[Ocular myasthenia gravis]. / Miasteniya, glaznaya forma.

The article describes an urgent medical and social problem - the diagnosis and treatment of the ocular myasthenia gravis. Despite recent advances in the study of synaptic pathology, the diagnosis of the ocular form of myasthenia gravis remains a challenging problem. This is due to the poor information content of laboratory and electromyographic methods in the diagnosis of ocular myasthenia gravis, and the generalized myasthenia gravis manifests in 90% of cases by external ophthalmoplegia and ptosis. The article highlights the features of the diagnosis and differential diagnosis of ocular myasthenia gravis. Data on the course and features of the clinical presentation of the disease are presented. The importance of analyzing anamnestic data and clinical manifestations of the disease is emphasized. The article discusses modern approaches to the treatment of the ocular myasthenia gravis. The authors present clinical observations of 2 patients with ocular myasthenia gravis.

[Myasthenia gravis and muscle atrophy]. / Miasteniya i myshechnye atrofii.

Muscle atrophy is uncommon in myasthenia gravis. There are some reports in the literature of muscle atrophy in MUSK-positive myasthenia gravis. The authors present eight cases of AChR-positive myasthenia gravis associated with muscle atrophy. Symmetrical atrophy of the forearms with preserved hand muscles was identified in six cases and scapular winging in two cases. Atrophy appeared 3-18 years after the onset of myasthenia gravis and after a period of progression, the condition was stabilized. In all cases, there were no pain syndrome, no sensory loss, normal creatine phosphokinase (CPK) levels, no significant abnormality on MRI of the cervical spine. Concomitant neuropathies, amyotrophic lateral sclerosis and other neuromuscular diseases were excluded. All patients underwent quantitative electromyography and repetitive nerve stimulation. The authors suggest that the atrophy is not the sign of myasthenia gravis in this case and is caused by other neuromuscular diseases.

[Seronegative nonparaneoplastic Lambert-Eaton myasthenic syndrome]. / Seronegativnyi neparaneoplasticheskii miastenicheskii sindrom Lamberta-Itona.

The authors studied two patients with Lambert-Eaton myasthenic syndrome (LEMS) in whom the repeated examination did not find specific of LEMS P/Q type voltage-gates calcium channel autoantibodies. The results of clinical testing and electrophysiological examination showed the typical character of movement disorders with the absence of tendon reflexes and signs of disautonomia as well as a decrease in M-response amplitude and phenomena of decrement with low frequency- and increment with high frequency stimulation. Both patients revealed no signs of paraneoplastic process. Autoimmune character of the damage was confirmed by the effectiveness of treatment with glucocorticoid hormones.

[A comparative analysis of the informative value of anti-AChR-antibody radioimmunoassay and laser correlation spectroscopy in myasthenia gravis]. / Sravnitel'nyi analiz informativnosti radioimmunologicheskogo opredeleniya antitel k atsetilkholinovomu retseptoru i lazernoi korrelyatsionnoi spektroskopii pri miastenii.

AIM: An aim of the study was to compare informative value of traditional approach (anti-AChR antibody radioimmunoassay) and evaluation of metabolic shifts by laser correlation spectroscopy in myasthenia gravis. MATERIAL AND METHODS: The search for the relationship between the disease severity in 77 patients, 12-80 years and the distribution pattern of subfraction serum components revealed three informative zones: 6-15, 27-67, and 127-223 nm. RESULTS AND CONCLUSION: In patients without disturbances of vital functions, the contribution of the first zone particles into light scatter increases and that of the third zone particles decreases. Considerable differences attaining the level of statistical significance in zones 6 and 20 nm were revealed in the spectra of serum from patients with myasthenia gravis of the same severity with and without thymoma. This opens prospects for dynamic monitoring of the efficiency of therapy.

[Decrement pattern of M-response amplitude in the low-frequency repetitive nerve stimulation in the muscles of patients with myasthenia gravis and Lambert-Eaton myasthenic syndrome]. / Dekrement amplitudy M-otveta pri nizkochastotnoi stimulyatsii myshts bol'nykh miasteniei i miastenicheskim sindromom Lamberta­Itona.

AIM: To investigate the pattern of decrement in the muscles of patients with myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). MATERIAL AND METHODS: Twenty-seven patients with MG and 39 patients with LEMS were studied using low frequency repetitive nerve stimulation (3/ sec). RESULTS AND CONCLUSION: The decrease of safety factor of neuromuscular transmission was equal in both groups. At the same time, a significant difference in the decrease of pattern of the amplitude compound of muscle action potential (CMAP) was found. In LEMS, by contrast with MG, another sequence of amplitude variability of CMAP from the second stimulus to the first and from the fifth stimulus to the fourth was noted. In LEMS patients, progressive decrement, manifesting by increasing ratios of late A9/A1 to early A4/A1 was found, whereas the MG patients showed retrogressive decrement expressed by the reduction in decrement ratio (from late to early). These differences reflect the mechanisms and status of acetylcholine mobilization and release from the axon terminal.

