Clinical Neuroscience

[Examination after the first epileptic seizure]

RÓZSAVÖLGYI Margit1

SEPTEMBER 20, 1997

Clinical Neuroscience - 1997;50(09-10)

[106 patients were examined after the first epileptic seizure; they were divided into two groups based on the clinical data. The two groups were: the group of provoked seizure and the group of non-provoked (isolated) seizure. 95 patients were in these two groups. The others showed focal epileptiform EEG alterations or generalized epileptiform activity without seizures and, because of the very low number of patients, the author excluded them from the study. A questionnaire was used to study and to search for the history of the disease and provocative factors. The familial occurrence of epilepsy was quite often among the patients, similar to primary generali zed epilepsy (17.9%). There were no important previous illnesses, except the provoked seizure. The provocative factors played a significant role in the occurrence of the first seizure. The different provocative factors appeared in greater numbers in the different groups. The anxiety occurred as a possible provocative factor in the group of non provoked seizure. The EEG after the first epileptic seizure showed epileptiform activity in the lower percentage than was predictable from the literature. The mobile long term EEG was shown to be an effective method of examination for epileptiform activity.]

AFFILIATIONS

  1. Magyar Honvédség Központi Honvédkórház, Neurológiai Osztály, Budapest

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Clinical Neuroscience

Management of cavernous angiomas of eloquent brain areas by means of image (MRI or CT) - guided key-hole craniotomy

KÖVÉR Ferenc, DÓCZI Tamás, VON JAKO C., BALÁS István

Details of neurosurgical management of four patients suffering from intractable epilepsy caused by cavernous angiomas of subcortical eloquent brain areas are presented. Two of four cavernomas were not visualized by CT or angiography but only by MRI. MRI or CT stereotactic image-guided key-hole craniotomy and resection of the lesion from the dominant anterior central gyrus (2 cases), from the dominant superior temporal gyrus or from the dominant supramarginal gyrus were performed without any morbidity. The postoperative hospital stay was 3-5 days and all four patients experienced improved seizure control. We conclude that stereotactic image-guided key-hole craniotomy and microsurgical resection offer significant advantages in the treatment of cavernous malformations. Surgical indications include medically refractory epilepsy, intracerebral haemorrhage and/or progressive neurological deficit.

Clinical Neuroscience

[Interpretation of the resoults of DNA image analysis in astrocytomas - biology and prognosis]

SZŰCS István, LEEL-ŐSSY Lóránt, KINDLER Miklós

[Ploidy and proliferative activity of 72 astrocytomas (except glioblastomas) were examined with nuclear DNA content by computed image analysis. The majority of samples originated from paraffin embedded material but they were partly obtained from surgical biopsy by printing or smear technique. The 72 astrocytomas were classified according to Kernohan's grading system. Different areas of tumours were evaluated according to their ploidy (euploid, diploid, aneuploid and heterogenous ranges), proliferative parameters and DNA indices. The transitional zone sometimes revealed higher proliferative index with euploidy. The DNA distribution showed greater population even in the case of moderate malignancy. Uniform – single population - DNA distribution characterized the benign type in 79% with DNA index within the diploid range. The remaining 21% had aneuploidy with another population (heterogenous). The higher the malignancy the greater the population may be found with the higher percentage of S phase cells. The mitotic forms displayed higher DNA index as well as G 2 phase even in the transitional zone of the tumour. The heterogeneity with higher percentage of S- phase correlated well with the histological type of the tumour. The follow-up study of some astrocytomas sometimes did not show a good correlation with the DNA values. However a good correlation was found between the number of cells with higher S and G 2 phases and the type of histogram in the majority of cases studied. The different parts of the tumours also varied in these respects, which should be taken into consideration during needle biopsies.]

Clinical Neuroscience

[Suggestion for new classification of ischemic stroke]

KOPA János, RÁDAI Ferenc, SZÁSZ Krisztina, REPA Imre, HUSZÁR Péter, BESZTERCZÁN Péter, GYŐRBÍRÓ Zsolt

