Citations

Objectives: The Patient Health Questionnaire-9 Modified (PHQ-9M) is a self-report tool used to assess the presence and severity of depressive symptoms in teenagers. Despite widespread use in primary care clinics and psychiatric settings, the PHQ-9M has not been validated nor are its psychometric properties adequately understood for the adolescent population. This study sought to examine the psychometrics of the PHQ-9M in treatment-seeking, depressed adolescents at a psychiatric psychopharmacology clinic who were concurrently assessed with the Children's Depression Rating Scale Revised (CDRS-R) and Quick Inventory of Depressive Symptomatology-Adolescent (17-item) Self-Report (QIDS-A17-SR). Methods: Adolescents ( N = 160) aged 13 through 18 years with a diagnosis of major depressive disorder, determined on the basis of a clinical interview and semi-structured interview using the Kiddie Schedule for Affective Disorders and Schizophrenia—Present and Lifetime Version, were assessed for severity of depressive symptoms with the PHQ-9M, CDRS-R (adolescent interview only), and QIDS-A17-SR assessments at baseline, 4, and 8 weeks. Classical test theory analysis was used to evaluate the internal consistency and dimensionality of the PHQ-9M. Convergent validity was evaluated via intraclass correlations of the PHQ-9M with the CDRS-R and QIDS-A17-SR. Sensitivity to treatment response was also evaluated. Results: The internal consistency (Cronbach's coefficient α) at baseline, 4, and 8 weeks was 0.879, 0.859, and 0.827 for the PHQ-9M; 0.739, 0.835, and 0.867 for CDRS-R; and 0.712, 0.777, and 0.804 for QIDS-A17-SR, respectively. The PHQ-9M had moderate convergent validity with the CDRS-R but good convergent validity with the QIDS-A17-SR. The PHQ-9M was less sensitive to changes in symptom severity than the CDRS-R and QIDS-A17-SR. Conclusions: The PHQ-9M appears to be a valid and reliable assessment tool for the severity of depressive symptoms in a psychiatric clinic setting. However, its utility as a treatment outcome measure may be limited compared with other available rating scales.

Objective. To evaluate longitudinal cognitive/behavioral change over 12 months in participants enrolled in the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS). Methods. We analyzed data from 294 ALS participants, 134 of whom were studied serially. Change over time was evaluated controlling for age, sex, symptom duration, education, race, and ethnicity. Using multiple regression, we evaluated associations among decline in ALS Functional Rating Scale-Revised (ALSFRS-R) scores, forced vital capacity (FVC), and cognitive/behavioral changes. Change in cognitive/behavioral subgroups was assessed using one-way analyses of covariance. Results. Participants with follow-up data had fewer baseline behavior problems compared to patients without follow-up data. We found significant worsening of behavior (ALS Cognitive Behavioral Screen (ALS CBS) behavioral scale, p<0.001; Frontal Behavioral Inventory-ALS (FBI-ALS) disinhibition subscale, p=0.044). Item analysis suggested change in frustration tolerance, insight, mental rigidity, and interests (p<0.05). Changes in ALSFRS-R correlated with the ALS CBS. Worsening disinhibition (FBI-ALS) did not correlate with ALSFRS-R, FVC, or disease duration. Conclusion. We did not detect cognitive change. Behavioral change was detected, and increased disinhibition was found among patients with abnormal baseline behavioral scores. Disinhibition changes did not correlate with disease duration or progression. Baseline behavioral problems were associated with advanced, rapidly progressive disease and study attrition.

A 70-year-old female presented with a three-year history of evolving macroglossia causing dysphagia and dysarthria, with proximal muscle weakness. Given the classic physical finding of macroglossia, the patient underwent extensive evaluation for amyloidosis which proved to be negative apart from a bone marrow biopsy which stained positive for transthyretin without amino acid sequence abnormality, thus giving wild-type transthyretin amyloidosis. Since the wild-type transthyretin amyloidosis could not entirely explain her clinical presentation and evaluation, further studies were conducted in a sequential manner, thus leading to a diagnosis of Pompe disease explaining her presenting signs and symptoms including her macroglossia. Through this fascinating case, we attempt to highlight the approach for the diagnoses of two rare diseases in a patient by emphasizing the importance of having a broad differential diagnosis when presented with findings which may have been thought as pathognomonic for certain diseases.