Experience and philosophy with regard to case registers in health and welfare

A well designed and executed case register for mental illness or other conditions can provide information not readily obtainable by any other method. This includes unduplicated counts of diagnosed cases of the disease and longitudinal information such as changes in diagnosis, outcome, and survival information. Registers often are the only way in which new cases can be identified with any certainty. Also, the register can serve as a sampling frame for more intensive studies of services and of prevalence of the disease in the community, genetic studies, studies of the cost of treating various types of patients, before and after comparisons, and many other sociological and epidemiologic investigations. At the same time, there are many problems and pitfalls to case registers. This paper outlines some of these difficulties and suggests that registers be established only after careful planning and preparation.This paper is based on a presentation for a Workshop on Case Registers, American Orthopsychiatric Association Meeting, Chicago, Illinois, March 20, 1964. An extended bibliography on case registers is available from the author.     

Depression in Parkinson disease: a disabling but neglected factor

20 depressed and 20 non depressed patients with Parkinson disease were rated for disability on the Northwestern University Disability Scale and for severity of the typical motor symptoms on the Columbia University Rating Scale and were assessed for mental deterioration. The severity of the typical symptoms was practically equivalent in the two groups but the depressed patients not only presented mild mental deterioration but were significantly more disabled (less independent) than the patients without depression. A significant correlation was found between severity of depression and degree of disability but not between severity of depression and severity of typical motor symptoms. Depression was diagnosed much later than the typical symptoms. Only 25% of the depressed parkinsonians had received tricyclic antidepressants, about 20% had been treated with dopamine-antagonist psychotropic drugs and some 50% had received no treatment at all against depression. Timely diagnosis and appropriate treatment of depression in parkinsonian patients limits the effects of a major disabling factor.Party supported by Regione Piemonte, grant n. 17     

Diagnosing Depression in African Americans

Since the 1970s, articles have noted the increased presence of psychotic symptoms among depressed African Americans, the presence of diagnostic bias identified when structured clinical interviews are used, and the identification of misdiagnosis of affective illness among chronically, mentally ill, African Americans. This paper reviews this literature and describes three alternative presentations of depressive illness among African Americans that differ from the DSM IV criteria for Major Depressive Disorder: the stoic believer, the angry, evil one with a personality change, and the John Henry doer. Clinicians are encouraged to recall these presentations of depression when evaluating African American patients.     

Lack of correlation between DST results and urinary MHPG in depressed inpatients

Summary Abnormalities of noradrenaline metabolism and of the activity of hypothalamic-pituitary adrenal axis (HPA) have been reported in depression. To study the possible relationship between these 2 parameters, urinary excretion of 3-methoxy-4-hydroxy-phenylethyleneglycol (MHPG) and Dexamethasone Suppression Test (DST) were analyzed in 58 depressed patients. A positive correlation was found between the age of depressed patients and 24-h urinary excretion of MHPG. Twenty-two patients (38%) were DST non suppressors. Pre-DST plasma cortisol levels were significantly higher in non suppressors than suppressors. No difference was found however between urinary MHPG levels in supressors and non suppressors. There was no correlation between pre-DST plasma cortisol and levels of urinary excretion of MHPG. These results do not support the hypothesis of a relationship between these 2 parameters. However, when depressed patients were separated into two groups according to urinary excretion of MHPG (high MHPG and low MHPG), the high MHPG group included significantly more non suppressors then the low MHPG one. This result is not sufficient to demonstrate of link between HPA system activity and central noradrenaline metabolism.     

Postural control in children with autism

Postural control was evaluated in samples of autistic, normal, and mentally retarded children in this pilot study using a recently developed, computerized posturographic procedure. A battery of postural positions was administered including postures involving some degree of stress (e.g., occluded vision or standing on pads). The postural patterns of children with autism differed from those observed in normal children, in mentally retarded children, and in adults with vestibular disorders. In comparison to normal children the autistic subjects were less likely to exhibit age-related changes in postural performance and postures were more variable and less stable with more lateral sway. Autistic subjects also exhibited a paradoxical response of greater stability with more stressful postures, putting excessive weight on one foot, one toe, or one heel. The implications for neuroanatomical models of autism are discussed.This work was supported in part by grant NS25026 from the National Institute of Health.     

