Clinical Neuroscience

[Fine structure of brain capillaries in neonatal mice]

GOSZTONYI György1

OCTOBER 01, 1966

Clinical Neuroscience - 1966;19(10)

[Morphological features of brain capillaries from one-, six- and nine-day-old mice were studied by electron microscopy. The endothelial layer is complete and continuous, the endothelial-cell junctional interfaces are well developed, and the zonula occludens is characteristic. The endothelial layer is thus identical in all essential features to that found in adulthood. The basement membrane, on the other hand, is poorly developed, but its density increases with age. There are significant extracellular gaps between the perivascular and neuropil elements in the neonatal animal; these gaps gradually decrease in extent. In the neonate there is no perivascular asrtocyte sheath, but the formation of this structure begins early and progresses steadily, although it is incomplete at nine days of age. These morphological features of the immature central nervous system provide an opportunity to interpret certain phenomena of the blood-brain barrier.]

AFFILIATIONS

  1. Medical Research Council Víruskutató Intézetének Elektronmikroszkópos Laboratóriuma, Carshalton, Surrey, Anglia

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Clinical Neuroscience

[Changes in evoked potentials during cooling of the brainstem]

PÁSZTOR Emil, KUKORELLI Tibor

[In cats, we performed brief circumscribed surface cooling of the somatosensory area and examined changes in the evoked potentials. 1. After intensive cooling, the waves of the primary complex disappeared and only the irradiance potential with unchanged latency was detected. 2. 3. Our experiments with cooling suggest that other mechanisms besides the function of the more superficial cells of the cortex are involved in the generation of the negative wave of the primary complex. 4. The surface-positive afterglow of cooling can be interpreted as an irradiance wave of extralemniscal polysynaptic afferentatio. 5. A spreading depression state may occur during local cooling of the cortex. 6. By 30 sec of ice cooling, the evoked potentials disappear completely, but the impairment of cell function is always reversible. Restitution of the primary complex waves occurs in 3- 4 min. In addition to the extent of cooling, the rate of cooling also plays a significant role in the change in induced potentials. ]

Clinical Neuroscience

[Exploration and psychotherapy with psychotropic drugs (LSD25 and Cy39) in neurotic patients]

KORONKAI Bertalan, FÉBÓ Gzőző

[1. Authors describe two cases of psychotherapeutic treatment with psychotropic drugs in psychoneurotic patients. 2. They present their cases as a model of the change in psychic dynamics during drug exposure. 3. They explain the relationship between drug-induced imagery and pathological experiences. 4. They suggest that psychodysleptics can be used to shorten psychotherapeutic treatment and make it more effective. 5. They see the therapeutic effect in facilitating the identification of the associative relationship between current and past psychotraumas, and in the cathartic re-enactment and discussion of the associated affect.]

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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.

Clinical Neuroscience

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

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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.

Lege Artis Medicinae

[A short chronicle of three decades ]

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Clinical Neuroscience

Neuroscience highlights: The mirror inside our brain

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Over the second half of the 19th century, numerous theories arose concerning mechanisms involved in understanding of action, imitative learning, language development and theory of mind. These explorations gained new momentum with the discovery of the so called “mirror neurons”. Rizzolatti’s work inspired large groups of scientists seeking explanation in a new and hitherto unexplored area of how we perceive and understand the actions and intentions of others, how we learn through imitation to help our own survival, and what mechanisms have helped us to develop a unique human trait, language. Numerous studies have addressed these questions over the years, gathering information about mirror neurons themselves, their subtypes, the different brain areas involved in the mirror neuron system, their role in the above mentioned mechanisms, and the varying consequences of their dysfunction in human life. In this short review, we summarize the most important theories and discoveries that argue for the existence of the mirror neuron system, and its essential function in normal human life or some pathological conditions.

Clinical Neuroscience

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

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[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]