Lege Artis Medicinae

[Fiberscopic examination of posttracheotomy tracheae in myasthenic patients]

KAS József1, BARANYAI Lajos1, RIGEL András1, MOLNÁR János1, SZOBOR Albert2

MARCH 31, 1992

Lege Artis Medicinae - 1992;2(03)

[Posttracheotomy strictures are often detectable only long after decannulation. That observation led the authors to make endoscopic follow-up examination of the tracheae of tracheotomized patients. Since 1973, 103 of 600 myasthenic thymectomized patients required tracheotomy. 45 of them were examined with fiberscope under topical anesthesia two months to thirteen years after tracheotomies (47 cases). The tracheal lumen was expressed as an estimated percent age of the original cross section. Strictures of various sizes were detected in 33 cases (70%), of which 5 (11%) were symptomatic. The stridor of two patients was eliminated by the resection of nodular struma which were causing compression, and by the removal of granuloma formed at the site of the stoma. In three cases of verified stricture, repeated translaryngeal intubation was performed under optical control. In regular follow-up patients prefer fiberscopy to the rigid bronchoscopy. In addition, fiberscopy reveals severe strictures causing stridor and significant, but asymptomatic, stenoses. The awareness of these changes is essential in the management of myasthenic patients. Based on their own clinical experience, the authors suggest a follow-up protocol. ]

AFFILIATIONS

  1. MÁV Tüdőgyógyintézet Sebészeti Osztály
  2. Dél-pesti Kórház Neurológiai Osztály

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Lege Artis Medicinae

[A newly discovered vasoconstrictor peptide: endothelin]

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[The discovery of endothelium-derived relaxing (prostacyclin, EDRF) and contracting factors (EDCF) in the last decade opened up new vistas in investigating the regulation of the cardiovascular system. In this review, recent results of the research of a 21 amino acid residue EDCF, called Endothelin, including its discovery, isolation, biosynthesis, tissue specific expression, receptors and the characteristics of the Endothelin converting enzyme are summarized. Based on present knowledge of the wide variety of biological actions of Endothelins, we present their possible autocrine, paracrine and humoral actions, and their significance in the physiological regulation of the cardiovascular system as well as in pathological conditions. Despite the abundance of information however, the actual role of Endothelins in the short- and long-term modulation of the tissue function still remains to be determined.]

Lege Artis Medicinae

[ATP-sensitive potassium channels, and their modulators]

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[Recently great interest has been concentrated on potassium channels inhibited by intracellular adenosine triphosphate (ATP-sensitive potassium channels), which connect the metabolic state of cells with their electrical activity. Channel closure initiates the secretion of insulin from pancreatic ß-cells; a number of hormones altering the level of blood-sugar act via these channels. The channels are present in neurones; they regulate both in the cell bodies and the nerve terminals (e. g. peripheral nerves) the excitability. The reby, in the central nervous system, they influence the neuronal activity both in the satiety center and some catecholaminergic brain nuclei. These channels mediate the effects of endogenous vasodilators in vascular smooth muscle. They have a pathophysiological role in protecting neurones and muscle cells from hypoxic damage. The ATP-sensitive potassium channels are targets of the sulphonylurea antidiabetic agents and of a new class of drugs, the potassium channel agonists. The latter compounds are investigated or used for the treatment of hypertension, angina pectoris, cardiac insufficiency, bronchial asthma, irritable bladder syndrome, chronic occlusive arterial disease and even in impotence. They are relatively well-tolerated drugs. Their adverse effects are related to their basic vasodilator profile and most commonly include headache, dizziness, palpitation or oedema. ]

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[The high incidence of intra-articular ligament injuries of the knee justifies a more frequent use of noninvasive diagnostic procedures such as the native CT scan. In positive cases, a correct preoperative diagnosis may be produced; a negative result is especially important, since diagnostic arthroscopy may be avoided. Due to the anatomic arrangement of the knee joint ligaments and theis density, which differentiates them from adjacent structures, an accurate, reliable, and reproducible image can be obtained with the native CT scan. With the high resolution charactaristic of the native CT scan, the ligaments of Humphry and Wrisberg can be visualized, and the two bundles of the anterior cruciate ligament can be distinguished from one another. Exact localization of intra-articular ligament rupture is essential in the planning of an optimal treatment schedule. According to the literature and the authors experience, the native CT scan is equivalent to the MRI as a diagnostic procedure in knee intra-articular ligament lesions. It has the advantage of being far less expensive and it is presently available in Hungary. ]

Lege Artis Medicinae

[Dipyridamole test: comparative evaluation of simultaneous 2D echocardiography and 201TL scintigraphy in ischaemic heart disease]

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[Simultaneous 2D echocardiography and 2017 scintigraphy were performed during dipyridamole testing (DT) in 57 patients with suspected IHD. The dipyridamole dose was 0.56 mg/kg/bm in 4 minutes. If no effect was observed, the test was continued from the eighth minute with a dose of 0.28 mg/kg/bm in 2 minutes. 2D echomonitoring of the left ventricular motion using apical views was initiated at the beginning of DT and continued for 7 or 13 minutes in case of the longer test. After 6.5 or 12.5 minutes, 74 MBq 201TI was injected intravenously. Standard planar scintigraphy was performed in 3 views starting within 15 minutes. On the basis of the clinical results, 25 patients were subsequently referred for coronary angiography. The sensitivity, specificity and positive and negative predictive values of 2D echocardiography were 79%,55%,69% and 67%, respectively. The corresponding values for 201TIscintigraphy were 93%, 27%, 62%, and 75%. Where the results of both methods were positive, the combined sensitivity and specificity values were 71% and 73%. The results indicate the suitability of both methods for detecting IHD. Furthermore, they suggest a higher specificity, but lower sensitivity of 2D echocardiogaphy as compared to 201TI scintigraphy. Our observations demonstrate that the com binaton of these 2 techniques is very useful in clinical practice for the evaluation of IHD.]

