Folia Neuropathol
November 2024
Clear cell meningioma (CCM) is a rare subtype of meningioma, especially unusual as a neoplasm of the filum terminale. Clear cell meningioma seems to have a more aggressive nature and a higher risk of recurrence than WHO grade I meningiomas. A 44-year-old woman presented with lower back pain radiating to the left leg and mild weakness in the left leg.
View Article and Find Full Text PDFSubacute sclerosing panencephalitis (SSPE) is a fatal, slowly progressive brain disorder caused by a mutated measles virus. Both subacute inflammatory and neurodegenerative mechanisms appear to play significant roles in the pathogenesis. TAR DNA-binding protein 43 (TDP-43) inclusions are a common co-pathology in several neurodegenerative disorders with diverse pathogenesis.
View Article and Find Full Text PDFIntracranial collision tumours are rare pathologies in which two distinct neoplasms are found in the same location. We present an unusual case of an intracranial collision tumour composed of meningothelial meningioma (CNS WHO G1) and glioblastoma (IDH-wildtype, CNS WHO G4). This collision tumour was found in a 64-year-old man.
View Article and Find Full Text PDFTransactivation (TAR) DNA binding protein 43 kDa (TDP-43) inclusions frequently occur as a comorbid pathology in several neurodegenerative disorders, including Alzheimer's disease, Huntington's disease, Lewy body disease, and progressive supranuclear palsy, and may appear in association with nondegenerative neurological etiology, for example neoplastic, paraneoplastic, traumatic, or infectious. Relationships between various pathological proteins and mechanisms associated with TDP-43-induced neurodegeneration are still not fully understood. Thus, overlap of distinct neuropathological mechanisms frequently leads to greater brain atrophy and a more severe clinical course, suggesting the importance of co-pathologies in ante-mortem diagnosing and treatment.
View Article and Find Full Text PDFAcute disseminated encephalomyelitis (ADEM) is an immune demyelinating central nervous system (CNS) disorder, characterized by monophasic new onset neurological symptoms including encephalopathy, combined with neuroradiological evidence of multifocal demyelination. ADEM is extremely rarely diagnosed and is much more common in children and adolescents than in adults. The aim of this study is to present an extremely rare case of ADEM in a heroin-addicted patient with a very difficult diagnostic course.
View Article and Find Full Text PDFTransactive response DNA binding protein of 43 kDa (TDP-43) is considered to play an essential role in the pathogenesis of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Growing body of evidence indicate that pathological TDP-43 inclusions frequently occur in the context of other distinctive hallmark pathologies, referred to as secondary TDP-43 proteinopathies. Comorbid TDP-43 pathology is well-documented in several neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, multiple system atrophy, or progressive supranuclear palsy.
View Article and Find Full Text PDFGlioblastoma (GBM) is the most malignant type of glial tumor associated with a very unfavorable prognosis. Typical radiological features of GBM include the presence of a tumor with irregular contrast-enhancing margins and central necrosis surrounded by a wide area of vasogenic edema. Here, we presented an atypical clinical presentation of GBM mimicking autoimmune meningitis.
View Article and Find Full Text PDFA diffuse leptomeningeal glioneuronal tumours (DLGNT) are very rare tumours of the central nervous system, typically characterized by enhancement of subarachnoid space with cystic lesions, diffuse leptomeningeal infiltration, and no primary mass. We report an atypical clinical presentation of DLGNT. A 48-year-old male was admitted to hospital with symptoms of ischaemic stroke.
View Article and Find Full Text PDFBackground: Since the new World Health Organization (WHO) classification of nervous system tumors (2016, revised, 4th edition) has been released, gliomas are classified depending on molecular and genetic markers in connection with histopathology, instead of histopathology itself as it was in the previous classification. Over the last years, epigenetic analysis has taken on increased importance in the diagnosis and treatment of different cancers. Multiple studies confirmed that deoxyribonucleic acid (DNA) methylation and hydroxymethylation play an important role in the regulation of gene expression during carcinogenesis.
View Article and Find Full Text PDFWe performed ultrastructural studies of mitochondria and evaluated the appearance of small blood vessels of three middle-aged siblings affected by the same mutation in the NOTCH3 gene, causing CADASIL. CADASIL pathognomonic features include granular osmiophilic material (GOM), which we observed. GOMs were located in damaged and thickened basement membranes (BM) of capillaries and arterioles.
View Article and Find Full Text PDFIntroduction: Migraine is considered not only as a separate clinical entity but also as a symptom of various brain disorders, including cerebral small vessel diseases. Since cerebral small vessel diseases are usually general angiopathies, evaluation of biopsy material other than brain tissue may help in their diagnosis in vivo. In patients with migraine, brain magnetic resonance imaging (MRI) often shows hyperintense changes in the cerebral white matter.
