Autonomic Dysfunction is Associated with Increased Cardiometabolic Risk in Patients with Childhood-Onset Craniopharyngioma.

Patients with craniopharyngioma are susceptible to autonomic dysfunction as a result of hypothalamic damage. We evaluated indices of heart rate variability (HRV) in patients with childhood-onset craniopharyngioma to investigate autonomic function and its relationship with components of the metabolic syndrome (MetS). This cross-sectional, case-only study included 53 patients (10-30 years of age). We measured the standard deviation of all normal R-R intervals (SDNN) and total power indicating overall HRV, the root-mean square of the difference of successive R-R intervals (RMSSD) and high frequency indicating parasympathetic modulation, and low frequency. These indices were compared according to the presence of the MetS. During the mean 10.8 years of follow-up, 25% of patients were diagnosed with the MetS. Patients with the MetS showed significantly lower levels of SDNN (29.0 vs. 40.6 ms), total power (416.1 vs. 1129.6 ms2), RMSSD (20.1 vs. 34.5 ms), high frequency (94.7 vs. 338.5 ms2), and low frequency (94.5 vs. 289.4 ms2) than those without (p <0.05, for all). Individual components of the MetS including insulin resistance, serum triglycerides levels, and systolic blood pressure were inversely associated with SDNN, total power, RMSSD and high frequency. Higher overall variability and parasympathetic modulation were related to decreased odds ratios for having the MetS (OR 0.91, p=0.029 for SDNN; OR 0.91, p=0.032 for total power). In conclusion, autonomic dysfunction, as evidenced by reduced HRV indices, is associated with increased cardiometabolic risk in patients with childhood-onset craniopharyngioma.

[Childhood craniopharyngioma with hypothalamic obesity - no long-term weight reduction due to rehabilitation programs]. / Kraniopharyngeom mit hypothalamischer Adipositas - Stationäre Rehabilitation ohne Einfluss auf die Gewichtszunahme bei Kindern und Jugendlichen.

BACKGROUND: Severe obesity due to hypothalamic involvement has major impact on prognosis in long-term survivors of childhood craniopharyngioma. The long-term effects of rehabilitation efforts on weight development and obesity in these patients are not analyzed up to now. PATIENTS AND METHODS: 108 patients with childhood craniopharyngioma recruited in HIT Endo before 2001 were included in the study. Long-term weight development (BMI SDS after >10 yrs follow-up) was analyzed in regard to rehabilitation, which was performed in 31 of 108 (29%) patients (one rehabilitation in 4 patients (13%), more than one in 21 patients (68%), 6 patients unknown) in 13 German rehabilitation -clinics. RESULTS: 84% of patients underwent rehabilitation in order to reduce hypothalamic obesity (BMI>+ 2 SD), whereas 12% of patients were normal weight. Childhood craniopharyngioma pa-tients with rehabilitation presented with higher BMI at diagnosis (median BMI: +1.32 SD; range: -1.08 to + 7.00 SD) and at last evaluation (median BMI: +4.93 SD; range: -0.20 to + 13.13 SD) when compared with patients without rehabilitation (median BMI at diagnosis: +0.24 SD; range: -2.67 to + 6.98 SD; BMI at evaluation: +2.09 SD; range: -1.48 to + 10.23 SD). A long-term weight reducing effect of rehabilitation was no detectable regardless of degree of obesity, frequency of rehabilitation, and hospital of rehabilitation. CONCLUSION: Treatment options for hypothalamic obesity in terms of rehabilitation are limited. Accordingly, strategies for prevention of hypothalamic lesions and psychosocial effects of rehabilitation are currently in focus for improvement of prognosis in childhood craniopharyngioma patients.

Brief communication: cognitive rehabilitation of executive functioning in a case of craniopharyngioma.

