Clinical Neuroscience

[The brain that changes itself - Norman Doidge]

HALÁSZ Péter

JULY 20, 2011

Clinical Neuroscience - 2011;64(07-08)

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Clinical Neuroscience

[Recent changes in the paradigm of limbic encephalitis]

ILLÉS Zsolt

[In the recent years, novel antibodies associated with limbic encephalitis have been described, which target such extracellular receptors or proteins that have been already indicated in the pathogenesis of hereditary or degenerative diseases. In a number of cases, where pathogenic role of antibodies generated against the voltage-gated potassium channel (VGKC) had been presumed, antibodies against a trans-synaptic scaffolding protein, LGI1 were indicated. Antibody response against NMDA-receptors has been suggested as a major cause of limbic encephalitis especially in young females, resulting in a typical clinical syndrome sometimes triggered by an ovarian teratoma. Antibodies against other receptors essential in synaptic transmission and plasticity (AMPA and GABAB receptors) have been also indicated, partially elicited by paraneoplastic processes. Such antibodies against surface proteins result in severe but potentially treatable diseases due to reversible internalization of the antigens crosslinked by the bivalent antibodies. In contrast, the rare classical onconeural antibodies reacting with intracellular targets (anti-Hu, anti-Ta/Ma2, anti- CV2/CRMP5) may elicit additional symptoms beside limbic encephalitis and the prognosis of such syndromes is poor.]

Clinical Neuroscience

[Our first impact factor: 0.236 (2010)]

RAJNA Péter, TAJTI János

Clinical Neuroscience

[Modeling of human movements, neuroprostheses]

LACZKÓ József

[Modeling of human movements became very important as modern methods in informatics and engeniering are available to discern human movement characteristics that were hidden before. The construction of models of neural control and mechanical execution of human movements helps the diagnosis of movement disorders and predicts the outcome of clinical intervention and medical rehabilitation. Here I present methods for recording kinematic and muscle activity patterns. Measurements can be compared with predicted movement patterns based on mathematical models. There are an infinity of different muscle activity patterns or joint rotation patterns to perform a given motor task. I present the main approaches that are used to find such solutions from the infinity of choices that might be employed by the central nervous system. I present a practical application of movement modeling: In rehabilitation of spinal cord injured patients we develop and apply artificially controlled neuroprostheses to generate active cycling lower limb movements in the patients of the National Institute for Medical Rehabilitation.]

Clinical Neuroscience

[Extension of polynomial analysis of interstitial I-125 brachytherapy for 48 months]

KOLUMBÁN Zsuzsa, MAJOR Tibor, JULOW Jenő

[Objective - Previously we described from 20 patients’ data with our new “polynomial prediction approach” the volumetrical changes following gliomas I-125 brachytherapy. The aim of this study is to extend the polynomials for 48 months, and to carry out multivarial analysis of several different aspects. Methods - 20 inoperable low-grade gliomas were followed for a 48-month period after I-125 interstitial irradiation. The delivered dose on the tumor surface was 50-60 Gy. Dose planning and image fusion were done with the BrainLab Target 1.19 software, mathematical and statistical computations were carried out with the Matlab numeric computation and visualization software. Volumes of tumor necrosis, reactive zone and edema referred to as “triple ring” were measured on image fused control MRI and planning CT images. The measured volumes were normalized with respect to the reference volumes. Mean values of volumes were determined, then polynomials were fitted to the mean using the polynomial curve fitting method. The accuracy of our results was verified by correlating the predicted data with the measured ones. Results - We have found that the edema reaches its maximum two times after irradiation during the 48 months follow up period. We have shown that small tumors react more rapidly and creating greater volumes of the “triple ring” than bigger ones. Conclusions - The polynomial prediction approach proposed here reveals the dynamics of triple ring for 48 months. The derived polynomials and the multivarial analysis carried out afterwords help to (i) design the best treatment, (ii) follow up the patient's condition and (iii) plan reirradiation if necessary.]