View Article and Find Full Text PDFCoronavirus disease 2019 (COVID-19) poses a global challenge to healthcare and society in the early 21st century. We report neuropathological changes in 52 patients aged between 22 years and 88 years (median 58 years) who were infected with the CoV-2 coronavirus. Patients died under various circumstances and had various pre-existing diseases.
View Article and Find Full Text PDFEncephalitis/encephalomyelitis in the course of rheumatoid arthritis (RA) remains a matter of debate. We present a case of a patient with encephalomyelitis associated with RA confirmed with post-mortem neuropathological examination. A 68-year-old woman with a long-standing, seropositive history of RA presented progressive disturbances of consciousness.
View Article and Find Full Text PDFWe present a female patient with a mutation of the POLG gene (POLG DNA polymerase gamma, catalytic subunit; *174763) in which the clinical course suggested a mitochondrial disease, a neuropathological examination identified the syndrome more closely, and a genetic test confirmed the disease. Apart from the morphological lesions typical of Alpers-Huttenlocher syndrome, rarely observed symmetrical degenerative changes in the accessory olivary nuclei were found. It was unusual in the clinical course of the disease that pancreatitis was diagnosed before symptoms of liver failure appeared.
View Article and Find Full Text PDFThe contribution of glutamatergic transmission to generation of initial convulsive seizures (CS) is debated. We tested whether pretreatment with a glutamine synthetase (GS) inhibitor, methionine sulfoximine (MSO), affects the onset and progression of initial CS by cholinergic stimulus in juvenile rats. Male rats (24 days old, Sprague Dawley) sequentially received i.
View Article and Find Full Text PDFHerpes simplex encephalomyelitis (HSE) is a rare disease with a high mortality rate. Correct diagnosis is established on the basis of the combination of the clinical and investigative features. Unfortunately, precise diagnosis remains difficult due to several clinical similarities and false negative or inconclusive results of diagnostic tests.
View Article and Find Full Text PDFIntroduction: Adult neurogenesis includes proliferation and differentiation of progenitor cells as well as their migration and maturation. In the adult human brain, two neurogenic regions, the hippocampal dentate gyrus (DG) and the subventricular zone (SVZ) of lateral ventricles, have been identified. In the dentate gyrus, three types of transcriptionally active cells and in the subventricular zone, four types of transcriptionally active cells, including GFAP-positive neural stem cells (NSCs), have been differentiated.
View Article and Find Full Text PDFWe describe a case of 52-year-old woman with a medical history of Crohn's disease presented abrupt fever, asymmetrical multiple cranial nerve palsies and focal neurological symptoms localized to the brainstem. The patient was initially diagnosed with ischaemic stroke, because of acute clinical course and results of neuroimaging. Cerebrospinal fluid analysis revealed mild infection with negative Gram staining and culture.
View Article and Find Full Text PDFWriters' cramp is a movement disorder with dystonic co-contraction of fingers and hand during writing and is part of the clinical spectrum of focal dystonias. Previous studies showed reduced striatal dopamine receptor D2 (DRD2) availability in dystonia. The expression of D2 receptors is modulated by a DRD2/ANKK1-Taq1A polymorphism (rs1800497).
View Article and Find Full Text PDFSpinal and bulbar muscular atrophy (SBMA, Kennedy's disease) is an X-linked recessive disease affecting lower motor neurons. In the present case report, we describe morphological changes in a muscle biopsy obtained from a 62-year-old patient with gynecomastia and with the following neurological symptoms: dysphagia, dysarthria, wasting and fasciculation of the tongue, proximal weakness, fasciculations in the limb muscles, and an absence of all tendon reflexes. Neurogenic alternations were predominantly observed using light and electron microscopy.
View Article and Find Full Text PDFIntramedullary arteriovenous malformations (AVMs) in the cervical region are a rare clinical condition. They represent a therapeutic challenge, as the lesions may cause serious functional disorders due to their location within or immediately adjacent to the critical ascending and descending sensorimotor pathways. In this case report, we present a patient with a cervical intramedullary AVM that was treated with endovascular therapy.
View Article and Find Full Text PDFAnxiety, depression, and alcohol use disorders often go together. The aim of the present study was to evaluate anxiety- and depressive-like traits in selectively bred Warsaw alcohol high-preferring (WHP) and Warsaw alcohol low-preferring (WLP) rats. Alcohol-naïve WHP rats were more active in the open field test as compared to alcohol-naïve WLP rats.
View Article and Find Full Text PDFAims: An assessment of the acoustic startle response (ASR) and prepulse inhibition (PPI) of ASR in laboratory animals is used to model human anxiety and psychotic states, respectively. The aim of the study was to evaluate ASR and PPI in alcohol-naive male and female Warsaw alcohol high-preferring (WHP) and Warsaw alcohol low-preferring (WLP) rats.
Methods: ASR and PPI were assessed in two separate experiments by using the SR-LAB apparatus (San Diego Instruments, San Diego, CA, USA).