[Chordoid gliomas of the third ventricle]. / Opyt lecheniya khordoidnykh gliom III zheludochka.

BACKGROUND: Chordoid glioma is a rare slow-growing tumor of the central nervous system. Available world experience includes no more than 200 cases (lesion of the third ventricle in absolute majority of cases). Recognition and treatment of chordoid glioma are currently difficult problems due to small incidence of this disease. OBJECTIVE: To describe clinical manifestations and surgical treatment of chordoid glioma of the third ventricle considering literature data and own experience. MATERIAL AND METHODS: There were 12 patients (6 men and 6 women) with chordoid glioma between 2004 and 2023 (10 patients with lesion of the third ventricle, 1 - lateral ventricle, 1 - pineal region). Only patients with tumors of the third ventricle were analyzed. RESULTS: Total and subtotal resection was performed in 1 and 3 cases, respectively. Five patients underwent partial resection, 1 patient underwent biopsy. The follow-up data were available in 7 out of 10 patients (mean 25 months). Radiotherapy was performed in 4 patients (continued tumor growth in 2 cases). One patient died. CONCLUSION: Chordoid glioma is a benign tumor predominantly localized in the third ventricle. Preoperative MRI and CT in some cases make it possible to suspect chordoid glioma and differentiate this tumor from craniopharyngioma, meningioma and pituitary adenoma by such signs as isointense signal in T1WI, hyper- or isointense signal in T2WI, homogeneous contrast enhancement and edema of basal ganglia in T2 FLAIR images. The only effective treatment for chordoid glioma is surgery. Total resection is often impossible or extremely dangerous due to location of tumor, large size and invasion of the third ventricle. Postoperative mental disorders and diabetes insipidus, including severe hypernatremia, are common that requires mandatory monitoring of water and electrolyte balance.

[Predictors of postoperative remission of acromegaly. (Experience of the Burdenko Neurosurgical Center)]. / Prediktory posleoperatsionnoi remissii akromegalii. (Opyt FGAU «NMITs neirokhirurgii im. akad. N.N. Burdenko¼).

BACKGROUND: Treatment of acromegaly is still an unresolved problem. Overall postoperative remission rate ranges from 34 to 85%. These values are better for microadenomas (75-90%) and worse for macroadenomas (45-70%). Identification of predictors of acromegaly remission after surgical treatment is an urgent objective to improve the quality of medical care for these patients. OBJECTIVE: To analyze postoperative freedom from acromegaly and predictors of remission. MATERIAL AND METHODS: A retrospective single-center study included 227 patients with acromegaly who underwent resection of pituitary adenoma between August 2018 and August 2021. RESULTS: Remission (normalization of serum IGF-1) was achieved in 65 (55%) patients. Growth hormone and IGF-1 index decreased after surgery in all patients. Mean preoperative serum growth hormone was 12.45 [6.88, 29.85] ng/ml, early postoperative concentration - 1.54 [0.80, 3.38] ng/ml, in delayed period - 1.15 [0.57, 3.80] ng/ml. Mean IGF-1 index was 2.18 [1.69, 2.71], 1.47 [0.99, 1.90] and 0.99 [0.74, 1.43], respectively. CONCLUSION: Significant predictors of acromegaly remission after neurosurgical treatment were age, preoperative level of growth hormone, tumor size and location, growth hormone and IGF-1 index in early postoperative period and residual tumor after surgery. Multivariate analysis revealed a significant association of acromegaly remission with small tumor size, low postoperative level of growth hormone and no residual tumor within 3-6 month after surgery.

[Reset osmostat syndrome - when hyponatremia become «a normal¼: diagnostics, case report].

