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Notes: Summary The genetic hypothesis on the independence of the recently (1965) described three types of the progressive myoclonus epilepsy (Unverricht, Lundborg and Hartung) is critically evaluated under a clinical aspect by studying the symptoms in our own 35 cases from 15 sibships and in those from the literature by statistical means. 1. Type Unverricht — with an autosomal recessive mode of inheritance — is the “classical” form of progressive myoclonus epilepsy. It is characterized by an onset of the disease between the 6th and 19th year of age with an average of the 10th year, by a fast to very fast progression of myoclonic, epileptic, psychic and electroencephalographic manifestations, inconstant neurologic deviations from normal, a course of 1 to 20 years with an average of 10 years, leading to death in stage III of the disease between the 15th and 30th year with an average of 20 years. Relatively specific findings on autopsy are myoclonus (Lafora) bodies in the central nervous system (cf type Lundborg). 2. Type Lundborg — also with an autosomal recessive mode of inheritance — represents a marginal form of progressive myoclonus epilepsy. Its nosologic relation to type Unverricht can only be clarified by evaluations of additional cases. The disease begins between the 9th and 27th year of age; it shows fast to very slow progression of the myoclonic, epileptic, psychic and electroencephalographic manifestations; but progression may be more or less absent. Neurologic findings are rarer as compared to type Unverricht, and after a duration of 10 to 50 years — mostly 30 to 36 years — death occurs in stage II, II to III or III of the disease between the 16th and 72nd year of age with the highest incidence between 40 and 50 years. In our own 2 cases, we found myoclonus (Lafora) bodies in the central nervous system. 3. Type Hartung — with an autosomal dominant mode of inheritance — is also a marginal form of progressive myoclonus epilepsy. The disease begins between the 7th and 46th year with an average of 20 years; the progression of the myoclonic, epileptic, psychic and electroencephalographic manifestations is slow to very slow or can be absent in some regards. Just as in type Lundborg, the neurologic findings are also rarer as compared to type Unverricht. After a duration of 13 to 40 years with an average of 25 years — in stage II or II to III of the disease — the patients will die between their 23rd and 70th year of age with an average of 45 years. The only case which was autopsied showed loss of ganglion cells of the cerebrum and the cerebellum. We did not find an myoclonus (Lafora) bodies. 4. The genetic hypothesis quoted above appears to be verified by the different clinical variety as it increases from type Unverricht over type Lundborg to type Hartung. The relative number of cases with marked symptoms and early ages of manifestations is conversely decreasing — a statistical paraphrase of the clinical “malignancy” of type Unverricht and the relative “benignity” of types Lundborg and Hartung. 5. A new classification of the stages of progressive myoclonus epilepsy is suggested which deviates from Lundborg's terminology. The problems of objectifying extrapyramidal and cerebellar symptoms in chronic, generalized myoclonus have been shown. Delimitation of a functional (reversible) dementia of the middle stages of the disease course is made as opposed to an organic (irreversible) dementia in the final stage.

Notes: Summary The problem was examined, whether those cases of postinfantile (late infantile, juvenile, and adult) amaurotic idiocy, who showed myoclonies, belong to separate genetic types, or whether myoclonies are just an occasional symptom without genetic significance. For this purpose, three new families were analyzed together with the observations from the literature. Results 1. Within the same sibships, either all siblings show myoclonies, or none of them. There are only very few exceptions from this rule, all of which can be disputed. 2. Within the late infantile, juvenile, and adult group, age of onset and other symptoms were compared between the subgroups with and without myoclonies. Among the late infantile cases, no clearcut differences were found. Among the juvenile as well as among the adult groups, however, differences between the myoclonic and non-myoclonic cases could be demonstrated. 3. The age of onset of the disease in all cases with myoclonies shows a clearcut trimodal distribution. From these three results together, the conclusion seems to be warranted that there are at least six different types of post-infantile amaurotic idiocy: late infantile, juvenile and adult types without myoclonies, and late infantile, juvenile and adult types with myoclonies. 4. In near relatives of the patients, unspecific central nervous disturbances, especially epilepsies, seem to be more frequent than in the average population. In some of these persons, we observed mild EEG anomalies. 5. the EEG of propositi with postinfantile amaurotic idiocy seems to be more variable than expected. 6. Literature reports were confirmed, according to which heterocygotes differ from normal controls by showing a certain fraction of vacuolized lymphocytes. In the discussion some problems are mentioned which need to be solved for a more thorough analysis of the postinfantile amaurotic idiocies.

