Intracerebral hemorrhage in the context of cerebral amyloid angiopathy and varied time of onset of cerebral venous thrombosis: a case report.

In patients with cerebral venous thrombosis (CVT) the incidence of intracerebral hemorrhage (ICH) is estimated at about 37% and subarachnoid hemorrhage (SAH) at 1% of patients. A case with coincident occurrence of ICH, SAH and CVT in a patient with cerebral amyloid angiopathy (CAA) is reported. A 79-year-old woman was admitted to the Neurological Department after the occurrence of generalized seizures, the first in her life.

Lenticular nucleus hyperechogenicity in Wilson's disease reflects local copper, but not iron accumulation.

In patients with Wilson's disease (WD) transcranial brain sonography typically reveals areas of increased echogenicity (hyperechogenicity) of the lenticular nucleus (LN). Correlation with T2-hypointensity on magnetic resonance images suggested that LN hyperechogenicity in WD is caused by trace metal accumulation. Accumulation of both, copper and iron, in the brain of WD patients has been reported.

The association between cerebral amyloid angiopathy and atherosclerosis in patients with intracerebral hemorrhages.

Unlabelled: aim of the study: To analyze the incidence and grade of cerebral amyloid angiopathy (CAA) and atherosclerosis (AS) in cerebral vessels in patients who died from spontaneous intracerebral hemorrhage.

Material And Methods: The clinical diagnosis, based on CT scans of the brain, was made and immunohistochemic neuropathological examinations were performed in patients with intracerebral hemorrhages due to CAA. Cerebral amyloid angiopathy was diagnosed according to the Boston criteria.

β-amyloid deposits in veins in patients with cerebral amyloid angiopathy and intracerebral haemorrhage.

Aim Of The Study: To review the incidence and grade of cerebral amyloid angiopathy (CAA) in veins in patients who died due to spontaneous intracerebral haemorrhage (ICH).

Material And Methods: Neuropathological examinations were performed in the study group of 189 patients. Cerebral amyloid angiopathy was diagnosed according to the Boston criteria and confirmed during an autopsy.

Alcohol-induced changes in the developing cerebellum. Ultrastructural and quantitative analysis of neurons in the cerebellar cortex.

Maternal ethanol consumption during pregnancy may cause foetal alcohol syndrome (FAS). Our experiments of ethanol-treated female rats were based on the FAS model in humans; therefore, the results obtained may help explain the clinical mechanism of the disease development. The ultrastructural examination of the cerebellar cortex of ten-day-old rat pups of ethanol-treated dams during pregnancy (group IA), pregnancy and lactation (group IIA), and lactation (group IIIA) revealed that alcohol administration leads to a delayed maturation of Purkinje cells.

The value of magnetic resonance imaging in the early diagnosis of Creutzfeldt-Jakob disease - own experience.

Background: Creutzfeldt-Jakob disease (CJD) is a rare progressive neurodegenerative disorder, caused by the deposition of the pathological isoform of prion protein PrPsc in the central nervous system. The classic triad of symptoms consists of: rapidly progressive dementia, myoclonus and typical electroencephalographic findings (intermittent rhythmic delta activity and periodic sharp wave complexes). Detection of 14-3-3 protein in the cerebrospinal fluid plays an important diagnostic role as well.

Inflammatory cerebral amyloid angiopathy: the overlap of perivascular (PAN-like) with vasculitic (Aβ-related angiitis) form: an autopsy case.

Beside advanced age, cerebral amyloid angiopathy (CAA) and hypertension (HTA) are the two most important risk factors for haemorrhagic stroke. Inflammatory changes of amyloid-laden vessels have been reported only in rare sporadic CAA cases. We present the case of a 78-year-old woman with a history of hypertension, dementia and haemorrhagic stroke of the right frontal lobe 2 years before admission.

Astroglia disturbances during development of the central nervous system in fetuses with Down's syndrome.

Down's syndrome (DS), caused by aneuploidy of chromosome 21, is the most common chromosomal disorder. The most significant symptom of this disorder is mental retardation. Neuropathological changes found in the DS central nervous system (CNS), such as reduced number of neurons, alteration of synapses and synaptic spines or delayed myelination have been widely described.

Differential expression of calbindin D28k, calretinin and parvalbumin in the cerebellum of pups of ethanol-treated female rats.

