Clinical Neuroscience

[Pseudo abducens palsy]

RÓZSA Anikó, KOVÁCS Krisztina, SZILVÁSSY Ildikó, BOÓR Krisztina, GÁCS Gyula

JULY 20, 2011

Clinical Neuroscience - 2011;64(07-08)

[In this study, we present two cases of different eye movement disorders with variable case histories but with the same end stage; abduction paresis of one of the eyes, which ceased when the other eye was covered. Our differential diagnosis is that either the ocular form of myasthenia gravis, convergence spasm or ocular myotonia could explain the symptoms. However, we hypothesize that the clinical picture corresponds to pseudo abducens palsy or focal dystonia of the extraocular muscle, which in turn could be the result of impaired inhibition of the tonic resting activity of the antagonistic medial rectus muscle. We offer an explanation for the patomechanism of pseudoabducens palsy and the variants of internuclear ophthalmoplegia.]



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Clinical Neuroscience

[Recent changes in the paradigm of limbic encephalitis]


[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]


[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]


[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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[Two cases of uncommon manifestation of central nervous system sarcoidosis are reported. A 42 year-old man had a spinal cord sarcoidosis. MRI of the spinal cord showed myelopathy in the cervico-thoracic region, and the T2-weighted image showed increasing signal intensity. Neurological symptoms did not correllate with radiological abnormalities. Neurological manifestation was paucisymptomatic. Half a year later steroid and azatioprin therapy led to almost complet radiological and clinical regression. In the second case we present a 49 year-old woman who had left side internuclear ophthalmoplegia and the brainstem lesion. The patient was proven to have sarcoidosis. In this case no abnormalities were found in brain MRI. Neurological symptoms could not be detected by MRI, probably caused by brainstem parenchymal lesions consisting of microgranulomatosis that is sarcoid "brainstem encephalitis". Neurological symptoms improved after steroid treatment in this case too. In both of the cases pulmonary lymphadenopathy helped to diagnose sarcoidosis. In our cases there were interesting correllations between neurological symptoms and MRI abnormalities. At the spinal cord sarcoidosis the radiological abnormalities were more striking than the clinical manifestation. In the other case we found distinct brainstem symptoms but could not detect MRI abnormalities.]

Clinical Neuroscience

Single-hole, ruptured parenchymal arteriovenous fistula of the mesencephalon: not known vascular malformation of the brain or a posthemorrhagic entity?

KULCSÁR Zsolt, MACHI Paolo, VARGAS Isabel Maria, SCHALLER Karl, LOVBLAD Olof Karl

The subtypes of brain arteriovenous malformations, with direct, single-hole fistulas without co-existing nidus are not described as existing entities inside the brain parenchyma but on the pial surface. True parenchymal arteriovenous malformations present with nidal structure, even if they are small, whereas surface lesions may present a direct fistulous configuration. In this case of midbrain haemorrhage a direct arteriovenous fistula was detected at the level of the red nucleus between a paramedian midbrain perforator artery and a paramedian parenchymal vein, with pseudo-aneurysm formation at the fistulous connection, without signs of adjacent nidus structure. The hypothesis whether a pre-existing arteriovenous fistula ruptured or a spontaneous haemorrhage has caused the fistulous connection is discussed.

Clinical Neuroscience

[Epidemiology, cost and economic impact of cerebral palsy in Hungary]

FEJES Melinda, VARGA Beatrix, HOLLÓDY Katalin

[Objective - The purpose of our communication was to determine the total cost of cerebral paretic patients in Hungary between 0 and 18 years and to assess their impact on the national budget. Methods - Based on the data of Borsod county we calculated the CP characteristics. The cost of CP was determined by routine care of individuals. Lost Parental Income and Tax were calculated on the basis of average earnings. The ratio of GDP, Health and Social Budget and Health Budget to CP is based on CP annual average cost and frequency. We have developed a repeatable computational model. Results - Of the risk groups, premature birth (30.97%), low birth weight (29.64%), perinatal asphyxia (19.47%) were the most common. Source is unknown of 37.61% of the cases. CP prevalence was 2.1‰. The two-sided (59.7%) and the one-sided (19.0%) spastic pareses dominated. The most serious form is the two-sided spastic paresis (42.5% GMFCS 3-5 degrees). Epilepsy was 22.0%, incontinence was 27%, mental involvement was 46%. Care for one child up to 18 years of age costs an average of 73 million HUF (€ 251,724). The lost family income was 27.36 million HUF (€ 94,345), and lost tax and health care contributions were 14.46 million HUF (€ 49,862). Additionally, 0.525% of the GDP, 0.88% of the full health and social budget and 1.83% of direct medical costs were spent for CP families. Conclusion - The cost of CP disease is significant. Costs can be reduced by improving primary prevention. From the perspective of the family and government, it is better to care for families so they can take care of their disabled children.]

Clinical Neuroscience

Role of positioning between trunk and pelvis in locomotor function of ambulant children with and without cerebral palsy


Purpose - To understand if children with and without cerebral palsy share the same lumbar postural control threshold on the sagittal plane for the transition between each walking locomotor stage. Method - Observational analysis of sagittal trunk-pelvis kinematics of 97 children with cerebral palsy and 73 with typical development, according to their locomotor stage. Results - Among children with typical development, all average and minimum measurements of the sagittal lumbar curve during the gait events were correlated with age and the locomotor stages of development. Among children with cerebral palsy, there were significant correlations between all average and minimum values of the sagittal lumbar curve and locomotor stages of development but not age. Conclusions - We conclude that, for the same locomotor level, there are no common postural patterns between children with typical development and those with spastic bilateral cerebral palsy for the position between trunk and pelvis in the sagittal plane. Maximal lordosis reduction between trunk and pelvis may change with age or even training, but does not make a positive effect on the locomotor level, while basal and maintenance capacities could explain locomotor function. Trials that failed to assess quality of movement may now have a better understanding of how different interventions improve posture towards the next functional level.