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  • 1
    Electronic Resource
    Electronic Resource
    Creutzfeldt-Jakob disease with severe involvement of cerebral white matter and cerebellum (1990)
    Springer
    Virchows Archiv 417 (1990), S. 533-538 
    Details
    ISSN: 1432-2307
    Keywords: Creutzfeldt-Jakob disease ; Cerebellar de-generation ; Leucoencephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We describe a patient with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Postmortem examination revealed the characteristic lesions of CJD in the grey matter and profound white matter involvement was seen with immunocytochemical techniques. Ultrastructural white matter lesions were identical to those described in experimentally transmitted CJD. There was marked loss of cerebellar granule cells with virtual disappearance of parallel fibres, but Purkinje cells were only slightly reduced. Electron microscopic studies revealed extensive degenerative changes including cytoplasmic vacuoles in both cell types. Silver methods disclosed massive impregnation of white matter and striking abnormalities of Purkinje cells consisting of hypertrophy and flattening of thick dendritic branches, reduction in the number of terminal branchlets, segmentary loss of spines and polymorphic spines. These findings show the extensive involvement of all three cerebellar cortical layers and the reactive plasticity of Purkinje cells to deafferentiation. They favour the hypothesis that demyelination represents a primary lesion of the white matter.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01625735
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Friedreich's ataxia presenting with pure sensory ataxia: a long-term follow-up study of two patients (1993)
    Springer
    Journal of neurology 240 (1993), S. 177-180 
    Details
    ISSN: 1432-1459
    Keywords: Friedreich's ataxia ; Sensory neuropathy ; Cardiomyopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two patients with Friedreich's ataxia whose presenting symptomatology was for years progressive tabetic ataxia. Based upon the initial clinical, electrophysiological and nerve biopsy data, a diagnosis of idiopathic sensory neuropathy was established. Subsequent examination of the kin showed that three sisters of case 1 had Friedreich's ataxia. Upon serial clinical and electrocardiographic study, both patients eventually developed a florid Friedreich's ataxia, including cardiomyopathy. Our findings indicate that at onset Friedreich's ataxia may be indistinguishable from sensory neuropathy and also that serial examination and investigation of kinship are essential steps for accurate diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00857524
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Brain-stem auditory evoked potentials and blink reflex in friedreich's ataxia (1991)
    Springer
    Journal of neurology 238 (1991), S. 212-216 
    Details
    ISSN: 1432-1459
    Keywords: Friedreich's ataxia ; Brain-stem auditory evoked potentials ; Blink reflex
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The brain-stem involvement in Friedreich's ataxia (FA) was studied by using brain-stem auditory evoked potentials (BAEPs) and the blink reflex. Ten out of 18 patients had abnormal BAEPs, the main abnormality being complete absence of responses and disappearance of wave V. Combined degeneration of the peripheral and central acoustic pathways probably accounts for these findings. The blink reflex was abnormal in 50% of the cases. The outstanding abnormality was bilateral delay of late responses with normal early response, which could be correlated with the known pallor of the descending trigeminal tracts. In contrast with BAEP findings, blink reflex abnormalities did not correlate with either the age of patients or the severity and duration of the disease. These data suggest a difference in susceptibility to degeneration between the auditory system and neuronal system subserving the blink reflex. We conclude that systematic BAEP and blink reflex recording is useful in the electrophysiological evaluation of FA patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00314783
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    Paper (German National Licenses)
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