Clinical Neuroscience

[Hungarian Society of Neurologists and Mental Doctors]

SZÉL István

JULY 20, 1993

Clinical Neuroscience - 1993;46(07-08)

[Report of the two-day event "Neurorehabilitation - Practice and Perspectives", held in mid-May 1993, organised jointly by the MIET and the MRT Working Group on Neurorehabilitation and the Austrian Neurorehabilitation Society.]

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Clinical Neuroscience

Pathology of the vestibular system

GOSZTONYI György, ZILL Edith

The vestibular end organ, in spite of its small size, has extremely rich interconnections with other parts of the nervous system. The vestibular system can be damaged at the end organ, along the vestibular nerve, in its brain stem representations and in its cerebellar projections. The nature of the pathological process damaging the vestibular system is manifold: neoplastic, inflammatory, vascular, nutritional and degenerative. Neural complications of AIDS may also involve the vestibular system. The lesions may be focal, multifocal and diffuse. While in the past the results of neurootological examinations could only be correlated with post mortem findings, NMI opens new horizons for neurootological and topoanatomical correlative studies.

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[Vascular surgery in the prevention of stroke]

MOGÁN István, NEMES Atilla, NAGY Zoltán

[In Hungary the prevalence of cerebrovascular disorders has increased so that each year vascular reconstruction surgery is needed in 2800 cases. However, only a quarter of these eligible patients are operated. After a thorough examination if all the indications for carotid surgery are met, more and more patients will end up in vascular reconstruction units. Angiography and surgery are recommended if the carotid artery stenosis is asymptomatic and is more than 90%. Also, under special conditions an asymptomatic carotid stenosis may caused by indicate surgery (before coronary by-pass operation, etc.). Another indication is a transient ischemic attack, if carotid artery lesion and the stenosis is above 70%. Ulcerated plaques also need surgery because they are a likely source of emboli. After stroke surgery may be necessary if the angiologic status is unstable and further ischemic events, that may lead to disability are expected. An acute stroke rarely calls for surgery. In contrast to this, immediate surgery is needed after repeated, TIA, or crescendo TIA because the risk of stroke is very high in these cases. Finally, sometimes surgery is indicated because of the occlusion of internal and common carotid artery. With vertebrobasilar vascular reconstruction, we do not have enough experience. Cerebrovascular syndromes due to supraaortic vascular lesions are other indications for reconstruction surgery. However, surgery is never a satisfactory substitute for pharmacological treatment.]

Clinical Neuroscience

[Transcranial doppler detection of cerebral vasospasm following aneurysmal subarachnoid haemorrhage]

BARZÓ Pál, BORDA Lóránt, VÖRÖS Erika, KISS Mariann, BODOSI Mihály, DÓCZI Tamás

[In 22 patients with subarachnoid hemorrhage secondary to ruptured intracranial aneurysms serial neurological evaluations, transcranial Doppler examinations and computer tomographic scans were performed. Transcranial Doppler flow velocities were significantly elevated for the group with vasospasm on posthemorrhage day 2. The maximum blood flow velocities were recorded between days 9 and 18, with normalization occurring within the following 3 weeks. Increase in velocity preceded clinical symptoms and could therefore be used as a prognostic factor for the management of patients with subarachnoid hemorrhage. The data also indicated that the extent and location of blood in the subarachnoid space determine the severity and location of vasospasm.]

Clinical Neuroscience

[Spinal lipomas and their surgery]

PARAVICZ Ervin, TÓTH Katalin, TORMA Albert

[18 intraspinal lipomas were operated, mostly in childhood. Surgery seems to be indicated unavoidably in view of preoperative clinical progression and postoperative improvement. Prior MR investigation was indispensable to the surgical procedure.]

Clinical Neuroscience

[Elective extra-intracranial arterial bypass in the treatment of giant aneurysms of the carotid artery]

BODOSI Mihály, DÓCZI Tamás, BARZÓ Pál

[Clinical details are presented of 5 patients with a giant carotid aneurysm in whom both the occlusion of the aneurysm and the parent internal carotid artery were performed with an extra-intracranial arterial bypass. In the first case ligature of the giant carotid-ophthalmic aneurysm narrowed the parent artery critically and hemiplegia developed. The bypass operation did not improve the clinical outcome. In the second case the occlusion of the aneurysm was performed after an extra-intracranial anastomosis and in spite of the severely narrowed carotid artery the postoperative course was uneventful. In three cases of giant intracavernous aneurysm the occlusion of the carotid artery on the neck and just proximal to the ophthalmic artery was performed in the presence of an arterial bypass. All of the anastomoses were patent and no ischemic event developed during the follow up period. On the basis of these experiences the authors suggest that, if the preoperative tests (TCD, EEG, SPECT) reveal impending ischemic lesion after carotid compression, surgery should be performed with the combination of extra-intracranial bypass.]

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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

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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.

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

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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.

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[The role of sleep in the relational memory processes ]

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[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.]

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

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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.