A FAMILY WITH HEREDITARY SPASTIC ATAXIA: AN INVESTIGATION INTO THE EXISTENCE OF THE SO CALLED "FORMES FRUSTES".
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Biomedical subjects
Publications and source records attributed to A Staal.
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The interobserver agreement for the diagnosis of a cerebral transient ischemic attack (TIA) was investigated in a pool of eight senior and interested neurologists from the same department. They interviewed 56 patients in alternating pairs. The diagnosis was based on internationally accepted criteria. The agreement rates were corrected for chance (kappa statistics). Both neurologists agreed that 36 patients had a TIA and 12 had not, but they disagreed about 8 patients (kappa = 0.65; for perfect agreement kappa would be 1.0). The vascular territory (carotid or vertebrobasilar) was agreed upon in only 24 of the 36 patients in whom both diagnosed TIA's (kappa = 0.31). We concluded that currently the diagnosis of a TIA, made by a single neurologist, is a poorly defined entity.
To try and improve the interobserver agreement for the diagnosis of TIA, we used a checklist in which the symptoms were recorded in plain language, instead of in abstract diagnostic terms such as amaurosis fugax. Criteria for a diagnosis of TIA were similarly phrased and recommended to all observers. Eight senior neurologists and ten neurology residents interviewed 72 patients in random pairs. In 64 cases the observers agreed on the diagnosis (kappa value = 0.77). After a short discussion between the two observers the agreement increased to a maximum (kappa = 1.0). However, in 29 of the 144 interviews the diagnosis would have been different, had the recommended criteria been fully applied. In 28 of these the observer had diagnosed TIA on insufficient evidence. Six "misinterpretations" led to disagreement for the diagnosis and only these were corrected by the observers during their discussion. The present design has led to a maximal agreement for the diagnosis of TIA between two observers, but the agreement between such a pair and the common diagnostic criteria was not yet ideal. The precision of the diagnosis could be improved if details of the required symptoms are discussed in general as well as for each patient.
In this paper the basic requirements for a clinical therapeutic trial in Bell's palsy are discussed. The available therapeutic studies are analysed in view of these requirements. The following conclusions seem justified: (1) Galvanic stimulation has not been proved to lead to a better recovery. (2) There is no evidence that decompression of the mastoid part of the facial nerve in the second or third week has a positive effect. Whether surgery at an earlier stage or decompression of the complete intratemporal part of the nerve is effective, has not yet been studied. (3) There is no convincing proof that prednisone administration up to a daily dose of 60-80 mg during the first days has any effect.
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