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ABSTRACT: OBJECTIVE: This study is aimed at revealing the relationship between local interictal epileptic activity, cytoarchitectural disturbances and magnetic resonance imaging (MRI) findings. MATERIAL AND METHODS: We prospectively investigated a series of 25 patients with symptomatic epilepsy due to isolated forms of focal cortical dysplasia (FCD) or hippocampal sclerosis and low-grade tumours associated with FCD, all of whom underwent tailored surgical procedures under intraoperative electrocorticography; we conducted neuropathological examinations of 92 biopsies taken from different places. We examined the relationship between dysplastic changes in the cortex and the absence or presence of seizure patterns (SPs), including regular spikes/sharp waves, recruiting discharges, paroxysmal fast activity and rhythmic delta-theta activity. Comparisons with MRI findings were also performed. RESULTS: Complete removal of the SPs zone was associated with better results of surgical treatment. Areas with isolated architectural abnormalities were associated with SPs significantly more often than those where the cortex contains immature or giant neurons; these areas were associated with SPs more than areas containing dysmorphic neurons. The extent of MRI signs appearance in the neocortex correlated neither with the presence of SPs nor with the types of histological changes. CONCLUSIONS: We suppose an inverse relationship between the morphological changes in neurons and their ability to generate epileptic activity. Electrocorticography may be used for the identification of the MRI-negative epileptogenic lesions.
Acta Neurologica Scandinavica 12/2012; · 2.47 Impact Factor
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ABSTRACT: A comprehensive study with the assessment of reactive responses to motor tasks was performed in nine patients with a tumor
localized in the frontal divisions of the brain using two methodological approaches: functional magnetic resonance imaging
(fMRI) and EEG. The data obtained were compared to the results of a similar study on 12 healthy subjects. It was established
that cerebral pathology was associated with disorders of functional specialization and an increase in the diffuse component
of reactivity. The fMRI responses were characterized by greater intactness compared to the EEG parameters of reactive changes.
These features are especially marked when an afferent stimulus is sent to the damaged hemisphere. The characteristics of the
involvement of individual EEG bands in the formation of motor responses and changes in the fMRI response topography are determined
by the degree of cerebral dysfunction reflected by the pattern of baseline EEG reorganization and the severity of the motor
defect. The predominant increase in the coherence of slow rhythms in the damaged hemisphere irrespective of the target of
the afferent stimulus in patients with severe cerebral dysfunction reflects the dominant formation of a pathological focus
and is indicative of a greater, compared to healthy subjects, involvement of deep brain structures in the reactive process,
which is confirmed by the fMRI data.
Key wordsfMRI-EEG-tumor-motor tasks
Human Physiology 04/2012; 36(5):550-559.
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ABSTRACT: The functional magnetic resonance imaging (fMRI) and EEG responses to identical functional (visual and motor) loads have been
compared in ten healthy subjects with the use of individual structural MRIs of the brain. It has been established that an
increase in the coherence of the EEG α waves corresponds mostly to the zone of the fMRI response (as a +BOLD reaction). Reactive rearrangements, according to the data of fMRI and, particularly, EEG studies, are characterized by pronounced
interindividual variation, which increases along with the functional test complexity. The fMRI responses have shown a greater
locality and closer dependence on the modality of presented stimuli than EEG rearrangements, which underline the systemic
character of brain response to functional loads. The −BOLD response accompanying the local +BOLD effect is more generalized, without distinct topographic referencing to the functional load modality; it conforms most of
all to the decrease in the EEG’s coherence.
Human Physiology 04/2012; 35(3):274-284.
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Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 01/2012; 112(1):90-3. · 0.12 Impact Factor
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ABSTRACT: To evaluate clinical perspectives of proton multi-voxel magnetic-resonance spectroscopy (PMMRS) for monitoring the severity of multiple sclerosis (MS) and neurodegenerative process, we studied the changes of the NAA/Cr ratio in the brain tissue of patients with MS and calculated the correlations between this parameter and the clinical state of patients. Based on these results, we studied the potential neuroprotective effect of Β-interferon 1a (avonex) for intramuscular injection in patients with remitting MS. Twenty-six patients with remitting MS were enrolled in the study. The study of anamnesis, neurological examination using EDSS, neuropsychological testing and dynamic MRI using PMMRS were performed. The NAA/Cr ratio was decreased in patients compared to controls. An analysis of the NAA/Cr ratio after one year revealed the significant decrease of this parameter. The negative correlation between the NAA/Cr ratio in the brain tissue and the level of neurological and cognitive deficits was noted. The analysis of existing treatment of MS with Β-interferon 1a for intramuscular injection revealed the neuroprotective stabilizing effect during one year of treatment that is probably associated with the effect of this drug on the neurodegenerative process in MS.
