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  • 1
    ISSN: 1468-2982
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Isolated headache as the presenting clinical manifestation of intracranial tumors: a prospective study. Cephalalgia 1994;1'4:270-1. Oslo. ISSN 0333-1024We prospectively studied over two years the incidence of headache as the initial and isolated clinical manifestation of adult patients suffering from intracranial tumors (n = 183). Fifteen patients (8%) exhibited headache as their first and isolated clinical manifestation. Age, sex, neoplasm localization, or pathological diagnosis did not correlate with the presence of headache. Posterior fossa location and hydrocephalus, though not reaching statistical significance, were more frequent in patients who presented with headache as the first symptom. At the moment of diagnosis, 59 (31%) of the patients admitted to headache, though only I out of the 15 patients starting as headache still had this symptom as the only manifestation. From our experience in adults, isolated headache for longer than 10 weeks will only exceptionally be secondary to an intracranial neoplasm.
    Type of Medium: Electronic Resource
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  • 2
    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
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  • 3
    ISSN: 1432-1920
    Keywords: Adenosine deaminase (ED 3.5.4.4.) ; Brain tumour ; Cerebellopontine angle ; Lymphoma ; Meningitis ; Tuberculosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a primary leptomeningeal lymphoma (PLML) presenting as a cerebellopontine angle lesion. CT showed slight enlargement of the ventricular system, obliteration of the basal cisterns and a dense lesion in the left cerebellopontine angle which enhanced with contrast medium. Cerebrospinal fluid abnormalities included sterile lymphocytic pleocytosis without malignant cells, low sugar and high adenosine deaminase levels. An erroneous diagnosis of tuberulous meningitis was made, but autopsy revealed a leptomeningeal B-cell lymphoma with infiltration of the middle cerebellar peduncle giving the appearance of a cerebellopontine angle lesion. Seven cases of cerebellopontine angle lymphoma have previously been decribed, only one of which could be classified as PLML.
    Type of Medium: Electronic Resource
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  • 4
    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
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 238 (1991), S. 212-216 
    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
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neural transmission 96 (1994), S. 135-142 
    ISSN: 1435-1463
    Keywords: Alpha2-adrenoceptors ; autoradiography ; β-adrenoceptors ; 3H-bromoxidine ; 125I-iodocynaopindolol ; cerebellum ; olivopontocerebellar atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using autoradiographic techniques we studied the changes that in adrenergic receptors occurred in the cerebellum of two olivopontocerebellar atrophy (OPCA) patients as compared with a control group. In OPCA cerebellum the densities of total β-adrenoceptors were reduced along the cortex but increased in the white matter. Although mainly the β1 subtype was decreased along the cerebellar cortex, the increase of β-receptors over the white matter was due to a selective raise in the β2 subtype. These findings suggest a post-synaptic neuronal location for the β1 subtype and a glial location for the β2-adrenoceptor. On the other hand, alpha2-adrenoceptors were clearly reduced all along the cerebellar cortex of these OPCA brains, this probably being secondary to the loss of presynaptic adrenergic terminals arising from the locus coeruleus. These results help clarify both the subcellular location of adrenoceptors in human cerebellum and the neurochemical pathophysiology of OPCA.
    Type of Medium: Electronic Resource
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