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Clinical Neuroscience

MAY 30, 2019

[In memoriam Mátyás Papp]

KOVÁCS Tibor

[Mátyás Papp died on 4th of April, 2019, at the age of 92, following a long disease. He was working for nearly 60 years in the Department of Neurology, Semmelweis University. He was known about his works on the inclusion bodies in multiple system atrophy (Papp-Lantos bodies). He was a honorary member of the International Society of Neuropathology. ]

Clinical Oncology

FEBRUARY 10, 2015

[Molecular diagnostics of brain tumors - an up-date]

REINIGER Lilla, HANZÉLY Zoltán, BÁLINT Katalin, TURÁNYI Eszter

[In recent years there have been major advancements in the understanding of molecular events driving brain tumor genesis and progression. Although state-of-the-art techniques are not widely available, many of the molecular discoveries lead to novel antibodies that can assist in identifying the major molecular subgroups by immunohistochemistry. Molecular informations will likely be incorporated into the next World Health Organization (WHO) classifi cation of central nervous system tumors, but clinical practice in many centres have already taken on the available informations and therapeutic decisions are made based on genetic/epigenetic information. In the adult population IDH, ATRX and 1p/19q codeletion studies help to defi ne molecular subgroups that correlate better with prognosis and therapeutic response than traditional histology based diagnosis. The KIAA1549-BRAF fusion gene is a hallmark for pilocytic astrocytomas, while diffuse pediatric gliomas lack the IDH mutations and 1p/19q codeletions that are common in adult astrocytomas and oligodendrogliomas. Uncommon in adults, Histone H3.3 mutations are pathognomic in pediatric brainstem malignant gliomas. Molecular subgroups of medulloblastomas have also been identifi ed, and a corresponding set of antibodies are ready to guide treatment decisions in those centres where molecular techniques are not available. These genetic and epigenetic events determine a tumor’s behaviour, and integrating this level of informations into neuropathology practice is essential to provide the best possible care to both pediatric and adult patients.]

Clinical Neuroscience

OCTOBER 05, 2013

[The diagnosis of herpesencephalitis - a case-based update]

CSONKA Tamás, SZEPESI Rita, BIDIGA László, MÓZES Péter, KLEKNER Álmos, HUTÓCZKY Gábor, CSIBA László, MÉHES Gábor, HORTOBÁGYI Tibor

[Herpes simplex virus encephalitis (HSVE) is a rare and lifethreatening infection. The clinical signs are diverse and often misleading regarding the aetiology. However, focal seizure with fewer and typical CT/MRI finding should always raise the possibility of HSVE as early diagnosis and antiviral therapy is crucial. Before the advent of molecular techniques and high-tech imaging histological examination from multiple brain biopsies were often necessary. Although nowadays PCR and other molecular methods may provide an aetiological diagnosis some cases need neuropathological verification. Due to the high IgG seropositivity rate in the population the plasma IgG titer is not diagnostic and elevation of its plasma level requires several weeks. We report the case of a 25-years old male patient who initially presented with epileptic fits. There was no final diagnosis and causal treatment in the district general hospital. The patient was admitted to our institution in comatose state on day 9; the initiated diagnostic tests and therapy could not save the patient who died next day. The autopsy and subsequent neuropathological examination revealed HSVE. We present a flowchart on diagnostic work-up and special techniques to aid diagnosis in suspected viral encephalitis.]

Clinical Neuroscience

JANUARY 22, 2008

[FREQUENCY OF DIFFERENT FORMS OF DEMENTIA IN THE DEPARTMENT OF NEUROPATHOLOGY OF THE HUNGARIAN NATIONAL INSTITUTE OF PSYCHIATRY AND NEUROLOGY DURING A 3-YEAR PERIOD]

KOVÁCS Gábor Géza, KŐVÁRI Viktor, NAGY Zoltán

[Background - Dementia is an increasing problem of the society. The underlying cause of dementia may be difficult to diagnose during life. Only neuropathological examination gives definite diagnosis. Differences in the reported frequency may be related to factors such as the age or gender of subjects with dementia. Materials and methods - In our neuropathology-based study we examined 156 consecutive subjects clinically diagnosed with dementia during a 3-year period. Using histopathological criteria we calculated the frequencies of various disorders causing dementia. We studied the effect of age and gender on these frequencies. Results - Alzheimer’s disease was the most frequent pathologic finding (57.7%) followed by vascular dementia (43%); diffuse Lewy body disease (15.4%); argyrophilic grain dementia (12.1%), various forms of frontotemporal dementia (5.7%); and other (4.5%). The latter comprise prion disease, alcoholic encephalopathy, and hippocampal sclerosis. Mixed pathology was common: concomitant Alzheimer’s disease was present in 41.6% of diffuse Lewy body disease cases and in 49.2% of vascular dementia patients. Pure disease forms are rare: Alzheimer’s disease: 26.3%, vascular dementia: 17.3%, diffuse Lewy body disease: 5.1%, argyrophilic grain dementia: 2.5%. Females were overrepresented among those with Alzheimer’s disease with age at death above 75 years (p <0.02), while males were overrepresented in patients below 75 years with vascular dementia (p <0.05). Conclusions - Our study indicates that the frequency of neurodegenerative dementias is high in the examined patients, but vascular pathology frequently influences the clinical course.]