Craniopharyngiomas are rare brain tumors within the area of the craniopharyngeal duct. They are often diagnosed in children and young people and can be associated with significant cognitive impairments such as memory and executive problems. This single-case report describes a brief investigation into the effectiveness of goal management training for dysexecutive problems following the removal and treatment of a craniopharyngioma in a young woman. In contrast to previous laboratory-based studies, we exploited naturally occurring distractions within the patient's work environment as periodic alerts to facilitate goal management. Following a 2-month intervention, the patient reported improvements in her ability to focus attention at work and performed significantly better on neuropsychological tests requiring organized behavior posttraining relative to pretraining. Thus, the exploitation of naturally occurring external distractions as periodic alerts to facilitate goal management can be an effective and easy-to-adopt cognitive rehabilitation strategy for dysexecutive problems associated with craniopharyngioma.

Patients cured from craniopharyngioma or nonfunctioning pituitary macroadenoma (NFMA) suffer similarly from increased daytime somnolence despite normal sleep patterns compared to healthy controls.

OBJECTIVE: Adults patients previously treated for craniopharyngioma have increased general and physical fatigue compared to healthy controls. This could be related to disturbed sleep patterns. The aim of this study was to compare sleepiness and sleep patterns in those patients to healthy controls and to patients treated for nonfunctioning macroadenomas (NFMA) of the pituitary. DESIGN: Case-control study. PATIENTS AND METHODS: Sleepiness and sleep patterns were assessed in 27 adult patients [14 men, 8 patients diagnosed at childhood age, mean age of 53 years (range 27-80 year)] after long-term follow-up and compared to 50 healthy controls and 38 age-, gender- and BMI-matched patients with NFMA. We used two validated questionnaires for sleep parameters (Epworth sleepiness score and Münchener Chronotype Questionnaire). RESULTS: Sleep patterns (onset, sleep timing, duration and rise time) were not statistically different between the three groups. However, daytime sleepiness scores were increased in patients treated for craniopharyngioma compared to healthy controls, but not different from patients with NFMA. Thirty-three percent of patients with craniopharyngiomas had ESS scores above 10 compared to 8% of healthy controls (P = 0.005), indicating severe daytime hypersomnolence. Neither type of surgery, previous radiotherapy, or age at diagnosis influenced the sleepiness scores in patients with craniopharyngioma. CONCLUSION: Patients treated for craniopharyngioma or NFMA have increased daytime somnolence despite normal sleep patterns, compared to healthy subjects. The results indicate that increased daytime somnolence is a general consequence of large tumours, and/or their treatment, in the hypothalamic/pituitary region, rather than a specific feature of craniopharyngiomas per se.

[Rehabilitation of young craniopharyngioma patients]. / Rehabilitation jugendlicher Kraniopharyngeom-Patienten.

The course of rehabilitation in 11 young patients with craniopharyngeoma was investigated. With intelligence well-preserved, neuropsychological diagnosis revealed impairments in rate and persistence in virtually all patients; behaviour disorders were present in 8 of the 11 patients, with efforts to distinguish psychoorganic from psychoreactive causality however not always successful. The neuropsychological deficits and behavioural deviations mentioned, rather than complications of a medical nature (1 of 11), resulted in only 2 patients having been able to maintain their premorbid educational/vocational status. The rehabilitative outcomes obtained in patients with clear-cut indications of diencephalic lesion (among them variations in vigilance, polyphagia, adipsia) had been unsatisfactory, whereas, in the other behaviourally disordered patients, successful outcomes were achieved by a therapeutic approach tailored to the specific needs of the individual condition.

Results of treatment for craniopharyngioma.

A review of the results of treatment for 155 cases of craniopharyngioma during the past 47 years by the palliative operation was made. Steroid and operative microscope reduced operative mortality and gave more chance of total extirpation, but survival rate was almost unchanged. It is noteworthy that 3 cases who underwent palliative operation more than 30 years ago are still alive and leading useful lives. The quality of survival was somewhat less favorable in pediatric than adult cases. Morbidity frequently encountered in pediatric cases was growth retardaton. Craniopharyngioma is a benign tumor pathohistologically, but it should be considered as malignant because of the region it occurs in. Therefore, no forceful attempt to total extirpation should be made and the operations should, as a rule, be palliative.