[The differential diagnosis of amyotrophic lateral sclerosis and subacute herpes virus myelitis]. / Differentsial'naya diagnostika podostrogo gerpeticheskogo mielita s bokovym amiotroficheskim sklerozom.

Differential diagnosis of incurable and potentially curable neurological diseases is an urgent problem of modern neurology. The authors present a case report of subacute herpes virus myelitis, a rare complication of herpes infection by Varicella-Zoster virus. The differential diagnosis with amyotrophic lateral sclerosis is described.

[Differential diagnosis of multiple sclerosis with pediatric onset: the experience of the Moscow Division for treatment of children and adolescents with multiple sclerosis].

The diagnosis of multiple sclerosis in children and adolescence should be differentiated from a group of rare white matter diseases, with fuzzy diagnostic criteria. Some of these conditions require modern diagnostic techniques and wide knowledge of the doctor. The Moscow Division for treatment of children and adolescents with multiple sclerosis is a specialized advisory structure, which has specialists with experience in the differential diagnosis of multiple sclerosis with pediatric onset. The emphasis on the clinical aspects in differential diagnosis of early onset multiple sclerosis can reduce the time for the diagnosis and help to avoid diagnostic mistakes.

[Neurological manifestations of HIV-infection (review)].

Current data on the etiology, pathogenesis, diagnosis and treatment principles of HIV are reviewed. The special attention is focused on affections of the nervous system (neuro-AIDS). Primary and secondary manifestations of these affections are described.

[MRI in the diagnosis of hereditary leukoencephalopathies (review)].

The review addresses MR imaging of hereditary leukoencephalopathies (HL) as one of the most urgent problems of modern neurology and neuroradiology. HL is a group of rare human diseases that occur with a primary brain white matter lesions. Modern theoretical and methodological approaches for MRI of patients with hereditary leukoencephalopathies and data analysis are discussed. The authors demonstrate that MRI plays the important role in the diagnosis and differential diagnosis of HL, in-vivo imaging of structural changes in the brain that are correlated with pathogenetic mechanisms of these diseases.

[Data of MRI and neuropsychological tests predict the course of typical remitting multiple sclerosis during five years].

A complex study of 50 patients with active typical remitting multiple sclerosis (MS) was carried out. Neuropsychological testing using Wechsler and Stroop tests and MRI of the brain with the morphometric analysis of focal and diffusive changes were used in the study. Patients were stratified into two subgroups by the changes in the performance of neuropsychological tests. The disease course was assessed during five years. In all cases, the natural course of the disease, i.e. when patients did not receive disease modifying drugs, was analyzed. The transition to secondary progressive MS and the marked increase in MS severity on the EDSS were found in the subgroup of patients who demonstrated changes in the neuropsychological test performance. The strongest correlation was observed between EDSS scores and the diffusive atrophy of the brain white matter on MRI. The data of neuropsychological testing and some brain MRI parameters may be recommended as a predictive test in remitting MS.

[Comatose state caused by seizure-free status epilepticus in postoperative period].

We present a rare clinical observation of progressive development of neurological symptoms up to comatose state in a child with extra-intraventricular craniopharyngioma in delayed postoperative period due to seizure-free status epilepticus which was diagnosed only by EEG-monitoring. Concerning this case report, we discuss genesis of seizure-free status epilepticus and its possible role in development of severe condition in patients with different kinds of neurosurgical pathology in postoperative period.

[Megalencephalic leukoencephalopathy with subcortical cysts (van der Knaap disease)].

A history of the discovery, hypotheses of the pathogenesis, neuromorphologic data, clinical appearances, differential diagnostics and an own description of a single case of rare hereditary children leukoencephalopathy, with megalencephaly, changes of myelin structure and formation of subcortical cysts are presented. Taking into account non-specific clinical appearances of this disease and difficulties of genetic analysis, a priority role of MRI-diagnostics revealing typical signs of the disease is emphasized.

[Multiple cerebral abscess caused by Nocardia brasilientis].

Streptococcus aureus, streptococci, and enterobacteria were major causative agents of abscesses of the brain. At present, associated infection is responsible for 60% of cerebral abscesses. When systemic and specific immunities are diminished, a risk for cerebral abscesses caused by fungi increases. Among them, there are the most common fungi Candida species or Aspergillus species. The paper gives an example of successful complex treatment of multiple cerebral abscesses caused by an association of Nocardia brasiliensis and Staphylococcus epidermidis. The specific feature of the observation was the correct diagnosis before intraoperative verification of cerebral abscesses. The disease developed in the presence of diminished systemic immunity and in the absence of specific immunity to Nocardia. Immunodeficiency could be caused by prior herpetic infection, Epstein-Barr disease, a wasp bite. The pathogen that was able to cause microstrokes played an indubitable role in the pathogenesis of the disease, which predisposed to the development of cerebral abscesses in patients with immunodeficiency. Impaired blood circulation in the occipital region could be also induced by wasp venom.

[Aspergillosis of the central nervous system (clinical observation and review of literature)]. / Aspergillez (Aspergilosis) tsentral'noi nervnoi sistemy (klinicheskoe nabliudenie i obzor literatury).

Aspergillosis of the central nervous system presents a challenge faced with the problems arising from the establishing the diagnosis, the low efficiency of treatment, and high mortality rates (about 95% as shown by some data). This paper presents a clinical case of a patient with aspergillosis-induced central nervous system lesion verified by autopsy. Possible errors in the diagnosis and treatment of the patient are analyzed. The literature data including clinical cases, etiopathogenesis, and clinical manifestations and its possible complications are presented. Groups of patients at risk for invasive aspergillosis are considered. The paper gives the data available in the foreign literature on the methods and efficiency of treatment and mortality rates in different groups of patients. The urgency of the problem in the diagnosis and treatment of cerebral aspergillosis rises with the increased number of patients with immunological disorders due to infectious diseases (HIV), social (drug addiction, alcoholism), environmental, and other factors.

Five patients with a recently described novel leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate.

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.

[Three cases of non-differentiated leukodystrophy with involvement of cerebral hemisphere's white matter and cerebellum and specific lesions of cerebral pathways. Neuroimaging and clinico-biochemical characteristics]. / Tri sluchaia nedifferentsirovannoi leikodistrofii s porazheniem glubinnogo belogo veshchestva bol'shikh polusharii, mozzhechka i spetsifichnym porazheniem provodiashchikh putei. Neirorentgenologicheskie i kliniko-biokhimicheskie kharakteristiki.

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).

[The possibility of the use of several clinical, genetic and MRI characteristics for the prognosis of effects of beta-interferon-1beta treatment in multiple sclerosis]. / Vozmozhnost' ispol'zovaniia klinicheskikh, geneticheskikh pokazatelei i dannykh magnitno-rezonansnoi tomografii dlia prognozirovaniia éffektov lecheniia beta-interferonom-1-beta rasseiannogo skleroza.

Recently, beta-interferons have become the first line medicine of pathogenic treatment of multiple sclerosis (MS). 75 MS patients from Moscow received the treatment with beta-interferon-1b (betaferon). Clinical, genetic and MRI data on 48 of them were included in detailed statistical analyses. High basic frequency of relapses, absence of atrophy of the brain at the first MRI scans and the presence of HLA-DRB1*15 (less significant DRB1*0301 and DRB1*04) were associated with better clinical effects of one year Betaferon course--decrease of relapses frequency and delay in EDSS progression.