The evolution of Kraepelin's nosological principles

Emil Kraepelin developed a new psychiatric nosology in the eight editions of his textbook. Previous papers have explored his construction of particular diagnoses, including dementia praecox and manic‐depressive insanity. Here we are providing a close reading of his introductory textbook chapter, that presents his general principles of nosology. We identify three phases: 1) editions 1‐4, in which he describes nosological principles in search of data; 2) editions 5‐7, in which he declares the mature version of his nosological principles and develops new disease categories; 3) edition 8, in which he qualifies his nosological claims and allows for greater differentiation of psychiatric disorders. We propose that Kraepelin's nosology is grounded in three principles. First, psychiatry, like other sciences, deals with natural phenomena. Second, mental states cannot be reduced to neural states, but science will progress and will, ultimately, reveal how nature creates abnormal mental states and behavior. Third, there is a hierarchy of validators of psychiatric diagnoses, with the careful study of clinical features (signs, symptoms and course) being more important than neuropathologic and etiological studies. These three principles emerged over the course of the eight editions of Kraepelin's textbook and were informed by his own research and by available scientific methods. His scientific views are still relevant today: they have generated and, at the same time, constrained our current psychiatric nosology.     

Locating genetic disease: the impact of clinical nosology on biomedical conceptions of the human genome (1966–1990)

The variability of disease expression often complicates clinical classification. Since the 1960s, medical geneticists have sought to address this problem by associating diseases with discrete locations in the human genome. While this nosological approach was quite successful in the 1980s, unanticipated complications arose. In 1987, two historically distinct disorders, Prader-Willi and Angelman syndromes, were unexpectedly associated with the same genomic “address.” Does genomic overlap imply nosological sameness? This paper explores the clinical and biological implications of this finding, and argues that the process by which it was resolved represented new modes of thinking and practice in late-twentieth-century biomedicine. In the decades before the completion of the Human Genome Project, the genome was understood to be, at once, a standardized scientific object and an observable part of the human anatomy. Depicted and analyzed at the level of chromosomes, the genome became an important conceptual space and experimental system for late-twentieth-century biomedicine, producing novel research questions that drew the attention of both clinically oriented physicians and basic laboratory geneticists.     

The syndromal approach to the diagnosis of toxic food poisonings]

The authors criticize the nosological principle of the diagnosis of toxic infections and believe that in a vast majority of cases such diagnosis must be based on syndromes. The only exceptions are cases of mass outbreaks and hospital infections. The definition of alimentary toxic infection is given, the difficulties of their clinical diagnosis and the ways to overcome these difficulties are indicated.     

Clinico-roentgenologic diagnosis of disseminated and diffuse lung diseases

The results of clinical and x-ray investigations of 398 patients with disseminated pulmonary lesions were analyzed. Two groups were identified: patients with interstitial type of lesions (101) and patients with focal type of lesions (297). Proceeding from roentgeno-morphological correlations, the authors presented specified roentgeno-semiotics of 10 nosological entities with the syndrome of pulmonary dissemination. The possibilities of optimization of differential diagnosis of disseminated pulmonary lesions were shown basing on combined (clinical, functional and roentgenological) investigation.     

Differential diagnosis of an accumulation of fluid in the pleural spaces

Altogether 345 patients with pleural effusion were investigated; 22 nosological entities were diagnosed. Of them four diseases (tuberculosis, metastatic cancer, pneumonia, and pleural mesothelioma) accounted for 79.7%. Failures of outpatient clinical investigation and late referral of patients to special diagnostic departments were noted. Possibilities to diagnose the type of disease on the basis of x-ray symptoms, clinical and laboratory tests and their combinations were analyzed. A necessity of the use of various types of biopsy with respect to an x-ray picture was recommended. Early thoracoscopic biopsy was indicated for progressive or stable pleural effusion of obscure etiology.     

Medico-genetic study of the residents of the Kostroma province. XI. Diversity of hereditary pathology in Kostroma

The diversity of hereditary pathology in Kostroma was studied. An attempt was made to classify all isolated cases by genetic and clinical analysis. 57 nosological forms of autosomal dominants, 41 autosomal recessive and 14 X-linked recessive disorders were found. The analysis of marriage distances in the whole population and in the families of the probands was carried out. The spectra of hereditary pathology in Kostroma and Kostroma Province were compared. The sources of the load of hereditary pathology in Kostroma are discussed.     

