One hundred and fifty years of hebephrenia. A review.

INTRODUCTION: The publication of Hecker's article on hebephrenia in 1871 was a fundamental milestone for clinical psychiatry. Despite the initial recognition, many voices were raised against this diagnostic category and its limits were attenuated throughout the 20th century until its disappearance at the beginning of this century (along with the other subtypes of schizophrenia) in the DSM and ICD. DISCUSSION: However, given the consistency of the clinical picture, there is the possibility of other criteria emerging that would lead its systematic study to continue or recommence. In this sense, the concepts of deficit schizophrenia, hebephrenia as a replacement for schizophrenia as a whole, and Leonhard's hebephrenias as systematic schizophrenias stand out. This article discusses the main diagnostic conflicts of the category of hebephrenia over time, with emphasis on the problems of recent decades. CONCLUSIONS: The concept of hebephrenia has begun to be revalued in recent years, and the concepts of deficit schizophrenia, of hebephrenia as a major category, and of systematic hebephrenias allow further investigation of this foundational picture of clinical psychiatry.

An overview on Hebephrenia, a diagnostic cornerstone in the neurodevelopmental model of Schizophrenia.

Pre-Kraepelinian observations converged in Kahlbaum's and Hecker's description of Hebephrenia. For Kraepelin, Hebephrenia was an 'idiopathic incurable dementia whose onset is in adolescence'. It became the core of 'Dementia Praecox', and then Bleulerian 'Schizophrenia'. In recent decades, the resurgence of the 'late neurodevelopment' hypothesis of schizophrenia has brought into focus Hecker's clinical reports of adolescents who, as a result of a putative loss of psychic energy, showed a rapidly progressive cognitive impairment leading to functional and behavioural disorganization. This paper summarizes the nineteenth-century conceptualization of Hebephrenia as a developmental illness.

The development of Kraepelin's mature diagnostic concept of hebephrenia: a close reading of relevant texts of Hecker, Daraszkiewicz, and Kraepelin.

In developing his mature concept of hebephrenic dementia praecox (DP) in his 4th (1893) through 6th textbook editions (1899), Kraepelin worked from the hebephrenic syndrome first described by Hecker (1871) and then carefully studied by his student Daraszkiewicz (1892). Working under Kraepelin's supervision, Daraszkiewicz followed Hecker in emphasizing several key features of hebephrenia (distinctive deteriorative course, importance of silliness and minimal positive psychotic symptoms) but expanded the syndrome to include cases developing severe dementia, rejected the link to prodromal depressive and manic phases, and reduced the emphasis on thought disorder. Daraszkiewicz proposed a soft subtyping of hebephrenia based on level of deterioration, which Kraepelin adopted in his 4th edition with an additional emphasis on severe positive psychotic symptoms. In his 5th edition, Kraepelin created a third subform with even more pronounced and bizarre delusions and hallucinations. In his 6th edition, which contained his first articulation of DP, Kraepelin eliminated his hebephrenia subforms presenting a single syndrome, which, compared to Hecker, included more emphasis on positive psychotic and catatonic symptoms and severe dementia. Kraepelin's paths to hebephrenic and paranoid DP differed in important ways. Paranoid DP was a de novo syndrome created by differentiation from paranoia. Hebephrenia, by contrast, evolved from a disorder created in the Kahlbaum/Hecker paradigm of the iterative study of clinical features, course and outcome. Kraepelin further implemented this approach in substantially reworking, over several drafts, the hebephrenic syndrome to fit into his emerging construct of dementia praecox.

Heboidophrenia and Pseudo-Psychopathic Schizophrenia: Current Knowledge and Critical Perspective.

In this article, based on literature review, we present an integrated description of heboidophrenia and pseudo-psychopathic schizophrenia. Both diagnostic constructs describe latent psychotic processes inextricably bound with psychopathic features. Although both have been described in different eras and research threads, they are that similar that we could not find divergences. We formulated operational criteria for clinical and research purpose. The recognition of this syndrome improves risk management, treatment, and legal decisions.

