Clinical Neuroscience

[Text analysis of the diary of a schizophrenic patient ]

KUNCZ Elemér1, THORDAI István1

MARCH 01, 1963

Clinical Neuroscience - 1963;16(03)

[Authors examine the diaries of 33-year-old female patients - 70 handwritten pages spanning a year - which can be used to track phases of immersion in a world of closed, autistic experience and self-medication tendencies. They find that, on the one hand, the diary as a form corresponds to a written monologue by which the patient, isolated from the outside world, deceives his own solitude, and, on the other hand, a certain striving for autonomy and the role of self-preservation tendencies can be discerned in the bizarre turns of phrase and conceptual changes.]


  1. A budapesti XIII. ker. Tanács Róbert Károly körúti Közkórháza III. sz. női elme- és idegosztálya



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Clinical Neuroscience

[Reactivity, pharmacological influence and association with respiration of the periodic eeg complex in subacute panencephalitis]


[1. The stimuli we used could not influence the shape or periodicity of the periodic complexes. It appears that periodic eeg phenomena in penencephalitis can no longer be provoked by external stimuli when periodicity is stable. 2. No rapid activity typical of the initial stage of barbiturate anaesthesia was seen in any of our patients after intravenous Evipan. The lack of rapid activity in response to Evipan is associated with panencephalitic cortical lesions. 3. After Megimide injection, spasm potentiation initially occurred only in the section corresponding to the periodic complex. On this basis, it seems reasonable to conclude that the periodic complex represents a state of increased excitability that facilitates the appearance of spasm potentials. 4. There is a statistically highly significant difference between the number of eeg complexes occurring with increased frequency during the inspiratory and expiratory phases. If we assume that the firing of inspiratory and expiratory bulbopontine neurons facilitates the emergence of the panencephalitic eeg complex, it is reasonable to conclude that the primary anatomical lesion responsible for the emergence of the periodic complex may be the bulbopontine phase of the formatio reticularis, or at least a system intensely related to it. 5. In some cases, intravenous Relaxil G caused the characteristic periodicity of the curve to disappear and the diffuse polymorphic deltaic activity characteristic of end-stage panencephalitis to develop. The Relaxil effect suggested that the structure controlling the autorhythmicity of the periodic complex may be the diffuse thalamic system. Thus, the presence of a periodic complex implies the relative integrity of the diffuse thalamic system. 6 We have discussed the differences between the panencephalitic periodic complex, the K-complex and the Forbes' secondaerial response and, on the basis of these differences, we believe that the periodic complex cannot be identified with either the Forbes' secondaerial response or the K-complex.]

Clinical Neuroscience

[Amnesticus syndrome due to Härtel's alcohol injection ]

PAP Zoltán, ADLER Péter

[We reported a patient with severe trigeminal neuralgia in whom, by Härtel's method, part of the alcohol given into the Gasser's duct was presumably introduced into the cisterna chiasmatis and caused sterile meningitis, with encephalitis in the infundibulum area and severe damage to the mammillary bodies. This resulted in acute unconsciousness, febrile delirium, a slowly recovering foggy state of consciousness and a permanent amnestic syndrome. However, neuralgia - except for the unpleasant sensation of phonia, which still exists in branch I, has disappeared. The case is interesting not only from a psychological point of view, because of the memory disturbance, but also because of the lessons to be drawn from it. Although Dandy and Frazier-Spiller's trigeminal surgery cannot be said to be completely without risk, not to mention Sjöquist's intramedullary tractotomy, the situation is really different today from what it was in Fedor Krause's time. Härtel's confident statement, quoted above, made in 1924, has lost much of its legitimacy today. His proposal for surgical interventions, like Kirschner's electrocoagulation, was restricted to a narrower field. Despite the undoubtedly positive results, its use must always be accompanied by undesirable consequences. Its recommendation should therefore be limited to patients who are not suitable for or do not agree to intracranial surgery and who cannot be made painless by other means. ]

Clinical Neuroscience

[Dr. Adolf Juba]


[The author gives an account of the life and work of Dr. Adolf Juba.]

Clinical Neuroscience

[Glioma behaviour in tissue culture]

PÁLYI Irén, TÓTH Géza, CSÁKY László, ÁFRA Dénes, ZOLTÁN László

[1. In tissue cultures of gliomas after a single high dose of 6000 r., phosphorus metabolism was investigated 6 and 22 h after irradiation. 2. After irradiation, glioma cultures showed higher activity in the acid-soluble phosphorus fraction than controls. The lipid and DNA fractions, on the other hand, had lower activity compared to controls. 3. Normal tissues show no consistent changes in their phosphorus metabolism after a single high dose of radiation. ]

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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.

Clinical Neuroscience

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


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.]