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 01/2012; 112(9 Vypusk 2 Rasseyannyi skleroz):129-135. · 0.12 Impact Factor
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ABSTRACT: Glomus jugulare tumors (GJT) (AKA paraganglioma or chemodecroma) make 0.6% of head and neck tumors and 80% of jugular foramen lesions. Recently different types of stereotactic radiotherapy are actively used in their treatment. The given paper summarizes our first experience of using CyberKnife system in management of patients with paragangliomas. Since April 2009 till September 2011 34 patients with GJT were treated in department for radiation therapy of Burdenko Neurosurgical Institute using CyberKnife system. Mean age was 50.5 years (range 26-75 years). Mean volume of treated lesions was 14.6 +/- 10.96 cm3 (range--1.04-37.4 cm3). Stereotactic radiosurgery was performed in 4 patients. Mean prescribed dose was 17 +/- 3.1 Gy (13.7-22 Gy). 30 patients received hypofractionation treatment (3-7 fractions with total dose 18-35 Gy). Mean follow-up period in our series was 8 months (1-20 months). Control of tumor growth reached 100% because none of the patients needed any additional therapy (microsurgical resection or repeated radiotherapy). Persistent trigeminal neuropathy was observed in 1 case (5%). There were no constant impairment of VII, IX, X, XII and XIII cranial nerves in the series. Stabilization of auditory function was present in 9 (82%) of 11 patients with initially preserved hearing. Preservation of effective hearing was observed in 75% of patients. Stereotactic radiosurgery and hypofractionation using CyberKnife system are effective modalities in management of such complex neurosurgical disease as GJT. Neurological deterioration after radiation treatment is extremely rare and in most cases is transient.
Voprosy neĭrokhirurgii 01/2012; 76(1):30-6; discussion 36.
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ABSTRACT: Currently stereotactic radiosurgery has become the treatment of choice in small vestibular schwannomas. This paper discusses our first experience of application of CyberKnife system for stereotactic irradiation of these tumors. From April 2009 till June 2011 we treated 62 patients (35 female and 27 male) with vestibular schwannomas. Stereotactic radiosurgery using CyberKnife system was performed in 33 patients. Mean tumor volume was 2 +/- 1.4 cm3. Hypofractionated treatment was used in 30 cases (31 tumor). Mean tumor volume reached was 7 +/- 6.2 cm3 (range - 0.5-31.3 cm3). In a case of a patient with NF2 simultaneous irradiation of bilateral tumors was performed. Most frequently we applied 3 fractions 6 Gy each (17 observations of 31, or 55%) and 5 fractions with mean dose 5 Cy (10 cases, or 32%). Follow-up period varied from 1 to 26 months (mean 9 +/- 4.5 months). By the end of this study (June 30, 2011) surgical resection was required in the only case of 47-years old male patient with cystic schwannoma of left vestibular nerve 5 months after radiation treatment, due to progressive growth of the cyst and increased brainstem compression. Tumor growth control was established in 97.5% of cases. Stabilization of auditory function was achieved in 77.5% of series. Effective hearing was preserved in 75% of patients. Facial nerve palsy after stereotactic radiation treatment was observed in 2 cases (3%). Incidence of trigeminal nerve dysfunction was significantly higher: sensation disturbances occurred in 6 (10%) patients: 3% after radiosurgery and 16.7% after hypofractionation. We did not obtain significant correlations between risk of cranial nerve complications and dosimetric or demographic factors. However we observed stable tendency: larger initial volume of the tumor and presence of trigeminal nerve dysfunction before treatment were poor prognostic factors for trigeminal neuropathy. Stereotactic irradiation using CyberKnife system is effective and sufficiently safe technique for management of vestibular schwannoma. The paper demonstrates high rates of tumor stabilization, hearing preservation and minimal incidence of complications associated with trigeminal or facial nerve.
