Magnetic resonance imaging in planning limb-salvage surgery for primary malignant tumors of bone

In defining the linear extent of a malignant tumor in a long bone, radiographs, computerized tomography, and scintigraphy are routinely employed, especially when non-ablative surgery is being considered. The drawbacks of these modalities in defining the true intracompartmental extent of disease within a bone can largely be overcome with the use of magnetic resonance imaging. We did a prospective analysis of magnetic resonance imaging in sixteen consecutive patients with a primary malignant tumor of a long bone, and it showed that this modality has clinical promise of being more precise than the other modalities in defining the true proximal and distal extent of a tumor in a long bone. Coronal images permit easier planning of surgical techniques for salvage of a limb using an allograft than do a multiplicity of transverse images.     

Total lymphoid irradiation in chronic polyarthritis--a new therapeutic concept

Eleven patients with refractory rheumatoid arthritis were submitted to a total lymphoid irradiation up to a dose of 20 Gy. A constant improvement of clinical symptoms was observed in four out of the eleven patients already during the treatment and in the other patients not later than two months after. The frequency of attacks decreased and the number of joints involved in the attack was reduced. Morning rigidity and joint swellings decreased. One patient developed joint empyemas 4 and 26 months after the treatment. Four patients died in the meantime. In two patients the cause of death were renal insufficiency and a postoperative cardiogenic shock associated with generalized amyloidosis. The third patient died because of a toxically induced left cardiac decompensation with sepsis that could not be controlled by antibiotic drugs and multiple joint empyemas. The fourth patient developed an abscess after surgical treatment of a Kaposi syndrome. She died three months later from acute left cardiac decompensation. The therapy induced a lymphocytopenia with decrease of T helper lymphocytes and unchanged number of T suppressor lymphocytes. The constant therapy results of total lymphoid irradiation in primary chronic polyarthritis is probably due to this modification in the immune regulation.     

Priapism: a refined approach to diagnosis and treatment

The recent introduction of intracorporeal injections of papaverine and phentolamine for the diagnosis and treatment of impotence has resulted in an increased incidence of iatrogenic priapism. Based on our research into penile hemodynamics we propose a refined approach to all types of priapism. Intracorporeal blood gas and pressure monitoring should be used to differentiate ischemic (low flow) from nonischemic (high flow) types. Most cases of papaverine-induced or phentolamine-induced priapism will respond to aspiration alone or in combination with intracorporeal instillation of a diluted alpha-adrenergic agent. In spontaneous priapism alpha-adrenergic agents can be tried first if patients have only mild or no ischemia. In patients with severe ischemia stagnant blood should be evacuated and a shunt procedure should be performed to allow metabolic replenishment of tissue. Intracorporeal pressure monitoring will help to determine the size and number of shunts needed to re-establish corporeal circulation.     

Timing deficits in apraxia of speech

Summary This paper deals with a particular aspect of speech motor control in patients suffering from apraxia of speech. Three experiments are reported concerning the phase relations between individual speech gestures. These include the timing of laryngeal, velar and labial movements relative to lingual gestures.A total of 8 patients and 12 normal controls were examined using speech material which was designed according to appropriate phonetic paradigms. Evaluation was performed on the basis of speech signal parameters referring to the kinematics of inter-articulatory phasing. Deviations of the patient group were found in all three experiments. This suggests that disturbed phase relations of individual speech movements are a general feature of apraxic speech. It is further hypothesized that the described motor symptoms are the origin of a variety of phonemic errors. Support for this view is provided by appropriate examples which refer to the examined paradigms. By this argument, much of the disturbed phonemic structure of apraxic speech may be accounted for by timing deficits.     

The pattern evoked response in disorders of the optic nerve

The pattern evoked electroretinogram (PERG) was investigated in 11 patients with unilateral optic nerve disease and in a series of age-matched controls. The visually evoked potential (VEP) was also measured. The PERG showed a similar reduction to the VEP in optic nerve disease. Serial studies indicate that the PERG may not be affected immediately in some instances but may show a gradual decline over several months.     

Clinical and molecular findings in IPEX syndrome.

IPEX (immunodysregulation, polyendocrinopathy, enteropathy, X linked syndrome) is a rare disorder which usually results in death in early infancy or childhood. Clinical awareness remains the cornerstone of diagnosis, and provided that the diagnosis is entertained, mutation analysis for FOXP3 gene mutations can be confirmatory. Two new patients in whom IPEX was diagnosed retrospectively are reported.