Clinical Neuroscience

[MULTIPLE SYSTEM ATROPHY: THE BEGINNING OF A NEW ERA IN THE HISTORY OF NEURODEGENERATIVE DISEASES]

PAPP Mátyás, KOVÁCS Tibor

SEPTEMBER 30, 2006

Clinical Neuroscience - 2006;59(09-10)

[Multiple system atrophy (MSA) belongs to the neurodegenerative diseases of the nervous system, but it is different from them in many aspects: it has no familiar form and no genetic factor was identified in the pathomechanism. Its neuropathology is unique too, because oligodendroglial cells are harbouring the main pathological burden. It was described in MSA that there is no elective neuronal degeneration in neurodegenerative disorders: the glial cells show the same patochemical and structural abnormalities as found in the neurones. The discovery of the glial cytoplasmic inclusions, as a pathognostic marker for MSA, has directed attention to the glial cells in other neurodegenerative disorders. As a result of this, there are several neurodegenerative diseases nowadays in which glial inclusions were described, similar to the neuronal inclusions in their structural and biochemical properties and some of them became the diagnostic marker of the disease. In our review we summarize the clinical features, the history and the neuropathology of MSA and we discuss its special features.]

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Endogenous psychopharmacology]

BÁNKI M. Csaba

[Endogenous psychopharmacology 2006;59(09-10)]

Clinical Neuroscience

[TEMPORAL LOBE EPILEPSY - STATE OF ART REVIEW]

HALÁSZ Péter, FOGARASI András

[The temporal lobe epilepsy (TLE) is the most important kind of partial epilepsy both from practical and research point of view, where studies brought many new results in the last years. This article is a state of art review with a special emphasis on medial temporal lobe epilepsy (MTLE). We show the clinical symptoms, EEG and neuroimaging signs, the psychiatric co-morbidities and psyhosocial consequences. Etiological factors, among them hippocampal sclerosis and hippocampal reorganisation is assessed in a more detailed form. The possibilities of pharmacological and surgical treatment are also shown, together with the brief outline of the Hungarian situation. TLE is presented as a model for the development of partial epilepsies.]

Clinical Neuroscience

[PREDICTIVE FACTORS OF TEMPORAL LOBE SURGERY]

KELEMEN Anna, RÁSONYI György, SZŰCS Anna, FABÓ Dániel, HALÁSZ Péter

[The most effective type of epilepsy surgery in adults is temporal lobe epilepsy (TLE) surgery. Three quarter of the patients become seizure free, however the remaining patients experience seizures after resection. In our study we analyzed retrospectively the possible electro-clinical, neuroimaging and surgery-related outcome predictors in 94 adult patients who had anterior temporal lobectomy (ATL) from the material of Epilepsy Centre of the National Institute of Psychiatry and Neurology, Budapest since the beginning of the surgery program in 1989 until 2001. Three outcome endpoints were chosen: the seizure status at the last visit, the longest seizure free period and the time to the first non-acute postoperative seizure. The predictors were assessed by multivariate and Cox regression methods. After one year of surgery 72% of the patients were seizure free, after two years 67% and after five years 59%. Factors predicting favorable outcome in TLE surgery were: typical temporomesial aura, strictly unilateral interictal anterotemporal spikes, unilateral ictal onset, slow contralateral propagation, hippocampal sclerosis (HS) as etiology. Factors predicting poor outcome in TLE surgery were: increase in seizure frequency in the last two preoperative years, presence of preoperative psychiatric disturbances, ictal contralateral propagation, MRI lesion distant from the surgery site, incongruency of data of preoperative investigations, postoperative sequels and non-HS type MR residuum.]

Clinical Neuroscience

[CONGRESS CALENDAR]

[congress calendar 2006;59(09-10)]

Clinical Neuroscience

[We do need change in the education of neurosurgeons]

CSÓKAY András

All articles in the issue

Related contents

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

[LAM 30: 1990–2020. Facing the mirror: Three decades of LAM, the Hungarian medicine and health care system]

KAPÓCS Gábor

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.]

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.]