Axonal swellings in human intramuscular nerves.

The presence, morphology, distribution, and abundance of axonal swellings in intramuscular nerves were evaluated. Axonal swellings were present in intramuscular nerves in 42% of 127 muscle biopsies from patients with a variety of conditions. The incidence was highest in muscle from patients with peripheral neuropathy, but swellings were present in muscle from patients with motor neuron disease, primary muscle diseases, and some individuals without clinical or histological evidence of neuromuscular disease. The greatest number of swellings in intramuscular nerves was in muscle from patients with chronic inflammatory demyelinating neuropathy. Swellings were spherical or elliptical, 4-20 microns in diameter, 5-30 microns in length, and composed of neurofilaments. Swellings were present only in myelinated axons of intramuscular nerves, proximal to nodes of Ranvier or in internodal regions. Swellings were not associated with axonal degeneration. They were probably not transported. The formation or accumulation of swellings may reflect altered axonal dynamics common to a number of disease processes.     

Diffuse peripheral odontogenic fibroma: report of 3 cases

Since peripheral odontogenic fibroma (POF) is characteristically described as a solitary lesion and no diffuse POF had been reported in the literature, our cases should be considered as extremely unusual. Three diffuse cases of POF are described of which one case was seen in association with ocular and skin lesions. The question arises whether POF should be considered as a true odontogenic tumor rather than a diffuse hamartomatous lesion caused by uncontrolled induction of the gingiva. It is also possible that such lesions could be part of a yet undescribed syndrome.     

Activity and distribution of phosphoinositidase C in rat sciatic nerve.

The hydrolysis of phosphatidylinositol-4,5-bisphosphate (PIP2) by rat sciatic nerve cytosolic phosphoinositidase C [phosphoinositide-specific phospholipase C (PIC)] was studied at neutral pH and at ionic concentrations that approximate intracellular conditions. The principal water-soluble product formed was shown to be inositol trisphosphate by anion exchange chromatography. The maximum hydrolysis rate (2.5 nmol/min/mg protein) was achieved at less than 100 nM Ca2+. Hydrolysis was markedly increased to 15 nmol/min/mg protein by inclusion of K+ in the reaction mixture. In the presence of 200 mM K+, the optimum Ca2+ was increased to approximately 600 nM. Higher Ca2+ concentrations progressively inhibited PIP2 hydrolysis. Mg2+ also inhibited the reaction, but the presence of equimolar amounts of ATP and Mg2+ had no effect. Appreciable degradation of phosphatidylinositol-4-phosphate (PIP) also occurred in the nanomolar Ca2+ range, whereas breakdown of phosphatidylinositol (PI) required millimolar Ca2+. The presence of PIP but not PI inhibited PIP2 hydrolysis. Upon subcellular fractionation of nerve, more than 50% of recovered PIC activity was in the cytosol and about 20% was located in a myelin-enriched fraction. Using PIP2 as substrate, PIC activities in nerves from normal and streptozotocin-induced diabetic animals were not different. However, the myelin-associated enzyme from diabetic animals was more labile to freezing and thawing.     

Macrophage-like cells from explant cultures of rat sciatic nerve produce apolipoprotein E

Apolipoprotein E is synthesized and secreted by degenerating peripheral nerve, but the role of resident endoneurial cells in this process is not clear. To exclude the involvement of nonresident cells, we examined the cellular source of endoneurial apolipoprotein E in explant cultures of rat sciatic nerve. The cellular outgrowth from these explant cultures released apolipoprotein E into the culture medium. The cellular outgrowth contained fibroblasts, Schwann cells, and a population of cells with many phenotypic characteristics of macrophages, including the production of apolipoprotein E. No other cell type in the cultures appeared to contribute to this production. These data suggest that apolipoprotein E is produced by resident endoneurial cells in explant cultures and that these cells are macrophages.     

Neuropathy in children and adolescents with diabetes mellitus

Nerve conduction velocities were studied in the median, posterior tibial, radial and sural nerves of 50 juvenile diabetics, average age 13 +/- 1.3 years and mean duration of diabetes 2.3 +/- 1.4 years. Motor conduction velocity (MCV) in the median nerve was reduced in 10% of the subjects, and in the posterior tibial in 32%. Sensory conduction velocity (SCV) in the radial nerve was reduced in 30% of the subjects, and in the sural in 44%. No relationship was found between the reduction in conduction velocity and the duration of diabetes; nevertheless, a correlation was observed between this reduction and the degree of glycaemic control represented by the glycosylated haemoglobin concentration. The authors emphasize the importance of good glycaemic control for the prevention of diabetic neuropathy.     

New approach to management of intraaortic balloon pumps in patients with peripheral vascular disease: Case reports of four patients requiring urgent IABP insertion

Four selected cases of emergent IABP insertion in PV patients are presented. After angiographic documentation of critical iliac stenosis, conservative peripheral angioplasty was performed prior to IABP insertion. No patient experienced a peripheral ischemic event associated with IABP use. © 1992 Wiley-Liss, Inc.     

One-year follow-up of the outcome of a randomized controlled trial of a home-based intervention programme for children with autism and developmental delay and their families

Introduction There is debate about the type and intensity of early childhood intervention that is most helpful for children with developmental problems. The aim of the study was to determine whether a home‐based programme provided over 12 months resulted in sustained improvement in development and behaviour 12 months after the intervention ceased. The characteristics of the children and families who benefited most from the intervention were also studied. Method Randomized controlled trial. Participants A total of 59 children, aged 3–5 years, attending two early childhood intervention centres in Melbourne, Australia. Intervention Half of the subjects received an additional home‐based programme consisting of 40 weekly visits. Main outcome measures Bayley Scales of Infant Development and Wechsler Preschool and Primary Scale of Intelligence Revised, Preschool Behaviour Checklist, Bayley Behaviour Rating Scale and Behaviour Screening Questionnaire. All tests administered pre‐intervention, following the intervention and 12 months later. Secondary outcome measures Family stress, support and empowerment. Results Fifty‐four children completed the assessments 12 months after conclusion of the intervention. Compared with the control group, improvement in aspects of cognitive development in the children who received the extra intervention was sustained 1 year later (P= 0.007) while significant behavioural differences post intervention were not. Analyses of the data by the Reliable Change Index indicated improvement of clinical significance occurred in non‐verbal areas. In contrast to the control group who deteriorated, language skills in the intervention group remained stable. Improvements were significantly associated with higher stress in the families. Conclusion Improvements following the provision of a home‐based programme to preschool children with developmental disabilities were sustained 1 year later. Children from highly stressed families appeared to benefit most, reinforcing the importance of involving families in early childhood intervention programmes.     

Fatal encephalitis in a patient with refractory celiac disease presenting with myorhythmia and carpal spasm.

We report the case of a woman with refractory celiac disease who developed abnormal spontaneous movements of the extremities and face consistent with myorhythmia. Investigation led to a diagnosis of encephalitis, confirmed by postmortem examination. The movements were likely caused by nonparaneoplastic encephalitis associated with refractory celiac disease. Etiologic and diagnostic considerations and treatment options are discussed.     

Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.