Reset osmostat syndrome (ROS) is characterized by a change of normal plasma osmolality threshold (decrease or increase), which leads to chronic dysnatremia (hypo- or hypernatremia). We have described a clinical case of ROS and chronic hyponatremia in a patient with chordoid glioma of the III ventricle. It is known that the patient had previously been diagnosed with hyponatremia (131-134 mmol/l). She has not hypothyroidism and hypocorticism. There is normal filtration function of the kidneys was (CKD-EPI 91.7 ml/mi/1,73m2). Urine osmolality and sodium level were studied to exclude of concentration kidney function disorder. During first three days after removal of the tumor of the third ventricle (chordoid glioma, WHO Grade II), the sodium level decreased to 119 mmol/l. Repeated infusions of 200-300 ml hypertonic 3% sodium chloride solution, gluco- and mineralocorticoid therapy was ineffective, increasing plasma sodium levels by 2-3 mmol/l with the return to the initial level during 6-8 hours. Hypopituitary disorders did not develop after surgery. With further observation, the sodium level remained within 126-129 mmol/l for 6 months after surgery. The water load test make exclude the classic syndrome of inappropriate secretion of antidiuretic hormone, and confirmed the diagnosis of RSO. Because of absence of clinical symptoms associated with hyponatremia, no medical correction was required, patient was recommended to clinical follow-up.

[Permanent central diabetes insipidus after traumatic brain injury. Case report and literature review]. / Razvitie postoyannogo tsentral'nogo nesakharnogo diabeta posle travmaticheskogo povrezhdeniya golovnogo mozga. Klinicheskii sluchai i obzor literatury.

The authors report permanent central diabetes insipidus (CDI) in a patient after severe traumatic brain injury (TBI) in traffic accident. A 16-year-old boy entered to a medical facility in coma (GCS score 6) with the following diagnosis: acute TBI, severe cerebral contusion, subarachnoid hemorrhage, depressed comminuted cranial vault fracture, basilar skull fracture, visceral contusion. CDI was diagnosed in 3 days after injury considering polyuria and hypernatremia (155 mmol/l). Desmopressin therapy was initiated through a feeding tube. Thirst appeared when a patient came out of the coma after 21 days despite ongoing desmopressin therapy. Considering persistent thirst and polyuria, we continued desmopressin therapy in a spray form. Under this therapy, polyuria reduced to 3-3.5 liters per a day. Symptoms of CDI persisted in long-term period (2 years after TBI) while function of adenohypophysis was intact. This case demonstrates a rare development of permanent diabetes insipidus after TBI. CDI manifested only as polyuria and hypernatremia in coma. Thirst joined after recovery of consciousness. Probable causes of CDI were damage to neurohypophysis and partially injury of pituitary stalk because of extended basilar skull fracture and/or irreversible secondary lesion of hypothalamus following diffuse axonal damage after TBI.

[Adipsic diabetes insipidus after transsphenoidal surgery for suprasellar intraventricular].

Presented case demonstrates a rare diencephalic pathology - adipsic diabetes insipidus (ADI) with severe hypernatremia in a 58-year-old woman after ttranssphenoidal removal of stalk intraventricular craniopharyngioma. ADI was diagnosed because of hypernatremia (150-155 mmol/L), polyuria (up to 4 liters per day) and absence of thirst. Normalization of water-electrolyte balance occurred on the background of desmopressin therapy and sufficient hydration in postoperative period. After release from the hospital, the patient independently stopped desmopressin therapy and did not consume an adequate amount of fluid of the background of polyuria. This led to severe hypernatremia (155-160 mmol/L) and rough mental disorders.Patients with ADI need closely monitoring of medical condition and water-electrolyte parameters, appointment of fixed doses of desmopressin and adequate hydration.

[Modern aspects of surgery for cushing's disease]. / Sovremennye aspekty khirurgicheskogo lecheniya bolezni Itsenko­Kushinga.

Cushing's disease is a severe neuroendocrine disorder caused by chronic hypersecretion of ACTH by pituitary adenoma (corticotropinoma). Surgical resection is a gold standard for this disease resulting stable remission in about 65-95% of cases. Despite benign nature of corticotropinoma, recurrence rate is still higher (25-35%) even in specialized neurosurgical centers. Modifications in surgical strategy can prolong recurrence-free period.

[Pituitary microadenomas - current diagnostic and treatment methods]. / Mikroadenomy gipofiza: sovremennoe sostoyanie metodov diagnostiki i lecheniya.

