Clinical Neuroscience

[Hungarian Society of Neurologists and Mental Doctors]

JANUARY 20, 1994

Clinical Neuroscience - 1994;47(01-02)

[Department of Neurology, Semmelweis University of Medicine and II. After a 5-year break, the Institute of Pathology held a clinicopathology conference at the University II. s. In the classroom of his Gynecology Clinic on November 5, 1993, at 1 p.m. Collective Report on the 9th World Congress of Psychiatry June 6-12, 1993 Rio de Janeiro.]

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Clinical Neuroscience

The changing face of neuroscience

LORD WALTON of Detchant

This paper is based upon three lectures one given in Australia in a symposiom in honour of Professor James Lance on his retirement, another delivered to the Russian Academy of Medical Sciences on 26 January 1993 and published in the procceedings of that annual symposium of the Academy. It is reproduced here with permission.

Clinical Neuroscience

The treatment of cluster headache

KARL Ekbom

Cluster headache attacks occur in series, lasting for weeks or months, that are separated by remission periods. However, in less than 15 %, patients suffer from a chronic syndrome, that is regular attacks for one year or longer. The spontaneous course of cluster headache may cause some problems when evaluating clinical trials. For instance, it may be difficult to decide whether an observed improvement is due to effects of the trial drug or to a spontaneous remission. Most studies published hitherto have used an open treatment app- roach – using a concomitant treatment – which is understandable, because the placebo effect has been regarded as being small in cluster headache. In fact, there are in the previous literature only few well-designed, randomised, double-blind clinical trials.

Clinical Neuroscience

[Visual P300 and early components in chronic schizophrenic patients (clinical, neurocognitive and biochemical correlates)]

BARTKÓ György, KUNDRA Olga, BOLLA Mariann, ZÁDOR György, SÁNTA Zsuzsa, HORVÁTH Szabolcs, ARATÓ Mihály

[P300 and early components of the visual event related potentials were compared in 26 chronic schizophrenic patients and 20 healthy subjects. The correlation between visual evoked response and clinical, neurocognitive, biochemical variables was analysed in schizophrenic group. Event related potentials in response to rare visual stimuli were recorded from central and occipital sites and 20 electrophysiological parameters were determined. Reaction time and proportion of correct recognition were also detected. The schizophrenic patients showed significant reduction is P300 amplitude. Differences in other components between groups were also demonstrated. The seven most important parameters were evaluated by discriminant analysis. The prolonged negative components latency and delayed reaction time suggest that the stimulus classification process is slower in schizophrenics, Using canonical correlation analysis three factors were found to be significant. The data showed that electrophysiological abnormality was highly correlated with chronicity of the illness, severe psychopathological features and cognitive deficit but was uncorrelated with negative symptoms and serum dopamine-beta-hydroxylase activity. These findings are compatible with other studies suggesting visual P300 has the characteristic of a state marker in schizophrenia.]

Clinical Neuroscience

[Intracerebral hemorrhages and their neurosurgical management in early infancy]

PARAICZ Ervin, KÓNYA Eszter

[The number of spontaneous intracerebral hemorrhages over early infancy has increased in the last years in Hungary. As possible cause maybe the lack of Vitamin K prophylaxis in our 19 cases. With regard to management mostly the semiinvasive treatment was succesful, the external drainage of the hemorrhage and CSF. Craniotomy was indicated only in 3 patients. The functional result was good in the majority of cases.]

Clinical Neuroscience

[Provocative myasthenia gravis and myasthenic syndromes]

SZOBOR Albert, KLEIN Magda

[Myasthenia gravis as a disease entity has long been known. With the inclusion of the paraneoplastic myasthenia syndrome, a wider area was encompassed by the disease, which became still wider by the description of different myasthenic syndromes in childhood. Recently quite a few provocative factors became known which can cause myasthenia gravis or some similar syndromes. One such-prominent-factor is D-penicillamine a drug widely used in rheumatology practice. A great number of cases were studied involving the provocative factors: D-penicillamine, the infectious diseases, drugs and other possible causes. After provocative factors myasthenia gravis disease, myasthenic syndrome with different clinical course and transitive myasthenic reaction with spontaneous remission may develop. The ability to distinguist between these conditions is important from both the therapeutic and prognostic points of view. Mild not known, or not recognized myasthenia gravis can in some cases be diagnosed by careful neurological examination and diagnostic tests. ]

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Cases of inborn errors of metabolism diagnosed in children with autism

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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

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.

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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.]

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.