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  • 1
    Digitale Medien
    Digitale Medien
    The clinimetrics of hypokinesia in Parkinson's disease: Subjective versus objective assessment (1994)
    Springer
    Journal of neural transmission 8 (1994), S. 117-121 
    Details
    ISSN: 1435-1463
    Schlagwort(e): Hypokinesia ; assessment ; Parkinson's disease
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary In this study we evaluate the feasibility of measures that reflect different characteristics of motor activity and immobility in the objective quantification of hypokinesia. Because by definition hypokinesia can only be assessed over a period of time, continuous activity monitoring was used during 5 successive days in the home setting in 64 patients with Parkinson's disease (PD) and 104 healthy elderly subjects. In the patients we also evaluated the relation between the monitor measures and subjective measures of hypokinesia and age. Compared to the healthy elderly subjects, PD patients have a decreased activity level, increased proportion of time without movement, elevated mean duration of immobility, and decreased percentage of short-lasting immobility periods. Differences between both groups were most prominent for those measures that incorporate or reflect immobility. Moreover, in the PD patients the mean duration of immobility and percentage of short-lasting immobility periods show an apparent lack of relation with age and clinical ratings obtained from the UPDRS. In conclusion, our findings underscore the poor representation of hypokinesia in the UPDRS and value of objective quantification of this fundamental impairment of PD.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02250922
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  • 2
    Digitale Medien
    Digitale Medien
    Sleep, excessive daytime sleepiness and fatigue in Parkinson's disease (1993)
    Springer
    Journal of neural transmission 5 (1993), S. 235-244 
    Details
    ISSN: 1435-1463
    Schlagwort(e): Sleep ; daytime sleepiness ; fatigue ; Parkinson's disease
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The objective of this questionnaire-based survey was to evaluate the prevalence and causes of sleep disturbances in 90 nondepressive patients with Parkinson's disease (PD) and 71 age-matched healthy subjects. We also assessed the prevalence and characteristics of excessive daytime sleepiness (both groups) and excessive fatigue (PD patients). A high prevalence of sleep disturbances in PD patients was found; this is to a large extent probably the result of aging. As compared with controls, patients had a more severely disturbed sleep maintenance because of nycturia, pain, stiffness, and problems with turning in bed. The prevalence of excessive dreaming is similar in both groups, but altered dream experiences almost exclusively occurred in PD. Patients rated themselves more often to be morning-types than controls. This finding may account for the reported adaptation effects in experimental settings and the reduced REM latency in PD patients. The prevalence of daytime sleepiness was similar in both groups. Excessive daytime sleepiness showed a clear diurnal pattern with a peak in the early afternoon. As for excessive fatigue, the majority of the patients did not report a preferential time for this symptom. Our findings further argue against an association of fatigue with any circadian factor, and instead suggest a relationship with the motor deficits of PD.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02257678
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  • 3
    Digitale Medien
    Digitale Medien
    Morphology of cerebral plaque-like lesions in hereditary cerebral hemorrhage with amyloidosis (Dutch) (1992)
    Springer
    Acta neuropathologica 84 (1992), S. 674-679 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Hereditary cerebral hemorrhage with amyloidosis ; Dutch type ; Amyloid β/A4 protein ; Diffuse plaques ; Immunohistochemistry
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary We studied the presence and morphology of plaque-like lesions in the frontal cortex of six patients, aged 40 to 76 years, with hereditary cerebral hemorrhage with amyloidosis — Dutch type (HCHWA-D), using β/A4 immuno-, silver, Congo red and thioflavin S staining. Two types of β/A4 immunoreactive and Congo red-negative plaques were detected. The first type was composed of argyrophilic fibrous material in periodic acid-methenamine silver (PAM) and modified Bielschowsky staining and lacked silver-stained degenerating neurites. Therefore, this type of plaque has the same staining properties as the diffuse plaque described in Alzheimer's disease, Down's syndrome and nondemented elderly. The second type of plaque, occurring only in the three oldest patients and numerically increasing with age, consisted of a spherical non-argyrophilic area of granular texture with a rim of PAM-positive material. The PAM-positive fibrous material of both types of plaques was mingled with coarser and compact, irregular-shaped argyrophilic structures in the oldest patient. The described plaques did not show bright fluorescence with thioflavin S staining. These results indicate, that the morphology of plaques, encountered in HCHWA-D, is diverse and changes with age.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00227745
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  • 4
    Digitale Medien
    Digitale Medien
