Lege Artis Medicinae

[A huge GIST]

KRUTSAY Miklós, CHANIS William

JUNE 20, 2010

Lege Artis Medicinae - 2010;20(06-07)

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Clinical Neuroscience

Quality of life of patients with non-diabetic peripheral neuropathic pain; results from a cross-sectional survey in general practices in Hungary

BRODSZKY Valentin, PÉNTEK Márta, KOMOLY Sámuel, BERECZKI Dániel, EMBEY-ISZTIN Dezső, TORZSA Péter, GULÁCSI László

Background and purpose - There is a lack of data on the impact on health related quality of life of peripheral neuropathic pain in Hungary. The main aims of the study were to assess the health related quality of life of nondiabetic PeNP patients identified in general practices through screening, and to assess the relationship between condition specific pain scores and health state utilities. Methods - Non-diabetic patients aged ≥30 years were recruited in 10 general practices in Hungary. At first, patients filled in the PainDETECT Questionnaire (PD-Q) and those who have achieved ≥13 PD-Q score (unclear or possible neuropathic pain) were further assessed by the DN4 questionnaire. Patients with PD-Q score >18 or DN4 score ≥4 were considered to have PeNP and they completed the EQ-5D health questionnaire. Results - Among the 111 patients identified as non-diabetic PeNP patients the mean age was 62 (SD=14) years, 69% were women. Average EQ-5D score was 44% lower than the gender and age matched Hungarian norm (0.42 vs. 0.75, p<0.001) and it worsened with increasing pain intensity. The pain/discomfort and anxiety/depression were the most affected EQ-5D dimensions. Strong relationship was demonstrated between the PD-Q and EQ- 5D score. Most of the PeNP patients (86%) were undiagnosed. Conclusions - Non-diabetic PeNP pain has a huge negative impact on health related quality of life. Although PeNP is a serious chronic condition, the disease burden is seriously underestimated, both on the level of individuals and society, due to the fact that patients are rarely identified.

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PÁPAI Zsuzsanna, KISS Nóra

[Conventional adjuvant therapy is, in most cases, either the well-known standard doxorubicin monotherapy or the combination of doxorubicin + ifosfamide. No clear guideline has been developed yet - adjuvant therapy is recommended in cases with high grade, larger than 10 cm, sarcoma, where surgery hasn’t been suffi ciently radical, and adjuvant radiotherapy may not be advisable. In locally advanced tumors, due to the requirements of limb salvage, isolated limb perfusion is recommended. As a new compound, hafnium-oxide nanoparticles (NBTXR3) can be useful in local therapy: combining intratumoral injection and radiotherapy may be a fl agship initiative, however further investigations are necessary. In the treatment of metastatic tumors, beside the standard methods, new, targeted treatments are becoming more and more prevalent: in leiomyosarcomas trabectedine, pazopanib and olaratumab; in liposarcomas trabectedine and eribulin; in synovial sarcomas pazopanib; and in imatinib-resistant GIST, sunitinib and regorafenib. Soft tissue sarcomas are rare tumors categorized as heterogeneous histological subtypes. In their treatment, it is key to customize the treatment based on these subtypes and interdisciplinary collaboration with the orthopedic surgeon, the pathologist and the radiotherapist to determine the suitable therapy for each individual.]

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[NONSENSE MUTATION 193C>T OF NEUROFIBROMATOSIS TYPE 2 - A NEUROSURGICAL CHALLENGE]

BOBEST Mátyás, TÓTH Csaba, GYURCSÓ Mária, MOLNÁR Mária Judit, GARZULY Ferenc

[A 15 years old male was operated because of incidentally found intercostal schwannoma. Two years later severe cerebellar ataxy and left sided anacusis developed. MRI revealed bilateral vestibularis tumors and multiple cervical intradural extramedullar myelon compressing lesions. After partial resection of the huge left sided cerebello-pontin tumor, histologically schwannoma, and the exstirpation of the multiple cervical meningiomas the patient died three months later due to septic complications. The 24 years old mother had been operated on similar lesions 12 years earlier, after two weeks postoperative period she died. Her 14 years old twins are living, a boy also with bilateral acustic tumours and a girl who is intact. Genetic investigation revealed C>T nonsense mutation at position 193 in the exon 2 of the NF2 gene. This mutation cause premature truncation of the gene protein and is probably in connection with the clinically severe phenotype. Early diagnosis of this type of neurofibromatosis is mandatory concerning the therapy.]

