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Biomedical subjects

S Apostolski

Publications and source records attributed to S Apostolski.

At least 55 records · Page 3Linked to original sources

Poland syndrome associated with 'morning glory' syndrome (coloboma of the optic disc).

A 12 year old girl with the Poland syndrome and the 'morning glory' syndrome is described. The patient presented with absence of the left pectoralis major muscle, hypoplasia of the left arm, symbrachydactyly, and ipsilateral coloboma of the optic disc. This is the first report of the association of these two congenital anomalies.

Abnormalities, Multiple↗

[Diagnostic problems in patients with myasthenia gravis].

Myasthenia gravis (MG) is an antigen-specific autoimmune disease in which antibodies directed against nicotinic acetylcholine receptors of the postsynaptic muscle membrane (nAChR) impair neuromuscular transmission. MG is clinically characterized by abnormal muscle fatigue and weakness. The initial symptoms and signs are often unrecognized. Therefore, we analyzed the diagnostic errors and duration of diagnostic delay in patients affected with MG (n = 444) in a ten-year period (January 1, 1983-December 31, 1992) in Yugoslavia. The initial diagnosis was correct in 44.4% of patients and erroneous in 38.4%; 17.2% of patients were admitted without an initial diagnosis. The average duration of diagnostic delay was 11 months. We present the differential diagnostic difficulties in MG and discuss the principles of diagnostic strategy which may reduce the risk of diagnostic errors in MG.

Adult↗

Plasma exchange in myasthenia gravis and multiple sclerosis.

During the last twenty-year period therapeutic plasma exchange (TPE) was used in the treatment of 68 patients with myasthenia gravis and 61 patients with multiple sclerosis. The therapeutic effects were evaluated on the basis of neurologic deficit changes, electrophysiological findings, necessary laboratory analyses and patient's general conditions. It was shown that the therapeutic effects mosty depended on the nature and stage of the basic disease, adequate selection of the patients and timely applied therapeutic procedure. Significant positive effects of the TPE treatment applied with the anti-inflammatory and immunosuppressive therapy were observed in patients with myasthenia gravis and multiple sclerosis upon clinical findings and some paraclinical tests.

Adolescent↗

[McArdle's disease].

Glycogenosis type V (McArdle disease) is a serious metabolic disorder with an exercise intolerance, myalgia, early fatigue and stiffness of exercising muscles, relieved++ by rest. The authors present a case report of patient with McArdle's disease, and diagnostic procedures which can be used in different diagnostic of metabolic myopathies, especially between myoadenylate deaminase deficiency and different types of gly(geno)lytic myopathies. The importance of "ischemic forearm test" and muscle biopsy is emphasized.

Adult↗

[Modern approach to the diagnosis and therapy of myasthenia gravis].

Only two decades ago myasthenia gravis (MG) has been considered to be a severe neuromuscular disease with a very high mortality rate. Contemporary diagnostic and therapeutic approach resulted in standardized methodology for clinical management of myasthenia gravis. As an example, we present a case of generalized MG in a 22-year-old female patient. The diagnosis was based on the results of characteristic clinical, pharmacologic and electrophysiologic tests. Preoperative treatment with anticholinesterase drugs, thymectomy and postoperative course of the disease are presented. The importance of the thymus pathology and immunological serum abnormalities is emphasized. The results of this kind of clinical approach are compared with the published experience of other neurological groups.

Adult↗

[von Recklinghausen's neurofibromatosis].

We present a case of unusual clinical picture of the well defined Morbus Von Recklinghausen. In a 35-year-old male patient, after an uncontrolled rapid movement of the neck, the disease was suddenly manifested with spastic quadriparesis. Three years later the clinical picture showed spastic quadriplegia. Antalgic torticollis confirmed by X-ray of the neck clearly showed vertebral subluxation at the level of C3-C4. However, additional magnetic resonance imaging revealed large extradural tumorous formation at the level of C1-C2, and two small oval tumours in the splenium of the corpus callosum as well as in the left lateral ventricle. "Café au lait" spots, "molluscum fibrosum" and few subcutaneous fibromas appeared as skin changes which explained the possible nature of the registered intracranial and intraspinal tumours. Neurosurgery has been performed at the spinal level with good results; it confirmed the diagnosis of Von Recklinghausen's Neurofibromatosis.

Adult↗

[Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes: the POEMS syndrome].

