Clinical Neuroscience

[Reversible hepatocerebral degeneration-like syndrome due to portovenous shunts]

SIBEL Güler, UFUK Utku, AHMET Tezel, ERCÜMENT Ünlü

NOVEMBER 30, 2013

Clinical Neuroscience - 2013;66(11-12)

[Ataxia and tremor are rare manifestations of hepatocerebral degeneration due to portovenous shunts. Ammonia is a neurotoxin that plays a significant role in the pathogenesis of hepatic encephalopathy. A 58-year old male patient was assessed with the complaints of gait disturbance, hand tremor, and impairment of speech. His neurological examination revealed dysarthric speech and ataxic gait. Bilateral kinetic tremor was noted, and deep tendon reflexes of the patient were hyperactive. Serum ammonia level was found to be 156.9 μg/dL. Cranial magnetic resonance (MR) imaging revealed increased signal intensity in bilateral globus pallidus on T1-weighted axial sections, and bilateral prominent hyperintense lesions in the middle cerebellar peduncles on T2-weighted axial sections. On his abdominal MR portography, multiple portohepatic venous collaterals were noted in the right and left lobes of liver parenchyma in 2D FIESTA axial MR sections. To our knowledge, we reported the first case of acquired hepatocerebral degeneration presenting with cerebral symptoms without any hepatic findings in which clinical improvement was noted, and hyperammonemia disappeared following medical treatment.]

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Clinical Neuroscience

[Post-operative management of primary glioblastoma multiforme in patients over 60 years of age]

DARÓCZI Borbála, SZÁNTÓ Erika, TÓTH Judit, BARZÓ Pál, BOGNÁR László, BAKÓ Gyula, SZÁNTÓ János, MÓZES Petra, HIDEGHÉTY Katalin

[Background and purpose - Optimal treatment for elderly patients with glioblastoma multiforme is not well defined. We evaluated the efficacy of post-operative radiotherapy with or without concomitant and/or adjuvant temozolomide in patients aged ≥60 years to assess survival and identify prognostic factors of survival. Methods - A retrospective analysis of overall survival and progression-free survival in patients with newly diagnosed glioblastoma multiforme aged ≥60 years treated with postoperative radiotherapy with or without temozolomide chemotherapy was conducted at our institutions. Prognostic factors were determined by univariate and multivariate analyses. Results - Of 75 study participants (54.7% male; median age at first diagnosis, 65.1 years), 29 (38.7%) underwent gross total resection, whereas others underwent partial resection or biopsy only. All but 1 patient received radiotherapy. Twenty patients received concomitant temozolomide only. Adjuvant temozolomide (1-50 cycles) was administered in 42 patients; 16 received ≥6 cycles. Median overall survival was 10.3 months. One- and 2-year overall survival rates were 42.6% and 6.7%, respectively. Median progression-free survival was 4.1 months. Radiochemotherapy was generally well tolerated. Median overall survival was 15.3 and 29.6 months for patients who received 6-12 cycles and >12 cycles of adjuvant temozolomide, respectively. There were no significant differences in overall survival between age groups (60-64, 65-69, and ≥70 years). Adjuvant temozolomide, Karnofsky performance status ≥70, and additional surgery after progression were significant prognostic factors of longer overall survival (p<0.05). Conclusions: Radiochemotherapy, including ≥6 cycles of adjuvant temozolomide, was safe and prolonged survival of glioblastoma patients aged ≥60 years. Aggressive therapy should not be withheld from patients aged ≥60 years with good performance status because of age.]

Clinical Neuroscience

[Treatment possibilities in advanced Parkinson’s disease]

TAKÁTS Annamária, NAGY Helga, RADICS Péter, TÓTH Adrián, GERTRÚD Tamás

[In the course of Parkinson’s disease, advanced and late stages can be distinguished. In the advanced stage, levodopa has good effect on motor symptoms, but patient care is often hindered by levodopa-induced complications such as motor fluctuation and dyskinesias. In the late stage levodopa response becomes poor, falls, dementia and psychotic symptoms appear and patients often need hospitalization. In the advanced stage, the quality of life may be improved better by device-aided therapy than by best oral medical treatment. The alternatives are apomorhin pump, levodopa carbidopa intestinal gel with pump and deep brain stimulation. The therapy plan should be based on the principle: “the right treatment, to the right patient, in the right time”.]