Three calcium-binding proteins (CaBPs), calbindin D28k, calretinin and parvalbumin, were immunohistochemically examined in the cerebellum of ten-day-old rat pups of ethanol-treated dams. Dams were treated with ethanol during pregnancy and/or lactation. In the cerebellar cortex of the pups from control groups, Purkinje cells with their processes and Golgi cells were positive for calbindin D28k, whereas interneurons (Lugaro, Golgi and unipolar brush cells) and sometimes Purkinje cells were positive for calretinin.

Complications of severe cerebral amyloid angiopathy in the course of dementia with Lewy bodies. A case report.

A 68-year-old male who suffered from dementia, progressing for four months without Parkinson's symptoms, was admitted to the Department of Neurology because of vertigo, slight left hand paresis and positive Romberg test. During hospitalization the patient's status deteriorated. The intracerebral lobar haemorrhage, subarachnoid haemorrhage and ischaemic lesions observed on CT scans suggested the clinical diagnosis of CAA.

Cerebral amyloid angiopathy as a cause of an extensive brain hemorrhage in adult patient with Down's syndrome - a case report.

A case of 54-year old woman who deceased due to consequence of extensive brain hemorrhage is presented. The patient was admitted to our Department of Neurology due to progressive quadriparesis as a complication of the cervical spinal cord compressive myelopathy. On the third day after neurosurgical decompression of the spinal cord sudden worsening of neurological and general condition was observed, finally caused death.

Capillary vessel wall in CADASIL angiopathy.

The study was aimed at investigating the morphology of capillaries in four skin and muscle biopsy specimens obtained from CADASIL patients. In all cases diagnosis confirmed at the ultrastructural level, and additionally in three cases, the genetic test revealed the Notch3 gene mutations. Using histological and immunohistochemical (IHC) markers for components of capillary vessel wall we showed the reduction and loss of pericytes and and fibrous vessel wall including the thickened basement membrane.

[Aortic dissection and carotid arteries dissection complicated by ischaemic stroke in a patient with cystic medial necrosis].

We present a case of a 62-year-old man who was admitted in grave condition to the Institute of Psychiatry and Neurology because of ischaemic stroke. Neurological examination re- vealed left-sided pyramidal hemiparesis. Computed tomography (CT) showed the ischaemic focus in the right cerebral hemisphere.

Kufs' disease: diagnostic difficulties in the examination of extracerebral biopsies.

Kufs' disease or NCL4 (neuronal ceroid lipofuscinosis type 4) is a rare and poorly characterized, adult-onset form of NCL. The mutation in gene CLN, underlying Kufs' disease, still remains unknown. The diagnosis of this disease is difficult because it is based only on clinical and ultrastructural examinations.

Amyloid angiopathy in idiopathic Parkinson's disease. Immunohistochemical and ultrastructural study.

The prevalence of cerebral amyloid angiopathy (CAA) and its association with intellectual decline in idiopathic Parkinson's disease (iPD) remain unclear. To identify the role of CAA in iPD dementia the prevalence and severity of CAA were investigated, with particular respect to changes in vessel wall structure. Twenty-eight autopsy Parkinsonian brains and fourteen age-matched controls, post-mortem revised histopathologically for the presence of alpha-synuclein and Alzheimer's disease (AD)-type pathology, using standardized clinico-neuropathological criteria, underwent further investigation.

Giant cell ependymoma of the spinal cord and fourth ventricle coexisting with syringomyelia.

This report presents a case of widespread intramedullary giant cell ependymoma arising from the central canal of the C4 segment of the spinal cord in a 28-year-old man admitted to hospital with tetraplegia and signs of increased intracranial pressure, eight months after surgical spinal cervical decompression without tetraplegia improvement. Magnetic resonance imaging and autopsy revealed a tumour extending from segment C3/C4 of the spinal cord to the lower half of the fourth ventricle with coexisting syringomyelia. This slow-growing ependymoma of low-grade malignancy exhibited unusual morphology as well as degenerative and ischaemic changes.

Small cerebral vessel disease in familial amyloid and non-amyloid angiopathies: FAD-PS-1 (P117L) mutation and CADASIL. Immunohistochemical and ultrastructural studies.

Three patients (of two unrelated Polish families) with early-adult onset dementia were subjects of the study. Two cases, previously diagnosed as familial Alzheimer's disease (FAD) with cerebral amyloid angiopathy (CAA), were confirmed by genetic and neuropathological studies, and one case of CADASIL was ultrastructurally confirmed by the presence of vascular granular osmiophilic material. Now the brain autopsy material has been reinvestigated using immunohistochemical (IHC) markers for vascular smooth muscle cells, paying special attention to collagen markers for extracellular matrix components and ultrastructural microvascular changes.

