Clinical Neuroscience

[Report]

OROSZ Éva

APRIL 01, 1959

Clinical Neuroscience - 1959;12(04)

[The author reports on the meeting of the French-speaking Society of Neurosurgery held on 2-3 December 1958.]

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Clinical Neuroscience

[Data on the cerebral hemisphere and lobes sclerosis]

TARISKA István

[It can be concluded that the extensive cortical damage that is the hallmark of hemispheric atrophies and lobar scleroses is, according to the literature and our own case studies, the result of an anoxic-vascular disturbance, which is particularly predisposed to occur as a consequence of a disturbance of the flow through relatively large pial arterial trunks or arterial border zones. This is associated with convulsions in the majority of cases, but convulsions must be accompanied by some other factor perhaps more pathogenetically important than convulsions ; respiratory disturbances, stagnant anoxia, fever, hypoglycaemia, immunobiological processes. ]

Clinical Neuroscience

[A case of pubertal psychosis ]

DRIETOMSZKY Jenő

[Authors have taken different paths, emphasising the dominance of different characteristics in relation to the pubertal period. It is generally accepted that this age is characterised by a breakdown of the previous relative somatic and psychic equilibrium, which, together with the heightened susceptibility to stimuli from the outside world, which is also observed at this age, may be the basis for a number of dangers, including undesirable, possibly pathological, personality development. A particular interpretation of this aspect is given by Ruckert, who says that "in this age, ... everyone has before his eyes the image of what he should be, not as an abstract formula of a categorical imperative, but as a plastic image, an entelechy of the ideal constitution of his own soul..."]

Clinical Neuroscience

[Diffuse CSF invasion of oligodendroglioma ]

BALAJTHY Béla

[In our oligodendroglioma case, the histopathological examination revealed a ",total metastasis" of the tu. liquor pathways. The connective tissue elements are actively involved in the process of expansion in the subarachnoid space and show excitatory phenomena which we assume to be related to the blastomatous stimulus acting on them. The term "tumorous meningitis" is not uninteresting to designate the picture. The fact that this phenomenon occurs only exceptionally in oligodendroglioma cases could be explained by the fact that tu does not always succeed in overcoming the resistance of the meninges. Diffuse subarachnoid expansion is likely to occur only in conditions where the tu. reaches the brain surface extensively and acts on the meninges over a large surface area. ]

Clinical Neuroscience

[A case of congenital word blindness ]

SZECSŐDY Imre

[In summary, we believe that congenital word blindness is not a single pathology, but rather a syndrome, in which in many cases there may indeed be an anatomical abnormality, but which, as in aphasia, never causes the loss of a specific isolated function, but which, by disrupting the dynamic unity of the nervous functions, is the main symptom of a general disorder. It is therefore plausible to assume a milder degree of mental retardation, with symptoms that are more dominant than dominant in literacy, since the literacy mechanism is the result of a complex and highly complex synthesising and analysing activity, the fragility of which can be easily assumed from the complexity of the reflex processes that are layered on top of each other. ]

Clinical Neuroscience

[Electroclinical study with temporal lobectomy in Ammon's horn sclerosis epileptics]

KAJTOR Ferenc, HABERLAND Katalin, HULLAY József, ÁNGYÁN András

[Seven patients with temporal lobe epilepsy in whom biopsy histology showed Ammon's horn sclerosis were subjected to electroclinical observations. The clinical features were high seizure activity during sleep, frequency of vegetative seizure phenomena and automatisms, personality abnormalities without intellectual impairment, intense mediobasal (garati) and hippocampal spasmodic activity. Temporal lobectomy provided the best results in the areas of EEG, seizure freedom and personality changes. Authors raise the possibility of an epileptogenic role of Ammon's horn sclerosis. ]

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Clinical Neuroscience

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

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Clinical Neuroscience

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

Clinical Neuroscience

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

[Effective therapy in highly active pediatric multiple sclerosis ]

MERÔ Gabriella, MÓSER Judit, LIPTAI Zoltán, DIÓSZEGHY Péter, BESSENYEI Mónika, CSÉPÁNY Tünde

[Multiple sclerosis (MS) is typically a disease of young adults. Childhood MS can be defined in patients under 18 years of age, although some authors set the limit un­der the age of 16 formerly known as “early-onset multiple sclerosis” or “juvenile multiple sclerosis”, seen in 3-5% of all MS patients. Nowadays, owing to ever-evolving, better diagnostic tools and well-traced, strictly defined diagnostic criteria, childhood MS is showing an increasing incidence worldwide (0.05-2.85/100 000). MS is characterized by recurrent episodes of the central nervous system with demyelination separated in space and time. In childhood almost exclusively the relapsing-remitting (RR) type of MS occurs. Based on experience in adults, the goal in the pediatric population is also the early diagnosis, to initiate adequate DMT as soon as possible and to achieve symptom relief and good quality of life. Based on efficacy and safety studies in the adult population, inter­feron β-1a and glatiramer acetate were first approved by the FDA and EMA for the treatment of childhood MS also. The increased relapse rate and rapid progression of childhood MS and unfavorable therapeutic response to nearly 45% of the first DMT necessitated the testing of more effective and second-line drugs in the population under 18 years of age (PARADIGMS, CONNECT). Although natalizumab was reported to be effective and well-tolerated in highly active RRMS in childhood, evidence based studies were not yet available when our patients’ treatment started. In this article, we report on the successful treatment of three active RRMS patients with individually authorized off-label use of natalizumab.]