[The use of low-field MRI for visualization of epileptogenic brain lesions in children]. / Primenenie nizkoinduktivnoi magnitno-rezonansnoi tomografii dlia vizualizatsii strukturnykh épileptogennykh porazhenii golovnogo mozga u detei.

AIM: To analyze MR-images of patients with focal epilepsy using a method close to the protocol of epileptic scanning on a MRI-device with the magnetic field tension of 0.4 Tesla. MATERIAL AND METHODS: MRI data of 50 children who underwent examination due to difficult-to-treat or drug-resistant forms of focal epilepsy were analyzed. MRI study was conducted using open-ended device of static magnetic field HITACHI 'APERTO' with the magnetic field tension of 0.4 Tesla. The thickness of the slices and the scan step was performed at 3.0 and 3.5 mm with the use of special positioning of slices in the coronal and axial projections, T2, T1, STIR, FLAIR weighted images (WI) perpendicular and parallel to the long axis of the hippocampus. RESULTS: Potentially epileptogenic structural changes were identified in 37 patients. Abnormalities of brain development of different severity were identified in 16 patients. In 21 cases, the changes were due to the consequences of cerebral vascular catastrophes, neuroinfections, brain traumas affecting the cortical plate. A method close to the epileptic scanning protocol allowed the identification of signs of acute cerebral catastrophes in two patients. In 8 patients, potentially epileptogenic changes after false-negative descriptions of the results of previous MRI studies were newly identified. The most difficult for the diagnosis was the visualization of small structural changes in mediobasal regions of the temporal lobes. Confirmation of this supposition according to the recommended thorough investigation was obtained only in 5 out of 12 patients with suspected small-bore pathological changes in mediobasal temporal regions. CONCLUSION: Low-tension technique approximated to the epileptic scanning protocol does not allow the reliable diagnosis of small and similar in signal characteristics changes in the visualization of mediobasal temporal lobes regions. It was not possible to reliably differentiate some cortical-subcortical neoplastic formations from various forms of focal cortical dysplasia.

[MRI-characteristics of epileptogenic supratentorial brain tumors in children].

OBJECTIVE: To analyze MR-images in patients with symptomatic epilepsy associated with the brain tumor. MATERIAL AND METHODS: MRI results of 52 patients with symptomatic epilepsy operated for tumors of supratentorial localization were analyzed. The most epileptogenic tumors with atypical MRI signs and subtle clinical presentation were identified. All patients with tumors were operated using different methods of surgical intervention. RESULTS: Dysembryoplastic neuroepithelial tumors (DNET), diffuse astrocytomas (DA) and gangliogliomas (GG) were the most frequent epileptogenic tumors. In all the cases of DNET and in 4 patients with GG, epileptic seizures were the first, and in 4 of 5 cases of DIO were the only clinical sign of tumor presence. In DNET, DA and GG, there was an iso- or hypointensive signal on T1 WI and a signal varying in intensity from moderate to hyperintense in T2 and FLAIR WI, while in cases with DNET and GG, no mass effect and perifocal edema was practically seen. The so-called «spume-like¼ (multicystic) structure was most clearly observed in FLAIR WI. No significant changes in the dimensions of the DNET and GG were identified. The combination of DNET with focal cortical dysplasia was noted in one case. In DA, it was difficult to distinguish the perifocal edema from tumorous tissue and normal brain tissues, and the growth potential of malformation was slow. CONCLUSION: Epileptogenic tumors can imitate the x-ray characteristics of each other, and mimicry to gangliogliomas, oligodendrogliomas and astrocytomas Gr I, II, and others. They are the most frequent causes of symptomatic focal epilepsy. The presence of these malformations is necessary to exclude first of all in all cases of pharmacoresistant epilepsy.

[Glutaric aciduria type 1: clinical presentations, diagnostics and treatment.].

Glutaric aciduria type I is a rare autosomic recessive neurometabolic disease, which develops in the first year of life and is characterized by progressive extrapyramidal disorders as a result of the basal ganglia damage. We describe first cases of this disease in Russian population. The clinical observations are compared to the literature data. The disease usually develops after infections and features by seizures, vomiting, metabolic acidosis and deprivation of consciousness up to coma. These crises lead to the development of necroses of the basal ganglia that results in dystonias, dyskinesias and choreoatethosis. The secondary complications of the disease are difficulties with feeding, speech delay, chronic aspiration syndrome and severe delay of movement development. Diagnostics of the disease is based on urine and blood tests using methods of tandem mass spectrometry and gas chromatography. Treatment is based on dietary lysine or protein restriction and supplementation with carnitine. The data on the treatment of this disease are presented.