Psychological and neuropsychological issues in the care of children with disabilities.

Pediatric specialists are faced with many challenges when confronted with young patients diagnosed with acquired or congenital disabilities. In addition to the myriad of presenting medical issues, team members also need to acknowledge and address contributing psychological, social and environmental factors when working with medically involved children. Specifically, the understanding of developmental, cognitive, familial, and emotional considerations is essential for tailoring more successful individual treatment plans. The purpose of this article is to address the multidimensional aspects associated with the treatment of children presenting with physical or neurological impairments. Specifically, psychological and neuropsychological perspectives will be discussed and case examples will be presented in an effort to propose a comprehensive approach for the promotion of better outcomes in pediatric patient care.

The relationship of the Rorschach Schizophrenia Index to psychotic features in a child psychiatric sample.

In this study we investigated the reliability and validity of the Rorschach Schizophrenia Index (SCZI) from Exner's (1978, 1993) Comprehensive System for a sample of 413 child psychiatric inpatients by examining relationships with the Personality Inventory for Children-Revised (PIC-R) and chart diagnoses. Interscorer reliability and internal consistency were acceptable. Multivariate analyses of variance results revealed significantly different PIC-R profiles for those with and without elevated SCZI scores, with significant differences emerging on the PIC-R Psychosis (PSY) scale and 2 cognitive triad scales (Intellectual Screening and Development), which have been reported to be more frequently elevated in PIC-R profiles of children with psychotic disorders. Significant differences were found across SCZI groups for the PSY scale, Reality Distortion scale, reality testing critical items and chart diagnoses of psychotic disorder. Implications for clinical interpretation of the SCZI with children and issues for further research with this population are discussed.

Diagnosis of manic episodes in adolescent inpatients: structured diagnostic procedures compared to clinical chart diagnoses.

This study examined the accuracy of clinical chart diagnoses of manic episodes in adolescent psychiatric patients, as well as treatment selection and patient outcome. A consecutive sample of 120 consenting adolescent patients was assessed at admission, discharge, and 30 and 120 days post-discharge. Clinical chart diagnoses were compared to research-quality diagnoses involving structured interview, chart review, and consensus. Agreement statistics were computed, and the symptom and treatment differences were compared between patients for whom there was and was not diagnostic agreement. Clinical diagnoses of manic episodes were more common than research diagnoses, and the rate of agreement between diagnoses was low (kappa = 0.15). Patients diagnosed as experiencing a manic episode by the clinical chart, but not via the research procedure, had reduced severity scores on elation and activity, and higher scores on depression. These patients also had more severe scores on depressive symptoms at follow-up. Manic episodes were diagnosed more frequently by clinicians relative to research-quality procedures. Patients who were diagnosed as experiencing manic episodes by the clinician, but not the research procedure, appeared to have depression and hostility, but not elation. The depression in these patients may not be adequately treated, and there are potential clinical implications of over-diagnosis of manic episodes in adolescents.

Development of an Infrequency-Psychopathology scale for the MMPI-A: the Fp-A Scale.

This article describes the development and initial validation of the Infrequency-Psychopathology scale, Fp-A, for the MMPI-A (Butcher et al., 1992). The scale parallels the Infrequency-Psychopathology scale, F(p), that has been developed for the MMPI-2 (Butcher, Dahlstrom, Graham, Tellegen, & Kaemmer, 1989). Results demonstrated that the 40-item Fp-A scale is superior to the F scale at discriminating between faking-bad and accurate reports of psychopathology, although the improvement over F was modest, particularly when compared to the improvement found for the F(p) scale. The difference seemed to reflect the superiority of the MMPI-A F scale to the MMPI-2 F scale. Even so, the findings suggest that the identification of overreporting on the MMPI-A could potentially be enhanced by using Fp-A as an adjunct to the F scale.

Validity of the family history method for identifying schizophrenia-related disorders.

We examined the family history method's validity for identifying schizophrenia related disorders (SRD) by comparing family history and family study derived diagnoses. First degree relatives (n = 284) of 48 psychiatrically disordered probands, predominantly with schizophrenia, were diagnosed using the Family History RDC (FH-RDC) which include three psychotic schizophrenia related disorders (P-SRD): schizophrenia, chronic SAD and chronic unspecified functional psychosis (CUFP). Supplementary criteria for schizophrenia related personality disorders (SRP), derived to identify schizotypal and paranoid personality disorders (PD), were also assessed. About two thirds of these relatives (n = 196; 69.0%) were independently diagnosed by RDC and DSM-III-R on both axis I and axis II in a family study. The specificity was 1.0 (178/178) and the sensitivity of the family history derived diagnosis for P-SRD was 0.72 (13/18). Sensitivity for P-SRD was improved, however, by inclusion of SRP which captured three of the five false negative relatives. The sensitivity of SRP for schizotypal or paranoid PD was 0.39 (15/38) and the specificity was 0.92 (127/138). The FH-RDC have moderately good sensitivity and excellent specificity for the psychotic schizophrenia related disorders. While family history criteria for SRP are not a good proxy for schizotypal or paranoid PD, they can enhance the family history method's sensitivity for SRD.