Attitudes to the mentally ill: A trait attribution approach

Summary In order to investigate the cognitive component of attitudes to the mentally ill, a questionnaire based on the Stereotype Measure of Katz and Braly was administered to a random sample of 400 adults in Dublin. The results from the survey show that the public hold stereotyped conceptions of mental patients, insane people and neurotic people. The neurotic are, clearly differentiated but the stereotypes of mental patients and the insane differ from eachother in degree and emphasis rather than in content. There is little evidence in any of the stereotypes of misinformation that could be corrected by education programs. Indeed, it seems that these groups are generally perceived in terms of unhappiness, confusion and withdrawal, terms that are predictable concomitants of being admitted to a psychiatric hospital. On the other hand a factor analysis of responses to the questionnaire revealed something of the cognitive structure of the public's conceptions and strongly suggested the existence of minority stereotypes. For instance, it appears that a significant minority of the sample perceive mental patients as violent and dangerous. An analysis of the effects of age, sex and socio-economic class on conceptions of the mentally ill was also carried out. The youngest group were shown to hold a generally more positive view of the mentally ill. Class membership was not a major influence on the content of the stereotypes except in the case of the lowest socioeconomic group, a substantial minority of which appear to confuse the mentally ill with the mentally retarded.     

Cognitive functioning in female patients with 21-hydroxylase deficiency

The cognitive functioning of 27 female patients with congenital adrenal hyperplasia (CAH) (aged 11–41 yrs) and 13 of their healthy sisters (13–31 yrs) was compared using short versions of age-appropriate Wechsler scales. In contrast to other studies, neither a higher than average IQ level for CAH patients (mean: 99.0) nor for their sisters (97.7) was found. Unexpectedly, and in contrast to other reports, the subgroup of salt-wasting (SW) patients>16 yrs (N=6; mean score: 111.5) differed from their sisters as well as from simple-virilizing (SV) patients in full IQ (p<0.05) and subtest scorings for Information, Similarities, and Picture Completion (p<0.05–<0.10). SW patients displayed more masculine behaviour (vs. SV patients and sisters) which, in turn, was related to differential prenatal hormonal influences. No clear-cut relationships between IQ/cognitive (subtest) findings and gender-role behaviour were found.

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Zusammenfassung 27 Patientinnen mit dem Adrenogenitalen Syndrom (AGS) (11–41 J.) und 13 ihrer Schwestern (13–31 J.) wurden hinsichtlich intellektueller Funktionen verglichen (Kurzformen von HAWIK, HAWIE). Im Unterschied zu den meisten früheren Untersuchungen wurden weder für Patientinnen (mean: 99.0) noch für Kontrollen (97.7) über dem Durchschnitt liegende IQ-Werte gefunden. Im Gegensatz zur Literatur unterschied sich die Teilgruppe der Salzverlust-Patientinnen (SW)>16 J. (N=6, mean: 111.5) von den Schwestern und den Patientinnen mit einfachem AGS (SV) im Gesamt-IQ (p<0.05) und in den Untertests Allgemeines Wissen, Gemeinsamkeiten und Bilderergänzen (p<0.05–<0.10). SW-Patientinnen hatten signifikant männlichere Verhaltensmuster gezeigt (vs. SV-Patientinnen und Schwestern), die auf differentielle Hormoneffekte pränatal bezogen worden waren. Es fanden sich aber keine klaren Zusammenhänge zwischen IQ-bzw. Untertest-Resultaten und Ergebnissen für Geschlechtsrollenverhalten.

