Clinical Neuroscience

[Analysis of the arginine content of liquid protein components]

SZILÁGYI A. Katalin1

JULY 01, 1968

Clinical Neuroscience - 1968;21(07)

[The Vorfractione of CSF contains much higher amounts of arginine than the other protein components of CSF. CSF albumin is arginine-poor. This finding, together with previous observations on the spatial distribution of arginine and Vorfractio in CSF, suggests that metabolic processes may occur between the two substances in the CNS. ]

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  1. Országos Ideg-és Elmegyógyintézet Kutatólaboratórium

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Clinical Neuroscience

[Transient electroencephalographic phenomena in the generalisalt spike-wave pattern and the so-called grand mal type ictal generalisalt repetitive spasm discharge ]

HALÁSZ Péter, VELOK Gyula, HIDASI József, BOCZÁN Gábor

[In search of an answer to the pathophysiological reasons for the difference between petit mal and grand mal mechanism, we described four patients with intertwined generalized spike-wave mechanism and generalized repetitive spasm; two cases with 3 c/s spike-wave mechanism within an ictus and two cases with 3 c/s spike-wave mechanism within an ictus. two spike-wave variants during two different types of seizures in the same patient. From our observations the following findings can be filtered. 1. Transitory EEG phenomena can be detected between the ictal electrographic phenomena of petit mal with spike-wave and grand mal seizures with repetitive rapid discharge. 2. We can confirm Gastaut's observations that in epileptic encephalopathies with some spike-wave variants, repetitive rapid discharge tonic seizures with mild motor symptoms and significant vegetative symptoms are frequently observed in sleep. 3. Sleep - both natural and Evipan anaesthesia - and benzodiazepine preparations also contribute to the appearance of transient phenomena. On the basis of our electrographic observations, the spike wave mechanism and the previously discovered facts about the neuronal mechanism of grand mal repetitive rapid discharge (Pollen 1964, Matsumoto and Ajmone Marsan 1964) and the interneuronal recurrent collateral inhibition shown to be crucial in rhythmic EEG phenomena (Eccles 1965), we attempt to provide a unified view of the grand mal and petit mal mechanism and to explain the electrographic and clinical differences.]

Clinical Neuroscience

[The structure and dynamics of resistance in psychotherapy in psychotherapy groups]

HIDAS György

[In psychotherapy, resistance is present all the time with varying intensity. It is also an obstacle to psychotherapeutic change, but it provides valuable insights into the resistance mechanisms, character and history of the participants in the process, as well as their present functions. Experience has shown that the recognition and discussion of resistance moves the psychotherapeutic process forward towards improvement, positive change and dissolves the current form of resistance. Since the cause of resistance is often not exclusively in the individual, but often in the family, it logically follows that family or couple therapy is necessary. ]

Clinical Neuroscience

[The effect of labyrinth stimulation on cerebellar blood flow]

POÓR Gyula, KOPA János, MOLNÁR László

[Authors investigated the effect of stimuli from the semicircular arcuate ducts of the labyrinth on cerebellar blood flow in rabbits. The flow was studied using an electrical system based on the negative feed-back principle, which allows the measurement of the change in thermoconductivity. In chloralose-urethane narcosis, changes in general blood pressure and respiration were simultaneously recorded, as well as electrical activity and blood flow in the cerebellar cortex. On stimulation of one side of the labyrinth with cold water, an increase in blood flow was found in the opposite cerebellar cortex and a decrease in the same side; on warm stimulation the change was reversed. Galvanic stimulation resulted in a biphasic change. Rotation increased blood flow in both cerebellar hemispheres, and this remained unchanged after bilateral transection of the vago-sympathicus, and became more pronounced and prolonged after KCl was dripped onto the cerebral cortex. ]

Clinical Neuroscience

[In patients with Dupuytren's contracture electroclinical studies ]

SZENDRŐI Mária, HASZNOS Tivadar, GALAMBOS József

[The authors performed detailed neurological examinations in 50 patients with Dupuytren's contracture; they also determined the conduction velocity of the motor fibres of the ulnar nerve in 23 patients. The mild clinical symptoms and the decrease in conduction velocity suggest that ulnar nerve lacunar lesions are involved in the pathogenesis of Dupuytren's contracture. In this sense, they evaluate 5 cases of their patients in which the symptoms of circumscribed ulnar nerve laesio were followed shortly by the appearance of Dupuytren's contractura. ]

Clinical Neuroscience

[Transaminase in cerebrospinal fluid ]

LÁNG Sándor

[The CSF cerebrospinal fluid glutamic acid oxaloacetic acid transaminase (GOT) level is normally low, 5-30 units. There is no significant difference between cisternal and lumbar CSF. In old age (over 50 years), transaminase levels may also rise above normal in healthy subjects. GOT is not elevated in epilepsy (except status epilepticus), multiple sclerosis, polyneuritis, polyradiculitis - No change in meningitis of various origins except for 1 or 1 spike. Normal values in benign CNS tumours and higher values in extensive malignancies. In vascular disease (atherosclerosis, stroke, cerebral thrombosis), CSF transaminase levels are increased in one third to one half of cases. Elevated CSF GOT is not specific to a single disease and should only be evaluated in conjunction with clinical signs and other laboratory tests.]

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Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

NALBANTOGLU Mecbure, AKALIN Ali Mehmet, GUNDUZ Aysegul, KIZILTAN Meral

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

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

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. ]