[Intracerebral hemorrhages and their neurosurgical management in early infancy]
PARAICZ Ervin1, KÓNYA Eszter1
JANUARY 20, 1994
Clinical Neuroscience - 1994;47(01-02)
PARAICZ Ervin1, KÓNYA Eszter1
JANUARY 20, 1994
Clinical Neuroscience - 1994;47(01-02)
[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
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
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
[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
[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. ]
Clinical Neuroscience
[OITI C3-11 monoclonal antibodies were produced against GFAP positive human glioblastoma tumour cells. The specificity of these antibodies was tested on different type of brain tumours and on normal adult brain both on tissue cultures and paraffin-embedded sections. Such OITI C3-11 monoclonal antibodies reacted with 16 of 18 malignant and 1 of 6 benign gliomas but did not react with meningioma, pituitary, adenoma, metastatic brain tumours and normal adult brain tissue.]
Clinical Neuroscience
The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histological examination excluded mycotic etiology of the aneurysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.
Clinical Neuroscience
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.
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
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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