No abstract

Quantitative assessment of patient data is a pertinent part of controlled clinical studies. When several centers are involved, the degree of agreement between different observers becomes important. Therefore, in addition to developing a multicenter study on acute ischemic stroke, we have estimated the interobserver agreement expressed in terms of K statistics. Twelve patients suffering from neurologic deficits due to acute ischemic stroke were examined by four investigators, and the results were assessed using the Mathew scale. Considerable interobserver variability was found. Agreement on items based on subjective information from the patient was low, and it is also possible that this information changes with time. It is advised that in the development of assessment scales, items with low interobserver agreement should be avoided. 4 in what they believed to be consistent findings. After starting a multicenter study on acute ischemic stroke, we decided to determine in a separate study the number of interobserver differences in recording neurologic signs. This article deals with the results of that study. Subjects and MethodsTwelve patients, aged 62-84 years, who were hospitalized in a nursing home consented to participate in our study. All suffered from neurologic deficits as the result of acute ischemic strokes that had occurred 3-11 months earlier. They were selected randomly from patients available in the wards on one day by an independent and nonparticipating physician. The patients were unknown to the four participating investigators, and all patients were considered to be in a stable state.The investigators were the four senior neurologists from the participating centers involved in a multicenter study on acute ischemic stroke who were directly responsible for patient care in their own units. All investigators were trained in clinical neurology, had at least 15 years' experience, and were especially interested in cerebrovascular diseases. The neurologic deficits were assessed using the single items of an ordinal scale developed by Mathew et al, 5 modified in only minor detail for the assessment of language disturbance.6 These items are listed in Figure 1 and were considered separately in the statistical analysis because there may be different levels of agreement for the separate parts of the neurologic investigation represented by the items. Definitions of the terms for different items were predetermined and specified in a protocol.The sequence of the four observers' scoring of each patient was randomized to balance the interaction between investigator and patient ( Figure 2). All evaluations were done the same morning to avoid dayto-day effects.At the beginning of each session, each neurologist was given a case record form containing the scoring system for assessment of neurologic deficits and a list of the patients. The only information that the investigators had was the name and age of the aphasic patients. Each patient was assessed separately by the investigators. The case record form was filled out im...

A family with Charcot-Marie-Tooth disease (CMT) is described. An irregular dominant sex-linked inheritance is observed. No linkage relationship between the Xg(a) blood group locus and the CMT locus was established. Abnormal values of the serum alkaline phosphatase level were found in affected and unaffected members.

In this study 289 individuals from 5 pedigrees with Leber's optic atrophy (L. O. A.) were examined neurologically and the symptoms observed were expressed by numerical values, according to an arbitrary number scale; 34 of the 289 were patients suffering from the optic atrophy. Of these 34 patients with L. O. A., 24 were found to have minor neurological abnormalities, a finding which was corroborated by an analysis of previously published reports. A gradual decline in the percentage of individuals with abnormalities was observed in different groups in the following order: Leber patients–brothers of Leber patients–sisters of Leber patients–group of individuals connected through females to the pedigrees (“female line” group)–external control group–group of individuals connected through males to the pedigrees (“male line” group or internal control group). Three of the pedigrees were included in the study because in each of them a patient with a severe neurological disorder was present, diagnosed as spastic paraplegia (1) and multiple sclerosis (2). Evidence is presented that the association, mostly in females, of Leber's optic atrophy and a multiple sclerosis‐like neurological disorder cannot be coincidental. Leber's optic atrophy seems to be an affection of the central nervous system not necessarily restricted to the optic nerves.

A family with Charcot-Marie-Tooth disease is described. An irregular autosomal dominant transmission is observed. Some unusual clinical symptoms were present, such as trophic ulcers and neural deafness. In one generation of sibships with affected and unaffected individuals, some younger sibs had raised serum alkaline phosphatase activities. A connection between the neurology cal disorder and these abnormal values may be assumed.