LAM Extra for General Practicioners

[NEBIVOLOL: THE LONG-ACTING, VASODILATOR BETA-BLOCKER]

LÉGRÁDY Péter

APRIL 20, 2010

LAM Extra for General Practicioners - 2010;2(02)

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LAM Extra for General Practicioners

[THE IMPORTANCE OF VITAMIN D DEFICIENCY IN PRACTICE]

TAKÁCS István

[The effects of vitamin D in bone health have been known since the 1920s. Recently, it has been proven that its role in the body is much more complex. Activated vitamin D is a steroid hormone that regulates transcription of more than 200 human genes through its receptor that is detectable in almost all types of cells. In contrast to the former conceptions, it can be activated not only in the kidneys; moreover, local 1-α-hydroxylation plays a greater role in its extraskeletal effects. Vitamin D deficiency, currently defined as serum levels of <30 ng/ml, is caused by the lack of ‘effective’ sunlight exposition. Thus, vitamin D deficiency is one of the most frequent deficiencies in the developed world that plays a role not only in the development of skeletal conditions but many other diseases, as well. A low vitamin D level causes a reduced calcium absorption, a higher bone remodelling rate and increased bone loss. It also reduces muscle strength and increases the risk of falling. Normal vitamin D status is required for the effectiveness of drugs for osteoporosis treatment; however vitamin D treatment in itself is not effective in osteoporosis. An increasing number of studies show the benefits of vitamin D supplementation and treatment in extraskeletal conditions. Vitamin D plays an important role in the prevention of several auto-immune diseases, infections, cardiovascular diseases, and cancers. Therefore, all UV-B radiation-deprived adults require an intake of vitamin D to maintain a level of >30 ng/ml. Vitamin D3 treatment is safe. The necessary dose can be reliably approximated by the calculation that an incremental consumption of 100 IU/day raises serum vitamin levels by 1,0 ng/ml. Clinical trials suggest that for the vast majority of individuals, a prolonged intake of 10,000 IU/day does not pose any risk.]

LAM Extra for General Practicioners

[SUDDEN CARDIAC DEATH IN ATHLETES - CARDIAC ELECTROPHYSIOLOGY POINT]

VARRÓ András

[Sudden cardiac death of athletes is very rare (1/50 000 to 1/100 000 annually) but it is still 2 to 4 times more frequent than that of the agematched normal population. In addition, it attracts peculiar media attention. Sudden cardiac death in athletes is supposed to not primarily have an ischemic origin but most likely relates to repolarization abnormalities. These may be caused by several independent and/or dependent factors such as benign cardiac hypertrophy developing normally in athletes (athlete’s heart), hypertrophic cardiomyopathy, increased sympathetic activity, genetic defects, seemingly harmless drugs, doping agents, food, and dietary supplements. These factors together can increase inhomogeneities in myocardial repolarization (“substrate”). In this case, an otherwise harmless extrasystole (“trigger”) occurring with unlucky timing may - although very seldomly - elicit fatal arrhythmias. Thus, effective prevention of sudden cardiac death may include new types of cost-effective cardiac electrophysiological screening methods (ECG or echocardiography) and, in case of a high level of suspicion, more costly genetic tests can be considered.]

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Acute effect of sphenopalatine ganglion block with lidocaine in a patient with SUNCT

KOCATÜRK Mehtap, KOCATÜRK Özcan

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache with cranial autonomic features (SUNCT/SUNA) is a rare severe headache. At the time of an attack, it can hinder a patient from eating and requires acute intervention. The sphenopalatine ganglion is an extracranial parasympathetic ganglion with both sensory and autonomic fibers. Sphenopalatine ganglion block has long been used in the treatment of headache, particularly when conventional methods have failed. Here, we present a patient who was resistant to intravenous lidocaine, but responded rapidly to sphenopalatine ganglion block during an acute episode of SUNCT/SUNA.

Clinical Neuroscience

Coexistence of cervical vertebral scalloping, pedicle deficiencies and dural ectasia in type I neurofibromatosis

YALDIZ Mahizer

Neurofibromatosis type 1 (NF-1; also known as Von Recklinghausen’s disease) is a common autosomal dominant disease that occurs in the general population at the rate of 1 in 3000. Many NF-1 patients present with spinal malformations. A 54-year-old female patient was admitted to the Outpatient Clinic of Dermatology with gradually increasing swelling and spots on the body that had been present for a long period of time. Cervical vertebral scalloping, pedicle deficiencies and dural ectasia (DE) were also detected. She was diagnosed with NF-1. NF-1 is routinely seen in dermatology practice. Coexistence of NF-1 with vertebral scalloping, pedicle deficiencies and DE rarely occurs. Our case is the second reported instance in the literature of NF-1 with a spinal anomaly in the cervical region, and the first reported instance of the coexistence of NF-1 with cervical vertebral scalloping, pedicle deficiencies and DE.

Clinical Neuroscience

[Vinpocetin in neurological diseases]

SZAPÁRY László, KÉSMÁRKY Gábor, TÓTH Kálmán, MISNYOVSZKY Melinda, TÓTH Tímea, BALOGH Ágnes, NAGY Krisztián, NÉMETH György, FEHÉR Gergely

[Introduction - Stroke is the third leading cause of death worldwide (following cardiovascular and cancer mortality) and associated with serious disability for the vast majority of patients. There is no salvage therapy for irreversibly damaged brain areas, improving the circulation of the surrounding hypoperfused territories may be associated with benefitial clinical states. Cerebral hypoperfusion may play a role in the pathogenesis of other kind of neurological diseases, improvement of global circulation may have a preventive effect on these conditions. Aims - The aim of our study was to review the experimental and clinical articles focusing on the role of vinpocetin in different neurological conditions. Results - Vinpocetin appears to have several different mechanisms of action that allow for its antiinflammatory, antioxidant, vasodilating, antiepileptic and neuroprotective activities in experimental conditions. On the other hand, several meta-analysis of the existing studies in acute stroke examining short and long term fatality rates with vinpocetin was unable to assess efficacy. In chronic cerebrovascular patients, vinpocetin improves impaired hemorheological variables, has significant vasodilating properties, improves endothelial dysfunction, neuroimaging studies showed selective increase in cerebral blood flow and cerebral metabolic rate, all of which are potentially beneficial in cerebrovascular disease and may improve cognitive functions. Summary - Based on the above mentioned results vinpocetin plays an important role both in basic research and in clinical management of different neurological diseases.]

Clinical Neuroscience

A case report of Morvan syndrome

AYTAC Emrah, ACAR Türkan

Morvan syndrome is a rare disease characterized by peripheral nerve hyperexcitability, encephalopathy, dys­autonomia and significant insomnia. The patient, who was included in the present study, was followed-up at our clinics for confusion, myokymia, hyperhidrosis, epileptic seizures, tachycardia, agitation, hypokalemia, and hyponatremia. The cranial MRI of the patient demonstrated hyperintensities at the T2 and FLAIR sections of the medial temporal lobe and insular lobes. Electromyography and neurotransmission examination results were concordant with peripheral nerve hyperreactivity. Contactin-associated protein-like 2 antibodies and leucine-rich glioma inactivated protein 1 antibodies were detected as positive. The patient was diagnosed with Morvan syndrome; intravenous immunoglobulin and corticosteroid treatment was started. Almost full remission was achieved. This very rare syndrome implies challenges in diagnosis and treatment; however, remission can be achieved during the follow-up. In addition, caution is needed in the long-term follow-up of these patients regarding the development of malignancies.