Clinical Neuroscience

Observations upon the So-colled Idiots Savants


JANUARY 01, 1968

Clinical Neuroscience - 1968;21(01)

In drawing the foregoing generalisations it is necessary to realise their limitations. We must agree with the conclusions arrived at by Mitchell, who recognised at least three psychological categories: (1) the “calculating prodigies — who may be persons of inferior intellectual calibre and who rely upon ingenious shortcuts; (2) arithmetical prodigies like Colburn, and Dase, with a moderately well developed knowledge of arithmetic; and (3) mathematical geniuses, such as the elder Bidder. These are endowed with exceptional abilities, and their knowledge of pure mathematics is profound.



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Clinical Neuroscience

[Pemphigus cases with lesions found in the spinal ganglia ]

BALÓ József

[Based on our experience with zoster cases, we have examined the spinal ganglia of 82 cases of pemphigus over the last 20 years to see if there are any phenomena that could explain the skin lesions. The lesions found, partly macroscopic but mainly histopathological, suggest that such a link between lesions in the spinal ganglia and skin disease exists. In addition to the acute signs of inflammation, there are also lesions that can be classified as chronic, such as those involving nerve fibres, nuclei, supporting tissue of the ganglia and lesions of the meninges. Diseases of the spinal ganglia are projected onto the skin, which makes pemphigus a cutaneous trophoneurosis. In addition to the morphological phenomena, the question is what aetiological factors are involved in its creation. This remains to be determined in the future. ]

Clinical Neuroscience

Sur la sémiologie des idioties amaurotiques du type Tay-Sachs en survie prolongée

L. van Bogaert, J. J. Martin

Etude clinique d'une idiotie amaurotique de Tay-Sachs à évolution prolongée sous l'angle des signes de décérébration, des réflexes primitifs et des manifestations d'automatisme médullaire.

Clinical Neuroscience

Thorium granulomas in the brain


Thorotrast was used in 1936 and 1937 to demonstrate the lesions of prefrontal lobotomy. Four patients came to autopsy after 10-22 years, and in each, one or more thorium granulomas were found. These masses ranged from 6 X 8 mm to 8X12 mm in size, were composed of hyaline material enclosed by a thick capsule of mixed connective and glia tissue, and surrounded in part by large phagocytes filled with thorium dioxide particles. Dense connective tissue developed in sulci where thorotrast escaped into the subarachnoid spaces, and marked gliosis with desquamation of the ependyma occurred when it entered the ventricles. The phagocytes in the cases with longer survival often showed vacant cavities where the nuclei should have been. Neurons in the vicinity showed no obvious lesions. The material was described as containing "a very strong thorium source.” It is believed that the alpha particles given off by the thorium are responsible for the formation of the granulomas and, after many years, for the death of the phagocytes. Thorium can safely be used in the brain only for the demonstration of cysts and abscesses which can then be completely removed. A case of such employment was described by Lehoczky in 1939.

Clinical Neuroscience

Télangiectasies de la moelle dorsale révélées à l'âge de 75 ans par une myelopathie transverse, avec une digression sur l'atrophie spinale segmentaire


L'observation que nous rapportons tire son intêret de la révélation extrêmement tardive (75 ans) d'un angiome de la moelle de type capillaire, resté jusque là cliniquement muet. Elle illustre la longue latence possible des mal formations vasculaires de la moelle, et elle montre qu'il faut toujours penser à cette étiologie devant une affection médullaire dont la cause nous échappe.

Clinical Neuroscience

[Maladies infectieuses extraneurales du système nerveux complications]


[Overview of neurological complications of common infectious diseases it is most useful to start from the following classification of encephalomyelitis based on its pathophysiological features, although only certain types are associated with our thymus: 1. meningo-encephalitis; 2. metastatic nodular encephalitis (abscess); 3. diffuse encephalitis, mainly involving the cerebral cortex; 4. polioencephalomyelitis, with pre-dilection areas of the brainstem disease of the prefrontal lobes of the brain; 5. panencephalitis; 6. leukoencephalitis.]

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Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.

Clinical Neuroscience

[The importance of patient reported outcome measures in Pompe disease]

MOLNÁR Mária Judit, MOLNÁR Viktor, LÁSZLÓ Izabella, SZEGEDI Márta, VÁRHEGYI Vera, GROSZ Zoltán