[Hebephrenia - a viable psychopathological construct?] / Hebephrenie ­ ein brauchbares psychopathologisches Konstrukt?

Ewald Hecker was the first psychiatrist to describe the disease entity of hebephrenia in some detail, focusing mainly on disturbances of affect. Later Emil Kraepelin and Eugen Bleuler saw hebephrenia as a subtype of dementia praecox or schizophrenia. Willy Mayer-Gross and Karl Leonhard characterized hebephrenia with highly differentiated psychopathological descriptions, whereas this construct only played a minor role in the works of Klaus Conrad and Kurt Schneider. The International Classification of Diseases (ICD-10) lists hebephrenia as a subtype of schizophrenia but in the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) no subtypes of schizophrenia are mentioned and the concept of hebephrenia is thus lost. Hebephrenia can be seen as an ideal type describing a psychopathological course pattern. This construct can be useful to conceptualize a group of disorders of affect which otherwise escape description, especially since these psychopathological alterations of affect are difficult to operationalize. To have a viable concept of these disorders is relevant for the prognosis and therapy planning. If the concept of hebephrenia is abolished, important psychopathological knowledge might be lost for future generations of psychiatrists.

The Curse of the Dolphins: Cognitive Decline and Psychosis.

CASE: Isela is an 11-year-old Mexican-American girl with mild intellectual disability. During a vacation with her family, she went swimming with dolphins. A few days later, Isela awoke at night with laughing spells; during the day, she was pacing, aggressive, and had a decline in self-care and communication skills. Her parents attributed the symptoms to the dolphins. She was evaluated by a pediatric neurologist. The sleep-deprived electroencephalogram, brain magnetic resonance imaging, lumbar puncture, and thyroid function tests were normal. A genomic microarray was sent. The neurologist initiated empirical therapy for seizures with lamotrigine, which caused a rash. It was discontinued. She was then treated with oxcarbazepine followed by topiramate for several months without any change in symptoms. Comparative genomic hybridization revealed a small deletion at 14q13.1, which includes the NPAS3 gene. Psychiatry was consulted after several months of persistent symptoms. Isela seemed to be laughing in response to internal stimuli. Owing to the decline in communication and her apparent preoccupation with visual and auditory internal stimuli, Isela could not be interviewed adequately to confirm that she was experiencing hallucinations, but her laughter seemed to be in response to hallucinations. Isela was diagnosed with disorganized schizophrenia with psychosis. Risperidone was prescribed. A psychology evaluation was completed a few months later. Parents noted significant improvement after starting risperidone with reduced inappropriate laughing spells, reduced pacing, as well as improved eating, sleeping, communication, and self-care. Cognitive assessment with the Wechsler Abbreviated Scale of Intelligence-II indicated the following: verbal estimated intelligence quotient (IQ) = 70, perceptual estimated IQ = 71, and full-scale estimated IQ = 68. There was no cognitive decline compared with testing at school 4 years previously. Although psychotic symptoms were significantly improved on antipsychotic medication and function appeared to have been restored to her previous level, her parents continued to perceive a significant decline of functioning, and they continued to attribute the psychosis to swimming with the dolphins.

[The use of acatinol in the treatment of stable patients with schizophrenia with predominance of behaviour disorganization and subcatatonic features].

Our study was based on the hypothesis that a non competitive antagonist of NMDA receptors can improve clinical effects of antipsychotic therapy in a subgroup of patients with schizophrenia with clinical signs of glutamatergic hyperfunction such as catatonic symptoms and disorganization. The study design was open and non-comparative. The duration of the study for each patient was 6 months, the target dosage of acatinol was 20 mg. Forty stable patients with schizophrenia with predominance of signs of disorganization and subcatatonic symptoms were included. The following instruments were used: PANSS, NSA, CGI, BACS, UKU. Adding of acatinol to the antipsychotic treatment improved clinical symptoms, cognitive functioning and social functioning and decreased the number of side effects. The drug was well-tolerated.

Verbal working memory in individuals with schizophrenia and their first degree relatives: relationship with negative and disorganized symptoms.

