Clinical Neuroscience

[Pathology of CNS tumors]

BÁLINT Katalin, JULOW Jenő

SEPTEMBER 30, 2010

Clinical Neuroscience - 2010;63(09-10)

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Clinical Neuroscience

[Examining the psychometric properties of a new quality of life questionnaire in migraineurs]

MANHALTER Nóra, PALÁSTI Ágnes, BOZSIK György, ÁFRA Judit, ERTSEY Csaba

[Background - The deleterious effect of primary headaches on the sufferers’ quality of life (QOL) has been abundantly documented using both generic and headache-specific instruments. The currently used questionnaires focus on a limited number of factors and therefore may not be sensitive enough to detect the effect of headache type and headache characteristics on QOL, despite the obvious clinical differences. We have devised a comprehensive questionnaire that may be more sensitive to the burden of headache. Objective - To assess the psychometric properties of the new questionnaire on a group of migraineurs. Patients and method - We studied 117 migraineurs who completed the validated Hungarian version of the SF-36 generic QOL measure and our new, 25-item questionnaire. Reliability was assessed by internal consistency, measured by Cronbach’s a of all items. Content validity was exam- ined by calculating the correlation of the items with subscales of the SF-36 measure. The correlation of the patients’ migraine characteristics with the questionnaire’s items was used to assess criterion validity. Results - The questionnaire was quick and easy to administer. The questionnaire demonstrated good reliability, with Cronbach’s alpha being 0.893. Content validity was adequate; most “physical” items of the new questionnaire showed significant correlations with the bodily pain and role physical SF-36 subscales and most “psychical” and “social” items were correlated with mental health and social functioning SF-36 subscales. Criterion validity was adequate, with headache severity being correlated with most of the items. Discussion - In this study the new headache-specific quality of life instrument showed adequate psychometric properties.]

Clinical Neuroscience

[The concept of epileptic networks. Part 1.]

HALÁSZ Péter

[In this paper we investigate evidences supporting the network concept of epilepsies from different approaches. Firstly the functions of cortical networks in which most of the epileptic networks are embedded, are treated. Then the tentative characteristics of an epileptic network are enumerated and the conversion mechanisms from physiological to epileptic networks are analyzed. Later the role of neuronal oscillations in epileptic networks and aspects of epilepsies provoked by sensory and cognitive tasks is studied. Lastly new fMRI data in mapping BOLD networks underlying spike and seizure discharges are used as arguments in favour of the epileptic network hypothesis. In a second part the well-known epilepsies related, or probably related to physiological networks are shown. Finally consequences of the network approach for creating a new unified epilepsy classification are discussed.]

Clinical Neuroscience

[Analysis of antiparkinsonian drug reduction after bilateral subthalamic deep brain stimulation]

FEHÉR Georgina, BALÁS István, KOMOLY Sámuel, DÓCZI Tamás, JANSZKY József, ASCHERMANN Zsuzsanna, BALÁZS Éva, NAGY Ferenc, KOVÁCS Norbert

[Background - Bilateral deep brain stimulation of the subthalamic nuclei (STN) is a well-established and cost-effective treatment in advanced PD. Objectives - To quantitatively analyze the change in use of antiparkinsonian drugs one year after subthalamic deep brain stimulator (DBS) implantation in patients with idiopathic Parkinson’s disease (PD). Patients and methods - Eighteen consecutive patients with advanced PD underwent bilateral STN DBS implantation were involved in the study. The stimulation achieved a stable and clear clinical benefit in all of the cases. One year after the implantation, drug usage of patients was analyzed and correlated with the postoperative symptomatic improvement measured by the modified Hoehn-Yahr, Schwab and England, and Unified Parkinson’s Disease Rating Scales. Because none of the investigated variables followed the normal distribution, non-parametric Wilcoxon signed-rank, McNemar and Kendell’s τ tests were applied. Results - Preoperatively, the patients used 12.05±4.57 tablets a day out of 3.19±0.97 different antiparkinsonian drugs, which was significantly reduced by deep brain stimulation to the application of 7.00±2.96 tablets out of 1-3 (1.84±0.76) drugs (p<0.001). Meanwhile, the usage of amantadine, MAO-B and COMT inhibitors was also significantly decreased (p<0.05). The dosage of dopaminerg medication was significantly lowered from 1136 mg to 706 mg expressed in levodopa equivalent dosage (p<0.001) whereas the UPDRS-III also improved by 48.6%. Conclusion - Our study is in accordance with previously published international findings that antiparkinsonian medication can be significantly lowered after bilateral STN DBS. Because not only the dosage, but also the applied number of tablets were decreased, it may have resulted in a better compliance and quality of life.]