Voprosy neĭrokhirurgii 01/2012; 76(1):13-20; discussion 20.
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ABSTRACT: During recent decade development of frameless techniques of fixation enabled introduction of stereotactic radiation therapy in metastatic brain lesions and made possible irradiation of large foci involving or proximal to eloquent and critical brain areas. This paper focuses on comparative analysis of effectiveness of hypofractionation (HRST) and radiosurgery (SRS) using CyberKnife system in cerebral metastases. Since November 2009 till June 2011 54 patients with cerebral metastases were treated using CyberKnife system. Age of patients ranged between 25 and 77 years (mean 54 years). 16 patients received radiosurgical treatment (mean total dose was 22.5-35 Gy, number of fractions varied from 2 to 7, mean volume of irradiation was 22.69 cm3) and 8 patients were treated by HRST with RS of selected foci (mean total dose reached 23 and 30 Gy, mean volume of irradiation was 1.02 cm3 and 11.19 cm3, respectively). Indices of overall regression and stabilization of disease for HRST and SRS groups were 81% and 79%, respectively. With mean follow-up period of 12.3 (1-16.1) months median survival for SRS and HRST reached 6.38 (1-15.8) and 6.2 (0.2-16.1) months and median recurrence-free survival was 3.6 (1-13.6) and 5.5 (2-14.2) months, respectively. Obtained results confirmed biological advantages of fractionated stereotactic radiotherapy of large cerebral tumors in comparison with radiosurgery. Prospective studies with rigid criteria of inclusion are required to determine optimal dose/volume/fractionation interrelations in stereotactic radiation treatment of cerebral metastases.
Voprosy neĭrokhirurgii 01/2012; 76(1):37-45; discussion 45.
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Iu Iu Trunin,
A V Golanov,
A N Konovalov,
L V Shishkina,
G E Gorlachev,
S K Gorelyshev, I N Pronin,
E A Khukhlaeva,
N K Serova,
A G Korshunov,
A G Melikian,
M V Ryzhova,
Sh U Kadyrov,
V S Sorokin,
N A Mazerkina,
S A Mariashev,
S R Il'ialov,
V V Kostiuchenko
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ABSTRACT: Pilocytic astrocytoma (PA) is a low-grade glial tumor (WHO grade I) with predominant occurrence in pediatric patients. According to many authors, stereotactic radiosurgery (SRS) and radiotherapy (SRT) promote long-term remission or retardation of tumor progression in patients with in inoperable lesions after incomplete resection or recurrence. Therefore it is essential to determine the role of SRS and SRT in complex management of patients with deep-seated PA. Since April 2005 till May 2010 101 patient with intracranial PA was treated in department for radiation therapy of Burdenko Neurosurgical Institute. The series consisted of 70 pediatric patients (below 17 years inclusively) and 31 adults, of them--51 male and 50 female patients. Mean age was 15.1 years (9.8 years in children and 28.7 in adults). In 90 patients (89.2%) tumors were previously histologically verified (tumor resection in 83 cases and biopsy in 7). In 11 (10.8%) patients diagnosis of PA was based on clinical and neurovisualization data. In most cases SRT (66 (66.3%) patients) was preformed, the rest 35 (34.7%) patients were treated by SRS. Median follow-up from the onset of disease reached 52 months (2-228 months). Catamnestic data were available in 88 (87%) patients. By the end of catamnestic follow-up (December 2010) 87 (98.8%) patients treated by SRS and SRT were alive. Median follow-up from the start of radiation treatment was 22.7 months (6-60 months). Progression of tumor was observed in 20 patients (22.7%), in 18 of them due to cyst growth. 18 patients were reoperated. In 12 operated patients histological examination and its comparative analysis were performed. We found that alterations in the tumor tissue, accompanied by regression of solid component and progression of cystic portion, represent reactive-degenerative changes in the tumor as a consequence of radiation-induced pathomorphism. SRS and STR are effective techniques for treatment of patients with primary and recurrent PA despite regardless of localization of the tumor. There procedures should be performed shortly after non-radical resection. Control of tumor growth by the present time (median follow-up is 22.7 months) reaches 98%. "Progression" of the tumor due to enlargement of cystic portion shortly after SRT and SRS represents reactive-degenerative alterations in the tumor tissue and should not be evaluated as true recurrence; without neurological deterioration these cases do not require special treatment.
