Clinical Neuroscience

[Application of robot hand technique in the course of microneurosurgical operations]


JANUARY 20, 2009

Clinical Neuroscience - 2009;62(01-02)

[Introduction - The aim of this study was to determine whether the new robot hand technique can help to avoid the complication in the course of high precise microneurosurgical operations. Methods - The physical efficacy was measured by tremorometry. The comperative study of the incidence of complications measured the clinical efficacy. Results - The tremors of the operating hand and the number of complications have decreased effectively. Conclusion - The precise level of robots could be available by novel robot hand technique. By this technique the microsurgical work has become more effective.]



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Clinical Neuroscience

[Neuroprotection in Parkinson’s disease and other neurodegenerative disorders: preclinical and clinical findings]


[The authors summarized the evidence supporting neuroprotection based on the data available in the literature. In vivo and in vitro studies have indicated that many compounds can decrease neurodegeneration, excitotoxicity, oxidative stress, protein aggregation, disturbance of Ca2+ homeostasis and compensate the energy impairment. Selegiline, rasagiline, dopamine agonists and other molecules (ubiquinone, kynurenic acid, tocopherol, creatine, glatiramer acetate) exert neuroprotective effects in preclinical studies. Much less clinical data are available regarding neuroprotection in different neurological disorders. In this review, such preclinical and clinical evidences are summarized.]

Clinical Neuroscience

[Genetically determined neuromuscular disorders of some roma families living in Hungary (in English language)]


[The authors discuss the clinical and molecular genetic aspects of genetically determined neuromuscular disorders of some Roma families living in Hungary. Among the autosomal recessively inherited spinal muscular atrophic (SMA) group, 8 Caucasian children had the typical 7-8 exonal deletions of the SMA gene, but only 2 patients belonged to the Roma population. There was no difference in the molecular genetic findings among the Caucasian and the Roma SMA patients. All of them had 7- 8 exonal deletions of the SMA gene. We wanted to call attention to the founder mutation of the Roma population in 7 patients suffering from congenital myasthenia (CMS) from 3 Roma families. The 1267G deletion for CMS was detected by molecular genetic method. Clinical onset was pubertal and relatively slow progression of specific and phenotypic features for this founder mutation of acetyl-cholin receptor epsylon gene. In 2 patients (sister and brother) the sarcoglycanopathy 2C type C283Q mutation was proven in one Roma family suffering from limb-girdle muscular dystrophy (LGMD). Two out of the three facioscapular-humeral dystrophy (FSHD) Roma families carried 21.8 kb and 18.5 kb alleles in FSHD A1 gene (D4S139). In one family together with prenatal diagnosis founder mutation in FSHD A1 gene was detected, according to the autosomal dominant (AD) inheritence. In (F2) prenatal diagnosis was carried out, 18.5 kb/18.5 kb homozygosity was proven in the fetus, so the pregnancy was interrupted. In the CMS, LGMD and FSHD Roma patients ancient typical Roma founder mutations were found.]

Clinical Neuroscience

[Conductive education for children with neurological diseases]

BALOGH Erzsébet, KOZMA Ildikó

[Conductive education, developed by the 40-s last century, spread out in the world in spite the lack of hard scientific evidences for its benefit. There are sorts of cerebral palsies (athetosis, ataxia) in which conductive education might have the unique role to help. In cerebral palsies of other sorts it is much helpful if the disturbance of body scheme and degree of somatomotor neglect are superior to the palsy. Short term results of conductive education are to see in the better movement coordination whilst the long term outcome is the increased activities of daily living.]

Clinical Neuroscience

[Measurement of mental fatigability by task related spectral EEG. A pilot study (in English language)]

RAJNA Péter, HIDASI Zoltán, PÁL Iván, CSIBRI Éva, VERES Judit, SZUROMI Bálint

[Background - Task related EEG spectra are promising markers of mental activity. But the cooperation of the patients necessary for the registration limits its application in the neuro-psychiatry. Methods - EEG difference spectra on counting (EDSC) - was developed to detect the effect of a short calculation task on the spectral EEG. The originality of the task situation is a continuous mental work in a very short period of time, while the level of task difficulty is adapted to the patient’s actual mental capacity. While the rest pre-task and the post task EEG sections were compared, the results show the mental “EEG fatigability” caused by the short intensive cognitive activity. The first preliminary results have been demonstrated by a comparative study of two healthy and three patient (probable Alzheimer disease, post-stroke state without mental deficit and mixed type of dementia) groups. Results - Similarly to the findings of other authors, in addition to the differences of the alpha band seen on the temporo-parieto-occipital regions, the frontal localization and the beta band seem to be prominent, too. Demented patients had stronger EEG reactions than post-stroke patients without mental deficits and healthy elder persons had more extensive changes than the younger ones. Conclusions - The test can be considered as indirect marker showing the different mental fatigability in diverse pathological conditions and during the aging process. Effect of therapeutic processes can also be followed based on “key-lock principle”. Standardization of the test is essential for the introduction of EDSC to the every-day routine of clinical neuropsychiatry.]

