Clinical Neuroscience

[Report]

HÁRDI István

JULY 01, 1968

Clinical Neuroscience - 1968;21(07)

[Author reports on the round table conference of the Psychiatric Section of the Hungarian Rehabilitation Society. ]

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Clinical Neuroscience

[Transient electroencephalographic phenomena in the generalisalt spike-wave pattern and the so-called grand mal type ictal generalisalt repetitive spasm discharge ]

HALÁSZ Péter, VELOK Gyula, HIDASI József, BOCZÁN Gábor

[In search of an answer to the pathophysiological reasons for the difference between petit mal and grand mal mechanism, we described four patients with intertwined generalized spike-wave mechanism and generalized repetitive spasm; two cases with 3 c/s spike-wave mechanism within an ictus and two cases with 3 c/s spike-wave mechanism within an ictus. two spike-wave variants during two different types of seizures in the same patient. From our observations the following findings can be filtered. 1. Transitory EEG phenomena can be detected between the ictal electrographic phenomena of petit mal with spike-wave and grand mal seizures with repetitive rapid discharge. 2. We can confirm Gastaut's observations that in epileptic encephalopathies with some spike-wave variants, repetitive rapid discharge tonic seizures with mild motor symptoms and significant vegetative symptoms are frequently observed in sleep. 3. Sleep - both natural and Evipan anaesthesia - and benzodiazepine preparations also contribute to the appearance of transient phenomena. On the basis of our electrographic observations, the spike wave mechanism and the previously discovered facts about the neuronal mechanism of grand mal repetitive rapid discharge (Pollen 1964, Matsumoto and Ajmone Marsan 1964) and the interneuronal recurrent collateral inhibition shown to be crucial in rhythmic EEG phenomena (Eccles 1965), we attempt to provide a unified view of the grand mal and petit mal mechanism and to explain the electrographic and clinical differences.]

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[The structure and dynamics of resistance in psychotherapy in psychotherapy groups]

HIDAS György

[In psychotherapy, resistance is present all the time with varying intensity. It is also an obstacle to psychotherapeutic change, but it provides valuable insights into the resistance mechanisms, character and history of the participants in the process, as well as their present functions. Experience has shown that the recognition and discussion of resistance moves the psychotherapeutic process forward towards improvement, positive change and dissolves the current form of resistance. Since the cause of resistance is often not exclusively in the individual, but often in the family, it logically follows that family or couple therapy is necessary. ]

Clinical Neuroscience

[The effect of labyrinth stimulation on cerebellar blood flow]

POÓR Gyula, KOPA János, MOLNÁR László

[Authors investigated the effect of stimuli from the semicircular arcuate ducts of the labyrinth on cerebellar blood flow in rabbits. The flow was studied using an electrical system based on the negative feed-back principle, which allows the measurement of the change in thermoconductivity. In chloralose-urethane narcosis, changes in general blood pressure and respiration were simultaneously recorded, as well as electrical activity and blood flow in the cerebellar cortex. On stimulation of one side of the labyrinth with cold water, an increase in blood flow was found in the opposite cerebellar cortex and a decrease in the same side; on warm stimulation the change was reversed. Galvanic stimulation resulted in a biphasic change. Rotation increased blood flow in both cerebellar hemispheres, and this remained unchanged after bilateral transection of the vago-sympathicus, and became more pronounced and prolonged after KCl was dripped onto the cerebral cortex. ]

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[Analysis of the arginine content of liquid protein components]

SZILÁGYI A. Katalin

[The Vorfractione of CSF contains much higher amounts of arginine than the other protein components of CSF. CSF albumin is arginine-poor. This finding, together with previous observations on the spatial distribution of arginine and Vorfractio in CSF, suggests that metabolic processes may occur between the two substances in the CNS. ]

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[In patients with Dupuytren's contracture electroclinical studies ]

SZENDRŐI Mária, HASZNOS Tivadar, GALAMBOS József

[The authors performed detailed neurological examinations in 50 patients with Dupuytren's contracture; they also determined the conduction velocity of the motor fibres of the ulnar nerve in 23 patients. The mild clinical symptoms and the decrease in conduction velocity suggest that ulnar nerve lacunar lesions are involved in the pathogenesis of Dupuytren's contracture. In this sense, they evaluate 5 cases of their patients in which the symptoms of circumscribed ulnar nerve laesio were followed shortly by the appearance of Dupuytren's contractura. ]

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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

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.

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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.

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

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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.

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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.]