Voprosy neĭrokhirurgii 01/2012; 76(1):64-78; discussion 78.
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ABSTRACT: Trigeminal neuralgia (TN) is a disabling disease with severe impairment of quality of life and everyday activity of patients. Range of treatment modalities is wide and includes conservative therapy, surgical methods and stereotactic radiosurgery (SRS). SRS is a minimally invasive technique which provides good results and minimal rate of complications. High geometrical and clinical precision of frameless SRS using CyberKnife system allows its application in TN when high dose is delivered to a small volume. The given paper focuses on the first Russian experience of frameless SRS of TN using robotized system CyberKnife. Since April 2009 till June 2011 4 patients with TN were treated in department for radiation therapy of Burdenko Neurosurgical Institute using CyberKnife system. Mean age was 63 years. Mean and maximal target dose reached 70 and 90 Gy, respectively. Data of catamnestic clinical examination were available in all 4 patients. Follow-up period reached 8 months. Response to treatment was observed in all cases. In 2 (50%) patients full effect was reached while in the rest only partial effect was present. No complications were encountered. Effectiveness and safety of SRS using CyberKnife system is comparable with framed SRS.
Voprosy neĭrokhirurgii 01/2012; 76(1):79-83; discussion 84.
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A N Konovalov,
A V Golanov,
G E Gorlachev,
V N Kornienko,
Iu Iu Trunin,
T M Kotel'nikova,
S V Zolotova,
E R Vetlova,
M V Galkin,
N A Antipina,
S A Mariashev, I N Pronin,
N V Arutiunov,
A Iu Lubnin,
S B Iakovlev
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ABSTRACT: Robotized system for radiosurgery CyberKnife (Accuray Inc., USA) is the first device dedicated and optimized for advanced irradiation during 1-7 fractions (i.e. radiosurgery and hypofractionation). CyberKnife is characterized by elaborate guidance system, high precision of dose delivery, possibility of conformal dose distributions with high gradient of target borderline dose which is most important in proximity of critical structures. The first CyberKnife system in Russia was installed in Burdenko Neurosurgery Institute. The paper presents 2-year experience of treating patients using CyberKnife. From April 2009 till October 2011 896 patients were treated using CyberKnife. Mean age was 48 years. Overall number of sessions was 2626. Radiosurgical procedures were performed in 21.8% of patients. 91% of cases were treated for intracranial lesions. Limited follow-up period in all kinds of pathology demonstrated results consistent with standard fractionation or radiosurgery. The rates of observed complications were also comparable with accepted techniques. CyberKnife system plays significant role in everyday activity of department of radiation therapy. In careful and thorough selection of patients it allows efficient and high-quality treatment of patients with neurosurgical diseases.
Voprosy neĭrokhirurgii 01/2012; 76(1):3-12.
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ABSTRACT: The aim of the study was to investigate the change of NAA/Cr ratio in the brain parenchyma in patients with MS with the help of protonic multivoxel magnetic resonance spectroscopy (PMMRS) and to detect the correlation between this factor and clinical status of the patients. The study was also performed in order to investigate whether this method can be potentially used for monitoring of the severity of the disease and severity of the neurodegenerative process. On the basis of this knowledge potential neuroprotective effect of the interferon beta 1a (IFN Β1а) i.m. (avonex) was investigated. Twenty six patients with relapsing-remitting multiple sclerosis (RRMS) were included in the study. The procedures included examination of the history of the disease, neurological examination, EDSS, neuropsychological tests, dynamic MRI with PMMRS. The decrease of the NAA/Cr ratio in patients with RRMS compared to healthy controls was detected. The dynamic control in a year revealed the decrease NAA/Cr in patients with MS. Negative association was revealed between NAA/Cr in the brain and the level of neurological and cognitive deficit. The analysis of the 1 year therapy with IFN Β1а i.m. revealed neuroprotective effect which can be related to the possible positive effect of the drug on the neurodegenaration process.
