Corticomotoneuronal dysfunction in ALS patients with different SOD1 mutations.

OBJECTIVE: To examine corticomotoneuronal function in amyotrophic lateral sclerosis (ALS) patients carrying superoxide dismutase 1 (SOD1) mutations using peristimulus time histograms (PSTH). METHODS: Six I113T, 3 A4V, one G41D and one G114A patient were studied along with 21 healthy control subjects. Analyses included comparison with previously reported data from 8 D90A homozygous and 12 sporadic ALS (SALS) patients examined by the authors using identical methodology. RESULTS: Cortical threshold was significantly reduced in A4V patients (41.3%) compared to I113T (58%), SALS (57%) and D90A (71%) patients, as well as healthy controls (49.7%). Estimated excitatory postsynaptic potentials (EPSPs) were significantly larger in A4V patients (4.39 mV) compared to healthy controls (2.95 mV), I113T (2.71 mV) and SALS (2.39 mV) patients. Clinical features and PSTH parameters in I113T were similar to SALS, however, PSTH primary peaks (PP) were significantly more dispersed, 9.5 ms compared to 4ms in SALS. PSTHs from single G41D and G114A patients were unremarkable, apart from large EPSP amplitudes in the G114A patient. CONCLUSIONS: ALS patients with A4V and I113T SOD1 mutations have distinctive corticomotoneuronal changes that are different from those in D90A homozygous and SALS patients. SIGNIFICANCE: PSTH studies should be considered for future in vivo studies of SOD1 pathophysiology in ALS.     

Video-enhanced visualization of the larynx and intubation with the Bullard laryngoscope — equipment report

PURPOSE: A simple technical solution is presented to provide video transmission from the tip of a Bullard laryngoscope to a bedside video display, while the operator is still able to look through the viewing ocular of the Bullard laryngoscope during tracheal intubation. EQUIPMENT: This is achieved by insertion of an ultrathin fibreoptic video-endoscopic system into the working channel of the Bullard laryngoscope. Thereby the view from the distal blade tip is transmitted to a bedside monitor, without interfering with the use of the Bullards laryngoscope's original eyepiece. The presented technical solution allows video transmission without considerable additional weight normally associated with attaching video endoscopy cameras, light and camera cables to endoscopic devices. Thus, the Bullard laryngoscope remains lightweight and easy to maneuver. A screw-threaded adapter with a side-port is proposed to prevent displacement of the fibreoptic cable while still allowing application of oxygen. CONCLUSION: Experience and skills with tracheal intubation using the presented video-enhanced Bullard laryngoscope can be achieved in the originally intended way, while the supervisor or attending viewers can follow the tracheal intubation procedure on the video display.     

Comparison of three different purification methods for the routine preparation of [11C] Metomidate.

PET with (R)-[O-methyl-11C] metomidate ([11C] MTO) is an attractive method for the characterisation of adrenal masses discriminating lesions of adrenal cortical origin from noncortical lesions. [11C] MTO was prepared by the reaction of [11C] methyliodide with the corresponding free acid. Three purification methods have been compared. The method of choice uses preparative HPLC with a ready-to-use weak acidic solvent.     

Neue Entwicklungen in der systemischen Psoriasistherapie

The current standard systemic therapeutic modalities for psoriasis have many potential side effects. Progress made in the understanding of the pathophysiology of psoriasis as a T‐cell‐mediated dermatosis provide options for new more precise therapeutic approaches. These immunological therapeutic strategies involve the inhibition/depletion of activated T‐lymphocytes, the inhibition of antigen presentation and thus the regulation of T‐cell activation, the inhibition of adhesion of inflammatory cells, the inhibition of effects of proinflammatory mediators and the administration of antiinflammatory cytokines. This article summarizes these new systemic therapeutic approaches. Clinical results in the early studies have been mixed. In the next years further results of phase II‐ and phase III‐studies may be expected, which should allow better assessment of the potential of those particular approaches. Some of these approaches could lead to the approval of new drugs to treat psoriasis and to enhance or replace already existing therapeutic options. Furthermore results of therapeutic experiments should contribute to a better understanding of the disease. As we learn which mechanisms are more or less important for the disease, we will be better able to plan intervention strategies.     