[In recent decades it has become increasingly important to involve patients in their diagnostic and treatment process to improve treatment outcomes and optimize compliance. By their involvement, patients can become active participants in therapeutic developments and their observations can be utilized in determining the unmet needs and priorities in clinical research. This is especially true in rare diseases such as Pompe disease. Pompe disease is a genetically determined lysosomal storage disease featuring severe limb-girdle and axial muscle weakness accompanied with respiratory insufficiency, in which enzyme replacement therapy (ERT) now has been available for 15 years. In our present study, patient reported outcome measures (PROMs) for individuals affected with Pompe disease were developed which included questionnaires assessing general quality of life (EuroQoL, EQ-5D, SF36), daily activities and motor performance (Fatigue Severity Score, R-PAct-Scale, Rotterdam and Bartel disability scale). Data were collected for three subsequent years. The PROM questionnaires were a good complement to the physician-recorded condition assessment, and on certain aspects only PROMs provided information (e.g. fatigue in excess of patients’ objective muscle weakness; deteriorating social activities despite stagnant physical abilities; significant individual differences in certain domains). The psychological effects of disease burden were also reflected in PROMs. In addition to medical examination and certain endpoints monitored by physicians, patient perspectives need to be taken into account when assessing the effectiveness of new, innovative treatments. With involvement of patients, information can be obtained that might remain uncovered during regular medical visits, although it is essential in determining the directions and priorities of clinical research. For all orphan medicines we emphasize to include patients in a compulsory manner to obtain general and disease-specific multidimensional outcome measures and use them as a quality indicator to monitor treatment effectiveness.]

Clinical Neuroscience

[Current questions of multiple sclerosis: the secunder progressive form of the disease]


[Recent data suggest that long-term worsening is common in relapsing-remitting multiple sclerosis patients and is largely independent of relapses or new lesion formation on brain MRI. The current definition of secunder progressive multiple sclerosis is worsening of disability independent of relapses over at least 6-month interval. Early focal inflammatory disease activity and spinal cord lesion are predictors of very-long term disease outcomes in relapse - onset multiple sclerosis. The potential of PET imaging to visualize hidden inflammation in MS brain in vivo is an important contribution for better understanding the progression of the disease. Therefore, PET imaging is a promising tool in detecting the conversion from relapsing remitting multiple sclerosis to secunder progressive form of multiple sclerosis. Furthermore, neuro-axonal damage is the pathological substrate of permanent disability in different neurological disorders including multiple sclerosis. The neurofilament proteins have promise in this context because their levels rise upon neuro-axonal damage not only in the cerebrospinal fluid but also in blood. Patients with increased serum levels of neurofilament at baseline, independent of other clinical and MRI variables, experience significantly more brain and spinal cord volume loss over 2 years and 5 years of follow-up. The kynurenine-pathway abnormalities may be associated with the swich from early-mild stage multiple sclerosis to debilitating progressive forms of the disease. Analysis of these metabolites in serum may have application as multiple sclerosis disease biomarkers. Free radical action has been suggested as a causal factor in the illness. Increased free radical production and consumption of the scavenger molecules were found during the active phase of the disease. Based on the clinical findings (EXPAND Study) and pathomechanism of the disease siponimod is approved by the US Food and Drug Administration for the treatment of relapsing remitting forms of multiple sclerosis, to include secunder progressive multiple sclerosis with active disease, relapsing-remitting multiple sclerosis and clinically isolated syndrome.]

Clinical Neuroscience

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report


Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

Clinical Neuroscience

Wnt pathway markers in low-grade and high-grade gliomas

NAGY Ádám, TOMPA Márton, KRABÓTH Zoltán, GARZULY Ferenc , MARÁCZI Alexandra , KÁLMÁN Bernadette

Aberrant activation of the Wnt pathway contributes to differentiation and maintenance of cancer stem cells underlying gliomagenesis. The aim of our research was to determine as to what degrees some Wnt markers are expressed in gliomas of different grades, lineages and molecular subtypes. Nine grade II, 10 grade III and 72 grade IV surgically removed, formalin-fixed paraffin-embedded glioma specimens were included. Mutation status of IDH1 codon 132 was defined by immunohistochemistry and pyrosequencing in all tumors. Grade II and III astrocytic and oligodendroglial tumors were further tested for the expression of p53 and ATRX by immunohistochemistry, and codeletion of 1p19q by fluorescent in situ hybridization. Expression levels of the non-canonical Wnt5a and Fzd2, and the canonical Wnt3a and beta-catenin Wnt pathway markers were determined by immunohistochemistry, and compared between subgroups stratified according to grade, lineage and the presence or absence of IDH1 R132H/C mutations. In the normal brain – grade II-IV glioma comparisons, a gradual increase was observed for the expressions of Wnt5a, Wnt3a, Fzd2 and beta-catenin. In the astroglial and oligodendroglial lineages of grade II and III gliomas, only the Wnt5a expression was significantly higher in the astroglial subgroup. Stratification according to the IDH1 status resulted in a significant increase of the Wnt3 expression in the wild type grade II-IV gliomas. These data extend previous observations and show a correlation of Wnt pathway activity with glioma grade. Further investigations of the Wnt marker expression regulation according to glioma lineage or IDH gene mutational status are in progress by using more exact molecular approaches.