Clinical Neuroscience

[Hungarian Society of Neurologists and Mental Physicians SZOTE neurological round table]

MAY 20, 1997

Clinical Neuroscience - 1997;50(05-06)

[Extrapyramidal diseases. The activity of SZOTE Neurology "Extrapyramidal Outpatient Clinic" (diagnostic and therapeutic principles). Autonomic nervous system disorders and their treatment in Parkinson's disease. Options for neuroprotective treatment in Parkinson's disease. Therapy for epilepsy. Strategy for the use of antiepileptic drugs. Relationship between antiepileptic drugs and the endocrine system. Teratogenic effects of antiepileptic drugs. Difficulties in caring for patients with epilepsy in some cases. Cases of untyped leukodystrophy in the setting of symptomatic focal epilepsy. Evaluation of surgical outcome of epilepsy using quality of life measurement. Interactions between antiepileptics and oral anticoncipients: the experience of a survey.]

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Clinical Neuroscience

[Effects of Propofol and Thiopental on median nerve somatosensory evoked potentials and cerebral blood velocity]

MÉSZÁROS István, KASÓ Gábor, BÜKI András, HUDVÁGNER Sándor, PFUND Zoltán, DÓCZI Tamás, NAGY Ferenc

[Effects of induction doses of thiopental and propofol on median nerve somatosensory evoked potentials and cerebral blood flow velocity were investigated in 50 patients suffering from degenerative lumbar spine diseases before operation and during anaesthesia. The propofol influences the cerebral electric activity and the blood flow velocity to a less extend than thiopental. Based on our results we prefer to employ propofol during neurosurgical procedures when using intraoperativ somatosensory evoked potentials and/or transcranial Doppler monitoring. ]

Clinical Neuroscience

[Three dimensional time of flight magnetic resonance imaging in trigeminal neuralgia]

VÖRÖS Erika, SZENTGYÖRGYI Réka, KUNCZ Ádám, MENCSER Zsolt, KARDOS Lilla, MILASSIN Péter

[The purpose of our study was to evaluate the role of three dimensional time of flight magnetic resonance angiography in detection of neurovascular compression in patients with trigeminal neuralgia. 53 patients (26 males, 27 females mean age 57 years) with trigeminal neuralgia underwent 3D TOF MRA. Examinations were performed on 0.5 T Elscint Gyrex V Dlx equipment. The imaging parameters were 33-38/9/25 TR/TE/flip angle with 30-50 mm slab thickness and 1-1.5 mm slice thickness. Contrast material was administered in every case. Maximum intensity projection and thin slice reconstruction (pixel by pixel) were performed in three standard directions (axial, coronal and sagittal). To evaluate the presence or absence of vascular contact, we used both the source slices and reconstructed pictures. Vascular contact with the trigeminal nerve in the entry zone was identified on the symptomatic side in 26 cases (superior cerebellar artery in 20, superior cerebellar artery and vein in one, anterior inferior cerebellar artery in 2, basilar artery or a vein in 1-1 case) and on the asymptomatic side in 3 cases (superior cerebellar arteries). No contact was detected in 24 patients. The examination was not of diagnostic value in three cases, because of head motion artefacts. Veins were better visualized on the contrast pictures. Microvascular decompression sec. Janetta was performed in 9 cases. The surgical and neuroradiological findings were identical in every case. Complete pain relief or significant diminshing of the symptoms were achieved following surgery in all patients. 3D TOF MRA is a useful method in demonstration of vascular contact with the trigeminal nerve at the entry zone, which is valuable information in planning surgical treatment for patients with trigeminal neuralgia.]

Clinical Neuroscience

[Uremic polyneuropathy - clinical and electrophysiological analysis]

PFUND Zoltán, CZOPF József, NAGY Ferenc

[Uremic polyneuropathy is a consequence of chronic renal failure, and is one of the symptoms of the uremic syndrome. Its etiology is not well known, several neurotoxins are presumed as pathogenic factors. Both axonal lesions and demyelinization are involved in the development of neuropathy 66 electrophysiological studies (EMG, ENG) in 57 uremic patients were performed in our department between 1978 and 1988. The correlation between clinical and electrophysiological findings are discussed. Clinical or neurographic and myographic signs of neuropathy were found in 50/57 of the patients. The number of electrophysiological signs changes parallely with the severity of uremic neuropathy. There was significant correlation between the decreased plasma calcium levels, the elevated plasma phosphate levels, and the severity of neuropathy. The electrophysiological symptoms are more frequent in cases of rapidly progressive neuropathy. Peripheral nerve pathophysiology reveals a combination of segmental demyelinization and axonal degeneration.]

Clinical Neuroscience

[Mental disorders after stroke (treatment of post-stroke depression with moclobemide)]

VARGA Dániel, PÉK Márta, NAGY Zoltán

[Post-stroke states are usually characterized by residual neurologic symptoms and movement performance, while psychological disturbances remain hidden. Stroke patients frequently present mood disturbances, cognitive decline, anxiety disorders, sometimes serious schizophorm and paranoid states. Among them, post-stroke depression is the most common and possibly amenable form to therapeutic intervention. Depression has negative effect on rehabilitation, quality of life and even long term survival. Biological, psychological and social factors play an important role in the etiology of post stroke depression. The psychotherapeutic approach is so far undeveloped; considering drug therapy, in the past decade tricyclic drugs have been replaced by newly developed second generation antidepressants with lower side-effects profile. In our experience 6-week treatment with moclobemide in 33 patients resulted in symptom reduction in 80%. Appropriate therapy extends the possibilities of rehabilitation, improves the quality of life, and reduces mortality.]

Clinical Neuroscience

[The genetic diagnosis of leber's hereditary optic neuropathy]

HORVÁTH Rita, ERIC A. Shoubridge, KATHERINE Fu, SOMLAI Judit, HAJDA Márta, KARCAGI Veronika, KOMOLY Sámuel

[We observed the occurence of Leber's hereditary optic neuropathy associated mitochondrial point mutations in patients with bilateral optic neuropathy. DNA samples isolated from peripheral venous blood by the phenol/chloroform method were tested with the help of PCR for the pathogenic Leber's hereditary optic neuropathy associated mitochondrial point mutations. From 14 patients suffering from bilateral visual impairment Leber's hereditary optic neuropathy associated mitochondrial point mutations were found in 5 cases. The patients were previously treated with optic neuritis or with toxic optic neuropathy. By testing the relatives of the patients some asymp tomatic Leber positive cases were observed, too. The diagnosis of Leber's hereditary optic neuropathy is based on the occurence of mitochondrial point mutations. To test for these mutations can help in the clinical practice in diagnosing bilateral optic neuropathies. In patients with the pathogenic Leber mutations the development of the clinical symptoms does not occure necessarily, however the other mechanisms determining the clinical picture are not known so far.]

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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

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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.