Clinical Neuroscience

[Surgically cured resistant epilepsy - caused by hemispherical dysgenesis - Case report]

HEGYI Márta1, SIEGLER Zsuzsa1, BARSI Péter2, RUDAS Gábor2, LENGYEL Zsolt3, SZAKÁLL Szabolcs3, BOGNÁR László4, KOZÁK Lajos Rudolf5, NEUWIRTH Magdolna1, FOGARASI András1

JUNE 02, 2009

Clinical Neuroscience - 2009;62(05-06)

[A part of patients with the therapy resistant epilepsy can be cured by surgical interventions. The more concordant the presurgical evaluation data, the better prognosis the patient has postoperatively. In case of discordant examination data, multimodal evaluation or case-specific decision might be successful. We report on a five-year-old boy with bilateral (left-dominated) cortical dysplasia and therapy resistant epilepsy. The ictal EEG did not help to localize the seizure onset zone, semiology had little lateralization value; however, FDG-PET showed left hemispherial hypermetabolism. The child became almost seizure-free and showed improved development after left-sided hemispherotomy.]

AFFILIATIONS

  1. Magyar Református Egyház Bethesda Gyermekkórháza, Budapest
  2. Semmelweis Egyetem, Szentágothai János Tudásközpont, MR Kutatóközpont, Budapest
  3. Pozitron Diagnosztika Kft., Budapest
  4. Debreceni Egyetem, Orvos- és Egészségtudományi Centrum, Idegsebészeti Klinika, Debrecen
  5. MR Kutatóközpont, Szentágothai János Tudásközpont, Semmelweis Egyetem, Budapest

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[Pain sensitivity changes in schizophrenic patients and animal models - Part II.]

TUBOLY Gábor, HORVÁTH Györgyi

[Diminished pain sensitivity in schizophrenic patients has been reported for more than 50 years, however little is known about the substrate and the basic mechanisms underlying altered pain sensitivity in this disease, therefore, relevant animal models are of decisive importance in the study of psychiatric diseases. The authors report a review consisting of two parts focusing on pain sensitivity changes in patients and in different animal models which proved the eligibility as schizophrenia models and pain sensitivities have also been determined. The second part of this article analyzed the results regarding knock out mice as schizophrenia models. These data proved that several genes have significant role in the pathomechanism of schizophrenia; therefore deficiency in one gene does not produce animals showing all signs of this disease. As regards the pain sensitivity changes, only a few data are available with controversial results. Data originated from complex chronic animal models indicate that they might be more adequate methods for studying the mechanisms of schizophrenia including the pain-sensitivity changes.]

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[Genetics and present therapy options in Parkinson’s disease: a review]

BEREZNAI Benjámin, MOLNÁR Mária Judit

[In the past years, six monogenic forms of Parkinson disease have clearly been associated with this movement disorder. The most frequent forms are LRRK2- and Parkin-associated Parkinson disease. Currently, a genetic diagnosis does not change the therapy, the genes involved in genetic Parkinson disease help to understand the underlying pathophysiologic mechanisms of Parkinson disease. Beside the overview of the molecular-genetic basis, we give a review about genetic testing, pharmacological and other multidisciplinary treatment options.]

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[Hypertension and it’s therapy in acut phase of stroke]

KÁPOSZTA Zoltán, RÁCZ Klára

[The elevation of blood pressure above normal and premorbid values within the first 24 hours of symptom onset in patients with stroke is relatively common. This acute hypertensive response is usually managed by different group of physicians, including general practitioners, emergency physicians, neurologists, internists, intensivisists. Management strategies of this phenomenon vary considerably. The first consideration in blood pressure management in this clinical setting is to determine whether the patient might be a candidate for thrombolytic therapy. For those patients are not entitled to that therapy premorbide blood pressure values and the type of stroke are the key data for sufficient control of hypertension. In patients with chronic hypertension, the lower end of the autoregulation curve is shifted toward high pressure and an impaired autoregulation due to acute stroke may increase the risk for further brain tissue damage if the blood pressure is inadequately controlled. The current guidelines recommend lowering blood pressure in patients with an intracranial haemorrhage below 160- 180/100-105 mmHg, if the patient is normotensive, while the target level is 180/105 mmHg in hipertensive patients. However, in ischaemic stroke no treatment is recommended if systolic blood pressure <220 mmHg and/or diastolic blood pressure <120 mmHg in the acute stage. Clinical studies are rare which assess the effectiveness of different antihipertensive drugs in acute stroke. The first strong evidence came from the ACCESS (The Acute Candesartan Cilexetil Therapy in Stroke Survivors) trial which suggested that a 7-day course of candesartan after an acute ischaemic stroke significantly improves cardiovascular morbidity and mortality.]