Clinical Neuroscience

[Account on the scientific meeting of the Környey Society in 2008]

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[The importance of vitrectomy in the management of diabetic retinopathy in proliferative stage]


[Vitrectomy is a closed microsurgical technique, by which intraocular tissue, blood and foreign bodies can be removed through a small incision in the pars plana with an automated suction-cutting device (vitrectom), while maintaining normal or slightly elevated intraocular pressure. This technique requires many accessory instruments (microscope, endoillumination, intraocular instruments, endolaser). The main application of vitrectomy is the treatment of severe complications in diabetes mellitus: diabetic retinopathy in the proliferative stage. Vitrectomy is indicated in diabetic retinopathy for the removal of intraocular bleeding and for the treatment of complications of diabetic proliferative vitreoretinopathy (recurring bleeding, tractional and rhegmatogenous retinal detachment). In the case of intraocular bleeding, early vitrectomy is better than delayed, providing good visus (V: 0,5) in 25% of all patients (based on literature data). In retinal detachment cases of tractional origin, surgery provides satisfactory visus for self-care in 2/3rd of all patients.]

Clinical Neuroscience

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Clinical Neuroscience

The effects of the level of spinal cord injury on life satisfaction and disability

GULSAH Karatas, NESLIHAN Metli, ELIF Yalcin, RAMAZAN Gündüz, FATIH Karatas, MÜFIT Akyuz

Introduction - Spinal Cord Injury (SCI) may often lead to significant disability in affected individuals and reduce life satisfaction. Herein we aimed to investigate the effects of the level of injury on disability and life satisfaction as well as the relation between life satisfaction and disability. Methods - Patients with at least one-year history of SCI were included. Demographic-clinical data of patients were recorded. The Craig Handicap Assessment and Reporting Technique-Short Form (CHART-SF) was used for quantifying the degree of patients’ disability. Life satisfaction was assessed by the Satisfaction with Life Scale (SWLS). Results - Of the 76 patients, 21 (27.6%) were tetraplegic and 55 (72.4%) were paraplegic. SWLS was found to be similar in tetraplegic vs. paraplegic patients (P=0.59), whereas CHART parameters such as physical independence, mobility, occupation, and total CHART value were significantly higher in paraplegic patients (P=0.04, P=0.04, P=0.001 and P=0.01, respectively). Social integration was found similarly high in both groups. There was a positive correlation between elapsed time after the injury and CHART physical independence, occupation and the level of economic sufficiency (P<0.01, P<0.01, P=0.01). Excluding the economic sufficiency (P=0.02), there was not any other association between the rest of CHART parameters and SWLS. Conclusions - According to our findings, although the level of injury seems to be influential on disability, it seems to have no significant effect on life satisfaction. Since the only thing that positively affects life satisfaction is economic sufficiency, more emphasis should be placed on regulations that increase the return to work in patients.

Clinical Neuroscience

[LADA type diabetes, celiac diasease, cerebellar ataxia and stiff person syndrome. A rare association of autoimmune disorders]

SOÓS Zsuzsanna, SALAMON Mónika, ERDEI Katalin, KASZÁS Nóra, FOLYOVICH András, SZŰCS Anna, BARCS Gábor, ARÁNYI Zsuzsanna, SKALICZKI József, VADASDI Károly, WINKLER Gábor

[Celiac disease - in its typical form - is a chronic immunemediated enteropathy with typical clinical symptoms that develops against gliadin content of cereal grains, and is often associated with other autoimmune diseases. In cases of atypical manifestation classic symptoms may be absent or mild, and extra-intestinal symptoms or associated syndromes dominate clinical picture. The authors present a longitudinal follow-up of such a case. A 63-years old woman was diagnosed with epilepsy at the age of 19, and with progressive limb ataxia at the age of 36, which was initially thought to be caused by cerebellar atrophy, later probably by stiff person syndrome. At the age 59, her diabetes mellitus manifested with type 2 diabetic phenotype, but based on GAD positivity later was reclassified as type 1 diabetes. Only the last check-up discovered the celiac disease, retrospectively explaining the entire disease course and neurological symptoms. By presenting this case, the authors would like to draw attention to the fact that one should think of the possibility of celiac disease when cerebellar ataxia, progressive neurological symptoms and diabetes are present at the same time. An early diagnosis may help to delay the progression of disease and help better treatment.]