullout test with three lumbar interbody fusion cages.

Study Design: In vitro biomechanical testing was performed on 12 cadaveric human lumbar spines.

Objective: To determine the initial dislocation resistance, as quantified by the pullout force of three different cage designs.

Summary Of Background Data: Interbody cage devices frequently are used as stand-alone cages in the surgical treatment of degenerative conditions in the lumbar spine.

Resistance of the lumbar spine against axial compression forces after implantation of three different posterior lumbar interbody cages.

Background: The aim of using interbody fusion cages is to distract the degeneratively decreased disc height to decompress the neural structures in the intervertebral foramina and allow bony fusion. Prerequisite for a successful fusion therapy is a high resistance against subsidence and breakage.

Method: Three types of implants, a cylindrical threaded titanium cage (Ray) (1c), a bullet shaped PEEK cage (Stryker) (1a) and a rectangular titanium cage with an endplate anchorage device (Marquardt) (1b) were implanted in eight monosegmental lumbar spine specimens (L 2/3 and L 4/5).

Stabilizing effect of posterior lumbar interbody fusion cages before and after cyclic loading.

Object: The function of interbody fusion cages is to stabilize spinal segments primarily by distracting them as well as by allowing bone ingrowth and fusion. An important condition for efficient formation of bone tissue is achieving adequate spinal stability. However, the initial stability may be reduced due to repeated movements of the spine during everyday activity.

[An unusual cause of hepatorenal symptoms].

Case Report: A 65-year-old patient with normal blood pressure had an exclusive elevation of the cholestasis enzymes (alkaline phosphatase 297 U/l, gamma-GT 315 U/l) and elevated bilirubin levels (1.4 mg/dl) since August 1994. A biopsy of the liver in March 1995 showed features of a "subacute viral hepatitis"; DD drug-induced or toxic lesions.