Clinical Neuroscience

[Hungarian Society of Neurologists and Mental Doctors]

CSANDA Endre

JULY 20, 1994

Clinical Neuroscience - 1994;47(07-08)

[Themes for the Congress of the European Federation of Neurological Societies (EFNS), 24-26 November 1994.]

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Clinical Neuroscience

[The history of Hungarian neurology (Part II)]

BEKÉNY György

[1935-1945: László Benedek and his time]

Clinical Neuroscience

Personality traits in scheuermann's disease - a controlled study

SOMHEGYI Annamária, JÁNOSFI Zsuzsa, BALÁZS István, RATKÓ István

High muscle tone in various muscle groups is typical in Scheuermann's disease, and secondary fibromyalgia often occurs in young adults after this condition. There are clinical observations of emotional-psychological disturbances in adolescents with Scheuermann's osteochondritis. For these reasons it was of interest to determine whether any particular personality traits were manifested in adolescents with Scheuermann's disease, that - in addition to the spinal pathology – would contribute to the high muscle tone. Using four personality diagnostics tests (Brengelmann's questionnaire, Taylor's anxiety scale, Neurosis Scale, Lüscher's 8-colour test) we investigated the emotional life of 52 adolescents with Scheuermann's osteochondritis and that of 52 matched healthy controls. Test results of the two groups were statistically evaluated using chi square tests, with a significance limit of p < 0.05. The most striking difference between the two groups was that the patient's volitional and diffuse psychic tensions were increased (c = 1.00 and p < 0.0005) and, at the same time, were accompanied by a significant deficiency of the means to discharge them ( c = 1.00 and p < 0.0005). The results indicate that Scheuermann-patients do not answer to the stresses of everyday life in the usual way but by an increase in muscle tone. Their muscles act as their stress organ. This - in addition to the spinal pathology - may contribute to the tightness of their muscles and might be considered as a possible predisposing factor to later secondary fibromyalgia. Therapy should seek to interrupt this vicious circle by complementing regular exercise with relaxation techniques.

Clinical Neuroscience

Management proposal for the treatment of acute odontoid fractures

RÓBERT Veres, GILES Hamilton Vince

Optimum management of the acute odontoid fracture has always been extensively discussed. Nowadays, with the availability of new operative techniques such as anterior odontoid screw fixation and the posterior C1-C2 transarticular screw fixation the management policy has to be reevaluated. The authors review 115 cases of type II and type III acute odontoid fractures admitted to the National Institute of Traumatology in Budapest between 1980 and 1990. For study purposes a modified Anderson-D'Alonzo classification was introduced allowing a more detailed description of the fracture components and thus enabling to find more clear guidelines for treatment planning. The patients were treated with: a. various types of non-Halo external fixation in 27 cases; b. Halo immobilization in 13 cases; c. various surgical procedures resulting in a loss of atlantoaxial joint function in 22 cases; d. anterior odontoid screw fixation in 53 cases. Modern therapy should be focused on preserving the function of the atlanto-axial joint whenever possible. This can be sufficiently achieved using anterior odontoid screw fixation. However, the main determining factor in the choice of the appropriate treatment - providing the ligaments are intact - is the direction and course of the fracture line. If the fracture line runs horizontal or oblique posterior anterior screw fixation is the most favourable treatment. On the other hand if the fracture line runs oblique anterior Halo immobilization or C1-C2 posterior fixation is the method of choice.

Clinical Neuroscience

[CT guided stereotactic radiosurgical treatment of cerebral metastatic tumors]

FEDORCSÁK Imre, HORVÁTH Ákos, KONTRA Gábor, SLOWIK Felicia, OSZTIE Éva

[Radiosurgery has in recent years been used with promising results in the treatment of cerebral metastases. Between July 1991 and January 1993 we treated 23 patients harbouring brain metastases (39 lesions) with our Linear accelerator based radiosurgery system. The median tumor size was 1.9 cm (1.0-3.0 cm) and the median delivered dose was 18.7 Gy (13–25Gy). Follow up CT scans at 4 months showed a complete remission or greater than 50% tumor volume reduction in 19 patients (82%). No change was noted in 2 patients (9%), and after a transitory decrease an increase in tumor size was seen in 2 patients (9%). Follow up time was minimum 6 month (or till death), and maximum 26 months. We have lost 13 patients during the study, and the cause of death was neurologic in only 2 cases. Our experience supports the cumulating evidence that radiosurgery is an effective treatment for metastatic brain tumors, is well tolerated by the patients, and can be applied also in cases where open surgery can not be performed.]

Clinical Neuroscience

[Real time and color doppler sonographic investigation in cases of fetal hydrocephaly and cerebral hemorrhage]

JAKOBOVITS Ákos, JÖRN Hendrik

[In three hydrocephalic and one fetus with intra- and periventricular hemorrhage real time imaging was used to identify cerebral changes. Color flow imaging was used to identify the fetal middle cerebral and umbilical artery for subsequent pulsed Doppler sonographic studies. Cerebral blood flow patterns of hydrocephalik fetuses seem to differ individually from case to case presenting normal, increased and decreased velocity waveform indices. The resistance index and pulsatility index of the middle cerebral artery in case of cerebral hemorrhage increased with advancing worsening of the fetal state of health. Loss of diastolic cerebral or umbilical flow followed by retrograde flow during diastole antenatally could be a bad sign prognostically. ]

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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

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

Clinical Neuroscience

[The role of sleep in the relational memory processes ]

CSÁBI Eszter, ZÁMBÓ Ágnes, PROKECZ Lídia

[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

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.