Multi-visceral upper abdominal resection and orthotopic liver transplantation--a surgical treatment concept for regionally metastatic tumors of the endocrine pancreas]

Islet cell carcinoma is rare. Even in advanced metastasizing tumors, relatively long survival times were reported. Aim of surgical treatment is complete removal of the tumor mass, if possible. When not, palliative procedures were described to be efficient in some of the cases. In the literature, among the first patients with regional metastasizing intraabdominal malignancies and upper abdominal exenteration, some cases with neuroendocrine tumors were reported. Aim of the following study is to describe the results in 4 patients with metastasizing islet cell carcinomas, which were treated by multi-visceral upper abdominal exenteration and combined orthotopic liver transplantation.     

Adolescent suicides with adjustment disorders or no psychiatric diagnosis

Detailed characteristics of adolescent suicides (aged 13–19 years) with adjustment disorders (AD) (N=11) or no psychiatric diagnosis (N=3) in a nationwide adolescent suicide population (N=53) from Finland are presented. The data were collected in a psychological autopsy study of all suicides in Finland (N=1397) during a 12-month period in 1987–1988. Data collection included thorough interviews with the victims' family members and professionals, and information from official records. All the suicides with AD or no diagnosis were males. Most of these victims used highly lethal suicide methods. Previous psychiatric treatment and previous suicide attempts were rare. They were seldom under the influence of alcohol when committing suicide. The process leading to suicide seemed to be of relatively short duration. According to informant reports, withdrawn or narcissistic individual characteristics predominated in many cases. Psychosocial stressors preceding suicide often involved interpersonal losses or conflicts. Talking of suicidal intentions prior to the act was common, indicating the need to take seriously all adolescents' expressions of intended suicide, even in the absence of explicit psychopathology.

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Zusammenfassung Es werden detaiilierte Beschreibungen von Selbst-morden im Jugendalter, verbunden mit Anpassungsstörungen (N=11) oder keiner psychiatrischen Diagnose (N=3) vorgestellt. Diese stammten aus der Gesamtheit aller jugendlichen Selbstmörder (N=53) aus Finnland. Die Daten wurden in einer psychologischen Obduktionsstudie aller Selbstmörder Finnlands (N=1397) während eines einjährigen Zeitraumes zwischen 1987 und 1988 gesammelt. Die Datenerhebung umfaßte ausgiebige Interviews mit den Familienmitgliedern, beteiligten Fachkräften und Informationen von offizieller Seite. Die Selbstmörder mit Anpassungstörungen oder keiner Diagnose waren alle männlich. Die meisten dieser Opfer benützten sehr letale Suizidmethoden. Vorangegangene psychiatrische Behandlungen und Selbstmordversuche waren selten. Sie standen nur selten unter dem Enfluß von Alkohol, als der Selbstmord vollzogen wurde. Der Prozeß, der zu dem Suizid führte, schien von relativ kurzer Dauer zu sein. Entsprechend den Angaben der Informanden prädominierten in vielen Fällen zurückgezogene oder narzißtische Persönlichkeitsmerkmale. Psychosoziale Stressoren, die dem Suizid vorangingen, umfaßten interpersonelle Verluste oder Konflikte. Häufig wurde über die Suizidabsichten vor der Tat gesprochen. Dies verdeutlicht die Notwendigkeit, alle Suizidäußerungen von Jugendlichen ernstzunehmen, selbst dann, wenn eine explizite Psychopathologie fehlt.

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Résumé Les caractéristiques détaillées de suicides d'adolescents (âgés de 13 à 19 ans) avec troubles avec l'adaptation (AD) (N=11) ou l'absence de diagnostic psychiatrique (N=3) sur une population de suicides d'adolescents s'étendant sur l'ensemble du pays (N=53), la Finlande sont présentées. Les faits ont été collectés dans le cadre d'une étude d'autopsie psychologique de tous les suicides en Finlande (N=1397) pendant une période de 12 mois 1987–1988. Le recueil des faits incluait des interviews avec les membres de la famille des victimes et les professionels ainsi que les informations provenant de documents officiels. Tous les suicides avec un trouble de l'adaptation ou sans diagnostic étaient masculins. La plupart de ces victimes utilisait des méthodes hautement mortelles. Les traitements psychiatriques précédents et les tentatives précédentes de suicides étaient rares. Ils étaient rarement sous l'influence de l'alcool quand ils ont commis les suicides. Le processus conduisant au suicide semblait être de durée relativement brève. Selon les rapports d'information, une attitude de retrait ou des caractéristiques individuelles de retrait prédominaient dans la majorité des cas. Les facteurs de stress psycho-sociaux précédent le suicide impliquaient souvent des pertes inter-personnelles ou des conflits. Parler du désir du suicide devant le passage à l'acte était habituel indiquant la nécessité de prendre au sérieux tous les adolescents exprimant le désir de suicide, même en l'absence d'une psychopathologie explicite.