Lege Artis Medicinae

[Multiple Risk Factor Intervention Trial]

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Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

Clinical Neuroscience

Myasthenia gravis, Guillain-Barré syndrome, or both?

ERDOGAN Cagdas, TEKIN Selma, ÜNLÜTÜRK Zeynep, GEDIK Korkut Derya

Myasthenia gravis (MG) and Guillain-Barré syndrome (GBS) are autoimmune disorders that may cause weakness in the extremities. The coexistence of MG and GBS in the same patient has rarely been reported previously. A 52-year-old male presenting with ptosis of the left eye that worsened with fatigue, especially toward evening, was evaluated in our outpatient department. His acetylcholine receptor antibody results were positive, supporting the diagnosis of MG. His medical history revealed a post-infectious acute onset of weakness in four extremities, difficulty in swallowing and respiratory failure, which was compatible with a myasthenic crisis; however, his nerve conduction studies and albuminocytologic dissociation at the time were compatible with GBS. With this case report, we aimed to mention this rare coincidental state, discuss possible diagnoses and review all other similar cases in the literature with their main features.

Clinical Neuroscience

[Myasthenia in a patient with sarcoidosis and schizophrenia (in English language)]

RÓZSA Csilla, KIS Gábor, KOMOLY Sámuel

[A 44-year-old male patient was hospitalised with paranoid schizophrenia in 1985. Depot neuroleptic treatment was started which successfully prevented further psychotic relapses for the next ten years. His myasthenia gravis started with bulbar signs in 1997 and the symptoms soon became generalized. The diagnosis of myasthenia gravis was confirmed by electromyography, by positive anticholinesterase test and by the detection of anti-acetylcholine receptor antibodies in the serum. Mediastinal CT examination showed enlarged hilar lymph nodes on the left but no thymic pathology was observed. Mediastinoscopy was performed and biopsies were obtained from the affected nodes. Histology revealed sarcoidosis. The patient suffered respiratory crisis following the thoracic intervention (in September 1998). Combined oral corticosteroid (64 mg methylprednisolone/e.o.d.) and azathioprine (150 mg/day) treatment regimen was initiated and complete remission took place in both the myasthenic symptoms and the sarcoidosis. The follow-up CT scans showed no mediastinal pathology (January 2000). During steroid treatment a transient psychotic relapse occured which was successfully managed by supplemental haloperidol medication added to his regular depot neuroleptics. The patient currently takes 150 mg/day azathioprine and receives 40 mg/month flupentixol depot im. His physical and mental status are stable and he has been completely symptome free in the last 24 months. The association of myasthenia gravis and sarcoidosis is very rare. To our best knowledge no case has been reported of a patient suffering from myasthenia gravis, sarcoidosis, and schizophrenia at the same time.]

Lege Artis Medicinae

[IMMUNE-MEDIATED NEUROLOGICAL DISORDERS]

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[Multiple sclerosis, myasthenia gravis and chronic inflammatory neuropathies share the common feature of chronic course with potential development of disability due to the damage caused by immunological processes. Early detection and precise diagnosis is very important, because most patients respond well to proper immunomodulatory treatment. The diagnosis requires extensive knowledge of the disease and is based on the clinical symptoms recognised by the GP, as well as on complex assessment of the results of special neurophysiological, radiological and laboratory examinations. The present paper reviews the major immune-mediated neurological disorders and discusses their targeted immunological treatment.]

Lege Artis Medicinae

[RARE ASSOCIATION OF HODGKIN’S LYMPHOMA, GRAVES’ DISEASE AND MYASTHENIA GRAVIS]

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[INTRODUCTION - In some cases other diseases associate with Hodgkin’s lymphoma, when it is diagnosed or relapses. Association of Hodgkin’s lymphoma with Graves’ disease and myasthenia gravis in one patient has not yet been reported in the literature. CASE REPORT - We report on a young female patient who had suffered from Hodgkin’s lymphoma since 1996. He had received polychemotherapy and mantle field irradiation previously. After treatment, complete remission was stated in 2000. Then she was treated because of Graves’ disease. In 2001 she complained of dysarthria, dysphagia, ptosis and diplopia. Thorough examinations proved myasthenia gravis. Considering the progression plasmapheresis was administered several times with cyclophosphamide and intravenous immunglobulin, besides conservative therapy. Recently she is euthyroid state, Hodgkin’s disease is in remission and her only complaint is dysarthria. CONCLUSION - The importance of this case on one hand is the rare association of these diseases, on the other is that Graves’ disease and myasthenia gravis occurred during in the remission of Hodgkin’s disease. Causal relation is not unambiguous but the role of disturbed immunregulation caused by Hodgkin’s lymphoma or the irradiation of the neck region can also contribute to it. The pure coincidental occurrence of Hodgkin lymphoma, Graves’ disease and myasthenia gravis is highly unlikely.]