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Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
A heterogeneous group of disorders with midline brain abnormalities: Hypoplasia or absence of the septum pellucidum and corpus callosum.Optic nerve hypoplasia.Pituitary/hypothalamic dysfunction.
Craniopharyngioma
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Hermann L. Müller, Claire Alapetite, Jeffrey Wisoff
The Puget classification defines extent of hypothalamic involvement on pre- and postoperative MRI scans and establishes a relative risk profile for postoperative morbid obesity. Hypothalamic invasion by the tumor (Puget 2) has been associated with a higher postoperative BMI.106,107 Studies promoting hypothalamic-sparing surgery plus irradiation for Puget 2 tumors demonstrated a class 3 obesity rate of 28% without increasing the local recurrence rate when compared with GTR.106 These findings have led to the recommendation of GTR if the tumor does not involve the hypothalamus (Puget 0 and 1) and incomplete resection of Puget 2 tumors followed by adjuvant irradiation.108 However, the Puget classification remains a relatively blunt instrument since even with a hypothalamic-sparing surgery and postoperative irradiation there remains a significant rate of hypothalamic morbidity measured primarily by obesity. There are obviously yet to be determined factors beyond MRI appearance of hypothalamic involvement that will affect outcome, especially with respect to short- and long-term hypothalamic dysfunction.
Gastrointestinal Aspects of Eating Disorders
Published in Kevin W. Olden, Handbook of Functional Gastrointestinal Disorders, 2020
Bruce D. Waldholtz, Arnold E. Andersen
A number of papers have described common physiological changes in anorexia nervosa, including the bulimic subtype, during starvation (3,4,14-22). A generalized metabolic slowdown occurs in the starved anorexia nervosa patient, reflected in resulting bradycardia, hypotension, and hypothermia. This adaptive hypometabolic state may resemble the euthyroid-sick syndrome, in which peripheral conversion of thyroxin to triiodothyronine is decreased (20), or result in a frankly hypothyroid patient with bradycardia. The mechanism of hypothermia present in starvation from anorexia is not well understood but most likely represents hypothalamic dysfunction.
Long-term central nervous system (CNS) consequences of COVID-19 in children
Published in Expert Review of Neurotherapeutics, 2023
Saskia Howe de la Torre, Valeria Parlatini, Samuele Cortese
In this section, we describe the most common neurological symptoms associated with long COVID in the pediatric population (Table 1). These include ‘brain fog’ and memory/attention deficits (2–81%), headache (3–80%), impairment of smell or taste (12–70%), and dizziness (3–20%) [16]. We have also included fatigue (3–87%) [16], as it has been associated with hypothalamic dysfunction, although multiple physiological and psychological factors are likely at play in its pathogenesis, as discussed. Finally, for completeness, we included rarer and more severe COVID-related neurological manifestations, such as seizures and encephalitis/encephalopathy. Seizures typically present as acute SARS-CoV-2 sequelae [69,70]; however, a large cohort study reported that the two-year cumulative risk of seizures/epilepsy was significantly higher in children (but not in adults) after COVID infection as compared to other respiratory infections [66]. Similarly, encephalitis/encephalopathy typically develop during the acute phase of disease or in the context of MIS-C [10,71], but delayed immuno-mediated onset has also been described [71]. Further, cognitive and neurological deficits may persist in those who survive [55,72].
Systematic review of the prognostic role of body mass index in amyotrophic lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2019
Pingping Ning, Baiyuan Yang, Shuangjiang Li, Xin Mu, Qiuyan Shen, Fayun Hu, Yao Tang, Xinglong Yang, Yanming Xu
In healthy people, food intake and nutrient absorption are generally in balance with basal (resting) and activity-induced energy expenditure. In ALS, energy expenditure exceeds intake by 10–27% (47), leading to energy imbalance. One reason is that bulbar muscle weakness leads to dysphagia, which leads in turn to lowered food intake (7). Another reason is that resting energy expenditure is increased by approximately 10% in ALS (48). This hypermetabolic state has been variously attributed to uncontrolled fasciculations, increased work of respiratory muscles, or mitochondrial defects (7). In addition, the previous study show a clear hypothalamic atrophy of about 22% was found in ALS patients as well as presymptomatic gene carriers compared to controls and a smaller hypothalamic volume is linked to an earlier disease onset and low BMI (49). Also, the intrinsic defects in the hypothalamic MCH neurons in ALS patients and mice resulted in reduced food intake in both patients and mice (50). These suggest that the hypothesis of a hypothalamic dysfunction may be involved in metabolic imbalance in patients with ALS.
Childhood-onset craniopharyngioma: latest insights into pathology, diagnostics, treatment, and follow-up
Published in Expert Review of Neurotherapeutics, 2018
Agnieszka Bogusz, Hermann L. Müller
Young age at CP diagnosis and presurgical functional impairments are risk factors for impaired quality of survival and reduced psychosocial and neurocognitive functionality during long-term follow-up after CP. Furthermore, prognoses in childhood-onset CP patients have been reported to be associated with tumor characteristics such as high tumor volume, third ventricle, and hypothalamic involvement at time of diagnosis [12,43]. GTR has also been implicated with worse prognosis when compared with a limited surgical approach followed by radio-oncological treatment and for multiple surgical interventions of tumor relapses. QoL is adversely affected by ophthalmological, neurological, and neuroendocrine long-term side effects [17,50,66,68,82,83]. Hypothalamic dysfunction has been reported as the most critical risk factor for severe impairments concerning physical function, social functionality, and body image perception [68,83,87].