     

The course of rehabilitation in patients with locked-in syndrome]

On the basis of observations of the course taken by 5 patients with so-called Locked-in Syndrome (LIS), the level of functional performance that can be achieved by long-term rehabilitation notwithstanding very severe initial symptoms is set out. The priority measures in the early phases of the condition and the particular problems of long-term rehabilitation are summarized in a checklist. Technical aids to ameliorate communication are dealt with in some detail. Special importance is attributed to patient-centered psychotherapeutic support and to family involvement.     

Fibre type characteristics of the lumbar paraspinal muscles in normal healthy subjects and in patients with low back pain

A knowledge of the alteration in the fibre type profile of paraspinal muscle associated with low back pain is essential for the design of successful rehabilitation programmes. In attempting to compare the muscles of patients with low back pain with those of controls, few previous studies have considered factors such as gender, age, and size of the subjects, each of which can potentially confound interpretation of the results. We obtained samples of lumbar paraspinal muscle during spinal surgery from 21 patients with low back pain and, using the percutaneous biopsy technique, from 21 control volunteers matched for gender, age, and body mass. The samples were subject to routine histochemicsl typcal analysis to determine characteristics of muscle fibre type. Compared with controls, the muscle of the patients had a significantly higher proportion of type-IIB (fast-twitch glycolytic) fibres than type- I (slow oxidatve) fibres. The mean size of a given fibre type did not differ between the patients and the controls. Consequently, the relative area of the muscle iccupied by type-IIB fibres was higher and that by type-I fibres Was lower in the patients. The patients had a greater number of muscle samples with more than 1% type-IIC fibres, and abnormalities that could be described as pathological were more marked in the patients than in the controls. In conclusion, the paraspinal muscles of patients who have low back pain display a more glycolytic (faster) profile; this can be expected to render them less resistant to fatigue.     

Fluorine-18 fluorodeoxyglucose positron emission tomography and iodine-131 whole-body scintigraphy in the follow-up of differentiated thyroid cancer

Metastases of differentiated thyroid cancer may show different uptake patterns for fluorine-18 fluorodeoxyglucose and [¹³¹I]NaI. FDG positron emission tomography (PET), iodine-131 whole-body scintigraphy (¹³¹I WBS) and magnetic resonance imaging were performed in 58 unselected patients, and spiral computed tomography (CT) of the lung in 25 patients. Thirty-eight patients presented with papillary carcinomas, 15 patients with follicular carcinomas and five patients with variants of follicular carcinoma. Primary tumour stage (pT) was pT1 in 3, pT2 in 19, pT3 in 11 and pT4 in 25 cases. For the detection of metastases, FDG PET was found to have a sensitivity of 50%, ¹³¹I WBS a sensitivity of 61%, and the two methods combined a sensitivity of 86%. When FDG PET was limited to patients with elevated thyroglobulin (Tg) levels and negative ¹³¹I WBS, the sensitivity of this algorithm was 82%. Of the 21 patients with lymph node metastases, seven presented with FDG uptake but no iodine uptake. In four of them, a second FDG hot spot appeared in a lymph node metastasis of normal size. Five of the seven patients underwent surgery. None of the eight patients with pulmonary metastases smaller than 1 cm exhibited FDG uptake, while five of them had iodine uptake. All had positive results on spiral CT. In conclusion, FDG PET cannot be substituted for ¹³¹I WBS. If the Tg level is elevated and ¹³¹I WBS is negative, FDG PET can be used to detect lymph node metastases and complements anatomical imaging. A spiral CT of the lung is useful to exclude pulmonary metastases before planning a dissection of iodine-negative lymph node metastases. Received 2 May and in revised form 8 July 1997