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  • 1
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Friedreich-Ataxie ; Heredoataxie ; Kardiomyopathie ; Todesursachen ; Interdisziplinäre Therapie ; Key words Friedreich’s ataxia ; Hereditary ataxias ; Cardiomyopathy ; Cardiac disease ; Interdisciplinary cooperation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Friedreich’s ataxia (FA) represents a degenerative, genetically determined disease of the nervous system in combination with myocardial affection and in some cases endocrinological disturbances. Manifestation of myocardial involvement usually follows symptoms of nervous system degeneration later in the course, but seems not to be secondary. These cardiac disturbances are the main cause of death in FA-patients. Therapeutic management of heart disease is possible and interdisciplinary neurologic-cardiologic cooperation should start early in the course of FA.
    Notes: Zusammenfassung Die Friedreich-Heredoataxie (FA) ist eine degenerative Erkrankung des Nervensystems, kombiniert mit kardialen und teilweise endokrinen Störungen. Das ätiologisch-pathogenetische Bindeglied zwischen der Erkrankung beider Organsysteme ist noch nicht identifiziert. Bei Beginn der Erkrankung steht zunächst die neurologische Symptomatik im Vordergrund. Die Myokardbeteiligung manifestiert sich meist erst im späteren Krankheitsverlauf, bei mehr als 90% der Patienten entwickelt sich eine zunehmende Herzinsuffizienz. Der überwiegende Teil der Patienten mit FA verstirbt aus kardialer Ursache. Histologisch wird eine fibrosierende Kardiomyopathie mit Ventrikelhypokinesie gefunden. Eine nosologische Einordnung der Myokard-Beteiligung ist bis jetzt nicht gelungen. Patienten mit FA sollten angesichts des konstanten Zusammentreffens von neurologischen und internistischen Symptomen schon in frühen Krankheitsstadien gemeinsam von Neurologen und Internisten betreut werden.
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  • 2
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Kraniektomie ; Intrazerebraler Druck ; Transtentorielle Herniation ; Therapie ; Key words Craniectomy ; Lumbar spinal drainage ; Pressure gradients ; Transtentorial herniation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The intracranial space is divided into two large compartments by the tentorium. The hydrostatic pressure of spinal fluid is responsible for buoyancy of the brain within these compartments. In patients with craniectomy this equilibrium is exposed to atmospheric pressure. We report on four cases of reversible herniation after either bilateral or unilateral decompressive craniectomy performed for increased intracranial pressure (ICP) and failure of conservative ICP treatment. All four patients had survived a severe neurological disease (encephalitis, subdural haematoma, stroke) which required craniectomy to control raised ICP. All were successfully weaned from the ventilator and awake and CT scans showed no space-occupying lesion anymore. The patients showed a typical „sunken pattern” at the trepanation site. All patients developed clinical signs of transtentorial herniation (i.e. unilateral dilated pupils, deteriorated alertness, and extensor posturing) shortly after either diagnostic or presumed therapeutic lumbar puncture. One patient developed herniation a second time while in the typical 30° upright position. After craniectomy, transtentorial herniation is possible even in the absence of increased ICP. It is related to a negative gradient between atmospheric and intracranial pressure, which is enhanced by changes in the CSF compartment following lumbar puncture. Lumbar puncture should be avoided if possible and, when necessary, only be performed in the head-down position. Acute therapy in these cases is quite simple; it requires flat or even head-down positioning and early cranioplasty.
    Notes: Zusammenfassung Der intrakranielle Raum wird durch das Tentorium in 2 Kompartimente geteilt. Der hydrostatische Druck des Liquors ist dabei für das Gleichgewicht des Gehirns innerhalb dieser beiden Räume verantwortlich. Bei Patienten mit Hemikraniotomien wird dieses Gleichgewicht dem Atmosphärendruck ausgesetzt. Wir berichten über 4 Patienten mit reversiblen Zeichen einer Herniation nach bilateraler und unilateraler Kraniotomie. Die Kraniotomie wurde zur Behandlung eines maximal erhöhten intrakraniellen Druckes durchgeführt. Nach Entlastungstrepanation waren alle Patienten vom Respirator entwöhnt, Raumforderungszeichen waren nicht mehr nachweisbar. Bei allen Patienten war das Gehirn über dem Trepanationsdefekt „eingesunken”. Zwei der Patienten entwickelten Tage nach durchgeführter Lumbalpunktion das reversibles Bild einer transtentoriellen Herniation (einseitig weite und nicht auf Licht reagierender Pupille, beidseitigen Pyramidenbahnzeichen und Bewußtseinstrübung). Ein Patient zeigte diese Symptomatik auch ohne vorangegangene Lumbalpunktion, hierbei war eine aufrechte Lagerung von 30° Grad ausreichend, die Symptomatik hervorzurufen. Die sofortige Therapie bestand in einer Flach- oder Kopftieflage, balancierter Flüssigkeitszufuhr und früher Deckung des Trepanationsdefektes. Obwohl durch Entlastungskraniotomien eine Herniation vermieden werden soll, sind die von uns beschriebenen „paradoxen” Herniationen seltene, aber unter Umständen fatale Folgen einer Kraniektomie.
