Membranous lipodystrophy

Summary The case is described of a 35-year-old housewife diagnosed as having membranous lipodystrophy (as described by Nasu et al. in 1970 and called lipomembranous polycystic osteodysplasia by Hakola in 1972). The main symptom of this patient was a slowly progressive dementia. Skeletal symptoms were not seen. The computerized tomogram of the brain showed calcification of bilateral basal ganglia and the plain roentgenograms of the bones revealed cystic radiolucent areas at the distal end of the bones of the patient's extremities. Histological examination of the curetted material from the right talus revealed a membranocystic pattern. The fatty tissue curetted from the cyst of the talus and the lysosomal enzymes of the white blood cells were biochemically normal. A possible relationship between this disease entity and connective disorders is considered.     

Bone involvement pattern in hypervascular lesions

The pattern of the focal bone lesion which consists partly or wholly of rounded holes with comparatively smooth edges is discussed.Twenty-two bone lesions were studied by angiography. The hypervascular pattern occurred in five cases of widely different histology, all with strong intraosseous hypervascularity. Different pathogenic mechanisms in the creation of this pattern are discussed. It is probably the result of both destructive and reparative processes in the bone.     

Mercury-induced necrosis of murine renal cell carcinoma

Administration of the nephrotoxic agent, bichloride of mercury (HgCl₂), to BALB/c mice bearing subcapsular renal tumor transplants induced early preferential necrosis of the tumor cells compared with mercury-treated normal kidney tubular cells and untreated control tumors. The principle is established that a selective proximal tubular poison is also toxic for a tumor of the same cellular origin.     

Osteoclastome-like giant cell thyroid carcinoma controlled by intensive radiation and adriamycin,in a patient with meningioma and multiple myeloma treated by radiation and cytoxan

The eighth case of osteoclastome-like giant cell carcinoma of the thyroid, and the first one to be treated with adriamycin in addition to surgery and radiation, is reported. This rare variant of anaplastic thryoid carcinoma appeared in a patient operated on for meningioma and treated for multiple myeloma with cranial radiation and chronic administration of cytoxan.     

Treatment of myasthenia gravis

Summary In the treatment of myasthenia gravis (MG) considerable progress has recently been achieved. Our experience is based on the observation of 139 patients with an average follow-up of 3 years and 4 months.A treatment plan and results are presented.Indications for thymectomy: all cases of MG in adult life, apart from ocular myasthenia without radiological thymoma and without electrophysiological and pharmacological signs of generalization; before puberty only cases with radiological thymoma and severely incapacitating or life-threatening signs.Median sternotomy is preferable for thymoma, the transcervical approach with a sternal split for non-neoplastic thymus. Mediastinal radiotherapy is indicated after removal of an invasive or adhesive thymoma.Indications for corticosteroids: 1) before thymectomy: respiratory weakness; 2) soon after thymectomy: life-threatening signs; 3) later after thymectomy: incapacitating or life-threatening signs; 4) as an alternative to thymectomy: when surgery cannot be performed or it is not indicated. Oral Prednisone was nearly always preferred: alternate-day high single dose (75 to 115 mg) has given good results in most cases even if in some cases a small dose was required in the off day; inversely a lower alternate-day or daily dose was often sufficient.Long-term results: following this schedule for adult patients good results were scored in 67% of thymomas, in 94% of hyperplasias, and in 62% of unthymectomized patients: in prepuberal life the few cases of severe MG have all shown a favorable evolution.

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Zusammenfassung Bei der Behandlung der Myasthenie wurden in letzter Zeit beachtliche Fortschritte erzielt. Wir teilen hier unsere Erfahrungen anhand von 139 Patienten mit einer durchschnittlichen Katamnese von 3 Jahren und 4 Monaten mit.Wir betrachten als Indikationen für eine Thymektomie: alle Fälle von Myasthenie beim Erwachsenen mit Ausnahme der rein okulären Formen ohne radiologisch nachweisbares Thymom und ohne elektrophysiologische oder pharmakologische Zeichen einer Generalisierung; bei Kindern vor der Pubertät empfehlen wir die Thymektomie nur in Fällen mit radiologisch nachweisbarem Thymom und mit schwerer Beeinträchtigung oder gar Lebensgefährdung durch die Symptome.Die mediane Sternotomie ist beim Thymom vorzuziehen, der transzervikale Zugang mit Spaltung des Sternums für die nicht neoplastischen Thymusvergrößerungen. Mediastinale Strahlentherapie ist nach Exstirpation eines invasiven Thymoms oder eines Thymoms mit Adhäsionen angezeigt.Als Indikation für die Corticosteroidtherapie betrachten wir: 1. wenn vor der Thymektomie Atemstörungen bestehen; 2. wenn bald nach der Thymektomie lebensbedrohliche Symptome auftreten; 3. wenn später nach Thymektomie nennenswert behindernde oder lebensbedrohliche Symptome in Erscheinung treten; 4. als Alternative zur Thymektomie, wenn diese nicht durchgeführt werden kann oder nicht indiziert ist. Die orale Prednisontherapie wurde fast immer vorgezogen: wir gaben an alternierenden Tagen jeweils hohe Einzeldosen (75–115 mg) mit gutem Erfolg in den meisten Fällen. In gewissen Fällen war eine kleine Dosis an den Tagen zwischen der Hauptdose aber genügend, ebenso in Einzelfällen eine allgemein niedrigere Dosierung. Die Langzeiterfolge mit diesem therapeutischen Vorgehen betrugen 67% gute Ergebnisse bei Thymomen und 94% bei Thymushyperplasie. Unter den nicht thymektomierten Patienten wiesen 62% ein gutes Ergebnis auf. Vor der Pubertät zeigten die allerdings wenigen Fälle schwerer Myasthenie alle ein gutes Ansprechen auf die Therapie.