Clinical Neuroscience

[Devic syndrome - case report, diagnostic and therapeutic principles]

ILJICSOV Anna, BARSI Péter, VÁRALLYAY György, TÁTRAI Erika, SOMFAI Gábor Márk, BERECZKI Dániel, RUDAS Gábor, SIMÓ Magdolna

[Neuromyelitis optica (NMO, Devic-syndrome) is a rare, relapsing autoimmun disease of the central nervous system, which is distinguished from other demyelinating disorders by a recently identified, specific autoantibody. By demonstrating the anti-aquaporin-4 IgG in the serum, a heterogenous group of syndromes can be defined, called NMO-spectrum. In the future, optical coherence tomography may support this diagnosis besides the clinical features, imaging examinations and presence of serum antibody. Early recognition and treatment can improve clinical outcome even in serious condition. Long-term immunosuppressive therapy is advised to prevent further relapses and to stabilize or improve clinical status. Hereby, we report a case of a 51-year-old woman, under treatment for 1.5 years. We summarize the up-to-date knowledge about the pathomechanism, diagnostic strategy and therapy of neuromyelitis optica. We review recent findings and the diagnostic value of a new, non-invasive ophtalmological examination, the optical coherence tomography. According to the first results, this method may be helpful in the early differential diagnosis of optic neuritis.]

Clinical Neuroscience

[Genetic risk factors in Alzheimer’s disease]

FEHÉR Ágnes

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Clinical Neuroscience

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

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

Clinical Neuroscience

[MR imaging of acute disseminated encephalomyelitis and multiple sclerosis in children. A review (in English language)]

PATAY Zoltán

[Inflammatory diseases of the central nervous system (CNS) are relatively rare in children, but their relevance to public health is considerable due to frequent and significant long term morbidity and even mortality. As in adults, acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) and their variants are the most common entities in this group of pathologies in the pediatric patient population. Recent efforts have focused on establishing standardized diagnostic criteria schemes to facilitate the diagnosis and differential diagnosis of these diseases, however especially with multiple sclerosis those have not been fully validated yet for disease occurring in children. In recent decades the role of MRI has been constantly increasing in the diagnostic work-up of suspected inflammatory diseases of the CNS as well as in the follow-up of patients with confirmed disease. Currently, MRI is the first-line diagnostic imaging modality in ADEM and MS and is fully integrated in the most widely used diagnostic criteria schemes, but it has a key role in clinical therapeutic research trials as well. This paper provides an update on the current concepts and strategies of MRI in inflammatory diseases of the CNS, as well as a review of the imaging semiology of the various disease entities and variants with emphasis on clinical and imaging particularities relevant to the pediatric patient population.]

Clinical Neuroscience

Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Clinical Neuroscience

Acute bilateral drop foot as a complication of prolonged squatting due to haemorrhoid

KOKSAL Ayhan, DOGAN Burcu Vasfiye

Drop foot is defined as difficulty of dorsiflexion of the foot and ankle due to weak anterior tibial, extensor hallucis longus and extensor digitorum longus muscles. Cauda equina syndrome, local peroneal nerve damage due to trauma, nerve entrapment, compartment syndrome and tumors are common etiologies. A 32-year-old male patient was applied with difficulty in dorsiflexion of both of his toes, feet and ankles after he had squatted in toilette for 6-7 hours (because of his haemorrhoid) after intense alcohol intake 2 weeks before. Acute, partial, demyelinating lesion in head of fibula segment of peroneal nerves was diagnosed by electromyography. This case was reported since prolonged squatting is an extremely rare cause of acute bilateral peroneal neuropathy. This type of neuropathy is mostly demyelination and has good prognosis with physical therapy and mechanical devices, but surgical intervention may be required due to axonal damage. People such as workers and farmers working in the squatting position for long hours should be advised to change their position as soon as the compression symptoms (numbness, tingling) appear.