Clinical Neuroscience

[Society of Pediatric Neurology, Neurosurgery, Child and Adolescent Psychiatry]

GYÖRGY Ilona, NEUWIRTH Magda

JANUARY 20, 1993

Clinical Neuroscience - 1993;46(01-02)

[Preliminary themes for the 1993 Congress. Summer camp for children with epilepsy.]

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Clinical Neuroscience

[Acute neurosurgical management of severe thoraco-lumbar spinal injuries]

ZSOLCZAI Sándor, PENTELÉNYI Tamás, TÚRÓCZY László, VERES Róbert

[Authors show their experiences with up-to-date segmental stabilization methods in the acute neurosurgical management of severe thoraco-lumbar spinal injuries. Among the 134 acute operations during 5 years with at least 1 year follow-up 81 were performed by Fixateur Interne (AO-ASIF, W. Dick), and 53 by angle-stable posterior plate-fixation (Steffee or Eger plates). Reduction, decompression and stabilization were achieved by these instrumentations. Results are evaluated from the points of view of neurological recovery, bony union, restoration of patients comfort and complications. Also the principles of modern management of spine-injured patients, developed through a long evolution in the last decade, are reviewed. It is stated that both segmental stabilization methods were used as routine, and they were suitable for the treatment of the most of severe thoraco-lumbar spinal injuries. Results of these methods are much better than those of the long-rod systems, but on condition that emergency neurosurgical treatment should be done in the first 6-8 hours together with early skilled and competent rehabilitation in a well trained center.]

Clinical Neuroscience

[Relation of acute and long-therm care of the spinal-cord-injured patients in up-to-date management]

KLAUBER András, ZSOLCZAI Sándor, PENTELÉNYI Tamás, MOLNÁR Ferenc

[The acute emergency management of the spinal-cord-injured patients is one of the most important steps in the long-lasting complex treatment which has great significance for the final results. The method of primary neurosurgical operation must be chosen with regard to the special aspects of subsequent rehabilitation. All conditions of emergency surgery have to be assured during the first 6-8 hours. According to the experiences of the last two decades the problems of the management of spinal-cord-injured patients originated in the shortage of financial sources, theoretical concepts and organization, lack of interest and scientific information. Even now there is no spinal-cord-injured center in Hungary which would provide for the complex management including primary neurosurgical treatment and all details up till the end of paraplegic rehabilitation. Primary treatment (either operative or conservative) and rehabilitation are performed in separate departments. Though there is a significant development in emergency spinal surgery during the last years (and it is a pity this can not be seen in rehabilitation in Hungary), it is still an important demand that emergency surgery should be performed with rehabilitation aspects. Until we have no proper financial conditions to organize spinal centers for both emergency and rehabilitation treatment, our most important task is to develop a unified rehabilitational view of all specialists involved in the complex treatment of spine-injured patients.]

Clinical Neuroscience

[Tethered cord syndrome in adults]

VÁRADY Péter, BENOIST György, PÁSZTOR Emil

[The tethered cord syndrome is resulted from the abnormally low position and traction of the conus medullaris in the lumbosacral region, that leads to progressive dysfunction of the caudal spinal segments. It originates from various ectodermal abnormalities, all due to errors of embryonic development in the 3rd-4th weeks. The disorder is manifested by progressive motor, sensory and trophic deficits in the legs and by incontinence. The adult onset is rare. The MRI is the most important procedure for pathoanatomic evaluation. The surgical aim is to stop progressivity and to cure the reversible deficits. We report our six cases and present the data of the literature concluding the caracteristics, the surgical indications and outcome of the tethered cord syndrome in adults.]

Clinical Neuroscience

[Operative treatment of craniocervical instabilities caused by rheumatoid arthritis]

TURÓCZY László, KENÉZ József, VERES Róbert, NAGY Aladár, PÁSZTOR Emil

[We have investigated and operatively treated 30 patients with craniocervical instabilities caused by rheumatoid arthritis. The evaluation of this patient-material seems to be important, because the indications of operations in the different stages of the disease are not completely clear, according to our knowledge. The atlanto-axial instabilities at the beginning stage, when the serious neurologic signs are absent, can be treated with low risk dorsal stabilization, while we believe the advanced instabilities need combined neurosurgical approach for successful treatment. We must emphasize the importance of detailed early diagnostics, and the prevention in neurosurgical approaches, in accordance of the opinions, expressed in different contributions in the literature, dealing with the subject. In 20 cases we had to perform combined surgical interventions, and only in 10 cases, the simple dorsal fixation proved to be satisfactory, which fact shows, that we got patients mainly in more serious stages. We detected a new sign ("ghost tumor") in the natural course of the disease.]

Clinical Neuroscience

[Our experiences in the conservative treatment of the compression fractures of the thoracolumbar spine]

URBÁN Ferenc, TAKÁCS Ferenc

[The authors at the DOTE Department of Traumatology made follow-up examinations of 136 patients treated for compression fractures of the thoracolumbar spine. 63 patients (46,3%) were treated with plaster corset, 73 patients (53,7%) with modified Magnus method. They compared the two methods of treatment for their possibilities and effectiveness. They used plaster corset after reposition in females under 55 years of age and in males under 60 years of age, if the patients' physical and general condition were suitable and the compression was more than 10 degrees. 47,9% of the patients treated functionally recovered well and 31,5% were in a satisfactory state. And 47,6% of the patients treated with plaster corset recovered well and 46,0% recovered satisfactorily. While in the first group i.e.those treated functionally, 17,8% of the patients had severe pain, only 6,4% of the patients treated with corset had similar pain. The authors ascertain that as the anatomical reposition of the fractures is not possible indefinitely, the insertion of the plaster corset when the compression exceeds 10–15 degrees is more suitable. When the deformity decreases, the subjective complaints of the patients decrease too. In the case of small compression, functional treatment is recommended and the patient must lie in bed to alleviate the pain. Both the methods used under appropriate conditions are useful and complement each other. ]

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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.

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

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.