     

The rotational component in the dynamics of the C2–3 spinal segment

Summary The technique of the PA axial projection of the arches of the upper cervical vertebrae into the occipital foramen and the normal findings, are described. The influence of maximum forced anteflexion at the craniocervical junction and rotation of the head on the relations of atlas and axis is demonstrated. It seems that in this position rotation at atlasaxis level is restricted and partly transmitted to the C_2–3 segment. Thus, selective clinical examination of the rotational component of the dynamics at C_2–3 can be achieved and the pathological restrictions of movement can be assessed.     

Release of vasoactive intestinal peptide in the central nervous system in man.

Recent work has shown that vasoactive intestinal peptide (VIP), one of the many candidate hormones of the gut, also occurs widely in neurones. To determine whether the neuronal peptide may have a neurotransmitter function, we studied changes in immunoreactive VIP in dog plasma and human cerebrospinal fluid after the infusion of choline esterase inhibitors (neostigmine and physostigmine, respectively). Immunoreactive VIP was released in both situations. The systemic changes (in VIP levels) were enhanced five weeks after portacaval shunting in dogs. Our results demonstrate that the immunoreactive VIP level increases as a result of choline esterase inhibitors. The plasma "release" may originate either from peripheral peptidinergic nerve terminals or from APUD cells of the gastroenteropancreatic system. The increase in immunoreactive cerebrospinal fluid VIP may very well originate from central neurons, since the peptide does not apparently cross the blood-brain barrier.     

Traumatic injuries of the abdominal aorta.

Despite immediate operation, patients with abdominal aortic injuries and profound hypovolemic shock do not respond to the usual methods of resuscitation and die soon after celiotomy, prior to control and repair of the aortic injury. The rate of aortic hemorrhage exceeds the ability to restore blood volume. Shock becomes "irreversible." In such patients tamponade of the aortic injury may be effected by the use of an external counterpressure device such as a G-suit or MAST suit. These devices, used in conjunction with transthoracic aortic occlusion, may raise blood pressure sufficiently to perfuse the sritical coronary and cerebral circulation, allowing time to correct acidosis and locate, control, and repair the aortic injury. Early aggressive therapy should result in increased survival.     

Glutamic acid concentration in human semen--its origin and significance

Glutamic acid content of semen was determined photometrically in over 400 semen specimens. Glutamatic acid content was found to increase proportionately to temperature in the first few hours post ejaculation. The glutamate estimation should therefore be performed on semen at 30 min. after ejaculation. The frequency distribution of glutamic acid concentration with 400 unselected specimens, 50 "normozoospermias" as well as 42 azoospermias is shown. The mean value of normozoospermias was 10.6 mg% (+/- 4.6 mg%) that of azoospermias 7.7 mg% (+/- 4.7 mg%). There was no correlation found between glutamic acid content and pH value. A correlation however, was demonstrated between glutamic acid and following: sperm count, citrate concentration, gamma-GT and carnitine. No correlation to fructose content was detectable. That suggests that glutamate gets to the ejaculate with the secretions from the prostate gland and epididymis whereas the seminal vesicles do not play a role in the level in the total semen.     

Study of preclinical changes in workers exposed to inorganic mercury in chloralkali plants

Summary The aim of the present work was to clarify the question of preclinical changes of Hg intoxication (micromercurialism) in man. The study to detect these disorders was performed on 39 chloralkali plant workers who had been exposed to mercury for more than 7 years. The ambient air, urine and blood values of the last few years were determined in extensive measurements by various methods and related to one another. The average ambient air concentrations were clearly below the currently applicable threshold limit value (German MAK) of 0.1 mg/m³. For the purpose of clarifying the mentioned question of preclinical changes of intoxication, the exposed persons were subjected to psychomotor-function examinations and compared with a group of nonexposed persons. The blood pressure and pulse frequency values of both groups were also determined and compared with one another. No significant differences between the two groups of persons examined were detectable.Presented at the 18th Congress of the Deutsche Gesellschaft für Arbeitsmedizin in Frankfurt, 24th May 1978