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FALUDI Béla, ROZGONYI Renáta

[Insomnia - one of the most prevalent sleep complain - has a great impact on the everyday life. Basically two different form of insomnia can be defined: the insomnia disorder and the co-morbid insomnias. To treat adequately determination of background pathology is essential, which is based on the help of Sleep Medicine Centers. According to the newest guidelines, the treatment of insomnia disorder is based on cognitive behavioural therapies followed by pharmaceutical intervention. In this review we provide the short description of cognitive behavioural therapies and basic principles of hypnotic drugs. Despite the availability of insomnia guidelines the huge variation of the insomnia medication can be seen in the daily practice. Due to the above mentioned reasons we summarize the good clinical practice of hypnotic drug administration for insomnia patients.]

Clinical Neuroscience

[Evolution of human brain and intelligence]

LAKATOS László, JANKA Zoltán

[The biological evolution, including human evolution is mainly driven by environmental changes. Accidental genetic modifications and their innovative results make the successful adaptation possible. As we know the human evolution started 7-8 million years ago in the African savannah, where upright position and bipedalism were significantly advantageous. The main drive of improving manual actions and tool making could be to obtain more food. Our ancestor got more meat due to more successful hunting, resulting in more calory intake, more protein and essential fatty acid in the meal. The nervous system uses disproportionally high level of energy, so better quality of food was a basic condition for the evolution of huge human brain. The size of human brain was tripled during 3.5 million years, it increased from the average of 450 cm3 of Australopithecinae to the average of 1350 cm3 of Homo sapiens. A genetic change in the system controlling gene expression could happen about 200 000 years ago, which influenced the development of nervous system, the sensorimotor function and learning ability for motor processes. The appearence and stabilisation of FOXP2 gene structure as feature of modern man coincided with the first presence and quick spread of Homo sapiens on the whole Earth. This genetic modification made opportunity for human language, as the basis of abrupt evolution of human intelligence. The brain region being responsible for human language is the left planum temporale, which is much larger in left hemisphere. This shows the most typical human brain asymmetry. In this case the anatomical asymmetry means a clearly defined functional asymmetry as well, where the brain hemispheres act differently. The preference in using hands, the lateralised using of tools resulted in the brain asymmetry, which is the precondition of human language and intelligence. However, it cannot be held anymore, that only humans make tools, because our closest relatives, the chimpanzees are able not only to use, but also to make tools, and they can be tought how to produce quite difficult ones. Some brain characteristics connected to human consciousness and intelligence, like brain asymmetry, the “consciousness” or “theory of mind” based on mirror neurons are surprisingly present in monkeys. Nevertheless, the human intelligence is extremly flexible and different, while the animal intelligence is specialised, producing one thing at high level. Based on recent knowledge the level of intelligence is related anatomically to the number of cortical neurons and physiologically to the speed of conductivity of neural pathways, the latter being dependent on the degree of myelinisation. The improvement of cognitive functions including language is driver by the need of more effective communication requiring less energy, the need of social dominance, the competitive advantages within smaller groups and species or against other species, which improves the opportunity for obtaining food. Better mental skills give also sexual dominance, which is beneficial for stabilising “cleverness” genes. The evolutionary history of human consciousness emphasises its adaptive survival helping nature. The evolution of language was the basic condition of conscious thinking as a qualitative change, which fundamentally differentiate us from all other creatures.]