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 01/2012; 112(7):36-41. · 0.12 Impact Factor
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ABSTRACT: The aim of the study was to reveal MRI features of focal cortical dysplasia and sclerosis of the hippocampus in patients with symptomatic epilepsy. Twenty-four patients who underwent the surgery with symptomatic epilepsy were studied. The results of pre-surgical MRI and histological studies of the removed cortex were compared. The signs of focal cortical dysplasia in the lesion area were found in patients with oncologic and non-oncologic lesions. In patients with sclerosis of the hippocampus, such specific sign as the T2-signal elevation was not seen in all cases. Though MRI is a highly sensitive method for detecting cortical dysplasia, it does not allow to differentiate significantly different types of this pathology. Even in the presence of typical signs of focal cortical dysplasia, its real boundaries may spread beyond the clearly visualized lesion. In the absence of typical signs of focal cortical dysplasia, one should take into account non-specific signs in the comparison with clinical and neurophysiologic data. In sclerosis of the hippocampus, the changes in the signal intensity were not constantly seen and the decrease in the volume of the structure was a main diagnostic sign. In the cases of so-called "medial temporal epilepsy", any changes in hippocampus volume may be diagnostically important.
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 01/2011; 111(10 Pt 2):15-9. · 0.12 Impact Factor
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A A Potapov,
N E Zakharova, I N Pronin,
V N Kornienko,
A G Gavrilov,
A D Kravchuk,
A V Oshorov,
A A Sychev,
O S Zaĭtsev,
L M Fadeeva,
S V Takush
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ABSTRACT: Forty patients with severe traumatic brain injury (GCS score 8 and less) aged 16-54 years treated in our clinic were analyzed. Correlations between clinical symptoms, CT signs of diffuse and focal traumatic lesions, intracranial hemorrhage, indices of cerebral blood flow (CBF) according to perfusion CT study, intracranial pressure (ICP) and cerebral perfusion pressure (CPP) were assessed. Main mechanism of injury in 27 of 40 (67.5%) patients was acceleration-deceleration due to traffic accidents which usually leads to diffuse axonal injury (DAI) of different severity. In the other 13 (32.5%) cases injury was associated with coup-countercoup mechanism which caused focal contusions mostly. Not only GCS score but CT-signs of DAI severity, intracranial hemorrhage and minimal levels of CPP had significant prognostic value. Results of perfusion CT studies demonstrated that in 37 of 40 (92.5%) patients cerebral blood flow decreased (below 28.6 ml/100 g/min) in one or more arterial blood distribution areas. Increase of CBF was registered in 9 cases (over 69 ml/100 g/min), in 6 of them elevation of CBF in one arterial distribution area was associated with reduction in the other. Generally, mean CBF values were higher in the middle cerebral artery circulation than in the other. The lowest CBF levels (16.3 +/- 6 ml/100 g/min) were observed in cortical and subcortical hemorrhagic foci while these values were significantly higher in the same contralateral intact zones (36.0 +/- 10.0 ml/100 g/min; p < 0.01). In 3 patients with DAI the CBF in the midbrain varied from 12.5 to 30.1 ml/100 g/min with the lowest levels in hemorrhagic focus in cerebral peduncle. It corresponded to cystic-atrophic alterations found on subsequent follow-up MRI. Thus, reduction of CBF and episodes of low CPP were the leading pathophysiological phenomena of diffuse and focal brain damages.
Voprosy neĭrokhirurgii 01/2011; 75(3):3-16; discussion 17-8.
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ABSTRACT: Range of methods for diagnostics of extracranial arterial stenosis includes both untrasonic techniques (transcranial Doppler study, duplex sonography, which are not only screening methods, but also used in surgery as well) and high-tech imaging methods--spiral CT scans and MR angiography with visualization of structural changes in vascular wall, quantitative and functional assessment of blood flow in vessels. Aim of this study was to determine the effective of non-invasive diagnostic techniques--MR angiography (MRA), including phase-contrast MRA (PCMRA), and spiral CT angiography (SCTA) in identification and comprehensive assessment of stenosis of internal carotid artery and comparison of capabilities of these methods with gold standard--direct angiography.