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Résumé Le fonctionnement cognitif de 21 patientes avec une hyperplasie congénitale surrénale (âgée de 11 à 41 ans) et de 13 de leurs soeurs saines (13–31 ans) a été comparé au moyen de versions raccourcies de l'échelle de Wechsler appropriée à l'âge. En contraste avec d'autres études, il n'a été retrouvé un Q.I. plus haut que la moyenne ni pour les patientes (moyenne 99.0) ni pour leurs soeurs (moyenne 97.7). De façon inattendue, et en contraste avec d'autres études, le sous-groupe de patientes déprivées en sel (SW)>16 ans (N=6), moyenne score: 111.5) différait de leurs soeurs aussi bien en tant que patientes présentant des signes de virilsation (SV) pour le Q.I. complet (p<0.05) et les scores aux subtests d'information, de similarité et de complément d'images (p<0.05–0.10). Les patientes déprivées en sel (SW) montraient un comportement plus masculin (vs. SV et leurs soeurs) qui en retour était relié aux influences hormonales prénatales différentes. Il n'y avait pas de relation de différences nettes entre les résultats aux sous-tests cognitifs du Q.I. et le comportement de genre.

     

Comparison of integrin adhesion molecules expressed by primary brain lymphomas and nodal lymphomas

The expression of 17 adhesion molecules was immunohistochemically examined in 5 primary cerebral lymphomas (PCL) and in 5 histologically similar nodal lymphomas (NL) to evaluate their possible involvement in selective targeting of lymphoma cells to the brain. PCL and NL tumor cells showed very similar expression patterns: they were consistently positive for ₃, ₄ and ₁ integrin chains; negative for ₂, ₆, ₃ and ₄ integrin chains; and heterogeneous for ₅, _L, _M, _X, ₂ and ₇ integrin chains, as well as for intercellular adhesion molecule-1 (ICAM-1) and the selectin LECAM-1. Loosely infiltrating PCL showed lower levels of the _L2 integrin than compact cell clusters. Vessels stained for ICAM-1 and vascular cell adhesion molecule-1 (VCAM-1). We conclude that the adhesion molecules implicated in the extravasation of non-neoplastic leukocytes (₄₁/VCAM-1 and _L2/ICAM-1) are also expressed by both PCL and NL. The adhesion molecules examined are apparently not selective mediators of lymphoma cell homing to the brain, but at least _L2 integrin might be related to the infiltration pattern of PCL within the brain parenchyma.Supported by the Sander Foundation     

Chronic mental hospital dependency as a character defense

Summary Prolonged hospitalization may cause atrophy of the patient's self-confidence, through excessive dependent gratifications of an oral-receptive nature. Hospital dependency results, and the possibility of discharge arouses such anxiety that the patient exhibits increased psychotic behavior with the effect of maintaining the hospital adjustment. Certain persons are excessively predisposed by previous experience to develop this dependency. The basic predisposition, however, is rooted in the normal course of childhood development.Hospitalization results in three major gains for the mentally disturbed patient: (1) primary relief from anxiety caused by instinctual impulses overwhelming the ego defenses; (2) secondary gains resulting from the socially-recognized sick role; and (3) the satisfactions of becoming an accepted member of a stable social system. If a mental patient is hospitalized, the danger of chronic dependency must be recognized and guarded against. Limited hospitalization is an important therapeutic modality, but as with other therapies, excess may cause secondary complications.Therapy of the chronic patient seeks first to convert dependence on the hospital as an ego-fortifying agent into a general confidence that help will be available if needed. Subsequently, the therapy aims to work through the secondary gains of illness byh viewing psychotic behavior as resistance to giving up the hospital. For the purpose of therapy, the chronic patient is viewed as environmentally-oriented, his behavior as based in present reality; and his active co-operation as essential to any change.The author, now program chief, mental health section, County Department of Public Health and Welfare, San Mateo, Calif., takes full responsibility for the contents of this paper, but wishes to acknowledge his intellectual debt to the staff of the Winter Veterans Administration Hospital, Topeka, Kansas.     