Clinical Neuroscience

[Status epilepticus and its treatment - Update 2013]

GYIMESI Csilla, JUHOS Vera, HORVÁTH Réka, BÓNÉ Beáta, TÓTH Márton, FOGARASI András, KOMOLY Sámuel, JANSZKY József

[Our study provides an overview of the results and guidelines published on the treatment of status epilepticus in the last five years. In recent years, as a result of scientific observations and collected data, the definition of and treatment approach to status epilepticus have been refined and novel therapeutic methods have been developed. The updated guidelines provide guidance in everyday medical practice. However, only a relatively small number of randomized studies are available on status epilepticus, especially in second-line treatment and third-line treatment, thus it is difficult to transfer the newest methods into clinical practice and into updates to treatment protocols. Due to the nature and epidemiology of the disease, the treatment of status epilepticus remains a daily challenge for healthcare providers. The key points of an effective treatment are: expeditiously initiating appropriate therapy, concurrent causal treatment and anticonvulsant therapy, early detection of nonconvulsive status epilepticus, as well as avoiding "overtreatment" and side effects.]

Clinical Neuroscience

[Psychosis as a process - New implications of staging models of schizophrenia]

HALMAI Tamás, TÉNYI Tamás

[The article discusses contributing factors in the pathogenesis of schizophrenia. In the last fifteen years, the emphasis has shifted from curative to prodromal and premorbid characteristics of later schizophrenia patients. Nevertheless, most studies are limited to the area of early detection and intervention of schizophrenia with much fewer focusing on actual prevention. A more general preventive approach not limited to psychotic condition is clearly underestimated. Following a review of current literature on prodromal approaches and identified premorbid markers of schizophrenia, the article outlines a possible trajectory of later psychotic condition with detectable, distinct stages from birth on. Based on this extended staging model involving neurotoxic impact and early prefrontal-limbic dysfunction, it argues for a refined, phase-specific treatment protocol including preventive interventions. Accepting a model of schizophrenia as an illness with detectable, phase-specific signs and symptoms from infancy on leads to the need to implement preventive interventions. Through this approach, we could, in the optimal case, be able to identify early signs of neuromotoric and cognitive dysfunction not specific for psychosis. Furthermore, it would be useful to lay greater emphasis on the detection of these early signs in the training of health care professionals. This approach calls for a close cooperation between psychologists, psychiatrists, neuropsychologists and special education experts and a change in the way we view psychotic illness.]

Clinical Neuroscience

[Diffusion MRI measured white matter microstructure as a biomarker of neurodegeneration in preclinical Huntington’s disease]

KINCSES Tamás Zsigmond, SZABÓ Nikoletta, TÓTH Eszter, ZÁDORI Dénes, FARAGÓ Péter, NÉMETH Dezsõ, JANACSEK Karolina, BABOS Magor, KLIVÉNYI Péter, VÉCSEI László

[Background - Huntington’s disease is a progressive neurodegenerative disease, genetically determined by CAG trinucleotide expansions in the IT15 gene. The onset of the symptoms is related to the number of CAG triplets. Because the patients are asymptomatic in the early phase of the disease, in vivo biomarkers are needed to follow up the neurodegeneration and to test putative neuroprotective approaches. One such promising biomarker is the diffusion MRI measured microstructural alteration of the white matter. Methods - Seven presymtomatic, mutation carriers and ten age-matched healthy controls were included in the study. Diffusion parameters were compared between groups and correlated with measures describing neurodegeneration. In order to reduce the possible misregistration bias due to atrophy the analysis was restricted to the core of each fibre bundles as defined by maximal fractional anisotropy (Tract- Based Spatial Statistics). Results - Decreased fractional anisotropy, along with increased mean, parallel and perpendicular diffusivity was found in white matter tracts, mainly in the corpus callosum. An inverse correlation was detected between the fractional anisotropy and neurodegeneration score (derived from the number of CAG triplets and the patient age) from the areas of the left precentral gyrus, frontal lobe, corpus callosum and the capsula extrema. Altered diffusion parameters are promising biomarkers of the neurodegeneration in Huntington’s disease.]