Voprosy neĭrokhirurgii 01/2011; 75(2):3-10; discussion 10.
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ABSTRACT: Since 2008 till 2010 years 22 patients with multiple aneurysms were examined in Burdenko Neurosurgical Institute (Moscow, Russia). The series included 13 female and 9 male patients, age ranged from 28 to 62 years (mean 45 years). 68 aneurysms of different localization were discovered in these 22 patients. We also analyzed results of examination of 2 patients with solitary aneurysms in which SW-MRI was performed to confirm or exclude the fact of hemorrhage due to rupture of the aneurysm. All patients were operated microsurgically. SW-MRI is effective technique for identification of source of hemorrhage in patients with multiple aneurysms thus allowing selection of correct tactics of management. Information value of the method declines with increase of time after hemorrhage (over 2 years). SW-MRI enables verification of the fact of hemorrhage due to aneurysm in disputable cases (minimal non-specific clinical presentation, absence of hemorrhage verification).
Voprosy neĭrokhirurgii 01/2011; 75(2):11-7; discussion 17.
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Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 02/2007; 107(1):63-7. · 0.12 Impact Factor
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S V Serkov, I N Pronin,
O V Bykova,
O I Maslova,
N V Arutyunov,
T I Muravina,
V N Kornienko,
L M Fadeeva,
H Marks,
C Bönnemann,
R Schiffmann,
M S van der Knaap
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ABSTRACT: Recently, a novel leukoencephalopathy syndrome was described in eight patients with a distinct pattern of MRI abnormalities. Here we describe the clinical, laboratory, and MRI findings in five new, unrelated patients. The clinical picture was homogeneous with onset in childhood, a slowly progressive course, variable mental deficits, signs of pyramidal and cerebellar dysfunction and sometimes dorsal column dysfunction. In two patients, a minor head trauma was followed by neurological deterioration and fever. No underlying metabolic defect was found. In two patients serum lactate was elevated, but no evidence of a mitochondrial defect was found. MRI showed variably extensive, diffuse, or spotty cerebral white matter abnormalities and a selective involvement of particular brainstem tracts. The tracts involved included the pyramidal tracts, sensory tracts, superior and inferior cerebellar peduncles, and intraparenchymal trajectories of the trigeminal nerve. In four patients spinal MRI was performed and revealed involvement of tracts over the entire length depicted. Single voxel proton MRS in three patients revealed increased lactate within the abnormal white matter. The uniform and highly characteristic MRI findings, in combination with the similarities in clinical and MRS findings, provide evidence for a distinct nosological entity.
Neuropediatrics 03/2004; 35(1):1-5. · 0.94 Impact Factor
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Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 02/2003; · 0.12 Impact Factor
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ABSTRACT: We presented three cases of the non-differentiated White Matter's disease. There are clinical resemblance and the similar MRI patterns including MR spectroscopy in all patients. They are outpatients: girl (K), 7 years old, boy (N), 13 yrs and young men (N), 24 yrs without any family relationship, and were investigated in Burdenko Neurosurgical Institute, in 2002 year. The cerebral MRI examinations all of that patients have shown extensive abnormalities in the hemispheric's white matter of cerebrum and cerebellum. Meanwhile there are some specific lesions of the brain stem's tracts (the posterior limb of the internal capsule, the splenium of the corpus callosum, the medial lemniscus and the mesencephalic trigeminal tracts). Step by step progression of suffer ness with unknown time of beginning, mild mnestic reduction and hemyparesis and moderate cerebellar disorder without loss of sensitivity were characteristic for all patients. The results of the biochemical markers for leucodistrophy testing are negative. A proton spectrum of the abnormal white matter showed elevated lactate in one case (girl, 7 yrs). In our opinion clinical diagnosis in all three cases is the new pathology that some body named as "WMDL" (White matter disease with lactate). It should be differentiated with adrenoleucodistrophy, Refsum disease and NARP syndrome (neuropathy, ataxia, pigmentive retinitis).
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 02/2003; · 0.12 Impact Factor