Cortical regulation of subcortical dopamine systems and its possible relevance to schizophrenia

Summary A unique model of DA system regulation is presented, in which tonic steady-state DA levels in the ECF act to down-regulate the response of the system to pulsatile DA released by DA cell action potential generation. This type of regulation is similar in many respects to the phenomenon proposed to mediate the action of norepinephrine on target neurons; i.e., an increase in the signal-to-noise ratio as measured by postsynaptic cell firing (Freedman et al., 1977; Woodward et al., 1979). However, in this model the signal and the noise are neurochemical rather than electrophysiological. Furthermore, the noise (tonic DA in the ECF) actually down-regulates the signal (phasic DA release) directly, and thereby provides a signal of its own that affects the system over a longer time-course. Therefore, the difference between signal and noise may also depend on the time frame under which such determinations are made.     

Quantitative analysis of “synaptic” ribbon profiles in the pineal complex of male and female Pirbright-White guinea pigs

Summary Previous studies have pointed in the direction of sex differences as well as regional differences in the pineal gland of guinea pigs. In the present investigation these aspects were studied at the electron-microscopic level by quantitating different types of synaptic bodies, intrinsic to pinealocytes. The two major types of synaptic organelles, ribbons and spherules, did not exhibit regional or sex differences. Synaptic structures intermediate in appearance to ribbons and spherules were significantly larger in number in males in the distal region of the pineal gland, compared to females. As previous studies have shown that ribbon and spherule numbers undergo characteristic changes depending on the functional state of the pineal gland, it is concluded that, as far as the synaptic organelles are concerned, no clear-cut sex or regional differences appear to exist in the guinea pig pineal gland.     

Severe impairments of social interaction and associated abnormalities in children: Epidemiology and classification

The prevalence, in children aged under 15, of severe impairments of social interaction, language abnormalities, and repetitive stereotyped behaviors was investigated in an area of London. A socially impaired group (more than half of whom were severely retarded) and a comparison group of sociable severely mentally retarded children were identified. Mutism or echolalia, and repetitive stereotyped behaviors were found in almost all the socially impaired children, but to a less marked extent in a minority of the sociable severely retarded. Certain organic conditions were found more often in the socially impaired group. A subgroup with a history of Kanner's early childhood autism could be identified reliably but shared many abnormalities with other socially impaired children. The relationships between mental retardation, typical autism, and other conditions involving social impairment were discussed, and a system of classification based on quality of social interaction was considered.     

Ultrastructure of peripheral nerve in metachromatic leucodystrophy

Summary An electron microscopical study of two consecutive nerve biopsies from a patient with metachromatic leucodystrophy (sulphatide lipidosis) was made. The ultrastructural changes observed consisted of: a) irregular whorls of myelin. The myelin in the whorls showed a thickened, sometimes doubled, intraperiod line, which was barely visible in compact myelin; b) inclusion bodies up to 1 in diameter in the cytoplasm of Schwann cells. These had a lamellar structure, with stacked membranes 60 Å apart; c) a loose pattern of the myelin in some nerve fibers, with loss of the intraperiod line, and d) presence of abnormally dense mitochondria with thickened cristae in Schwann cells. It is suggested that: a) the whorl formation and the ultrastructural abnormalities of the myelin in the whorls may be due to impaired myelin synthesis, and b) that the inclusion bodies may represent the accumulation of cerebroside sulfate in micellar aggregates. The loose pattern of myelin is considered artifactural until proven otherwise.

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Zusammenfassung Zwei aufeinanderfolgende Nervenbiopsien bei einem Patienten mit metachromatischer Leukodystrophie (Sulfatid-Lipoidose) wurden elektronenoptisch untersucht. Die beobachteten ultrastrukturellen Veränderungen bestehen in: a) unregelmäßigen Wirbelbildungen (whorls), in welchen das Myelin Verdickungen, manchmal Verdopplung des Zwischenstreifens (intraperiod line) aufweist, was im kompakten Myelin kaum sichtbar ist. b) Einschlußkörperchen mit einem Durchmesser bis zu1 im Cytoplasma der Schwann-Zellen. Diese weisen lamelläre Struktur mit einem Membranabstand von 60 Å auf. c) ein lockeres (loose) Myelinmuster mit Verlust des Zwischenstreifens in einigen Nervenfasern und d) Auftreten von abnorm dichten Mitochondrien mit verdicktem Cristae in Schwann-Zellen. Es wird angenommen, daß a) die Wirbelbildungen und die ultrastrukturellen Myelinabnormitäten in den Wirbeln einer gestörten Myelinsynthese entsprechen und b) daß die Einschlußkörperchen die Anhäufung von Cerebrosidsulfat in micellaren Verbänden darstellen. Das lockere Myelinmuster wird vorläufig als artifiziell angesehen.