    Age-related plaque morphology and C-terminal heterogeneity of amyloid β in Dutch-type hereditary cerebral hemorrhage with amyloidosis (2000)
    Springer
    Acta neuropathologica 99 (2000), S. 409-419 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Key words Amyloid β ; Cerebral amyloid angiopathy ; Hereditary cerebral hemorrhage with amyloidosis-Dutch type ; Senile plaques
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The evolvement of amyloid β (Aβ) deposition in the frontal cerebral cortex of 24 patients of increasing age with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) was studied using end-specific monoclonal antibodies to Aβx-42 (Aβ42) or Aβx-40 (Aβ40) and markers for degenerating neurites. Aβ42 immunostaining revealed parenchymal Aβ deposits with a heterogeneous morphology and distribution, i.e., clouds, fine/dense diffuse, coarse, and homogeneous plaques. Clouds and diffuse plaques were associated with glial Aβ granules. Aβ40 labeling was absent in clouds/fine diffuse plaques, inconsistent and variably intense in dense diffuse/¶coarse plaques and consistent in homogeneous plaques. In a subset of Aβ40-positive plaques, degenerating neurites – without tauopathy – and/or amyloid cores were observed. Electron microscopy revealed no apparent amyloid fibrils in fine diffuse plaques, small bundles of fibrils in dense diffuse/homogeneous plaques, and amyloid masses in coarse plaques. The parenchymal Aβ pathology was age-related: the ratio of fine to dense diffuse plaques decreased with age, clouds were limited to younger patients; coarse plaques to the oldest old. Homogeneous/cored plaques were present most consistently in older patients. Plaque density did not increase with age. Vascular Aβ deposits stained for both Aβ species, but exclusively Aβ42-positive, presumably recent deposits were also observed. This study suggests that HCHWA-D is a model of plaque evolution in which clouds leave fine diffuse plaques, which may become dense diffuse and ultimately coarse or homogeneous plaques.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004010051143
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  • 5
    Digitale Medien
    Digitale Medien
    Hereditary cerebral hemorrhage with amyloidosis (Dutch): a model for congophilic plaque formation without neurofibrillary pathology (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 371-378 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Key words Amyloid angiopathy ; Enzymehistochemistry ; Hereditary cerebral hemorrhage with amyloidosis (Dutch) ; Immunohistochemistry ; Senile plaques
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Plaque-like lesions and amyloid angiopathy were investigated in the frontal cerebral cortex of four patients with hereditary cerebral hemorrhage with amyloidosis (Dutch) (HCHWA-D), using immunohistochemical [antibodies to β amyloid protein (Aβ), β protein precursor (βPP), synaptophysin, ubiquitin (UBQ), cathepsin D, paired helical filaments (PHF) and glial fibrillary acidic protein (GFAP)], enzymehistochemical (acid phosphatase) and silver [methenamine silver (MS) and Palmgren] staining methods. Whereas Aβ- and MS-positive diffuse plaques were found in all patients, only the three older patients showed neuritic or congophilic plaques, which were acid phosphatase and cathepsin D positive and contained βPP-, synaptophysin- and UBQ-positive, but PHF-negative neurites. These plaques were surrounded by reactive astrocytes. Similar immuno- and enzymereactivity was found around congophilic blood vessels. Thus, apart from neuronal degeneration in a subset of plaque-like lesions and around blood vessels, this study shows an age-related morphology of the plaques in HCHWA-D, corresponding to that in Down's syndrome (DS), with the difference that neurofibrillary (NF) pathology is absent in HCHWA-D in contrast to DS. HCHWA-D may be considered as a model for congophilic plaque formation not associated with NF pathology.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00310382
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  • 6
    Digitale Medien
    Digitale Medien
    Hereditary cerebral hemorrhage with amyloidosis (Dutch): a model for congophilic plaque formation without neurofibrillary pathology (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 371-378 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Amyloid angiopathy ; Enzymehistochemistry ; Hereditary cerebral hemorrhage with amyloidosis (Dutch) ; Immunohistochemistry ; Senile plaques
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Plaque-like lesions and amyloid angiopathy were investigated in the frontal cerebral cortex of four patients with hereditary cerebral hemorrhage with amyloidosis (Dutch) (HCHWA-D), using immunohistochemical [antibodies to β amyloid protein (Aβ), β protein precursor (βPP), synaptophysin, ubiquitin (UBQ), cathepsin D, paired helical filaments (PHF) and glial fibrillary acidic protein (GFAP)], enzymehistochemical (acid phosphatase) and silver [methenamine silver (MS) and Palmgren] staining methods. Whereas Aβ-and MS-positive diffuse plaques were found in all patients, only the three older patients showed neuritic or congophilic plaques, which were acid phosphatase and cathepsin D positive and contained βPP-, synaptophysin-and UBQ-positive, but PHF-negative neurites. These plaques were surrounded by reactive astrocytes. Similar immuno-and enzymereactivity was found around congophilic blood vessels. Thus, apart from neuronal degeneration in a subset of plaque-like lesions and around blood vessels, this study shows an age-related morphology of the plaques in HCHWA-D, corresponding to that in Down's syndrome (DS), with the difference that neurofibrillary (NF) pathology is absent in HCHWA-D in contrast to DS. HCHWA-D may be considered as a model for congophilic plaque formation not associated with NF pathology.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00310382