We present a case of an unusual multi-systematic disorder whose cardinal signs were severe progressive sensorimotor polyneuropathy, hepatomegaly, endocrinopathy, plasma cell dyscrasia with osteosclerotic bone lesions and M-protein production as well as skin lesions (termed POEMS Syndrome) whose pathophysiology is still unknown, as well as its relationship with myeloma multiplex. The patient, 67 years old, had a history of progressive weakness and numbness of the lower legs, clinically revealed as sensomotor polyneuropathy, hepatomegaly, IgG lambda monoclonal protein in the serum, endocrine abnormalities and skin lesions. The final and definite diagnosis was established by open, surgical biopsy of the second lumbal vertebra.

Aged↗

[Cerebrospinal fluid findings in patients with acute and chronic inflammatory demyelinating polyradiculoneuropathies].

We studied the cerebrospinal fluid (CSF) in patients with acute (AIDP) and chronic inflammatory demyelinating poliradiculoneuropathy (CIDP). In order to assess its possible contribution in establishing the diagnostic approach, we analyzed the cell content, concentrations of total proteins, albumin and IgG, CSF/serum albumin quotient and the qualitative study of IgG. Sixteen patients with AIDP and 16 patients with CIDP, fulfilling the accepted diagnostic criteria, were included in this study. CSF features were not used as exclusion criteria. The cell count and concentration of total proteins were determined by standard procedures. CSF albumin was performed and IgG were done by single radial immunodiffusion, and the qualitative analysis of IgG by agarose isoelectric focusing of unconcentrated CSF. The incidence of elevated levels of total CSF proteins was identical in patients with AIDP and CIDP, reaching 94%. The mean level of CSF protein was similar in patients with AIDP and CIDP, 1100 and 1150 mg/l, respectively. The cell count was elevated in 3 (19%) patients with AIDP and normal in patients with CIDP. There were no significant differences in the mean level of any of CSF parameters between patients with AIDP and CIDP. Local synthesis of CSF oligoclonal IgG was found in 2 patients with CIDP and was not detected in patients with AIDP. No association between CSF and clinical parameters was recorded. The results of this study confirm that an elevated CSF protein and low CSF cell count are features highly supportive of the diagnosis of both AIDP and CIDP. The most intriguing finding is the detection of locally produced CSF oligoclonal IgG which may suggest the associated inflammation within the central nervous system in a number of patients with CIDP, and furthermore a pathogenic link between CIDP and multiple sclerosis.

Acute Disease↗

[Guillain-Barre syndrome and antibodies to ganglioside GM1. Case report].

A sudden onset of the syndrome Guillain-Barré in 48-year-old woman presenting as an acute motor neuropathy was associated with antibodies against ganglioside GM1 detected by ELISA. The neurological examination revealed flaccid quadriplegia without affection of the sensory system, and the additional investigation showed mild increase of the CSF protein content, demyelination of the peripheral motor nerves and significantly increased titer of the serum and CSF anti-GM1 antibodies. Several copruculture for Campylobacter jejuni gave negative results. There was a significant correlation between the severity of the clinical picture and the titer of the serum anti-GM1 antibodies. The patient completely and spontaneously recovered after five weeks. According to the clinical and laboratory parameters the patient could be classified as an axonal, and according to electromyographic findings and the course of the disease as the classical form of the syndrome Guillain-Barré.

Autoantibodies↗

[Amyotrophic lateral sclerosis in a defined population in Belgrade--an epidemiologic study].

The paper shows the results of the first epidemiological study of ALS in Belgrade. The incidence, prevalence and natural course of ALS were determined for the period 1985 through 1991. The distribution of 58 newly discovered cases in a 7-year survey period showed that the average annual incidence rate was 0.54 per 100000 population (95% confidence interval, 0.23-1.06), and age-adjusted incidence rate was 0.42 per 100000 population (95% confidence interval, 0.18-0.83). The rate for males was 1.5 times higher than the rate for females. The greatest age-specific average incidence rate was observed in patients between 60 and 64 (3.66 per 100000 population: 95% confidence interval, 2.17-5.78). The actual age-adjusted prevalence rate on December 31, 1991 was 1.07 per 100000 (95% confidence interval, 0.71-1.71). The mean age at the onset of the disease was 56.2 +/- 9.8 and it ranged from 24 to 74. The average interval between the onset of symptoms and the establishing of the diagnosis was 14.38 +/- 17.26 months. The mean duration of the disease was 27.7 +/- 18.2 months. The cumulative probability of survival was 27% for the whole population in a 5-year interval. Elderly patients and those with bulbar signs at the onset had a poorer prognosis.

Adult↗