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This investigation was supported in part by Public Health Service Research Grant No. FR-86 from the N.I.H. Division of Research Facilities and Resources.     

Kinematic properties of slow arm movements in Parkinson's disease

Present pathophysiological concepts of bradykinesia stress an impairment of fast movements in Parkinson's disease. It is, however, unknown whether bradykinetic movements are different from slow movements of normal subjects. We recorded trajectories of unrestrained natural arm movements from normal subjects and patients with Parkinson's disease. The experiment required the execution of pointing movements for different movement distances and velocities. The shape of trajectories was found to be changed in Parkinson's disease. The steepness of the initial segment and the relation between steepness of the initial segment and final segment both exceeded corresponding values in normal subjects. An analysis of velocity profiles showed an impaired synchrony of vertical and horizontal velocity components. The difference from normal subjects increased with movement velocity. Parkinsonian patients suffered from a fundamental defect in the composition of complex sequences of motor programs required to perform natural arm movements.     

Determinants of intellectual outcome after surgical revascularization in pediatric moyamoya disease: a multivariate analysis

Objects The aim of this study was to clarify predictors for poor intellectual outcome in pediatric moyamoya disease.Methods Fifty-two pediatric patients were included. Clinical diagnosis was transient ischemic attacks (TIA) in 35 and completed stroke in 17. Ten patients underwent indirect synangiosis through small craniotomy, whereas the other 42 underwent superficial temporal artery (STA)–middle cerebral artery (MCA) anastomosis and indirect synangiosis through large craniotomy. Full-scale IQ (FSIQ) was measured using the Wechsler intelligence scale for children (WISC) after surgery. Multivariate logistic regression models were applied to test the effect of clinical factors on intellectual outcome.Results and conclusion Eight patients revealed mentally impaired status (FSIQ<70). Multivariate analysis revealed that completed stroke and small craniotomy surgery were significantly associated with poor intellectual outcome. Odds ratios of each factor were 33.4 (95% CI, 2.4–474) and 19.6 (95% CI, 1.8–215) respectively. Early diagnosis and the revascularization procedure over as wide an area as possible may be essential to improve their intellectual outcome.     

Use of plasma levels for antiepileptic drug monitoring in clinical practice

A multi-center survey of antepileptic treatment was conducted in Italy on 245 previously untreated (new) patients with epilepsy and 355 patients treated for more than three months (old patients). Therapeutic drug monitoring (TDM) of antiepileptic drugs was evaluated in the context of routine clinical conditions, in relation to individual therapeutic problems and mode of treatment. Plasma levels (PL) were determined in 75% of new patients and 78% of old patients, with wide intercenter variability. TDM was done at 69% of the follow-up attendences for new patients and at 34% for old patients, but was apparently unrelated to specific therapeutic problems, such as poor disease control or adverse drug reactions. Plasma drug concentration measurements were made more often among patients on polytherapy. The age of the patient and the time elapsing since diagnosis did not seem to affect request patterns significantly. From these findings it appears that TDM is largerly influenced by factors unrelated to the common recommendations in the literature. In addition, the use of TDM in clinical practice reflects the limitations of the available techniques.