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[99-mTc-HMPAO single photon emission computed tomography examinations in genetically determined neurometabolic disorders]

ARANKA László, AMBRUS Edit, VÖRÖS Erika, SVEKUS András, KÓBOR Jenõ, BEREG Edit, PALATKA János, PÁVICS László

[The aim of our study was to determine regional cerebral blood flow (rCBF) abnormalities in different types of enzymopathies. Patients and methods - Among the patients with genetically determined enzymopathies 3 patients had aminoacidopathies, and 11 had different types of encephalopathies, from which 10 had mitochondrial encephalomyopathy (MEMP), and 1 patient had hyperuricaemic encephalopathy. Besides the mentioned 14 patients, 1 had ceroid lipofuscinosis and another patient had tuberous sclerosis. The further distribution of the MEMP patients’ group was the following - 5 patients had MEMP with lactic acidosis, 5 had Leigh’s disease (subacute necrotizing encephalopathy), from which 1 had cytochrome-c-oxidase deficiency (COX). Additionally in all patients were performed cerebral MRI and SPECT examination 10 min. after intravenous administration of 20 Mbq/kg 99 mTc-HMPAO. Results - Fourteen out of 16 SPECT findings were pathologic, showing decreased focal frontal/temporal/temporoparietal cerebral blood perfusion. Aminoacidopathic group - all the 3 patients revealed pathologic signs from the aminoacidopathic patients’ group. Among them the ornithine transcarbamylase (OTC) heterozygous female patient with left-sided hemiparesis caused by hyperammonemic stroke at 10 month-age, showed right sided temporoparietal, occipital and left frontal hypoperfusion, nearly 6 years after the cerebral vascular attack. This finding might be resulted because of diaschisis. Mitochondrial encephalo-myopathic (MEMP) group - all the four patients with MEMP and lactic acidosis showed focal hypoperfusion in the temporal region, while the perfusion was normal in the COX deficient patient and in 2 Leigh’s disease (subacute necrotizing encephalopathy) patients. In the remaining 1 Leigh’s patient frontotemporal hypoperfusion was found. In all patients there were non specific structural abnormalities detected by MRI - cortical and subcortical atrophy, and scattered demyelination foci. In the case of ceroid lipofuscinosis the MRI showed cerebral atrophy and cerebellar hypoplasia, and the SPECT showed right frontal and occipital hypoperfusion, bilateral parietal physiological riping process. The patient with tuberous sclerosis showed bilateral temporo-occipital hypoperfusion. Conclusion - 1. SPECT images demonstrated hypoperfusion rCBF changes in 14 out of all 16 patients. 2. Regional cerebral/cerebellar hypoperfusion was detected by SPECT in mitochondrial encephalomyopathies, with lactate acidosis and aminoacidopathies giving high informative value about the cerebral perfusion.]

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[Long term experience with Stalevo]

KLIVÉNYI Péter, VÉCSEI László

[The triple combination of levodopa, DDCI and entacapone (Stalevo) is used to treat motor complication in patients with Parkinson,s disease. In this study we summarized the clinical data of our patients treated with Stalevo for the longest period. We can concluded, that after switching to Stalevo due to wearing off, the average levodopa doses were lower then before and the motor complications were milder. After 3 years of Stalevo therapy the levodopa doses were increased but still did not reach the average doses before introducing Stalevo. After switching the patients, general well-being was improved as indicated by the visual analogue scale. In summary, the Stalevo treatment is safe and effective for long run and improves the patients, quality of life.]

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[Clonazepam can facilitate sensorimotor functional MRI examinations in status epilepticus during sleep]

[Functional magnetic resonance imaging examinations became an integral part of epilepsy surgery workup. In the pediatric population these examinations are usually carried out in full anesthesia, however in some forms of epilepsy, e.g. electrical status epilepticus in sleep, anesthesia could jeopardize the success of the examination. Here, we show on the example of an eight-year-old polymicrogyric epileptic child, that clonazepam can help suppressing the epileptic activity during propofol anesthesia, so it could facilitate performing passive sensorimotor functional MRI in such a case. Furthermore, among the methodological issues addressed, this case provides evidence for a post-hemispherotomy like functional redistribution of sensorimotor activations to the unaffected hemisphere in a case of childhood epilepsy.]

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Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.

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[What happens to vertiginous population after emission from the Emergency Department?]

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[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

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[The connection between the socioeconomic status and stroke in Budapest]

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[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]