[The interaction of nano-particles of titanium dioxide and blood serum in patients with autoimmune diseases.]

The article considers interaction of nano-particles of titan oxide and blood serum of patients with verified diagnoses of "myasthenia" and "allergy". The analysis of solution of nano-powder of titan oxide using technique of laser correlated spectrometry demonstrated that main input into light scattering is made by particles with radius of 4.64-8.42 nm. The data concerning dimensions of particles received with laser correlated spectrometry matches data of analysis of these particles with various physical techniques. The nano-particles of titan oxide stabilized with phosphoric acid at interaction with blood serum cause development and sedimentation of complexes with albumins and globulins. The interaction of nano-particles of titan oxide with blood serum of patients with myasthenia results in elimination from it the significant percentage (40%) of circulating immune complexes. In blood serum of patients with bronchial asthma, nano-particles of titan oxide bind proteins of small size mainly.

[Treatment approaches for bronchopulmonary infection in myasthenia gravis patients].

Nineteen patients with bronchopulmonary infection and myasthenia gravis were enrolled in the study. The microbiological analysis of the specimens of phlegm and bronchial secretion revealed both grampositive and gramnegative bacteria. All the isolates were susceptible to the antibiotic used (cefoperazone/sulbactam). Intravenous immunoglobulins (IvIgs) were used to increase the treatment efficacy, to opsonize the infection foci and to decrease the hospitalization terms. The antibiotic therapy and simultaneous use of intravenous immunoglobulins provided higher clinical efficacy in 16 out of 19 patients (84.2%).

[Thoracoscopic thymectomy in children by myasthenia gravis].

The authors' experience of surgical treatment of myasthenia gravis in children was analyzed. 32 patients were operated on, mean age was 13±3 (5-21 years); girls were 26, boys were 6 (19%). Disease severity was IIB-IVB by the MGFA classification, all patients were on anticholinesterase and pednisolone therapy. The operation was indicated by certified neurologists, specialized in myasthenia. All patient were thoracoscopically thymectomized using left- or rightside access. There were no conversions and intraoperative complications. The operation duration was 77±23 min. The postoperative hospital stay was 6.6±3.6 (3-9) days. Excellent long-term results were achieved in 6 (25%) patients, good - in 20 (62.5%) children, no changes were registered in 2 and deterioration of myasthenia in 2 (6.25%) patients. The study proved that thoracoscopic thymectomy obtains the radicalism of open technique, proposing the easier postoperative period and being the method of choice for the surgical treatment of myasthenia gravis in children.

[Thymectomy and antibodies to acetylcholine receptors (AChR) in patients with myasthenia gravis in the early postoperative period].

Thymectomy and removal of the hyperplastic thymus have been performed in 21 patients with myasthenia. In 14 patients, thymectomy is performed through thoracoscopic access (TA), while in 7 cases through thoracotomic access (TT). The serum levels of antibodies to acetylcholine receptors (AChR) determined before and after surgery were increased in both groups of patients. Thus, thymectomy, regardless of the degree of clinical improvement in early post operative period, significantly increases the level of anti-AChR antibodies in the serum of patients in the early postoperative period compared to baseline. These changes may reflect the immune response to a stressful situation related to the traumatic surgery. It should be noted that the level of anti-AChR antibodies was significantly higher in a group of TT that may be related with a larger volume of the removed tissue.

[The use of the QMGS for quantitative assessment of the severity of movement disorders in patients with myasthenia].

The MRC scale is traditionally used for assessment of the severity of movement disorders. The patients' effort is graded on a scale of 0-5. The use of this scale is limited by subjective biases and inadequate assessment of some functions. The quantitative scale for assessment of the severity of clinical symptoms of myasthenia (QMGS) allows to evaluate pathological muscle fatigability, the main syndrome of this disease, which is important for evaluating the severity of symptoms before and after the pathogenetic treatment or when probes with the introduction of acetylcholinesterase preparations are used.

[Efficacy and practicability of using intravenous human immunoglobulin in the pathogenetic treatment of patients with generalized myasthenia].

The treatment with immunoglobulin (IgG) has led to the clinical improvement in all 12 patients with generalized myasthenia and practically has not caused any side-effects. The highest efficacy was seen in patients with a threat of or developed mixed crisis who received high doses of IgG (0,4-0,5 g per one kg of body mass) in the course of 3-5 intravenous drop introductions administered along with basic treatment with glucocorticoid and immunosuppressive drugs. In patients who received IgG once in smaller doses (0,2-0,3 g per one kg of body mass) to stabilize the disease course or to hasten recovery from the exacerbation, the clinical effect was similar though less prolonged. The treatment course with IgG stopped myasthenic crises in all cases. The clinical efficacy of IgG was not correlated with anti-acetylcholine receptor antibodies.