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Cholinesterase inhibitors and memantine for the treatment of Alzheimer and non-Alzheimer dementias

BALÁZS Nóra , BERECZKI Dániel, KOVÁCS Tibor

In aging societies, the morbidity and mortality of dementia is increasing at a significant rate, thereby imposing burden on healthcare, economy and the society as well. Patients’ and caregivers’ quality of life and life expectancy are greatly determined by the early diagnosis and the initiation of available symptomatic treatments. Cholinesterase inhibitors and memantine have been the cornerstones of Alzheimer’s therapy for approximately two decades and over the years, more and more experience has been gained on their use in non-Alzheimer’s dementias too. The aim of our work was to provide a comprehensive summary about the use of cholinesterase inhibitors and memantine for the treatment of Alzheimer’s and non-Alzheimers’s dementias.

Hypertension and nephrology

[Monitoring of the blood pressure lowering effectiveness of ramipril-amlodipine fix combination – a non-interventional trial (RAMONA study)]

TOMCSÁNYI János

[Purpose: Monitoring the effectiveness and safety of the fix combination formulation Egiramlon® therapy containing ramipril and amlodipin in patients, suffering from mild or moderate hypertension despite antihypertensive treatment. Patients and methods: Open, prospective, phase IV clinical observational study, which involved 9169 patients (age >18) with mild or moderate hypertension [TUKEB No: 16927- 1/2012/EKU (294/PI/12.)]. Ramipril/Amlodipin 5/5, 5/10, 10/5, 10/10 mg combinations were administered/ titrated in three visits, during the four months period according to the physician’s decision Blood pressure was measured by validated blood pressure sphygmomanometry and ABPM (Meditech, Hungary). The dosis of the fix combination formulation was determined individually during the visits by the 923 doctors involved in the study. The target blood pressure value was 140/90 mmHg, but in case of high risk patients population (diagnosed cardiovascular disease, diabetes), 130/90 mmHg target value was determined. Results: In 70.1% of the patients had no protocoll deviation. Patients data and examination results were processed according to this 6423 patient population. The average age of the patients were 60.2 year, in 50-50% sex distribution. The average duration of the treated hypertension was 9.8 years and the average blood pressure value was 157/91 mmHg. Till the end of the study, systolic blood pressure has decreased with 26.4 mmHg and diastolic pressure with 11.8 mmHg. An average 5.5 bpm heart rate frequency decreasing was observed at the end of the study. As a result of the treatment 52.4% of the patient population has reached the target blood pressure value.]

Hypertension and nephrology

[May measurement month: analysis of the Hungarian results of years 2017 and 2019]

NEMCSIK János, PÁLL Dénes, JÁRAI Zoltán

[Cardiovascular (CV) diseases are not only the leading causes of mortality in Hungary, but also the mortality rate is excessively high compared with the average of European Union, so screening programs identifying subjects with elevated blood pressure (BP) is of utmost importance. May Measurement Month (MMM) is an annual global initiative which began in 2017 aimed at raising awareness of high BP. Hungary, through the Hungarian Society of Hypertension has joined the campaign of MMM from the beginning. The results of years 2017 and 2019 are presented in this paper. ]

Clinical Neuroscience

[Diseases with peripheral motor symptoms ]

DELI Gabriella, KOMÁROMY Hedvig, PÁL Endre, PFUND Zoltán

[Diseases with peripheral motor symptoms are a rare, but important subgroup of the all peripheral neuropathies, radiculopathies and neuronopathies. In these mostly progressive neuropathies, the clinical features include pure motor symptoms with weakness and wasting of the striated muscles. The differentiation of these diseases is frequently a challenge for qualified clinical neurologists. A careful history taking, the disease time course, the findings of routine clinical physical examination and the electrophysiological studies are all necessary in the diagnostic procedure. The aim of this publication is to overview the clinical characteristics of the pure motor peripheral neuropathies, to consider the diagnostic steps and the differential diagnosis, and finally to summarize the treatment options. ]

Hypertension and nephrology

[Subtype diagnosis, treatment, complications and outcomes of primary aldosteronism and future direction of research: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension – excerpts]

KOVÁTS Viktória, ALFÖLDI Sándor

[Primary aldosteronism is a frequent cause of secondary hypertension requiring a specific pharmacological treatment with mineralocorticoid receptor antagonist or with unilateral adrenalectomy. These treatments have shown to reduce the excess of cardiovascular risk characteristically associated with this disease. In this consensus, we discussed the procedures for the diagnosis of primary aldosteronism, we address the strategies for the differential diagnosis of primary aldosteronism subtypes and therapy. We also discuss the evaluation of outcomes and provide suggestions for follow-up as well as cardiovascular and metabolic complications specifically associated with primary aldosteronism. ]