Notes: Summary Some principles for a systematic therapy of early process schizophrenia are put forward on the basis of previous empirical research and our own observations in a special ward. The earliest symptoms are usually personality changes which are of two types: (1) A “pseudoneurotic” type which develops out of childhood tensions and conflicts and in which attitudes of defiance and protest are predominant. (2) An “egopathic” (Kisker) type characterised by passivity, inadequacy and autism. — These premorbid personality changes indicate a fixation at the level of primary process and a poor development of secondary processes, often with an unusually long attachment to the primary family. The defective ego is confronted by a strong ego-ideal. The deviant value orientation acquired from the primary family, together with the behavioural inadequacy, impede the normal development of verbal and behavioural communication skills, ranging from styles of thinking to emotional reactions. Psychotic symptoms usually become manifest after precipitating events which can be understood as inevitable in the light of the whole development. — It is important to interrupt the passivity, inadequacy and regressive defences against unresolved conflicts which underlie the progress of the psychosis. However, the patient's status in a deviant hospital sub-culture must not be allowed to undermine his social competence but, at the same time, he must not be discharged, without support, to a community in which he will often find other people's attitudes negative and intolerant. — Reducing the internal conflicts which cause the symptoms does not always, in itself, produce a satisfactory social adjustment. Treatment must also be directed towards producing attitudes and values which are more appropriate to the socio-cultural environment in which the patient will have to live while, at the same time, allowing a satisfactory degree of self-expression. The patient has lacked models of normal social behaviour, emotional communication and styles of thinking and these deficiences must be made up. Treatment procedure must therefore include not only somatic and psychological, but also social measures designed to produce social learning, a decrease in pathogenic primary relationships and an improved ability to cope with social problems after discharge. — We tentatively suggest that, during the course of such a long-term therapy, the process of psycho-social development of personality will be speeded up while, at the same time, the “schizophrenic process” will be reduced.

Notes: Zusammenfassung An den stationär behandelten Kranken der Psychiatrischen Universitätskliniken Freiburg und Heidelberg in den Zeiträumen 1900 bis 1960 und 1911 bis 1960 wird die relative Häufigkeit der Diagnose Schizophrenie, depressive Syndrome, reaktive Depression, Psychopathie und Manie untersucht. Unter den Parametern, von denen die Entwicklung der relativen Häufigkeit dieser Diagnosen abhängig ist, scheint die wissenschaftliche und diagnostische Auffassung der jeweiligen Klinikdirektoren die bedeutsamste Rolle zu spielen. Jedenfalls haben hier diagnostische Moden der psychiatrischen Diagnostiker einen wesentlich größeren Einfluß auf die von uns dargestellten Daten ausgeübt als die großen zeitgeschichtlichen Ereignisse wie etwa die beiden Weltkriege oder aber die Weltwirtschaftskrise mit Massenarbeitslosigkeit. Selbstverständlich geben unsere Ziffern nur eine grobe Orientierung und keine detaillierte Auskunft auf die Frage, inwieweit äußere Belastungen, ökonomische, soziale oder auch interpersonelle Faktoren als Ursachen oder Auslöser eines dieser Krankheitsbilder von Bedeutung sind. Aus unseren Untersuchungen kristallisiert sich der Eindruck heraus, daß es 1. Diagnosen gibt, die in ihrer relativen Häufigkeit vorwiegend von den, dogmatischen Auffassungen abhängen, die von den Klinikdirektoren vertreten und je nach dem Ausmaß ihrer innerklinischen Autorität in den Diagnosen realisiert werden (Depression, reaktive Depression, Schizophrenie). Die Entwicklung im Berichtszeitraum läßt erkennen, daß diese dogmatischen Einflüsse am Anfang des Jahrhunderts ihr Maximum erreichten und inzwischen deutlich im Schwinden begriffen sind. - 2. Andere Diagnosen sind weniger den speziellen Auffassungen der Klinikdirektoren unterworfen, sondern folgen einem allgemeinen Trend der psychiatrischen Lehre, beispielsweise die Psychopathie. - 3. Gibt es Diagnosen, die praktisch keine Abhängigkeit von den genannten Parametern zeigen. Bei diesen Syndromen ist offenbar das Erscheinungsbild relativ uniform und die Auffassung über ihre Ätiologie relativ einheitlich. Sie werden deshalb ohne allzu große Variabilität diagnostiziert (Manie).

Notes: Summary The administrative incidence of mental illness in the population of Mannheim, an industrial city with approximately 330,000 inhabitants, was calculated for the year 1965. Admissions to psychiatric and non-psychiatric hospitals, other psychiatric contacts, and all forms of welfare provision such as old people's and children's homes, educational facilities, and family and marriage advisory services were included. The results were in agreement with the zone-model proposed byParks andBurgess and applied to the distribution of mental illnesses in Chicago byFaris andDunham. The ecology of the incidence rates for schizophrenia, depressive psychoses, neurotic disorders and alcoholism and other conditions is described. The one-year incidence for all mental disorders in 1965 was 10.74 per 1,000.

Notes: Summary A newly-founded German university commissioned proposals for the organisation of a projected academic department of psychiatry which would provide psychiatric care for the University town, a small industrial and business centre of about 130,000 inhabitants. Hitherto, the only services available had been provided by a state mental hospital, 50 miles away, and several private psychiatrists and neurologists. A study of present-day psychiatric knowledge in teaching, research, prevention and therapy formed the basis for a proposal which was similar in conception to that of the American Community Mental Health Center. The central facilities — particularly the out-patients clinics, the in-patient unit and the day- and night-hospitals — were to be planned in strictly funtional relationship to the clinics and units of other medical specialties, so that psychosomatic medicine could provide a bridge between psychiatry and internal medicine. The whole organisation was, above all, to be community-oriented; from the preventive services, at the most fundamental level, to a community-care system for the chronically ill and mentally handicapped, set up in collaboration with lay and community bodies. In order to fulfil essential research and teaching requirements a part of the new service was to remain free to select certain types of patients for special programmes.