According to previously accepted criteria, pituitary microadenoma is characterized by a diameter of less than 10 mm. Improvement and widespread use of MRI are accompanied by increased incidence of diagnosis of these neoplasms. Pituitary microadenomas is an extremely heterogeneous group of tumors with different biological behavior, endocrine secretion and clinical symptoms despite the common MR characteristics. Treatment is mainly determined by endocrine secretion. Endocrine-active microadenoma requires medication (in case of microprolactinoma) and surgical treatment (in case of microsomatotropinoma and ACTH-releasing tumor). Follow-up is advisable for endocrine-inactive microadenoma (microincidentaloma). Modern data on the incidence, clinical and endocrine features, diagnosis and treatment of various pituitary microadenomas are discussed in the article.

[Mental activity of the children during the early stage of rehabilitation after acute severe brain damage]. / Psikhicheskaia aktivnost' u detei na rannem étape reabilitatsii posle ostrykh tiazhelykh povrezhdenii golovnogo mozga.

INTRODUCTION: Children who have suffered brain damage form a large group of patients in need of rehabilitation. For rehabilitation, patients require not only surgical, neurosurgical, traumatological, pharmacological and other medical support, but also the creation of special conditions for psychiatric care, psychological and pedagogical correction of their psychophysical potential. Neurological, motor and mental consequences of severe injuries of the nervous system in children lead to a high degree of disability with a subsequent restriction of their life, where social maladaptation and a violation of the quality of life are the primary problems. An integrated approach with differentiated qualified help will increase the effectiveness of rehabilitation programs and help the children adapt when they return to their normal environment. AIM: To identify the characteristics of mental activity during the recovery of the level of consciousness in children after acute severe brain damage at an early stage of rehabilitation. MATERIAL AND METHODS: We examined 210 children under the age of 18 years with severe brain damage (traumatic brain injury, hypoxia, hydrocephalus) who were admitted for treatment and rehabilitation. Clinical-psychopathological, pedagogical methods were used for examination as well as diagnostic scales, questionnaires. RESULTS: The main differentiating signs were divided into three groups, depending on the mental activity of the child, the level of consciousness: Group 1 - 37 (18%) patients with mental activity with physical, cognitive and social abilities with the minimal '+' consciousness (a-/hyperkinetic mutism with emotional reactions, understanding of speech); 2-nd group - 67 (32%) patients with dominant manifestations of physical and cognitive abilities with the minimal '-' consciousness (a-/hyperkinetic mutism without emotional manifestations and understanding of speech); 3rd group - 106 (50%) children with a weak manifestation of mental activity (physical abilities) in a vegetative status/exit from a vegetative status. CONCLUSION: Three variants of mental activity in children after acute severe brain injuries were distinguished - from minimal involuntary reactions or their absence during the vegetative status/exit from the vegetative status to arbitrary actions according to the instructions of an adult with a minimum '+' consciousness. Understanding the dynamics of the recovery of children's mental activity after neurotrauma may make it possible to have a differentiated approach to psychiatric, psychological and pedagogical rehabilitation in order to correct the recovery of mental functions in pediatric patients, and to prevent the developmental disorders as the child grows.

[The psychological and psychiatric care for the children after severe spinal cord injury in the framework of the combined early rehabilitative treatment]. / Psikhologicheskaia i psikhiatricheskaia pomoshch' detiam posle tiazheloi pozvonochno-spinnomozgovoi travmy v ramkakh kompleksnogo podkhoda k rannei reabilitatsii.

