Cavernous sinus involvement by extramedullary plasmacytoma of the sphenoid sinus. An argument for the use of adjuvant chemotherapy

A 63-year-old man with cavernous sinus involvement from extramedullary plasmacytoma (EMP) of the sphenoid sinus is described. Transient resolution of retro-orbital headache and continued progression of the locally extensive tumor were noted after chemotherapy was given following a poor response to 5400 cGy of local irradiation. To determine whether adjunctive chemotherapy will improve the outcome of these particular patients, we propose that a randomized trial comparing radiotherapy to chemoradiation be conducted.     

Axonopathy, tau abnormalities, and dyskinesia, but no neurofibrillary tangles in p25-transgenic mice

Neurofibrillary tangles, one of the pathologic hallmarks of Alzheimer's disease (AD), are composed of abnormally polymerized tau protein. The hyperphosphorylation of tau alters its normal cellular function and is thought to promote the formation of neurofibrillary tangles. Growing evidence suggests that cyclin-dependent kinase 5 (cdk5) plays a role in tau phosphorylation, but the function of the enzyme in tangle formation remains uncertain. In AD, cdk5 is constitutively activated by p25, a highly stable, 25kD protein thought to be increased in the AD brain. To test the hypothesis that p25/cdk5 interactions promote neurofibrillary pathology, we created transgenic mouse lines that overexpress the human p25 protein specifically in neurons. Mice with high transgenic p25 expression have augmented cdk5 activity and develop severe hindlimb semiparalysis and mild forelimb dyskinesia beginning at approximately 3 months of age. Immunohistochemical and ultrastructural analyses showed widespread axonal degeneration with focal accumulation of tau in various regions of the brain and, to a lesser extent, the spinal cord. However, there was no evidence of neurofibrillary tangles in neuronal somata or axons, nor were paired helical filaments evident ultrastructurally. These studies confirm that p25 overexpression can lead to tau abnormalities and axonal degeneration in vivo but do not support the hypothesis that p25-related induction of cdk5 is a primary event in the genesis of neurofibrillary tangles.     

Spectroscopic data following stroke reveal tissue abnormality beyond the region of T2-weighted hyperintensity

We examined the characteristic clinical features of one family of familial amyotrophic lateral sclerosis (FALS) with a His46Arg mutation in the enzyme Cu/Zn superoxide dismutase-1 (SOD1). The disease duration for this family was 18.1 +/- 13.2 (mean +/- S.D.) years, with the age at onset being 39.7 +/- 10.5 years old (mean +/- S.D.). The initial sign was distal weakness of the unilateral lower limb, extending to the lower limb of the other side. A wheel chair became necessary at 9.8 +/- 3.2 years after the onset. Upper limb weakness started at 15.5 +/- 8.9 years following from the onset. An autopsy was performed on a 71-year-old woman of the family with the mutation. Her disease duration was 47 years, and she died of pneumonia. She had no clear upper motor neuron involvement. Bulbar sign and respiratory muscle weakness had developed 2 years before her death. Neuropathological findings showed degeneration of corticospinal tracts, anterior/posterior spinocerebellar tracts, posterior columns, and Clarke's columns. There were few anterior horn cells in the lumbar spinal cord and no Lewy body-like hyaline inclusion bodies in these remaining anterior horn neurons. This is the first autopsy report of FALS with a His46Arg mutation in the SOD1 enzyme.     

A realistic approach to managing patients with fibromyalgia

Fibromyalgia syndrome is common and variable in impact, with some patients having a milder and shorter duration of symptoms and others suffering significant and prolonged pain. Disability also varies. It is thought that the syndrome arises from a disordered neurophysiology that, through links to central control inputs, involves emotions, thoughts, and cognitions. Social and psychological sequelae contribute to and result from this process. The biopsychosocial model of disease epitomizes fibromyalgia. Although management may be difficult at times, and much needs to be done, the growing appreciation of strategies that use this described model and the knowledge of the potential reversibility of the syndrome are resulting in improved outcomes.     

Lipoprotein-complexed C-reactive protein and the biphasic transmittance waveform in critically ill patients

The 'biphasic transmittance waveform' (BTW) refers to a decrease in light transmittance that often occurs prior to clotting in coagulation assays of critically ill patient plasmas. It correlates with disseminated intravascular coagulation and mortality. The present work shows that the BTW is due to the rapid formation of a precipitate and a coincident change in turbidity in re-calcified plasma. The precipitate was isolated from patient plasma and contained lipids typical of very low density lipoprotein (VLDL), plus the proteins apolipoprotein B-100 and C-reactive protein (CRP). Precipitation also occurred in normal plasma supplemented with CRP. In addition, CRP precipitated with VLDL and intermediate density lipoprotein, but not low density lipoprotein or high density lipoprotein. The Kd value for the CRP/VLDL interaction is 340 nM. The IC50 value of Ca2+ for complex formation is 5.0 mM, and epsilon-aminocaproic acid inhibits the process. In 15 plasmas with the BTW from critically ill patients, CRP was highly elevated (77-398 microg/mL) and VLDL cholesterol ranged from 0.082 to 1.32 mM. The magnitude of the turbidity change on re-calcification correlated well with the calculated level of the CRP/VLDL complex. Thus, the Ca2+-dependent formation of a complex between CRP and VLDL accounts for the BTW.     

Intraoperative magnetic resonance: the future of surgery

Intraoperative magnetic resonance imaging (iMRI) is a new development in medicine that bridges the specialties of surgery and radiology. Deficiencies in the visualization of anatomical architecture and the perception of tumour boundaries in conventional open surgery have led to the integration of imaging within surgery. The superior soft tissue and multiplanar imaging features of magnetic resonance (MR) make this imaging modality superior to that of alternatives. The unique properties of MR to detect heat change and perfusion, and diffusion characteristics of tissue enhance the usefulness of this medium. Concurrent developments in computer aided image guidance and thermoablative technology, herald the era of minimally invasive tumour ablation. Applications have been developed for areas such as neurosurgery, general surgery, gynaecology and urology.     

Ventricular tachycardia following ingestion of a commonly used antihistamine

This report describes ventricular tachycardia, likely to have been torsade de pointes, following ingestion of the non-sedating antihistamine loratadine. Documentation of the arrhythmia was made possible by the automatic electrogram storage facility of an implanted defibrillator in a patient with no prior history of cardiac arrhythmia.