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  • 7
    Digitale Medien
    Digitale Medien
    Juvenile Huntington disease (1986)
    Springer
    Human genetics 〈Berlin〉 73 (1986), S. 235-239 
    Details
    ISSN: 1432-1203
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie , Medizin
    Notizen: Summary Of 195 cases of juvenile Huntington disease gathered from case descriptions, the sex, age at onset, duration of disease, clinical type, sex of the affected parent, as well as sex, mean age at onset and at death of adult cases in the same pedigrees were noted when available, and the data were investigated for evidence of relationships between different features. Examination of a subset of 112 cases showed a preponderance of rigid cases, whose affected parent proved to be the father in a significantly high number of cases. Rigid paternal cases had a significantly lower age at onset, as well as a shorter duration of disease than choreatic paternal cases. No such relationship exists between rigid maternal and choreatic maternal cases. In rigid cases duration of disease proved to be shorter for those with a lower age at onset. Female juvenile cases tended to have a lower age at onset than male ones, regardless of clinical type or sex of the affected parent. The mean ages at onset and at death of adult members of the same pedigrees were not related to clinical type or age at onset of juvenile cases. It is argued that juvenile Huntington disease should not be regarded as a separate clinical entity, but as a manifestation of the rigid variant of the disease.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00401235
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  • 8
    Digitale Medien
    Digitale Medien
    Haemorrhagic necrosis of the grey matter of the spinal cord due to accidental injection of iopamidol in a patient with multiple neurofibromas; a clinico-pathological study (1988)
    Springer
    Neuroradiology 30 (1988), S. 178-183 
    Details
    ISSN: 1432-1920
    Schlagwort(e): Myelography, adverse effects ; Neurofibromatosis ; Syringomyelia ; Von Recklinghausen disease
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Cervical laminectomy was performed in a 34-year-old man with multiple spinal neurofibromas because of a slowly progressive medullary compression. Four weeks later a rapid deterioration necessitated iopamidol myelography by left lateral cervical puncture at C2 level. Despite the establishment of adequate spinal fluid contact, resulting in imaging of the subarachnoid space, part of the contrast medium entered the spinal cord, thus delineating a syrinx from the upper cervical extending to the upper thoracic level. After the puncture the patient developed triplegia, involving the left arm and both legs and a paresis of the right arm. He died from aspiration pneumonia. Autopsy revealed haemorrhagic necrosis of the spinal grey matter. This adverse effect of myelography is argued to have been conditioned by the extreme immobility and displacement of the spinal cord due to the presence of multiple neurofibromas. The deterioration four weeks after the operation was probably caused by a further compression of the spinal cord.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00395622
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  • 9
    Digitale Medien
    Digitale Medien
    Hereditary cerebral hemorrhage with amyloidosis-Dutch type (1990)
    Springer
    Neuroradiology 32 (1990), S. 142-145 
    Details
    ISSN: 1432-1920
    Schlagwort(e): SPECT ; Tc-99m HM-PAO ; Hereditary cerebral hemorrhage with amyloidosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary We performed single photon emission computed tomography (SPECT) and cerebral CT-scans in nine patients with hereditary cerebral amyloid angiopathy. CT-scans showed 23 focal hypodense lesions, 13 of which were visible on SPECT as a CBF-defect. One patient showed a CBF-defect on SPECT without CT-scan lesion and had a cerebral hemorrhage three months later in that particular region. In two additional patients, who were 50% at risk for this autosomal dominant disease, CBF-defects on SPECT, but no cortical lesions on CT-scan were found. CT-scans may be more sensitive than SPECT to detect chronic lesions caused by cerebral hemorrhages, but another possibility is that hemorrhages do not always lead to persistent CBF-defects. SPECT can show the effect of amyloid deposits on CBF before the angiopathy causes clinical symptoms.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00588564
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  • 10
    Digitale Medien
    Digitale Medien
    Magnetic resonance imaging in occult spinal dysraphism (1986)
    Springer
    Pediatric radiology 16 (1986), S. 412-416 
    Details
    ISSN: 1432-1998
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The value of magnetic resonance imaging (MRI), compared to myelography and computerized tomography, is illustrated in six patients with occult spinal dysraphism. MRI proved to be the diagnostic tool of choice.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02386821
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