Nozological Spectrum of Hereditary Diseases of the Nervous System in the Cities of Volgograd and Volzhsky

A spectrum of hereditary diseases of the nervous system (HDNS) was studied in the cities of Volgograd and Volzhsky. The estimates were obtained for the prevalence of major HDNS groups and individual nosological forms. The populations examined differed in prevalence of this pathology and in contribution of individual diseases and their nosological forms into HDNS load formation. The effect of the population dynamics in Volgograd and Volzhsky on the HDNS prevalence is discussed.     

Nozological spectrum of hereditary diseases of the nervous system in the cities of Volgograd and Volzhsky

A spectrum of hereditary diseases of the nervous system (HDNS) was studied in the cities of Volgograd and Volzhsky. The estimates were obtained for the prevalence of major HDNS groups and individual nosological forms. The populations examined differed in prevalence of this pathology and in contribution of individual diseases and their nosological forms into HDNS load formation. The effect of the population dynamics in Volgograd and Volzhsky on the HDNS prevalence is discussed.     

Naming and grouping illnesses in Feira (Brazil)

This paper examines the naming and grouping of illnesses in the popular medicine of Feira de Santana, a city in Northeastern Brazil, in a context of medical pluralism. It discusses the principles used to name illnesses in Feira and the semantic shifts evident in popular nomenclature; examines the grouping of illnesses in Feira; and shows the flexibility of these situationally negotiated categorizations. It also analyzes the place of symptoms in local nosological knowledge and examines three folk illnesses as an illustration of the discussion of illness classification.     

Hereditary blood factors and infectious diseases in children in the 1st 7 years of life

Infectious morbidity in respect to 23 nosological forms was studied in 958 children with known blood groups and Rh factors during the first 7 years of their life. The absence of statistically significant differences in morbidity rates in children with different age groups was revealed in respect to 16 nosological forms. Significant differences in morbidity rates in children with different blood groups were revealed in respect to parotitis, rubella, scarlet fever, E. coli infections, bronchitis and pneumonia; similar differences linked with Rh factor were observed only in cases of measles, rubella and tonsillitis.     

Lennert's lymphoma

Four cases (3 M, 1 F) of Lennert's lymphoma were presented. The age (38-76 years), the clinical aspect and the morphopathological features were characteristic. Despite an intensive chemotherapy, 3 of the 4 patients died between 2 and 16 months (mean survival time 7 months). The controversies about the nosological delimitation and the cellular nature of Lennert's lymphoma are presented. Because of the poor prognosis, it should be considered as a high-grade malignant non-Hodgkin lymphoma.     

The system of epidemiological surveillance as a reflection of the structure of the epidemic process

The socio-ecological concept in epidemiology, formulated by the author in his earlier works and regarding the epidemic process as a biosocial hierarchical, integral system, can serve as a theoretical basis for the epidemiological surveillance of infectious and parasitic diseases. In accordance with the structure of the epidemic process, the system of epidemiological surveillance is subdivided into the following monitoring levels: molecular, microbiological, clinical, biocenological, statistical and sociological. The functions of epidemiological surveillance should be performed by the existing and newly organized All-Union Centers for the corresponding nosological forms.     

Characteristics of bacteria in the genus Proteus isolated from patients with sporadic and group intestinal diseases

The biochemical and biological properties of 148 Proteus strains isolated from patients both in sporadic intestinal infections and in a case of group infection in children's hospital was studied. The study revealed that the etiological factor of the group infection was P. mirabilis belonging to rare serovar 48:2. Proteus organisms isolated in sporadic infections belonged to a great number of serovars. No relationship between the isolated serovar and the nosological form of the intestinal disease was established. Among the Proteus strains under study, 82 strains showed atypical biochemical properties in 1 test or more. No correlation between the clinical diagnosis and the occurrence of atypical strains was established.     

