The posterior fossa syndrome questionnaire: using science to inform practice.

INTRODUCTION: Up to 34% of patients with medulloblastoma develop posterior fossa syndrome (PFS) following brain tumor resection and have increased risk of long-term neurocognitive impairments. Lack of agreement in conceptualization and diagnosis of PFS calls for improvements in diagnostic methods. The current study aimed to describe psychometric properties of a new posterior fossa syndrome questionnaire (PFSQ). METHODS: The PFSQ was informed by prior research and developed by a multidisciplinary team with subject matter expertise. Participants (N = 164; 63.4% Male; 78.7% White; Mage at diagnosis = 10.38 years, SD = 5.09, range 3-31 years) included patients with newly diagnosed medulloblastoma enrolled in the SJMB12 clinical trial. Forty-four patients (26.8%) were classified as having PFS based on attending physician's post-surgical yes/no report. A PFSQ was completed by a neurologist within 2 weeks of coming to St. Jude Children's Research Hospital for adjuvant treatment, irrespective of suspicion for PFS. RESULTS: PFSQ items ataxia (100.00%), dysmetria (95.45%), and speech/language changes (79.55%) were most sensitive. However, ataxia (26.50%) and dysmetria (46.61%) demonstrated low specificity. Speech/language changes (81.36%), mutism (95.76%), orofacial apraxia (98.29%) and irritability (96.61%) had high specificity. A principal component analysis found four components: (1) speech/language changes, (2) apraxias (including mutism), (3) motor/oromotor, and (4) emotional lability. CONCLUSIONS: The PFSQ is a dimensional diagnostic approach that can be used to improve diagnostic consistency across clinical and research groups to help accelerate understanding of PFS etiology, identify surgical correlates of risk, predict long-term impairments, and develop targeted interventions. Additional measure validation, including correlation with symptom resolution, is required.

A Latent Profile Analysis of Sleep, Anxiety, and Mood in Youth with Craniopharyngioma.

Craniopharyngioma is a histologically benign brain tumor that arises in the suprasellar region affecting critical neurovascular structures including the hypothalamic-pituitary-adrenal axis and optic pathways. Children with craniopharyngioma often experience excessive daytime sleepiness (EDS) which may be compounded by anxiety and depression. The current study investigated disparate sleep profiles to better understand psychological adjustment among children diagnosed with craniopharyngioma. METHOD: Actigraphs recorded nightly sleep data, including measures of sleep onset latency (SOL) and wake after sleep onset (WASO), in a cohort of 80 youth between the ages of 2 and 20 years (median age = 9). Parent reports of behavioral and emotional functioning were included in the analysis. A latent profile analysis examined disparate sleep profiles, and a multinomial logistic regression examined differences of anxiety and depression among the sleep profiles. RESULTS: The latent profile analysis revealed three sleep profiles: "healthy sleepers" (68.8%), "night wakers" (21.3%), and "prolonged onset sleepers" (10.0%). Contrary to expectations, sleep profiles were not associated with daytime anxiety (ß = 2.26-4.30, p > .05) or depression (ß = -5.87-4.74, p > .05). CONCLUSIONS: Youth with craniopharyngioma demonstrate poor sleep and EDS. Those with delayed SOL and prolonged WASO are particularly vulnerable to disrupted nighttime sleep, which may significantly compound EDS. Disrupted sleep was not associated with anxiety or depression, which may be related to the overall poor sleep and daytime sleepiness or to timing, as patients were early in their treatment course. Further study should evaluate the factors underlying sleepiness and daytime function in patients with craniopharyngioma.

Do Anxiety and Mood Vary among Disparate Sleep Profiles in Youth with Craniopharyngioma? A Latent Profile Analysis.

INTRODUCTION: Craniopharyngioma is a histologically benign brain tumor that arises in the suprasellar region affecting critical neurovascular structures including the hypothalamic-pituitary-adrenal axis and optic pathways. Children with craniopharyngioma often experience excessive daytime sleepiness which may be compounded by anxiety and depression. The current study investigated disparate sleep profiles to better understand psychological adjustment among children diagnosed with craniopharyngioma. Method: Actigraphs recorded nightly sleep data, including measures of sleep onset latency and wake after sleep onset, in a cohort of 80 youth between the ages of 2 and 20 years (median age = 9). Parent reports of behavioral and emotional functioning were included in the analysis. A latent profile analysis examined disparate sleep profiles, and a multinomial logistic regression examined differences of anxiety and depression among the sleep profiles. Results: The latent profile analysis revealed three sleep profiles: "variable sleepers" (48.3%), "consistently poor sleepers" (45.4%), and "night wakers" (6.4%). Consistently poor sleepers had lower rates of anxiety (g = .76; p = .009) and depression (g = .81; p = .003) than variable sleepers and had significantly lower rates of anxiety than night wakers (g = .52; p = .05); all other differences were nonsignificant (ps > .05). Discussion: Youth with craniopharyngioma who have nightly variations in sleep may have worse psychological functioning than those with more consistent, albeit poor, sleep patterns. Patients with craniopharyngioma who report variable sleep should be assessed for anxiety and depression to prevent and intervene on emotional difficulties that may be reciprocally related to sleep.

What sensitivities matter in dental anxiety? Investigating sensitivity to anxiety, pain, and disgust.

Dental anxiety affects many people worldwide and interferes with oral health. Beyond emotional distress, avoidance of dental care visits can lead to serious dental and health consequences. Although emerging research implicates anxiety, pain, and disgust sensitivities in the etiology and maintenance of dental anxiety, no studies to date have concurrently investigated the unique contribution of these vulnerabilities in dental anxiety. As a step toward elucidating salient mechanisms of dental anxiety, the present study investigated the aggregate contribution of anxiety, pain, and disgust sensitivities in dental anxiety, after controlling for relevant covariates. In this study, participants (N = 717; 71.3% female) included an unselected sample of undergraduate students who completed a battery of online questionnaires. Consistent with community rates, 12% of this sample reported high levels of dental anxiety. The hierarchical regression model revealed anxiety and disgust sensitivities were positively associated with dental anxiety symptoms when adjusting for other model variables. Results highlight the roles of anxiety and disgust sensitivities in dental anxiety and indicate the potential benefit of targeting these emotional sensitivities through routine screenings and treatments for dentally anxious patients.

Characterizing Posterior Fossa Syndrome: A Survey of Experts.

BACKGROUND: Despite recent efforts, disagreement remains among frontline clinicians regarding the operational definition of a syndrome commonly referred to as posterior fossa syndrome or cerebellar mutism syndrome. METHODS: We surveyed experts in the clinical care of children with posterior fossa tumors to identify trends and discrepancies in diagnosing posterior fossa syndrome. RESULTS: All surveyed professionals conceptualized posterior fossa syndrome as a spectrum diagnosis. The majority agreed that mutism is the most important symptom for diagnosis. However, results highlighted ongoing discrepancies related to important features of posterior fossa syndrome. CONCLUSIONS: Greater posterior fossa syndrome conceptual alignment among providers is needed to formulate specific diagnostic criteria that would further research and clinical care. The authors propose preliminary diagnostic criteria for posterior fossa syndrome that require refinement through careful clinical characterization and targeted empirical investigation.