OBJECTIVE: To determine whether there are differences in verbal working memory amongst subjects with schizophrenia, their first degree relatives and controls, and to evaluate the influence of symptoms on these differences, as an initial step to assess whether this cognitive function is an endophenotype. METHODS: We examined 197 cases with schizophrenia, 197 first degree relatives and 200 controls through psychiatric interviews and the Letters and Numbers Sequencing test (LNS). Performance was compared among the three groups adjusting for age, sex and education level. Adjustment for "negative symptoms" and "disorganization" was performed afterwards. RESULTS: Subjects with schizophrenia showed lower performance in the LNS than their first degree relatives and the healthy controls; the effect sizes were 0.75 and 1.18 respectively. There was a small difference between relatives and controls (effect size =0.38). These differences were significant after adjustment for negative and disorganized symptoms, but the effect sizes became smaller: 0.26 for relatives vs. subjects with schizophrenia, 0.56 for controls vs. subjects with schizophrenia and 0.33 for relatives vs. controls. Among individuals with schizophrenia, performance in the LNS was not associated with disorder duration, disease onset age, antipsychotics, history of depressive episodes or substance use disorders. CONCLUSION: Results suggest verbal working memory may be considered as an endophenotype in schizophrenia.

[Life-threatening complication of schizophrenia]. / Lebensbedrohliche Komplikation bei Schizophrenie.

The Wernicke Encephalopathy (WE) as a result of a (sub)acute thiamine deficiency remains, unfortunately, still under-diagnosed, especially among non-alcoholics. It should be considered in each occurrence of delirium as well as in any ocular motor disorder and ataxia, in particular if there is a history of weight loss with or without gastrointestinal symptoms. In cases with suspected WE an immediate intravenous substitution of 3×200 mg/d is recommended.

Visual context processing deficits in schizophrenia: effects of deafness and disorganization.

Visual illusions allow for strong tests of perceptual functioning. Perceptual impairments can produce superior task performance on certain tasks (i.e., more veridical perception), thereby avoiding generalized deficit confounds while tapping mechanisms that are largely outside of conscious control. Using a task based on the Ebbinghaus illusion, a perceptual phenomenon where the perceived size of a central target object is affected by the size of surrounding inducers, we tested hypotheses related to visual integration in deaf (n = 31) and hearing (n = 34) patients with schizophrenia. In past studies, psychiatrically healthy samples displayed increased visual integration relative to schizophrenia samples and thus were less able to correctly judge target sizes. Deafness, and especially the use of sign language, leads to heightened sensitivity to peripheral visual cues and increased sensitivity to visual context. Therefore, relative to hearing subjects, deaf subjects were expected to display increased context sensitivity (ie, a more normal illusion effect as evidenced by a decreased ability to correctly judge central target sizes). Confirming the hypothesis, deaf signers were significantly more sensitive to the illusion than nonsigning hearing patients. Moreover, an earlier age of sign language acquisition, higher levels of linguistic ability, and shorter illness duration were significantly related to increased context sensitivity. As predicted, disorganization was associated with reduced context sensitivity for all subjects. The primary implications of these data are that perceptual organization impairment in schizophrenia is plastic and that it is related to a broader failure in coordinating cognitive activity.

Acerca de un caso de hebefrenia / No disponible

El término hebefrenia, acuñado por la psiquiatría clásica, fue sustituido por el de psicosis indiferenciada en los manuales modernos. Un caso clínico, que por su complejidad ha obligado la intervención de varios servicios de la red psiquiátrica, muestra que el concepto de hebefrenia sigue siendo útil y actual (AU)

The term hebephrenia, coined by classical psychiatry, was replaced by undifferentiated psychosis in modern textbooks. A clinical case, that complexity has forced the intervention of several psychiatric network services, shows that the concept of hebephrenia remains useful and current (AU)

Trastornos del Espectro Autista o Esquizofrenia Infantil: presentación de un caso clínico / Autistic Spectrum Disorder or Infantile Schizoprhenia: presentation of a clinical case