Clinical Neuroscience

[Phylo- and ontogenetic aspects of erect posture and walking in developmental neurology]

BERÉNYI Marianne, KATONA Ferenc, CARMEN Sanchez, MANDUJANO Mario

[The group or profile of elementary neuromotor patterns is different from the primitive reflex group which is now called the “primitive reflex profile.” All these elementary neuromotor patterns are characterized by a high degree of organization, persistence, and stereotypy. In many regards, these patterns are predecessors or precursors of from them the specific human motor patterns which appear spontaneously later as crawling, creeping, sitting, and walking with erect posture. On the basis of our experiences it can be stated that the elementary neuromotor patterns can be activated in all neonates and young infants as congenital motor functions. With regards to their main properties and functional forms, the normal patterns can be divided into two main groups: (1) One group is characterized by lifting of the head and complex chains of movements which are directed to the verticalization of the body; (2) The other group is characterized by complex movements directed to locomotion and change of body position. The neuromotor patterns can be activated by placing the human infant in specific body positions that trigger the vestibulospinal and the reticulospinal systems, the archicerebellum and the basal gangliae. Most of these systems display early myelinisation and are functioning very soon. Many of the elementary neuromotor patterns reflect the most important - spontaneously developing - forms of human movements such as sitting upright in space and head elevation crawling and walking. The majority of the human neuromotor patterns are human specific. When the infant is put in an activating position, crawling, sitting up, and walking begin and last as long as the activating position is maintained. Each elementary neuromotor pattern is a repeated, continuous train of complex movements in response to a special activating position. The brainstem is not sufficient to organize these complex movements, the integrity of the basal ganglia is also necessary. Elementary sensorimotor patterns during human ontogenesis reflect phylogenetic develpoment of species specific human functions. During ontogenesis spontaneous motor development gradually arises from these early specific sensorimotor predecessors.. The regular use of the elementary neuromotor patterns for diagnostic puposes has several distinct advantages. The neuromotor patterns have a natural stereotypy in normal infants and, therefore, deflections from this regular pattern may be detected easily, thus, the activation of the elementary neuromotor pattern is a more suitable method for identifying defects in the motor activity of the neonate or young infant than the assessment of the primitive reflexes. The “stiumulus positions,” which activate specific movements according to how the human neonate or young infant is positioned, do not activate such motor patterns in neonate or young primates including apes. The characteristic locomotor pattern in these adult primates, including the apes, is swinging and involves brachiation with an extreme prehensility. This species specific motor activity is reflected in the orangutan and gibbon neonates by an early extensive grasp. However, according to our investigations, no crawling, creeping, elementary walk, or sitting up can be activated in them. Neonates grasp the hair of the mother, a vital function for the survival of the young. In contemporary nonhuman primates including apes, the neonate brain is more mature. Thus, pronounced differences can be observed between early motor ontogenesis in the human and all other primates. The earliest human movements are complex performances rather than simple reflexes. The distinction between primitive reflexes and elementary neuromotor patterns is essential. Primitive reflexes are controlled by the brainstem. All can be activated in primates. These reflexes have short durations and contrary to elementary sensorimotor patterns occur only once in response to one stimulus, e.g., one head drop elicits one abduction-adduction of the upper extremities correlated to adduction and flexion of the lower extremities to a lesser degree with the Moro reflex. Elementary neuromotor patterns are much more complex and most of them including elementary walk may be elicited as early as the 19th-20th gestational week, though less perfectly than later.]

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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.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]

Clinical Neuroscience

[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

SANZ-MENGIBAR Jose Manuel , MENENDEZ-PARDIÑAS Monica , SANTONJA-MEDINA Fernando

[Vojta therapy has been reported as clinically beneficial for strength, movement and gross motor activities in individual cases and is being included within the second of three levels of evidence in interventions for cerebral palsy. The goal of this study is to understand the effect of Vojta therapy on the gross motor function. Our clinical trial followed a one group, pre-post design to quantify rates of changes in GMFM-88 after a two-months period undergoing Vojta therapy. A total of 16 patients were recruited. Post-intervention acceleration rates of GMFM-88-items acquisition (0.005; p<0.001) and Locomotor Stages (1.063; p<0.0001) increased significatively following Vojta the­rapy intervention. In this study, Vojta therapy has shown to accelerate the acquisition of GMFM-88-items and Loco­motor Stages in children with cerebral palsy younger than 18 months. Because functional training was not utilised, and other non-Vojta therapy intervention did not influence the outcome, Vojta therapy seems to activate the postural control required to achieve uncompleted GMFM-88-items. ]

Clinical Neuroscience

Neuroscience highlights: The mirror inside our brain

KRABÓTH Zoltán, KÁLMÁN Bernadette

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

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.