Clinical Neuroscience

NOVEMBER 30, 2007

[THE ROLE OF CHRONIC BRAIN HYPOPERFUSION IN THE PATHOGENESIS OF ALZHEIMER'S DISEASE - FACTS AND HYPOTHES]

ZÁDORI Dénes, DATKI Zsolt, PENKE Botond

[In Alzheimer’s disease, which belongs to the neurodegenerative disorders, the ethiopathogenetic role of several risk factors has been proved. A considerable number of them are mainly known as cardiovascular risk factors and can precipitate chronic brain hypoperfusion. Using functional imaging techniques, this hypoperfusion and the resulting hypometabolism become detectable in the watershed areas of the brain as early as in the stage of mild cognitive impairment. Hypoperfusion leads to the degeneration of capillaries in this area causing the deterioration of diffusion. The further reduction of nutrient and oxygen support of neurons is capable to initiate a neurodegenerative process which spreads along the glutamaterg system arising from the neurons of the association cortices. The neuropathological lesions of this neuronal system, such as the neurofibrillary tangles and the β-amyloid plaques, are known to be the characteristic markers of Alzheimer's disease. In our review we present the development of hypoperfusion and its consequences in the watershed areas of the brain and describe the neurodegenerative process of the neuronal system arising from the neurons of the association cortices in the early stage of Alzheimer's disease. Considering the previous hypotheses and the neuropathological lesions of Alzheimer's disease we give a new consensus model to characterize the pathomechanism of the disorder.]

Clinical Neuroscience

MAY 20, 2007

[NOVEL CELL-BIOLOGICAL IDEAS DEDUCIBLE FROM MORPHOLOGICAL OBSERVATIONS ON “DARK” NEURONS REVISITED]

GALLYAS Ferenc

[The origin, nature and fate of ”dark“ (dramatically shrunken and hyperbasophilic) neurons are century-old problems in both human and experimental neuropathology. Until a few years ago, hardly any cell-biological conclusion had been drawn from their histological investigation. On the basis of light and electron microscopic findings in animal experiments performed during the past few years, my research team has put forward novel ideas concerning 1. the nature of ”dark“ neurons (malfunction of an energystoring gel-structure that is ubiquitously present in all intracellular spaces between the ultrastructural elements), 2. the mechanism of their formation (non-programmed initiation of a whole-cell phase-transition in this gel-structure), 3. their capability of recovery (programmed for some physiological purpose), 4. their death mode (neither necrotic nor apoptotic), and 5. their relationship with the apoptotic cell death (the gel structure in question is programmed for the morphological execution of ontogenetic apoptosis). Based on morphological observations, this paper revisits these ideas in order to bring them to the attention of researchers who are in a position to investigate their validity by means of experimental paradigms other than those used here.]

Clinical Neuroscience

SEPTEMBER 30, 2006

[MULTIPLE SYSTEM ATROPHY: THE BEGINNING OF A NEW ERA IN THE HISTORY OF NEURODEGENERATIVE DISEASES]

PAPP Mátyás, KOVÁCS Tibor

[Multiple system atrophy (MSA) belongs to the neurodegenerative diseases of the nervous system, but it is different from them in many aspects: it has no familiar form and no genetic factor was identified in the pathomechanism. Its neuropathology is unique too, because oligodendroglial cells are harbouring the main pathological burden. It was described in MSA that there is no elective neuronal degeneration in neurodegenerative disorders: the glial cells show the same patochemical and structural abnormalities as found in the neurones. The discovery of the glial cytoplasmic inclusions, as a pathognostic marker for MSA, has directed attention to the glial cells in other neurodegenerative disorders. As a result of this, there are several neurodegenerative diseases nowadays in which glial inclusions were described, similar to the neuronal inclusions in their structural and biochemical properties and some of them became the diagnostic marker of the disease. In our review we summarize the clinical features, the history and the neuropathology of MSA and we discuss its special features.]

Clinical Neuroscience

MAY 30, 2006

[NEUROPATHOLOGICAL EXAMINATIONS IN AUTOPSIES WITH SPECIAL FOCUS ON FINDINGS IN ALZHEIMER’S DISEASE]

LEEL-ŐSSY Lóránt, KINDLER Miklós, SZŰCS Iván, SCHWARCZ Tibor

[Out of an average total of 1400 autopsies per year, neuropathological examinations were performed in 477 cases between 1997 and 1999 to investigate the incidence of dementias. The majority of the studied subjects were over 50 years old. Bielschowsky's and/or Gallyas's silver methods and, in some cases, protein tau (MAP) immuncytochemistry and amyloid staining were performed beside routine examination. Pathological changes were found in 212 of the 364 cases studied by the above methods but histological changes associated with dementia were only detected in 167 cases. The various forms of Alzheimer's dementia were also classified by age. The "incipient" form of Alzheimer's disease was verified in 23 cases. Old infarcts of various extensions were found in 42 percent of Alzheimer's dementias. Very mild or age-related degenerative changes were observed in 82 cases among subjects over 50 years old. Of these, eight patients died in their 90s. In some cases (n=38) the number of neuritic plaques dominated over the number of neurofibrillary tangles but a reverse finding also occurred (n=13). Neuronal degeneration was variable and was not always proportional to the number of neurofibrillary tangles. "Simple type of senile atrophy" was defined by the presence of minor or age-related Alzheimer changes and was considered a separate entity. The "incipient" form of Alzheimer's dementia was diagnosed in relatively young individuals where mild Alzheimer changes were found at the neuropathological examination. "Preclinical" Alzheimer's dementia could only be suspected by clinical data and could very rarely be supported by the neuropathological finding of "incipient" form. The ratio of pure Alzheimer’s to vascular dementias cases proved to be 54:41 in this study. The results suggest that dementias are considerably underdiagnosed both in the clinical and pathological practice and that the recently defined "preclinical" and "incipient" forms are very hard to recognize both clinically and pathologically. The neuropathological study of the degenerative, mainly Alzheimer's type, findings in the randomly selected autopsies revealed great variations which raises many questions concerning the normal and pathologic aging of the brain as well as the "incipient" and senile forms of Alzheimer's dementia.]