Objetivos: Diagnóstico diferencial entre Trastornos del Espectro Autista y Esquizofrenia. Métodos: Se expone el caso de un joven de 16 años con antecedentes psiquiátricos desde los 5 años de edad. Se revisa la evolución del mismo a lo largo del tiempo, así como los diagnósticos recibidos. Resultados: Se establece el diagnóstico inicial de Trastorno por déficit de atención e hiperactividad y Trastorno generalizado del desarrollo no especificado en paciente con importantes dificultades en la interacción social. La evolución clínica posterior, con presencia de síntomas psicóticos, comportamiento desorganizado y deterioro cognitivo, permiten realizar el diagnóstico de Esquizofrenia Desorganizada en la adolescencia. Conclusión: El diagnóstico diferencial entre Trastornos del Espectro Autista y Esquizofrenia es complicado en los primeros años vida. Solamente la evolución posterior, con la aparición de síntomas psicóticos propiamente dichos, permitirá un diagnóstico de certeza. En la literatura se han comparado los síntomas de diferentes tipos de esquizofrenia con los Trastornos del Espectro Autista y se ha observado que comparten rasgos, especialmente con la esquizofrenia hebefrénica o desorganizada

Objetive: Differential diagnosis between Autistic Spectrum Disorder and Schizophrenia. Methods: The case of a sixteen-year old boy who presents psychiatric antecedents since he was 5 years old is presented. His clinical evolution and the different diagnoses are revised. Results: A patient with difficulties in social interaction was diagnosed of Attention-Deficit Hyperactivity Disorder and Pervasive Developmental Disorders Not specified some years ago. Later the subsequent clinical evolution with presence of psychotic symptoms, disorganized behavior and cognitive deterioration made it possible to carry out the diagnosis of Schizophrenia Disorganized in his adolescence. Conclusion: The differential diagnosis between Autistic Spectrum. Disorders and Schizophrenia is complicated in the first years of life. Only the later evolution, with the presence of psychotic symptoms in strict sense, will make it possible to define a diagnosis of certainty. The symptoms of different types of schizophrenia have been compared in the literature with Autistic Spectrum Disorder, and it has been observed that they are very similar, specially, with the schizophrenia, disorganized subtype

An abnormal relation between basal prolactin levels and prolactin response to 12.5 microg TRH i.v. in drug-naïve patients with first-episode schizophrenia.

At doses lower than those needed to stimulate prolactin release directly, TRH almost completely antagonizes the inhibitory effect of dopamine on prolactin release. We have previously reported that prolactin response to administration of 12.5 microg TRH i.v. correlates with prolactin response to 0.5 mg i.m. haloperidol and negatively with 24-h urinary excretion of HVA in normal subjects, suggesting that the response reflects dopamine activity. An association between central dopamine hyperactivity and SANS scores relating to poverty of content of speech and inattention has been suggested by studies utilizing methylphenidate administration in patients with first-episode schizophrenia. The hypothesis that small plasma prolactin responses to administration of 12.5 microg TRH i.v. (Delta prolactin) correlate with SANS scores for these symptoms was tested in 19 drug-naïve patients with first-episode schizophrenia. Significant negative correlations were found between the response and scores relating to poverty of content of speech (r = - 0.55, p = 0.014) and inattention (r = - 0.52, p = 0.022), supporting the hypothesis of increased dopamine activity in association with disorganization symptoms. A significant positive correlation between basal prolactin levels and prolactin response to stimulation by 12.5 microg TRH was also found (r = + 0.61, p = 0.0058). Our previous study in normal subjects found a similar positive correlation between basal prolactin levels and prolactin response to stimulation by 200 microg TRH i.v., but not by 12.5 microg TRH i.v. As far as we know, this is the first study to report an abnormality in TRH-induced prolactin release in acute schizophrenia.

Are there valid subtypes of schizophrenia? A grade of membership analysis.

