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

[József Kómár is 75 years old]

KISS Gábor

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

[Congress calendar]

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

[Genetic risk factors in Alzheimer’s disease]

FEHÉR Ágnes

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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

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

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.

Lege Artis Medicinae

[The clinical importance of HER2 expression in breast cancer]

KAHÁN Zsuzsanna

[HER2 (neu/c-erbB-2) is a member of the EGF receptor family. It is activated without binding a specific ligand that leads to malignant transformation and tumor progression. Overexpression of HER2 is detected in approximately one quarter of human breast cancers. Immunohistochemistry and in situ hybridization (FISH) are the most widely used techniques in studying HER2 expression. HER2 positivity indicates worse outcome in node positive breast cancer and increasing number of studies show unfavourable prognosis in node negative cases as well. Recent data indicate that the knowledge of HER2 status may promote therapeutic decision. The generally applied cyclophosphamide- methotrexate-5-fluorouracil (CMF) polychemotherapy seems to provide no benefit in HER2 positive cases in contrast with HER2 negative breast cancer patients. Interestingly, doseintensive doxorubicin based chemotherapy gives better results in HER2 positive than in HER2 negative tumors. Determination of HER2 expression has great importance before therapeutic application of the humanized antibody trastuzumab (Herceptin). HER2 overexpression usually correlates well with estrogen and progesterone receptor negativity and hormone-resistance, therefore hormonal therapy is not justified for these patients. Some experimental and clinical data indicate that in case of simultaneous HER2 and ER positivity tamoxifen worsens treatment results which may be prevented by the coadministration of tamoxifen and trastuzumab. Emerging experimental and clinical data about HER2 has led to a new stage of individual treatment of breast cancer patients. The knowledge of HER2 status promotes antitumor intervention based on molecular characteristics of breast cancers. Therefore, reliable HER2 tests are needed in the everyday practice.]