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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Der Chirurg 69 (1998), S. 1345-1351 
    ISSN: 1433-0385
    Keywords: Key words: Myasthenia gravis ; Thymoma ; Transsternal thymectomy ; Postoperative chemoradiotherapy ; Prognostic factors. ; Schlüsselwörter: Myasthenia gravis ; Thymom ; transsternale Thymektomie ; postoperative Chemoradiotherapie ; prognostische Faktoren.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung. Zwischen Mai 1992 und Juni 1997 wurde bei 11 Patienten mit Myasthenia gravis und einem asymptomatischen Thymompatient eine erweiterte Thymektomie über den Zugang einer medianen Sternotomie durchgeführt. Sieben Patienten waren männlich und 5 weiblich. Das mittlere Manifestationsalter der Myasthenia gravis betrug bei den symptomatischen Patienten 46,5 (13–73) Jahre. Vom Erstsymptom bis zur Diagnosestellung vergingen 3,6 Monate (1 Woche – 7 Monate) und bis zur Thymektomie 8,3 Monate (2 Wochen – 36 Monate). Der postoperative Beobachtungszeitraum betrug 28,4 Monate (3–57 Monate). Eine klinische Besserung konnte bei 80 % der Patienten festgestellt werden. Vier Patienten wurden asymptomatisch bei verminderter Medikation. Von der Thymektomie profitierten sowohl die Mehrzahl der über 50 jährigen als auch Patienten mit einem rein oculären Typ der Myasthenia gravis. Wir konnten weder eine perioperative Letalität noch eine Langzeitmorbidität feststellen.
    Notes: Summary. Between May 1992 and June 1997, 11 patients with myasthenia gravis and 1 asymptomatic patient with thymoma underwent extensive thymectomy through a median sternotomy. Seven patients were male and 5 female. The mean age at onset of myasthenia gravis was 46.5 (13–73) years. The interval between the first symptom and diagnosis was 3.6 months (1 week – 7 months), between the first symptom and thymectomy 8.3 months (2 weeks – 36 months) and the mean follow-up period was 28.4 months (3–57 months). Clinical improvement after extensive thymectomy was noted in 80 % of patients. Four patients became asymptomatic under decreased medication. Thymectomy was found to be beneficial even in older patients or patients with the purely ocular type of myasthenia gravis. There was no perioperative mortality or long-term morbidity.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1433-0407
    Keywords: Schlüsselwörter APBD ; N.-suralis-Biopsie ; Branchingenzym ; Key words APBD ; N. suralis biopsy ; Branching enzyme
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We describe a 46 year old patient with adult polyglucosan body disease (APBD). She presented clinically with late onset pyramidal tetraparesis, sensory motor polyneuropathy and micturition difficulties. Magnetic resonance imaging of the brain revealed extensive leucencephalopathy and diffuse atrophy. The diagnosis based on the demonstration of polyglucosan bodies in the sural nerve biopsy. In search of a possible metabolic defect, we evaluated glycogen metabolism in this patient and her clinically unaffected daughters. Branching enzyme activity in the patients leukocytes was between 20–30% of the lower limit of normal range, whereas their children displayed values of 80%, suggesting a possible autosomal recessive mode of transmission. Branching enzyme deficiency in APBD with predominantly attack of the central and peripheral nervous system was so far described in 3 Jewish patients.
    Notes: Zusammenfassung Wir berichten über eine 46jährige Patientin mit adulter Polyglukosankörperkrankheit (APBD). Sie zeigte klinisch mit spätem Beginn eine spastische Tetraparese, sensomotorische Polyneuropathie und Blasenentleerungsstörungen. Die Kernspintomographie des Kopfes erbrachte eine ausgedehnte Leukencephalopathie und eine diffuse Hirnatrophie. Die Diagnose erfolgte durch den Nachweis von Polyglukosankörpern in der Suralisbiopsie. Zum Nachweis möglicher Stoffwechseldefekte untersuchten wir den Glykogenmetabolismus bei der Patientin und ihren klinisch gesunden Töchtern. Die Branchingenzymaktivität war in den Leukozyten der Patientin auf 20–30% und bei den Kindern auf 80% der unteren Grenze des Normalbereichs herabgesetzt, was auf einen möglichen autosomal-rezessiven Erbgang schließen läßt. Ein Branchingenzymdefekt bei APBD mit überwiegender Beteiligung des zentralen und peripheren Nervensystems wurde bislang bei 3 jüdischen Patienten nachgewiesen.
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Schwann cell inclusions ; Demyelination ; Myelinated nerve fibers ; Morphometry ; Peripheral ; neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30–600 μm in diameter. Some of these membrane-bound vesicular or tubular structures contained less dense or no osmiophilic inclusions. Morphometry revealed a reduction of the myelin area per endoneural area to approximately 13% (normal value: 20– 30%) and of the density of myelinated nerve fibers to 5,412/mm2 (normal value at this age: 6,000–9,000/mm2). Large myelinated nerve fibers were predominantly reduced in number, and no myelinated nerve fibers with diameters larger than 4.5 μm were seen. Numerous, usually small onion bulb formations indicated a predominantly demyelinating type of neuropathy. This is to the best of our knowledge the first case of a chronic demyelinating neuropathy in which this kind of presumably pathognostic deposits in the cytoplasm of Schwann cells was detected.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1364-6753
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 5
    ISSN: 1432-1459
    Keywords: Granulocytic sarcoma ; Meningeal extension ; Cerebrospinal fluid cytology ; Cytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Granulocytic sarcoma (GS) is a localized tumour of immature granulocytes that is usually associated with myelogenous leukaemia. We report an unusual case of mastoid GS with meningeal extension but no bone marrow involvement on presentation. Histological examination of the surgical specimen and the characteristic cerebrospinal fluid (CSF) cytology showing cytoplasmic granulations and Auer bodies led to the diagnosis of GS. Positive cytochemical staining of the immature CSF cells for naphtol-ASD chloroacetate esterase and myeloperoxidase confirmed their myeloid origin. Immunophenotyping did not reveal common acute lymphoblastic leukaemia antigen, cytokeratin, T or B-cell antigens. The patient underwent surgical resection of the localized tumour, followed by radiation therapy, intrathecal and systemic chemotherapy, as if he had acute myelogenous leukaemia (AML). He did not develop AML in the 21 months after the tumour resection. This case emphasizes the value of CSF cytological examination of tumour cells and the use of an immumocytochemical marker for differentiating GS from malignant lymphoma.
    Type of Medium: Electronic Resource
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