BACKGROUND/AIMS: Cluster analysis has had limited success in establishing whether there are subtypes of schizophrenia. Grade of membership (GoM) analysis is a multivariate statistical technique which has advantages when, as in schizophrenia, individuals conforming to pure types are uncommon and mixed forms are frequent. METHODS: GoM analysis was applied to 118 chronic schizophrenic patients. The patients were of all clinical subtypes, including 13 with simple schizophrenia. Both current and 'lifetime' symptoms were assessed, and two different rating systems were used. RESULTS: Specifying 3 pure types resulted in robust findings across analyses. One pure type corresponded to paranoid schizophrenia, one to simple schizophrenia and the third combined elements of hebephrenic and catatonic schizophrenia. Specifying 4 pure types split the original 3 pure types in ways which were not clinically intuitive. CONCLUSION: GoM analysis divides schizophrenia into subtypes along conventional lines, with the proviso that hebephrenic and catatonic schizophrenic patients are not separable, at least in the chronic stage of the illness.

Quantitative motor activity differentiates schizophrenia subtypes.

BACKGROUND: Motor symptoms are frequent in schizophrenia and relevant to the diagnosis of subtypes. However, the assessment has been limited to observations recorded in scales and experimental designs. The aim of this study was to use wrist actigraphy to obtain motor activity data in 3 schizophrenia subtypes. METHODS: In total, 60 patients with schizophrenia (35 paranoid, 12 catatonic, 13 disorganized) were investigated using continuous wrist actigraphy over 24 h in an inpatient setting on average 38 days after admission. Data of the wakeful hours of the day were analyzed. RESULTS: The activity level was predicted by schizophrenia subtype and by the type of antipsychotic medication. The movement index and mean duration of uninterrupted immobility were found to be predicted only by the schizophrenia subtype. Age, gender, duration of illness and chlorpromazine equivalents did not contribute to the variance of the activity data. A MANOVA demonstrated the significant differences in the 3 parameters between schizophrenia subtypes (p = 0.001). Patients with catatonic schizophrenia had lower activity levels, a lower movement index and a longer duration of immobility than those with paranoid schizophrenia. CONCLUSIONS: Schizophrenia subtypes can be differentiated using objective measures of quantitative motor activity. The increased duration of immobility appears to be the special feature of catatonic schizophrenia.

Organization of cognitive control within the lateral prefrontal cortex in schizophrenia.

CONTEXT: Cognitive control is highly affected in schizophrenia, but its overall functional architecture remains poorly understood. A recent study demonstrated that, in healthy subjects, cognitive control is functionally organized within the lateral prefrontal cortex (LPFC) as a cascade of representations ranging from premotor to anterior LPFC regions according to stimuli, the present perceptual context, and the temporal episode in which stimuli occur. OBJECTIVE: To determine the functional hierarchical organization of cognitive control within the LPFC in patients with schizophrenia. DESIGN: Case-control study. SETTING: Hospital-based research units. PARTICIPANTS: Fifteen schizophrenic patients and 14 controls. MAIN OUTCOME MEASURES: Behavioral performance and regional brain activity as measured by functional magnetic resonance imaging during a task, varying the amount of information conveyed by episodic and contextual signals. RESULTS: In patients and healthy controls, activity in caudal LPFC regions varied as episodic and contextual signals, whereas rostral LPFC regions only exhibited an episodic effect. However, patients made more errors than controls when information conveyed by contextual and episodic signals increased. These impairments were related to hypoactivation in caudal LPFC regions and hyperactivation in rostral LPFC regions, respectively. Activation in caudal LPFC regions negatively correlated with the disorganization syndrome score of patients. CONCLUSIONS: In schizophrenic patients, the architecture of cognitive control follows the cascading organization from rostral LPFC regions to caudal LPFC and premotor regions depending on the temporal framing of action and events. We found, however, that immediate contextual signals insufficiently bias the caudal LPFC activity required to select the appropriate behavioral representation. This specific deficit could thus alter the internal consistency of schizophrenic patients' behavior. To compensate for this weakening of contextual influence, schizophrenic patients may inefficiently use temporal episodic information through higher activation in rostral LPFC regions.