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  • 3
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background  Local injections of botulinum toxin type A (BTX-A) have been used successfully to treat focal hyperhidrosis, but because experimental data were lacking, doses have been chosen arbitrarily or empirically. Objectives  To analyse dose dependency and duration of BTX-A-derived suppression of sweat gland activity. Methods  Employing a standardized scheme (four injections, square 2 × 2 cm), different doses of BTX-A [Dysport®; 2·5–120 mouse units (MU)] were injected subcutaneously at the lateral aspects of both of the lower legs in 15 healthy volunteers. Sweat tests were performed before, and 3 weeks and 6 months after, BTX-A injections. Sweating was visualized by staining with iodine starch, and quantified by capacitance hygrometry after carbachol iontophoresis, the quantitative sudomotor axon reflex test (QSART). Results  Iodine starch staining indicated a threshold dose of 10 MU (2·5 MU cm−2) leading to visible anhidrotic skin spots after 3 weeks in all subjects. This was maintained for 6 months with doses of 50 MU (12·5 MU cm−2) or higher, but the size of the anhidrotic skin area decreased over time (P 〈 0·001) indicating partial recovery at the edges. After 3 weeks QSART was significantly reduced (P 〈 0·02) and completely suppressed by doses of 80 MU (20 MU cm−2) or more, although after 6 months QSART increased again to pre-BTX-A levels (P 〈 0·001). Both methods indicated that the suppression of sweating is dose dependent (QSART: r = −0·70, P 〈 0·001; iodine starch staining: r = 0·74, P 〈 0·001). Conclusions  Our findings suggest that BTX-A effectiveness can be quantified by testing sudomotor function. For the first time threshold doses for the suppression of sweating have been defined.
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  • 4
    ISSN: 1432-1238
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 5
    ISSN: 1432-1246
    Keywords: Neurotoxicity ; Organic solvents ; Spray painters
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A multidisciplinary cross-sectional study was performed to examine the chronic neurotoxicity of organic solvents. Participating in the study were 105 persons employed as spray painters and having long-term solvent exposure (10–44 years) and a control group consisting of 58 construction workers, electricians, and plumbers without occupational contact to solvents. Samples were matched for age, preexposure intelligence level, occupation, and socioeconomic status. After controlling for potentially non occupational confounding factors (neuropsychiatric diseases, metabolic disorders, high blood pressure, alcohol intake) 83 spray painters and 42 controls were entered finally into the study. The evaluation included work history, self-rating questionnaire, neurologic investigation, psychiatric analysis using the Present State Examination (PSE), psychological testing, and computerized axial tomography (CAT) of the brain. Physical and neurologic examinations demonstrated no case of overt disorders of the central or peripheral nervous system. An important result of the psychiatric analysis was that the syndromes “special features of depression” and “loss of interest and concentration” occurred significantly more frequently among spray painters than among controls. Further analyses demonstrated an association with chronic exposure over 30 years and repeated acute neurotoxic effects during solvent exposures. Neither psychological nor performance tests demonstrated any statistically significant differences in the performance sets after adjustment according to premorbid intelligence level; this finding supports the presumption of only a low grade of mental dysfunction. Correlation analyses indicated a relationship between subjective health complaints and long-term solvent exposure; however, the effect of age cannot be completely ruled out. Visual evaluation of CAT scans of the brain demonstrated significantly higher values for spray painters on the Cella media index, a measure of the inner liquor system of the brain. None of the other CAT parameters of inner or external brain atrophy showed significant differences. The rate of diffuse cerebral atrophy was not increased in spray painters. No statistical relationship between the solvent exposure index and CAT parameters was found by correlation analysis. In summary, the results do not support the hypothesis of an increased risk of solvent-induced encephalopathy among spray painters. It is not possible to establish a typical picture of central nervous system dysfunction due to chronic solvent exposure. Differences in the frequency of PSE symptoms “special features of depression” and “loss of interest and concentration” could be considered solvent related only if long-term (on average 30-year) exposure in combination with repeated acute neurotic effects had occurred. Cerebral atrophy beyond that of normal aging was not found in long-term exposed spray painters.
