Clinical Neuroscience

[Amyotrophic lateral sclerosis in the light of recent research]

LEHOCZKY Tibor1, HALASY Margit1

MAY 01, 1965

Clinical Neuroscience - 1965;18(05)

[35 years ago, in 1929, one of us in the histology laboratory of the Schaffer Clinic worked up an interesting case, which we observed clinically together for 2 years. Without going into details, we will summarise the essence of the clinical investigation.]

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  1. Fővárosi István Kórház Idegosztálya

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Further articles in this publication

Clinical Neuroscience

[The influence of Károly Schaffer on the development of neurology and neuropathology]

MISKOLCZY Dezső

[He was the child of the sculptor Károly Schaffer; forms and shapes also captured his attention, but behind them he immediately sought content and meaning. His lifework therefore became an example of the close interweaving of facts and ideas.]

Clinical Neuroscience

[The general neuropathological work of Károly Schaffer]

KÖRNYEY István

[Károly Schaffer was first commemorated by his admirers on the occasion of the fortieth anniversary of his literary activity, in October 1927. In his words of thanks, he expressed his pleasure that this occasion had been chosen to welcome him: while the usual anniversaries are determined by external circumstances, often by chance, the date on which one begins one's academic work is closely linked to one's personality.]

Clinical Neuroscience

[The relationship between lipids in the brain and blood]

BALÓ József

[Changes in the ratio of lipids in the blood to each other, or other changes in the blood, may be followed by abnormal changes in the brain. On the other hand, primary diseases of the brain may cause abnormal changes in the lipids of the blood. Austin's finding that in metachromatic leukodystrophy metachromatic staining lipoids are excreted in the urine is an important finding in the diagnosis of neurological diseases.]

Clinical Neuroscience

[The works of Károly Schaffer on suggestion and reflexes]

ANGYAL Lajos

[Schaffer summarises his final conclusions in the following sentences : 1. Intrahypnotic contractures are brain reflexes, but they can be influenced associatively. 2. The extent of a negative hallucination does not remain confined to the corresponding sensory cortical area, but spreads over the whole of the same hemisphere, with varying degrees of intensity. 3. Hysterical stigmata (deafness, blindness, anaesthesia, etc.) are not caused by dynamic damage to the corresponding central sensory areas, but are a disturbance (delusion ?) of the associative mechanism. ]

Clinical Neuroscience

[Alzheimer's disease-like fibril lesion in circumscribed brain atrophy]

TARISKA István

[The author discusses the pathophysiological and histopathological differential diagnosis of Pick's disease and Alzheimer's disease. He describes a case with a slow progression over 6 years, first classified as Pick's disease on the basis of moral personality changes, spontaneity, vocabulary loss and lack of disease insight, and later as Alzheimer's disease on the basis of dementia, mild aphasia and motor dysfunction. Asymmetric and eunomic cortical atrophy of the brain, as well as sparing of the projection areolae, the high number of AFEs in the atrophied cortex and the integrity of the fronto-pontine pathway were considered from the perspective of the possibility of Alzheimer's disease. Histochemical examination of AFE did not allow him to reach a definite conclusion. Although the author considers Pick's disease and Alzheimer's disease to be independent nosological entities, he rarely considers their symptomatic confusion to be possible.]

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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.

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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

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

EVRAN Sevket, KATAR Salim

Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.

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[Vaccines against COVID-19 pandemic]

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[The rapidly spreading SARS-CoV2 respiratory virus has evoked an epidemic with serious aftermath around the world. In addition to the health effects, the global economic damage is actually unpredictable. At the same time, the pandemic has launched a series of unprecedented collaborative scientific research, including the development of vaccines. This study summarizes up-to-date information on vaccines, immune memory, and some emerging clinical effects.]

Lege Artis Medicinae

[Focus on Lege Artis Medicinae (LAM)]

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[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.]