Evidence of a locus for schizophrenia and related disorders on the short arm of chromosome 5 in a large pedigree.

We attempted to identify a locus for schizophrenia and related disorders in 24 nuclear families of schizophrenic probands using a predefined classification system for affected cases that included those disorders most clearly identified as sharing a genetic relationship with schizophrenia--schizoaffective disorder and schizotypal personality disorder. Initially, we evaluated 8 markers on chromosome 5 on the first 12 families with available genotyping and diagnostic assessments and, assuming autosomal dominant transmission, found a lod score of 2.67 for the D5S111 locus (5p14.1-13.1) in one large nuclear family (no. 17; sibship: n = 12; schizophrenia: n = 3; schizotypal personality disorder: n = 2); the other 11 families were much smaller, less complete, and provided little additional information. Other branches of no. 17 were then assessed and the 2-point lod score for family 17 rose to 3.72; using multipoint analysis the lod score in 17 was 4.37. When only schizophrenia was used to define affectedness, the positive evidence for linkage to D5S111 was greatly reduced. Sensitivity analysis indicated that the lod score is heavily dependent upon the predefined diagnostic criteria. Our studies of other families of schizophrenic probands eventually totalled 23, but linkage to D5S111 in these yielded a -2.41 lod score. The results provide evidence for genetic linkage of the D5S111 locus to schizophrenia and related disorders in one family. It may be of interest that over several generations, almost all the ancestors of family 17 could be traced back to a small, relatively isolated, hill region of Puerto Rico.

Age at onset and familial risk in Alzheimer's disease.

OBJECTIVE: The authors investigated the relationship between probands' age at onset of Alzheimer's disease with the risk of primary progressive dementia in the probands' first-degree relatives. METHOD: Two hundred probands with clinically diagnosed Alzheimer's disease and 179 nondemented elderly probands were recruited from the Mount Sinai Alzheimer's Disease Research Center, located at Mount Sinai Hospital and the Bronx Veterans Affairs Medical Center. Demographic and diagnostic data were collected on 1,398 of the first-degree relatives of the probands with Alzheimer's disease and 955 first-degree relatives of the nondemented probands. RESULTS: Cox proportional hazards regression analysis showed a significant inverse relationship between age at onset of Alzheimer's disease in probands and greater familial risk in their relatives. Follow-up analyses indicated that the most commonly used age at onset cutoff point--65 years--was one of the points where an association with familial aggregation is least likely to be revealed; other onset cutoff ages (e.g., 55, 70, and 75) better identified Alzheimer's disease groups with differing familial/genetic risks. CONCLUSIONS: The authors conclude that patients with an earlier age at onset of Alzheimer's disease are more likely to have relatives with Alzheimer's disease than are patients with a later age at onset of the disease. An onset age of 70 best differentiated probands whose relatives were at higher risk from those whose relatives were at lower risk.

Patterns of risk in first-degree relatives of patients with Alzheimer's disease.

BACKGROUND: Although an increased cumulative risk for primary progressive dementia (PPD) has been repeatedly demonstrated in relatives of probands with Alzheimer's disease (AD), an examination of their rates of illness at different ages has not been previously undertaken. Such an examination might reveal possible age-related characteristics associated with a more familial variety of AD. METHODS: Using family history interviews and survival analysis, the cumulative risk for and 5-year age-specific hazard rates of PPD were assessed in the first-degree relatives of 200 probands with AD and two nondemented control groups--179 elderly ascertained through the Alzheimer's Disease Research Center (ADRC-derived controls) and 427 elderly ascertained from community senior centers (community controls). RESULTS: The PPD risk curve for the relatives of probands with AD rose to about 30% and was significantly higher than the curves for the relatives of the ADRC-derived and community controls, where comparable rates were observed (approximately 12%). The age-specific hazard rates of PPD were calculated in three groups of relatives for each 5-year interval from ages 45 to 49 years through ages 85 to 89 years. The age-specific relative risk (RRi) for PPD in the relatives of probands with AD began to steadily diminish from the 75- to 79-year age interval (RRi = 13.49) through the 85- to 89-year age interval (RRi = 0.96) compared with the relatives of ADRC-derived controls and from the 60- to 64-year age interval (RRi = 16.15) through the 85- to 89-year age interval (RRi = 2.03) compared with the relatives of the community controls. CONCLUSIONS: These data indicate that, for relatives of probands with AD, while the lifetime risk for PPD is greater than in relatives of controls, the familial contribution to the risk for PPD decreases with increasing age. The higher risk for PPD in relatives of probands with AD may be substantially diminished or even eliminated by the latter half of the ninth decade.