BACKGROUND: The number of children who have received severe spinal cord injury (SCI) is steadily increasing during the recent years. The clinical picture of the post-injury condition is characterized by psychological and psychiatric disorders of varying severity. The literature publications concern emotions experienced after SCI by the adult patients whereas the data on the emotional disorders in the children and the approaches to their medical treatment are virtually absent. Nor is enough information about the status of the children in the early period after the spinal trauma treated jointly by a psychiatrist and a psychologist. AIM: The objective of the present study was the evaluation of the emotions and motivations in the children following the severe spinal trauma in the course of the early rehabilitation period as well as the development of an algorithm for the interaction of a psychiatrist and a psychologist in their joint efforts to manage the emotional and motivational disorders. MATERIAL AND METHODS: The study included 35 children at the age from 8 to 18 years presenting with the severe spinal trauma who had been admitted for the treatment and rehabilitation based at the Research Institute of Emergency Pediatric Surgery and Traumatology, Moscow Health Department, during the period from 2016 to 2018. All the patients were examined by a psychiatrist and a psychologist in dynamics, viz. before the beginning of the rehabilitation course as well as within the 7-10th and 21-25th days after its initiation. The relevant diagnostic scales and questionnaires were used to detect depression and assess the severity of individual symptoms. RESULTS: Three groups of children who had experienced the severe spinal trauma were distinguished depending on the character of emotional disorders and their severity. Group 1 was comprised of the children with depression (n=6) including 2 boys and 4 girls at the age from 14 to17 years (17.2%). Group 2 consisted of the children with emotional disorders, such as high anxiety, impaired motivation with sub-depressive prerequisites (n=11) including 4 girls and 7 boys at the age from 12 to16 years (31.4%). Group 3 was composed of the children free from depression or depressive manifestations (n=18) including 15 boys, 3 girls at the age from 8 to17 years (51.4%). The algorithm for the combined rehabilitative treatment of the children during the early period after the severe spinal trauma with the participation of the psychiatrist and the psychologist has been developed. It was shown that the patients with a reduced intensity of emotional expression combined with a moderate or low level of anxiety and sufficient motivation needed the supervision by the psychologist alone. The children with a reduced intensity of emotional expression combined with a moderate or high level of anxiety and impaired motivation had to be supervised and treated by both the psychiatrist and the psychologist. The children with pronounced depression, high anxiety and low motivation required the supervision and treatment by a psychiatrist with simultaneous pharmacological correction of their condition. CONCLUSION: The results of this study give evidence that 48.6% of the children after severe spinal trauma suffer from the emotional and motivational disorders requiring specialized psychiatric care including the differentiated psychological and psychiatric treatment during the early rehabilitation period with the use of the algorithm for the combined treatment based on the joint efforts of the psychiatrist and the psychologist supplemented by the pharmacological correction.

[A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma]. / Redkii sluchai razvitiia diéntsefal'noi kakheksii u vzrosloi zhenshchiny s kraniofaringiomoi.

Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

[The rate of hyponatremia in neurosurgical patients (comparison between the data from the Burdenko Neurosurgical Instutite and the literature) and recommendations for the diagnosis and treatment].

UNLABELLED: Hyponatremia is a relatively frequent and serious complication in patients with various neurosurgical pathologies. OBJECTIVE: This study is aimed at assessing the incidence of hyponatremia in neurosurgical patients depending on the pathology. MATERIAL AND METHODS: This paper presents a retrospective analysis 39 479 cases of patients operated on at the Burdenko Neurosurgical Institute from 2008 to 2014. RESULTS: A total of 785 hyponatremic patients with Na level lower than 130 mmol/l (2% of all operated patients) were identified. Mortality in patients with hyponatremia was 14.3%, which is tenfold higher compared to the rest of population of patients without hyponatremia who were operated on during the same period. In adults, hyponatremia most frequently occurred after resection of craniopharyngiomas (11%) and as a result of acute cerebrovascular accident (22%). In children, it occurred after resection of craniopharyngiomas (10%), astrocytomas (7%), ependymomas (24%), and germ cell tumors (10.5%). CONCLUSION: This study, which was mainly statistical one, was not aimed at detailed investigation of hyponatremia in different groups of neurosurgical patients. We only tried to draw the attention of various experts to those categories of patients, where focused and in-depth developments are more than important. Obviously, already gained international experience should be taken into account for this PURPOSE: Therefore, this article presents the literature data on the etiology and pathogenesis of hyponatremia. We describe the details of the various classifications of hyponatremia, its clinical symptoms, diagnosis, and treatments, primarily based on the recommendations of the last European consensus of various specialists (2014).

[Clinical and morphological characteristics, diagnostic criteria, and outcomes of surgical treatment of TSH-secreting pituitary adenomas]. / Kliniko-morfologicheskie kharakteristiki, diagnosticheskie kriterii i rezul'taty khirurgicheskogo lecheniya TTG-sekretiruyushchikh adenom gipofiza.