The brain immune response in human prion diseases. Microglial activation and microglial disease. I. Sporadic Creutzfeldt-Jakob disease.

A study of microglial activation and its contribution to the CNS immune response was performed on the brain autopsy material of 40 patients with definite sporadic Creutzfeldt-Jakob disease (sCJD). Spatial patterns of microglial activation and prion protein disease-associated (PrPd) deposition were compared in cerebellar and cerebral cortices using immunohistochemical (IHC) activation markers. Morphological phenotype forms of microglial cells in activation stages were assessed immunohistochemically (IHC).

Limbic neuropathology in idiopathic Parkinson's disease with concomitant dementia.

To study pathological background of dementia in idiopathic Parkinson's disease (PD), 41 autopsy brains (31 cases with and 10 cases without dementia) were investigated. The severity of degenerative changes was evaluated in selected limbic regions (trans- and entorhinal cortex, hippocampus, and amygdala). The densities of Lewy bodies (LBs), Lewy neurites (LNs), neurofibrillary tangles (NFTs), and amyloid neuritic plaques (NPs) were determined on immunohistochemically stained sections using antibodies against alpha-synuclein, tau-protein, and amyloid-beta.

Ultrastructural evaluation of activated forms of microglia in human brain in selected neurological diseases (SSPE, Wilson's disease and Alzheimer's disease).

Activated forms of microglia were ultrastructurally evaluated in three neurological diseases of different aetiology (subacute sclerosing panencephalitis--SSPE, Wilson's disease and Alzheimer's disease). The occurrence of activated rod, ramified and amoeboid microglia was found in the investigated diseases. The widest ultrastructural variability of microglia was in SSPE, including the presence of mitotic chromosomes or centrioles in its cytoplasm, which indicates microglia proliferation.

CADASIL or CADVaSIL?

In the present study, morphological examination of patients from two unrelated Polish families with CADASIL was performed. Using light microscopy, there were evident changes characteristic to the disease. On electron microscopy, deposits of granular osmiophillic material (GOM) were found not only in cerebral arteries and veins but also in cerebral capillaries and vessels of the internal organs.

Haemangioblastoma of the posterior cranial fossa: clinico-neuropathological study.

Haemangioblastoma (HBs) may occur sporadically in the central nervous system, or in association with von Hippel-Lindau (VHL) disease. Haemangioblastoma of the central nervous system is often seen in the posterior cranial fossa. VHL is an autosomaly dominant disorder.

Degenerative axonal changes in the hippocampus and amygdala in Parkinson's disease.

The morphological background of cognitive and emotional impairments in Parkinson's disease (PD) has not yet been fully explained. We evaluated the expression of synaptic proteins: alpha- and beta-synuclein, synaptophysin and synaptobrevin and ultrastructural changes of perikaryons and axons in limbic structures at post-mortem from cases of PD to estimate degenerative axonal pathology in the hippocampus and amygdala [corrected]. Limbic structures (enthorinal cortex, hippocampus, and amygdala) are essential for the cognitive processes and emotional behaviour.

Neuropathological and anatomopathological analyses of acardiac and "normal" siblings in an acardiac-twin pregnancy.

Acardiac twinning is a very rare complication of multiple pregnancy. The authors present the neuropathological and anatomopathological description of the twins of the multiple pregnancy complicated by the acardiac foetus and terminated at 26 weeks of gestation. An anatomopathological examination of the "normal" twin showed hyaline membrane syndrome, cardiomegaly and hepatomegaly.

Morphological analysis of active microglia--rod and ramified microglia in human brains affected by some neurological diseases (SSPE, Alzheimer's disease and Wilson's disease).

The activation of microglial cells in pathological conditions is manifested primarily by their proliferation, as well as by the occurrence of a new morphological form--rod microglia. In the present study immunohistochemical identification of rod microglial phenotype against ramified microglia was performed on segments of 17 brains derived from 7 cases of encephalitis of viral aetiology (including 5 SSPE cases), 6 cases of Wilson's disease and 4 cases of Alzheimer's disease. Segments from frontal, temporal and occipital lobes, cerebellum and brainstem were subjected to histological, histochemical and immunohistochemical reactions.