Clinical Neuroscience

[A new version of Jacod's syndrome]

MARIA Filipowicz1

JULY 29, 1952

Clinical Neuroscience - 1952;5(02)

[Brain symptoms in nasopharyngeal tumours were observed as early as the second half of the 19th century. Observations on the frequency of certain brain lesions have been supplemented by descriptions of the pathways by which nasopharyngeal tumours penetrate the inside of the skull. For some time there were two opposing views on the origin and development of tumours. According to some authors, the tumour originates either from the nasopharynx or from the connective tissue of the skull base, and thus invades the skull from the outside, destroying the cranial nerves in its path. Others have argued that, on the contrary, elements within the skull are the starting point of the tumour, and that the process in the nasopharynx is independent or has been considered as a tumour metastasis from the inside of the skull to the outside. Doubts in this respect have only been dispelled by detailed study. It was found that in the majority of cases the nasopharyngeal space was the starting point. ]

AFFILIATIONS

  1. Varsói Egyetem Neurológiai Klinika

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Clinical Neuroscience

[Schizophrenia in childhood]

LÓRÁND S. Blanka

[According to the prevailing psychiatric view, schizophrenia is rare in childhood, and some authors, such as Vogt and Weichbrodt, have denied that it occurs before adolescence. However, a critical review of the older literature on childhood psychosis leads to the conclusion that it is not so much the rarity of childhood schizophrenia as its recognition and correct pathology: in the older descriptions, we often find images of childhood schizophrenia that are typified by the current understanding. ]

Clinical Neuroscience

[Data for the pathogenesis of morphinismus]

GARTNER Pál, KELEMEN Endre

[Since December 1950, authors have performed morphine withdrawal treatment on 32 morphine users referred from the KEKSZ. Their procedure is as follows: morphine is withdrawn immediately. On the first day of withdrawal, the patient receives 0.1-0.1 ccm of 2% morphine intracutaneously into the flexor aspect of both forearms, for a total of 4 mg. The same is given for the next 3 days. The fifth day is a break. On the sixth and seventh days he receives the same again. In addition: on days 1-3-6, another 3-5-10 ccm of foreign group blood is given intravenously. If there is an obstacle to hetero-blood injection, 10 ccm Resactor is given intramuscularly instead. This procedure is used to minimise withdrawal symptoms, and mood correctors are rarely needed. Patients usually leave on day 10 with no symptoms or complaints. The pathogenesis of morphinism is thought to be essentially immunobiological. They consider their new withdrawal treatment to be intracutaneous desensitisation and attribute the success of the treatment to the reduction in tolerance induced by the binding of morphine antibodies. Hetero-blood or Resactor injections mobilise antibodies through their effect on the RES system, which enhances the efficacy of the treatment. Treatment does not resolve the issue of relapse. ]

Clinical Neuroscience

[Fundus examinations in connection with electrohock]

KLIMES Károly, ERŐSS Sándor

[The introduction and use of cumulative electroshock (ES) in our clinic, in addition to the therapeutic results, has also provided an opportunity to further investigate the basic and concomitant phenomena of the spasm mechanism. In this brief, quasi-preliminary report, we describe the ocular fundus lesions associated with spasm. As the essence of shock therapy is seen by many authors (Abély, Delay, Morin, Gastant, Cain) in the stimulation of the diencephalon, and Krienberg and Eberhardt have drawn attention to the fundamental importance of cerebral blood circulation in the mechanism of shock, the study of the orbital vessels of the fundus is of prime importance. The behaviour of the fundus blood vessels provides an analogous picture of the probable identical behaviour of the cerebral blood vessels, and the vasovegetatively congruent state of the pupils and fundus blood vessels also provides information on the fluctuations of the vegetative tone.]

Clinical Neuroscience

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[A summary of the articles published in the issue in Russian and German]

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Clinical Neuroscience

Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

Clinical Neuroscience

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.