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Sommario In uno studio multicentrico condotto su 245 casi di epilessia di nuova diagnosi (trattati da meno di 3 mesi) e su 355 casi di epilessia di vecchia diagnosi (trattati da almeno 3 mesi)l'uso dei livelli plasmatici dei farmaci anticonvulsivanti è stato valutato in relazione alle modalità di trattamento e a problemi specifici indicati dai clinici. La percentuale di richieste di livelli plasmatici era del 75% e riguardava il 69% delle visite di follow-up nei pazienti di nuova diagnosi. Le percentuali erano rispettivamente del 78% e del 34% per i pazienti di vecchia diagnosi. In entrambi i casi vi era una marcata variabilità tra centri nelle percentuali di prescrizioni. Le richieste di livelli plasmatici erano più numerose nei pazienti in politerapia. Per contro, il monitoraggio dei farmaci antiepilettici non risultava influenzato dalla presenza di specifici problemi, quali il non completo controllo della crisi o la presenza di segni di tossicità farmacologica, né dall'età del paziente o dal tempo intercorso dalla formulazione della diagnosi. Da tutto ciò deriva che l'utilizzo dei livelli plasmatici dei farmaci antiepilettici nella pratica clinica è influenzato da fattori che spesso non riflettono le norme di comportamento comunemente suggerite dalla letteratura. Risultano inoltre ampiamente confermati i limiti delle metodiche attualmente in uso.

     

The effect of a 5-HT3 receptor antagonist,ondansetron, on brain stimulation reward,and its interaction with direct and indirect stimulants of central dopaminergic transmission

Summary 5-HT₃ receptors are abundant in central dopamine (DA) terminal areas. They do not affect basal DA turnover but appear to modulate DA release by e.g. morphine and nicotine. The interpretation of these findings is uncertain, and it is unclear whether 5-HT₃ receptors also influence the activity of compounds such as amphetamine and cocaine, which act more directly on the DA synapse. Variable-interval (VI), threshold-current hypothalamic self-stimulation can provide a continuous index of central dopaminergic activity, but is relatively insensitive to changes in 5-HT and thus offers a means of investigating this question. In the present study, a selective 5-HT₃ receptor antagonist, ondansetron (GR 38032F) (1.0 to 1000 g/kg sc), had no effect on VI self-stimulation, nor did a 100 g/kg dose affect facilitation of responding byd-amphetamine (500 jig/kg ip). Ondansetron (100 g/kg) reduced the initial depression of self-stimulation by high-dose nicotine (400 g/kg), but not the ensuing facilitation. Similar results were obtained in rats sensitized to nicotine by prior chronic exposure. These results support the proposal that 5-HT₃ receptors, normally quiescent under basal conditions, mediate the excitatory effect of compounds acting upstream from the DA neuron, such as nicotine, but do not affect the dopaminergic synapse directly.     

Soviet psychotherapy: The tasks and methodological problems

Soviet psychotherapy follows Western European traditional pre-psychoanalytical rationalistic methodology. On the other hand, Soviet therapists assign great importance to clinical diagnosis within the framework of the classical scheme of Kraepelin. Finally, methods close to behavior therapy, especially so-called training approaches, are popular.     

Electron microscopic structure of the alzheimer's neurofibrillary changes in case of atypical senile dementia

Summary A man aged 70, showed early disorientation, memory defects, delusions and rages at 66, later mental deterioration with muteness and dysphagia. He died of cardiac failure. The postmortem examination revealed macroscopically and light microscopically the neuropathological findings of atypical senile dementia.Moreover, it is the interesting characteristic in the presented case that there are electron microscopically two types of filaments making the neurofibrillary tangles. One showed the so-called paired helical filaments, which were observed in the cerebral cortex. The other showed parallel straight filaments. These straight filaments were found in the bilateral hippocampi.     

Hyperkinetic children: Early indicators of potential school failure

Hyperkinetic children are identified as a population-at risk upon admission to kindergarten. The etiology of hyperkinetic behavior is controversial. Organic driveness, hyperkinetic behavior disorder, postencephalitic behavior, brain damage with behavioral and conceptual deficit, Strauss syndrome, have all been used to label essentially similar symptom constellations. Bypassing the area of controversy, a study is reported that demonstrates that children who were identified as hyperkinetic (using behavioral criteria developed in an earlier study) were (1) absent from school more frequently, and (2) did remarkably less well on standardized tests of school readiness than their peers rated nonhyperkinetic. The implications are discussed and suggestions made for the development of intervention programs.An earlier version of this paper was presented at the Annual Meeting of the American Orthopsychiatric Association, March, 1967.