Thyrotropinomas (TSH-secreting tumors) are a rare type of pituitary adenomas, which account for about 0.5-2.0% of all pituitary tumors. The criterion of thyrotropinoma is visualization of the tumor in the presence of a normal or elevated level of the thyroid-stimulating hormone (TSH) in the blood and elevated concentrations of free T4 (fT4) and free T3 (fT3). OBJECTIVE: To study the clinical, diagnostic, and morphological characteristics and treatment outcomes of TSH-secreting pituitary tumors. MATERIAL AND METHODS: The study included 21 patients aged from 15 to 67 years with pituitary adenoma and a normal or elevated blood TSH level combined with elevated fT4 and fT3 levels who were operated on at the Neurosurgical Institute in the period between 2002 and 2015. Before surgery, in the early postoperative period, and 6 months after surgery, the patients were tested for levels of TSH, fT4, fT3, prolactin, cortisol, the luteinizing hormone (LH), the follicle-stimulating hormone (FSH), estradiol/testosterone, and the insulin-like growth factor (IGF-1). The thyroid status was evaluated using the following reference values: TSH, 0.4-4.0 mIU/L; fT4, 11.5-22.7 pmol/L; fT3, 3.5-6.5 pmol/L. An immunohistochemical study of material was performed with antibodies to TSH, PRL, GH, ACTH, LH, FSH, and Ki-67 (MiB-1 clone); in 13 cases, we used tests with antibodies to somatostatin receptors type 2 and 5 and to D2 subtype dopamine receptors. RESULTS: Thyrotropinomas were detected in patients aged from 15 to 67 years (median, 39 years), with an equal rate in males (48%) and females (52%). Before admission to the Neurosurgical Institute, 11 (52%) patients were erroneously diagnosed with primary hyperthyroidism; based on the diagnosis, 7 of these patients underwent surgery on the thyroid gland and/or received thyrostatics (4 cases). Hyperthyroidism symptoms were observed in 16 (76%) patients. The blood level of TSH was 2.47-38.4 mIU/L (median, 6.56); fT4, 22.8-54.8 nmol/L (median, 36); fT3, 4.24-12.9 pmol/L (median, 9.66). Tumors had the endosellar localization in 4 (19%) cases and the endo-extrasellar localization in 17 (91%) cases. Total tumor resection was performed in 7 (33%) patients. All these tumors had the endosellar and endo-suprasellar localization. No total resection was performed in patients with infiltrative growth of adenoma (invading the skull base structures). An immunohistochemical study of tumor resection specimens detected only TSH expression in 3 (14%) cases; 18 (86%) tumors were plurihormonal and secreted TSH and GH and/or PRL. Of 13 tumors, expression of the type 2 dopamine receptor was detected in 9 (69%) cases; expression of somatostatin receptors type 5 and type 2 was found in 6 (46%) and 2 (15%) cases, respectively. CONCLUSION: The criterion for total tumor resection was a postoperative decrease in the TSH level to 0.1 mIU/L or less. Total resection was performed in 33% of patients with tumors of only the endosellar and endo-suprasellar localization. In most cases, tumors were plurihormonal and secreted TSH and GH and/or PRL.

[Thyrotropin-producing adenomas and thyrotropic hyperplasia (clinical case reports and the review of the literature)]. / Tireotropinomy i tireotrofnaia giperplaziia (opisanie klinicheskikh sluchaev i obzor literatury).

An increased blood level of the thyroid stimulating hormone (TSH) is usually associated with primary hypothyroidism (PHT) but can also be observed in such rare cases as TSH-secreting pituitary tumor. The article describes four clinical cases of elevated TSH blood levels: 1) TSH-secreting pituitary adenoma with hyperthyroidism; 2) TSH-secreting adenoma with hypothyroidism; 3) hormonally inactive pituitary adenoma combined with primary hypothyroidism; 4) reversible thyrotropic hyperplasia. These clinical situations substantiate the importance of considering different diagnoses in a patient with a pituitary gland tumor associated with an increased TSH blood level.