Lege Artis Medicinae

[A short chronicle of three decades ]

KAPRONCZAY Katalin

[Hungarian professional periodicals started quite late in European context. Their publish­ing, editing and editorial philosophy were equally influenced by specific historical and political situations. Certain breaking points of history resulted in termina­tion of professional journals (War of In­de­pendence 1848-1849, First and Se­cond World Wars), however there were pe­riods, which instigated the progress of sciences and founding of new scientific journals. Both trends were apparent in years after the fall of former Hungarian regime in 1990. The structure of book and journal publishing has changed substantially, some publishers fell “victim” others started successfully as well. The latters include the then-established publishing house Literatura Medica and its own scientific journal, Lege Artis Me­di­cinae (according to its subtitle: New Hun­garian Medical Herald) issued first in 1990. Its appearance enhanced significantly the medical press market. Its scientific publications compete with articles of the well-established domestic medical journals however its philosophy set brand-new trends on the market. Concerning the medical community, it takes on its problems and provides a forum for them. These problems are emerging questions in health care, economy and prevention, in close interrelation with system of public health institutions, infrastructure and situation of those providing individual health services. In all of them, Lege Artis Medicinae follows consequently the ideas of traditional social medicine.]

Lege Artis Medicinae

[Focus on Lege Artis Medicinae (LAM)]

VASAS Lívia, GEGES József

[Three decades ago, LAM was launched with the goal of providing scientific information about medicine and its frontiers. From the very beginning, LAM has also concerned a special subject area while connecting medicine with the world of art. In the palette of medical articles, it remained a special feature to this day. The analysis of the history of LAM to date was performed using internationally accepted publication guidelines and scientific databases as a pledge of objectivity. We examined the practice of LAM if it meets the main criteria, the professional expectations of our days, when publishing contents of the traditional printed edition and its electronic version. We explored the visibility of articles in the largest bibliographic and scientific metric databases, and reviewed the LAM's place among the Hun­ga­rian professional journals. Our results show that in recent years LAM has gained international reputation des­pite publishing in Hungarian spoken by a few people. This is due to articles with foreign co-authors as well as references to LAM in articles written exclusively by foreign researchers. The journal is of course full readable in the Hungarian bibliographic databases, and its popularity is among the leading ones. The great virtue of the journal is the wide spectrum of the authors' affiliation, with which they cover almost completely the Hungarian health care institutional sys­tem. The special feature of its columns is enhanced by the publication of writings on art, which may increase Hungarian and foreign interest like that of medical articles.]

Lege Artis Medicinae

[Second game, 37th move and Fourth game 78th move]

VOKÓ Zoltán

[What has Go to do with making clinical decisions? One of the greatest intellectual challenges of bedside medicine is making decisions under uncertainty. Besides the psychological traps of traditionally intuitive and heuristic medical decision making, lack of information, scarce resources and characteristics of doctor-patient relationship contribute equally to this uncertainty. Formal, mathematical model based analysis of decisions used widely in developing clinical guidelines and in health technology assessment provides a good tool in theoretical terms to avoid pitfalls of intuitive decision making. Nevertheless it can be hardly used in individual situations and most physicians dislike it as well. This method, however, has its own limitations, especially while tailoring individual decisions, under inclusion of potential lack of input data used for calculations, or its large imprecision, and the low capability of the current mathematical models to represent the full complexity and variability of processes in complex systems. Nevertheless, clinical decision support systems can be helpful in the individual decision making of physicians if they are well integrated in the health information systems, and do not break down the physicians’ autonomy of making decisions. Classical decision support systems are knowledge based and rely on system of rules and problem specific algorithms. They are utilized widely from health administration to image processing. The current information revolution created the so-called artificial intelligence by machine learning methods, i.e. machines can learn indeed. This new generation of artificial intelligence is not based on particular system of rules but on neuronal networks teaching themselves by huge databases and general learning algorithms. This type of artificial intelligence outperforms humans already in certain fields like chess, Go, or aerial combat. Its development is full of challenges and threats, while it presents a technological breakthrough, which cannot be stopped and will transform our world. Its development and application has already started also in the healthcare. Health professionals must participate in this development to steer it into the right direction. Lee Sedol, 18-times Go world champion retired three years after his historical defeat from AlphaGo artificial intelligence, be­cause “Even if I become the No. 1, there is an entity that cannot be defeated”. It is our great luck that we do not need to compete or defeat it, we must ensure instead that it would be safe and trustworthy, and in collaboration with humans this entity would make healthcare more effective and efficient. ]