Neurasthenia as nosological dilemma

While researching concepts of neurasthenia as described by patients and physicians of various backgrounds, it was found that there is a great discrepancy between the two groups. In this study, questionnaires were administered to 70 psychiatric patients, 6 Chinese medicine men, 44 general physicians and 35 neuropsychiatrists, to inquire into the reasons for positive or negative attitudes toward neurasthenia. Half of the clinical patients believed that they were suffering from neurasthenia. Neurasthenia is a predominate term used for various types of distress arising mainly from psychiatric diseases. Chinese medicine men are aware that this term is a medical diagnosis introduced from the West. Through experience they regard neurasthenia as a kind of deficit of nerve. Apparently, the concept of neurasthenia has been integrated into the Chinese medical system, a fact substantiated by its longstanding, nosological use by the public.Younger generation physicians within both general and neuropsychiatric disciplines on the whole reject neurasthenia as a diagnostic term. However, one-third of neuropsychiatrists and 40% of general physicians use this term in their practice in order to improve the treatment of and to establish good communication and rapport with the patients whom they treat. Most of them, however, do not use the term in their formal diagnosis. The concept of the illness, neurasthenia, is historically rooted and today presents a nosological dilemma. It will eventually be transformed conceptually and disappear from the public mind.     

Medico-genetic study of the population of Uzbekistan. I. Incidence and variety of hereditary pathology]

In district of the Samarkand province the screening for families burdened with multiple cases of non-infectious diseases was performed. The principles of the applied screening procedure are described in the present paper. In the course of clinical examination 98 families were detected, 55 of which included more than one person suffering presumably with Mendelian diseases and 43--with multifactorial disorders. Over 30 nosological forms were found, among which orthopaedic and neurological forms were the most frequent. As a rule, identical cases were detected in one or two families. The role of certain genetic processes in the distribution of hereditary diseases in the Uzbek population is discussed.     

Effect of the site of microbial aerosol application on the pathogenesis and clinical picture of aerosol infection]

On the basis of experimental and clinical study of infections with the aerosol mechanism of infection there was revealed a relationship between the fractional-dispersive composition of the microbial aerosol, the porta of infection and the clinico-pathogenetic peculiarities of the course of the disease. On the example of tularemia, plague and other nosological forms it was demonstrated that coarse-dispersive aerosol caused development of oculo-bubonic and anginous-bubonic form of the disease, whereas the high-dispersive aerosol led to the appearance of primary pneumonia. In experimental aerosol infection with the causative agents in which the infection under natural conditions is not air-borne (botulism, American horse encephalo-myelitis, etc.) specific disease as a rule develops without any primary affection of the respiratory organs.     

Do we have any solid evidence of clinical utility about the pathophysiology of schizophrenia?

A diagnosis of schizophrenia, as in most of psychiatric practice, is made largely by eliciting symptoms with reference to subjective, albeit operationalized, criteria. This diagnosis then provides some rationale for management. Objective diagnostic and therapeutic tests are much more desirable, provided they are reliably measured and interpreted. Definite advances have been made in our understanding of schizophrenia in recent decades, but there has been little consideration of how this information could be used in clinical practice. We review here the potential utility of the strongest and best replicated risk factors for and manifestations of schizophrenia within clinical, epidemiological, cognitive, blood biomarker and neuroimaging domains. We place particular emphasis on the sensitivity, specificity and predictive power of pathophysiological indices for making a diagnosis, establishing an early diagnosis or predicting treatment response in schizophrenia. We conclude that a number of measures currently available have the potential to increase the rigour of clinical assessments in schizophrenia. We propose that the time has come to more fully evaluate these and other well replicated abnormalities as objective potential diagnostic and prognostic guides, and to steer future clinical, therapeutic and nosological research in this direction.     

Spectrum and territorial distribution of hereditary diseases in the population of the Krasnodar Krai]

The analysis of the spectrum of hereditary diseases in the population of the Krasnodar province is performed and the influence of the population dynamics factors on the spectrum is discussed. More than 130 nosological forms were discovered in the population of approx. 200,000. Among these, there are 63 autosomal dominant, 49 autosomal recessive and 17 X-linked recessive forms. Of the most frequent autosomal dominant diseases (more than 1 per 50,000) autosomal recessive and X-linked recessive disorders 13, 7 and 7 forms, respectively, were picked up. The coefficient of diversity of hereditary diseases (the number of nosological forms per 10 inhabitants) with different types of inheritance is higher in the Krasnodar population, as compared with the Kostroma population. The problem of similarity of the "nucleus" of autosomal-recessive disorders in Russian populations is discussed.