[Anti-acetylcholine receptor antibodies: effect of immunosuppressive drug therapy in patients with myasthenia gravis].

We studied anti-acetylcholine receptor antibodies (ARAb) in 25 patients with myasthenia gravis before and after the immunosuppressive drug (steroid) therapy and in 22 patients before and after thymectomy. The clinical effect after the treatment was correlated with the reduction of ARAb levels by more than 20% in 62% of patients. The ARAb levels did not change, or even increased, in 15% patients despite the improvement of their clinical state. We showed the direct correlation between the clinical improvement and the decrease of ARAb level.

[Clinical features of myasthenia related with autoantibodies to muscle specific tyrosine kinase (MuSK)].

Anti-acetylcholine receptor antibodies are detected in 80 to 90% of patients with generalized myasthenia gravis - seropositive myasthenia gravis (SPMG). Approximately 10% to 20% of patients with autoimmune MG do not have antibodies to acetylcholine receptor (AChR) - seronegative MG (SNMG). An immunological study of the blood serum of patients with SNMG revealed patients with or without antibodies to muscle specific tyrosine kinase (MuSK+ and MuSK-, respectively). A clinical study of 13 MuSK+ patients with myasthenia showed that this group was characterized by predominant affect of mimic and bulbar muscles with the rare involvement of eye movement and body muscles. In Musk+ patients with myasthenia, the clinical efficacy of antitoxin esterase drugs was minimal or absent. No unequivocal conclusion on the clinical pattern of this form of myasthenia has been made.

[Clinical and electrophysiological peculiarities of seronegative myasthenia].

Thirty-seven patients with myasthenia gravis (MG) underwent AChR-Ab analysis, clinical study and neurophysiological examination - repetitive nerve stimulation (RNS). About 16,2% of MG patients who were anti-AChR-negative constituted a so-called seronegative MG group (SNMG). Compared to the AChR-Ab positive patients (SPMG), the SNMG was characterized by the higher female/male ratio (6:1), higher frequency of infantile onset of MG (33,3%), absence of association with thymoma and highest frequency of myasthenic crisis (83,3%). The clinical pattern of SNMG differed from SPMG and was characterized by predominant affect of mimic bulbar and respiratory muscles that determined severity of the course and high frequency of myasthenic crises. The identical clinical pattern was found in 19,3% of SPMG patients. However the character of neuromuscular transmission in orbicularis oculi muscle was different in SNMG and this SPMG-group. The pathological decrement was observed in 83,3% muscles of the SPMG-group (from -20% to -74%) and only in one case in the SNMG-group (-48%). Besides, the absence of clinical and neurophysiological responses to anticholinesterase was noted in the SNMG-group. Cholinergic neuromuscular hyperactivity in SNMG patients manifested itself in clinical fasciculations and myokymic contractions of muscles which prevailed in facial muscles in 66,7% of SNMG patients. Neurophysiologic examination displayed extra repetitive discharges after the compound motor action potential (R-CMAP) at low-frequency stimulation after acetylcholine esterase inhibitors in 100% cases.

[Long-term results of surgical treatment of thymomas at the patients with generalized myasthenia].

Results of diagnosis and treatment of 150 patients with thymoma and generalized myasthenia are analyzed. The results of examination were registered before surgery, and also in 1, 3, 5 and 10 years after one. It is demonstrated that thymoma is potentially malignant tumor with risk of recurrence and requires long follow-up of patient after operation. Morphologic type and stage of tumor, severity of myasthenia are the main predictors in the prognosis of surgical treatment. Thymomectomy with excision of fatty tissue of anterior mediastinum is absolutely indicated for this severe category of patients, and it decreases the risk of tumor progression. Mortality of patients depends not only on tumor growth or recurrence but also on concomitant myasthenic disorders.

[Anti-Titin-antibodies at the patients with myasthenic and non-myasthenic thymoma].

Serum specimens from 52 myasthenic patients with thymoma of various histological characteristics and 4 patients with thymoma without myasthenia have tested for anti-Titin-antibodies titer. It has been demonstrated that organ-specific thymoma dominated at myasthenic patients, and organ-nonspecific thymoma - at the patients without myasthenia. There was no correlation between severe clinical symptoms and the level of anti-Titin-antibodies. The titer of antibodies at the patients with organ-specific thymoma was higher that at ones with organ-nonspecific thymoma, but there was no correlation between the level of anti-Titin-antibodies and histological type of organ-specific thymoma.