Distinctive hydrocephalus-like phenotype in NOTCH2NLC-related neuronal intranuclear inclusion disease: clinicopathological features and therapeutic implications.

The new ICD‐11 presents the first official version of a purely dimensional personality disorder (PD) classification with major implications for clinical practice and research (Blüml and Doering ). One of these implications is reflected in the focus on the overall personality functioning in relation to self and others, which stands for the core component of personality. The level of impairment in personality functioning provides the essential parameter for distinguishing PD from healthy personality as well as from other forms of mental disorders (World Health Organization ). The emphasis that ICD‐11 puts on the distinction between so‐called ‘personality difficulty’ and three degrees of PD severity (i.e., mild, moderate, and severe) resembles the psychodynamic tradition of personality evaluation, distinguishing between neurotic, borderline, and psychotic personality organization (Doering et al. ; Caligor et al. ). According to Kernberg (Kernberg ) and his colleagues, (Clarkin et al. ) assessing the degree of integration of self and the degree of normality or pathology in an individual's relationships with other people is the main criterion for evaluating any PD. In other words, when discussing the degree of impairment of personality functioning within the concept of personality organization, according to Kernberg, (Kernberg ) it is necessary to always assess all three structural characteristics, that is, integration versus diffusion of identity (and the related overall quality of object relations); the constellation of mature versus immature defensive operations; and the presence versus absence of reality testing. These characteristics subsequently distinguish between neurotic, borderline, and psychotic structural organizations. While individuals with a neurotic personality organization exhibit defensive operations centered around repression and other higher‐level defenses, patients with borderline and psychotic organizations exhibit predominantly lower‐level defensive operations centered around the splitting mechanism. Reality testing is preserved in neurotic and borderline organizations but is severely impaired in psychotic organizations. The most traditional way to evaluate the concept of personality organization is the Structural Interview. The Structural Interview combines features of a traditional mental state examination; a psychoanalytically oriented interview focused on the patient‐therapist interaction; and the clarification, confrontation, and interpretation of identity conflicts, defense operations, and reality distortions that the patient reveals in the interaction with the therapist in the “here‐and‐now,” especially if they express identifiable transference elements (Kernberg ). Although it is a clinically‐based approach deeply grounded in psychoanalytic theory, its administration is time‐consuming and requires a high level of knowledge of psychodynamic processes and clinical skills from the interviewer, which significantly affects its reliability (Reich and Frances ). To enhance its empirical soundness, Clarkin, Foelsch, and Kernberg (Clarkin et al. ) created the Inventory of Personality Organization (IPO)—a 57‐item self‐assessment tool that contributes to the structural diagnosis of the patient's personality usually received via semi‐structured, clinician‐rated interviews (Hörz‐Sagstetter et al. ). The IPO has been translated to various languages, for example, French, (Normandin et al. ) Japanese, (Igarashi et al. ) Dutch, (Berghuis et al. ) German, (Zimmermann et al. ) and Italian (Preti et al. ). Previous research has verified the IPO's internal consistency and test‐retest reliability in the general population (Normandin et al. ; Lenzenweger et al. ) and among psychiatric patients, (Hörz‐Sagstetter et al. ; Beheydt et al. ) as well as the criterion validity of the IPO in relation to established methods for assessing personality psychopathology (Berghuis et al. ; Preti et al. ; Lenzenweger et al. ). Nevertheless, from the factorial perspective, the initially proposed tripartite structure of the primary IPO scales in relation to Kernberg's model (i.e., identity diffusion, primitive defenses, and reality testing) was not confirmed by majority of studies. Some authors aimed to identify a subset of items for a unidimensional structure, (Zimmermann et al. , ) some authors pointed out a significant overlap of identity diffusion and primitive defenses factors, (Berghuis et al. ; Lenzenweger et al. ) others preferred a four‐factor solution, (Preti et al. ; Ellison and Levy ) yet others kept the original three factors, but at the cost of substantially eliminating items (Igarashi et al. ).