[The classifications of ischemic damage of the brain which have been in use cannot give a suitable orientation about the pathoanatomical background of this disease. As a result of this, the authors investigated the data of 1000 cerebrovascular indoor stroke patients who had been investigated by CT or MRI. The clinical and radiological data were compared. Their conclusion was: more than 80% of the cerebrovascular diseases are ischemic lesions. All of the ischemic patients have some vascular disturbances. In the cases of all patients having some ischemic event (transient ischemic attack, reversible ischemic neurological deficit, prolonged reversible ischemic neurological deficit, completed stroke) there are enduring ischemic lesions which can be demonstrated by CT or MRI. Comparing the neurological signs and the CT or MRI findings of the patients we could establish two groups of ischemic lesions: solitaire and multiplex infarcts. They can be divided into 3 subgroups. Among the solitaire infarcts is subgroup S-1: the patient has no neurological sign and in the CT or MR pictures there is only one lesion under 10 mm in diameter. S-2: mild neurological signs with one infarcted lesion about 15 mm in diameter; as a partial lesion in the territory of a cerebral vessel; S-3: severe neurological signs with total or almost total damage in the territory of a cerebral vessel. Among the multiple infarcted patients there is subgroup M-1: the patient has no neurological sign, but in the CT or MR pictures there are many small (under 5 mm in diameter) infarcted zones; M-2: the patient has mild neurological signs and multiple infarcted zones (5-15 mm in diameter) can be seen in the CT, MRI picture; M-3: the patient has very severe neurological signs and/or dementia. The infarcted areas consist of small and one or more sites of partial or total damage in the territory of a cerebral vessel. This classification is better in showing the state of the patients and the pathoanatomical distur bances. The authors have used this classification in clinical practice without any problems for a year.]

Clinical Neuroscience

Analysis of IQ and genotype in duchenne and becker muscular dystrophy

LÁSZLÓ Aranka, PÓR Erzsébet, CSEPREGI Zsuzsa, ENDERFFY Emőke, RASKÓ István

The authors analysed the association of genotype and the intelligence quotient (IQ) examined with age dependent psychological methods (Wechsler, Vineland, Binet, Raven IQ tests) in 41 Duchenne (DMD), 2 intermediate MD, and 14 Becker type muscular dystrophic (BMD) patients (mean age2.5-37 y). ln 61.4% of the DMD/BMD exon deletion of dystrophin gene was found, while in 38.6% no deletion was detected. The assessment of the genotype was made by using multiple PCR method, examining the most frequent deletion of 18 exons and the muscle specific promoter. Among the deletion patients the distribution of mentol retardation was 28.5%, among patients with non deletion 22.7%. ln 21.4% of BMD patients and in 29.2% DMD patients mentol retardation was detected, the differences were not significant. Similar to the findings of other authors in 4 patients having exon deletions near the 5' end of the gene no mentol retardation was found, but in 14 of 30 patients having deletions near the 5' end of the gene there was. One patient with the largest deletion (exons 12-44) proved to be debil.

Clinical Neuroscience

[Diastematomyelia a rare dysraphic lesion of the spine]

KISS Marianna, BUZA Zoltán, VÖRÖS Erika

[A split cord malformation may be either diastematomyelia or diplomyelia. The former is characterized by two dural sacs and an osseous or osseocartilaginous septum, while the latter has two hemicords within a single dural sac and a nonrigid septum. Consequently, both require a specific neurosurgical technique. The featured case emphasizes the importance of radiological investigations in the differential diagnosis of these rare entities. The 25 year old woman experienced progressive worsening of sensation of her left foot, lower leg atrophy on the same side and gait disturbance. The findings upon physical examination were slight weakness of the left peroneal muscles, diminished touch sensation over the left foot and position sense in the left toes, and an absent ankle jerk reflex. SSEP of the left lower extremity yielded 3-5 ms P40 peak latency. The plain X-ray films and CT images revealed multiple congenital osseous anomalies: scoliosis, lumbalization of s 1 vertebra, hemiblock of the L 1-3 vertebral bodies, spina bifida at the Th 4-5, L 2-5 and S 1 and a bony median septum at L 2-3. The Il and T2 weighted axial, sagittal and coronal MR images showed sagittal spinal cord cleft with asymmetrical hemicords at L 2-4, and rejoining of them below 14. Low position of the conus, tethered cord and lipomatous infiltration of the filum were also detected. The earlier the detection of a split cord malformation, the better the surgical outcome. From the technical point of view, the preoperative classification with CT and MR is paramount.]

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Cases of inborn errors of metabolism diagnosed in children with autism

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Clinical Neuroscience

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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.

Clinical Neuroscience

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Clinical Neuroscience

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

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Clinical Neuroscience

[The Comprehensive Aphasia Test in Hungarian]

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[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]