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  • 6
    ISSN: 1432-1920
    Keywords: Intracerebral hemorrhage ; Zieve-syndrome ; Intracranial lipid deposits
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In a 42-year-old man, admitted a few hours after an acute cerebrovascular event, CT demonstrated a hyperdense hemorrhage surrounded by a hypodense rim similar to perifocal edema or liquefying blood, thus raising doubts about the acuteness of the event. Laboratory findings revealed Zieve-syndrome (alcoholic hyperlipemia, hemolytic anemia, and alcoholic fatty liver) and negative Hounsfield Unit measurement of the hypodense rim finally identified it as a layer of fat around the clot.
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  • 7
    ISSN: 1435-1420
    Keywords: Key words Tetanus ; autonomic dysfunction ; magnesium sulfate ; Schlüsselwörter Tetanus-infektion ; autonome Störungen ; Magnesiumsulfat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Hintergrund: Für die hohe Letalität bei generalisiertem Tetanus sind häufig autonome Störungen infolge Sympathikusüberaktivierung verantwortlich. Anhand einer Kasuistik wird auf das therapeutische Potential einer adjuvanten Magnesiumtherapie hingewiesen. Fallbericht: Ein 54jähriger Patient entwickelte zwei Wochen nach einer oberflächlichen Hautverletzung zunächst eine Kau-, Schluck- und Sprechstörung und schließlich generalisierte Muskelkrämpfe mit Befall der Atemmuskulatur. Impfschutz gegen Tetanus bestand nicht. Die Basistherapie bestand in Antitoxingabe, Antibiose und hochdosierter Analgosedierung (Midazolam, Sufentanil) und Relaxierung (Cisatracurium) bei künstlicher Beatmung. Zur Beherrschung massiv auftretender autonomer Störungen wurde adjuvant Magnesiumsulfat 10% verabreicht. Hierunter verbesserten sich die enormen Blutdruckschwankungen, verringerte sich die Herzfrequenz und milderten sich die Muskelspasmen. Nach 10wöchiger intensivmedizinischer Therapie und 8wöchiger Rehabilitationsbehandlung war der Patient klinisch unauffällig. Schlußfolgerung: Magnesiumsulfat erwies sich bei einer schweren generalisierten Tetanuserkrankung als adjuvante Therapie zur Kupierung autonomer Störungen und zur Minderung der Muskelspasmen als nützlich.
    Notes: Summary Background: The high mortality rate in severe tetanus frequently is due to sympathetic overactivity. We present a case that demonstrates the usefulness of adjuvant magnesium. Case report: Two weeks after a superficial skin injury a 54-year old patient was admitted because of difficulties in chewing, swallowing, and speech. A few days later generalized muscle spasms occurred involving breathing muscles. He had not been immunized against tetanus by vaccination. Basic therapy included tetanusantitoxin, antibiotics, analgesia (sufentanil), sedation (midazolam) and relaxation (cisatracurium), and mechanical ventilation. There was pronounced autonomic nervous system instability. We used 10% magnesium sulfate to control sympathetic crisis, which resulted in normalization of blood pressure, slight depression of heart rate, and reduction of the muscle spasms. After ten weeks ICU treatment and eight weeks of rehabilitation the patient was in good clinical condition. Conclusion: This experience suggests that adjuvant magnesium is useful in the treatment of sympathetic overactivity and muscle spasms in severe tetanus.
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  • 8
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    German Medical Science GMS Publishing House; Düsseldorf
    In:  70. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Skandinavischen Gesellschaft für Neurochirurgie; 20190512-20190515; Würzburg; DOCV097 /20190508/
    Publication Date: 2019-05-09
    Keywords: ddc: 610
    Language: English
    Type: conferenceObject
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