Diencephalic cachexia (DC ) is a metabolic disorder characterized by a decrease in body weight. DC usually occurs in the presence of glioma brain tumors extended into the optic pathway. These tumors are very aggressive and have poor prognosis.Objective: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .Material and Methods. The study included 264 patients aged 0 to 18 years with an initial diagnosis of OPG s registered in the N.N. Burdenko National Medical Research Center of neurosurgery from 01/01/2003 to 12/31/2015. Patients were divided into two groups: without DC (204 people) and with DC (60 children). Results: neurofibromatosis type I (NFI) was much more common in children without DC , and pilomyxoid histology was much more prevalent in children with DC . Five-year overall survival (OS ) and event-free survival EFS were significantly lower in children with DC than in children without DC (82 ± 5 % and 96 ± 1 %, respectively versus 37 ± 7 % and 62 ± 3 %, respectively). It was found that in the DC group, the OS and EFS rates were significantly lower in girls, in children without NFI, in children without histological verification and in children with pilocytic astrocytomas. It was also found that in the DC group, OS rates were significantly lower in children under 1 year, and EFS rates were significantly lower in children aged more than 12 months. The number of patients without events were significantly higher in the group without DC (p=0.001). The number of deaths in the postoperative period was significantly higher in children with DC (p<0.001). Diabetes insipidus and hyponatremia were significantly more common in patients with diencephalic cachexia, and vision improvement after treatment was significantly more likely to occur in patients without DC .Conclusion. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation.

The potential local and systemic complications of superselective intra-arterial chemotherapy used to treat refractory and advanced forms of retinoblastoma are considered in conjunction with the currently available measures of their prevention. In addition, possible causes of unsuccessful outcomes of this treatment modality are discussed.

Gliomas of the anterior optic tract (AOT) (optic nerves, chiasms, and visual tracts) are benign slowly growing tumors usually detectable in childhood. These are a rather heterogenic group of tumors. The pattern and course of the disease are frequently affected by the presence of type 1 neurofibromatosis (NF-1) in the patient. The ophthalmological symptoms of AOT lesion in the presence of NF-1 were analyzed in 80 patients aged 1 to 24 years (median 5 years). According to the ophthalmological symptoms and the pattern and extent of lesions to AOT structures, the authors identified 4 groups by the data of neurovisualization techniques (magnetic resonance imaging, computed tomography) and revealed that the ophthalmological pattern reflected the site and degree of lesion to AOT structures. At the same time it is shown that there is a group of silent tumors attending without visual disorders. These tumors have the similar neurovisualization pattern, such as a moderate thickening of AOT structures and they occur in 13.8% of cases, as shown by the authors.

Fifty-nine patients with anterior optic tract (AOT) lesion concurrent with type neurofibromatosis (NF-1) were followed up for 1 to 36 years (median 5 years). Based on the study, the authors identified several grades of the disease: - stabilization without intervention; - stabilization after treatment; - insignificant negative changes as a slight tumor growth and a further stabilization of the process; - occasionally negative changes irrespective of treatment. The behavior of a tumor is unpredictable. The onset of progression in infancy is a poor predictor. Ophthalmological symptoms are not the criterion that can be used to make a prognosis. The prognosis is largely determined by neurovisualization techniques and primarily magnetic resonance imaging. A uniform slight thickening of AOT structures irrespective of the extent of the process along the AOT should be referred to as a good prognosis. There was no spontaneous tumor regression in any case. By taking into account the beneficial effect of radiotherapy, the authors consider it expedient to use it as monotherapy and in combination with surgery. Bypass surgery on the spinal fluid system frequently fails to entirely solve the problem intracranial hypertension.

Six clinical observations of a rare condition, delayed radiation-induced optic neuropathy (RON) are presented. RON developed in patients with brain tumors treated by radiotherapy and radiosurgery; in the majority of patients the condition developed during exposure to therapeutic doses; its incidence was 0.5% of the total number of patients treated by radiotherapy during this period. Asymmetrical chiasmal syndrome developed rapidly in all the patients. Clinical diagnosis of RON was confirmed by magnetic resonance tomography in all cases, and in one case by morphological analysis. Conservative therapy including hyperbaric oxygenation just stabilized the visual function. Remote period of observation was 42 months.

The paper analyzes tomographic scanning images of 32 patients with craniopharyngiomas in the late postoperative period. Computed tomographic data allowed the patients to be divided into 3 groups: 1) 10 patients without signs of tumor recurrence or hydrocephalus; 2) 14 patients with recurrent cystic craniopharyngiomas; 3) 8 patients with severe hydrocephalus. Single photon emission computed tomography (SPECT) of the brain indicated regional disturbances of brain tissue blood supply in the frontobasal or frontobasotemporal regions of the right hemisphere (the area of an surgical access and of the removed tumor) and revealed them in the distal areas (frontobasotemporal regions of the left hemisphere, parietal and occipital cortices of the cerebral hemisphere or cerebellar tissue). The compensatory reserves of cerebral circulation were assessed by the foci of relative physiological hyperemia of brain tissue (the cerebellum and the medial portions of the occipital regions of the brain). The findings provide evidence for that the vascular factor is involved in the late postoperative pathological picture in patients with craniopharyngiomas.

Craniocerebral injuries are known to involve the visual tract in 2-5% of cases. Fifty-nine patients aged 5 to 68 with visual tract involvement in craniocerebral injury were examined in N. N. Burdenko Institute of Neurosurgery of the Russian Academy of Medical Sciences. Unilateral optic nerve involvement was found in 48 patients, involvement of the chiasm and/or both optic nerves in 11. Involvement of a single optic nerve was associated with vision acuity reduction, 30 patients becoming blind or virtually blind, and with various defects of the visual field. Traumatic injury to the chiasm manifested as a rule by the asymmetric chiasmal syndrome. Follow-up of the patients in the acute period of craniocerebral injury showed that paling of the optic disc manifested in various periods after the moment of the injury, from 3-4 days to 1 month, this depending on the localization of the injury and its distance from the posterior pole of the eye. Besides visual disturbances and ophthalmoscopic changes, oculomotor disorders were found which were caused by traumatic impairment of the oculomotor nerves (in the orbit or skull) and muscles. Transcutaneous electrostimulation of the injured optic nerves was sufficiently effective, its efficacy directly depending on the period elapsed since the injury, excepting blind or virtually blind patients.