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. On admission she was unconscious with right hemiparesis and deviation of eyes to the left. On computed tomography (CT) scan many hemorrhagic infarcts were present in the frontal, parietal, temporal and left occipital lobes. Angio-CT revealed thrombosis in the right transverse sinus, right internal carotid vein and superior sagittal sinus. Her state slowly deteriorated. She died after 6 days. Neuropathologically, many hemorrhagic infarcts were observed in cortical regions in the vicinity of veins with thrombosis and in the white matter. The varied time of onset of thrombosis of the right sigmoid sinus, right superior petrosal sinus, superior sagittal sinus, right transverse sinus and the proximal part of the right internal carotid vein was confirmed. cerebral amyloid angiopathy in brain vessels was diagnosed. Subarachnoid hemorrhage is a very uncommon presentation of CVT and may coexist with CAA. We can only speculate that CAA may have an effect on vein destruction and can promote cerebral vein thrombosis and in consequence also predispose to intracerebral hemorrhage and subarachnoid hemorrhage. The most probable cause of extensive thrombosis was a coagulation disorder.

Neuropsychological characteristics of encephalopathy in Susac's Syndrome - Case report.

Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning. The initial assessment revealed attention, memory and executive dysfunctions, as well as behavioral changes including impulsivity, affective dysregulation and reduced self-awareness of disease deficits. After five weeks recovery process supported by rehabilitation program, improvement was observed, although some cognitive-behavioral deficits were still present in the follow-up assessment.

Temporal trends in vascular risk factors and etiology of urban Polish stroke patients from 1995 to 2013.

BACKGROUND: Despite estimates about general trends in stroke epidemiology worldwide, there are only a few reports of detailed longitudinal data and none of them reflects the economic transition that occurred in Central and Eastern Europe over the last two decades. The aim of this study was to investigate long term trends in risk factors and their pre-stroke control as well as acute stroke clinical presentation and etiology in Polish urban setting. METHODS: This is a retrospective registry-based analysis of consecutive acute stroke patients from a highly urbanized area (Warsaw, Poland) admitted to a single stroke center between 1995 and 2013. Patients were divided into four time periods: 1995-1999 (n=529), 2000-2004 (n=1253), 2005-2009 (n=1320) and 2010-2013 (n=871). RESULTS: During the study period 3973 first-ever stroke patients were admitted. The proportion of ischaemic strokes (88.2% to 90.9%) and male patients (45.2% to 46.2%) remained stable throughout the whole study period. Admitted patients became older (72, 73, 74 and 76years, consecutive time periods), were more likely to be diagnosed with hypertension (from 61.1% to 72.8%) and disable (84.3% to 67.4%) prior to stroke. There was an increase in pre-stroke use of antihypertensives in patients with hypertension (from 77.8% to 90.5%), antiplatelets in patients with coronary artery disease (from 33.9% to 56.5%), vitamin K antagonists in patients with atrial fibrillation (from 6.3% to 39.8%) and statins (from 7.6% to 26.3%). There was a decrease in mean stroke severity (9, 11, 8 and 6 points on the National Institutes of Stroke Scale) on admission and the proportion of strokes attributed to small-vessel disease (22.0%, 20.0%, 10.6% and 8.3%). CONCLUSIONS: Over the last two decades the profile of urban Polish stroke patients has changed significantly and it can be attributed to marked economic improvement in Poland since 1990s. Increasing age and better management of pre-existing vascular risk factors were accompanied by decreasing stroke severity and lower proportion of strokes attributed to small-vessel disease.

Impact of BDNF -196 G>A and BDNF -270 C>T polymorphisms on stroke rehabilitation outcome: sex and age differences.

BACKGROUND: Genetic factors, including gene polymorphisms, are promising in determining stroke rehabilitation outcome. Brain-derived neurotrophic factor (BDNF) is one of the most attractive because of its role in neuroplasticity and brain repair. OBJECTIVE: The aim of present study was to assess the role of BDNF -196 G≯A (val66met) and -270 C≯T on clinical parameters and functional outcome in patients with ischemic and hemorrhagic stroke. Additional analyses according to sex and age (≤55 and ≯55 years) were performed. METHODS: Three hundred thirty-eight patients (287 with ischemic and 51 with hemorrhagic stroke) were evaluated in terms of neurological deficit (National Institute of Heath Stroke Scale [NIHSS]), activities of daily living (Barthel Index [BI]), and everyday functionality (Rankin score [RS]) before and after rehabilitation. BDNF polymorphism genotyping was performed by polymerase chain reaction restriction fragment length polymorphism analysis. RESULTS: In multivariative analysis, unfavorable outcome of stroke rehabilitation (RS ≥2) was associated with independent factors: ischemic stroke (odds ratio [OR], 2.59; 95% CI, 1.03-6.47), female gender (OR, 2.80; 95% CI, 1.39-5.64), depression (OR, 4.24; 95% CI, 1.45-12.35), falls (OR, 2.61; 95% CI, 1.16-5.87), and BDNF -196 GG polymorphism (OR, 2.18; 95% CI, 1.09-4.35). The differences of functional parameters measured with BI and RS on admission and at discharge are apparent only for comparisons between patients ≤55 and ≯55 years old carrying BDNF -196 GA+AA genotypes but not in those carrying -196 GG genotype; the differences were evident in women but not in men. CONCLUSIONS: BDNF -196 G≯A polymorphism might affect functional outcome of stroke rehabilitation, but this hypothesis needs further verification.

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. The Vonsattel and Mountoy scales were used to assess the grade and score of CAA. Atherosclerosis was assessed according to a four-grade scale presented in the Coding Guide from Collaborative Study of Epidemiological Factors in Cerebral Vascular Disease. RESULTS: Of the 189 patients who died due to intracerebral hemorrhages 42 (22%) presented CAA. According to the Vonsattel scale this group comprised 32 (76%) patients who showed severe, 6 (14%) moderate and 4 (10%) mild CAA. Atherosclerosis was diagnosed in the CAA group of patients as follows: 6 (14%) with grade 1; 20 (49%) with grade 2; 9 (20%) with grade 3; and 7 (17%) patients with grade 4. CONCLUSIONS: There was no correlation between CAA and AS. The CAA was probably the direct cause of death in part of cases with advanced CAA. The different mechanisms presumably can cause CAA and AS.

ß-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. The Vonsattel and Mountjoy scales were used to assess the grade and scores of CAA. RESULTS: In the study group composed of 189 ICH patients, 42 presented CAA. In the microscopic examination, of the 42 patients 33 (78%) showed ß-amyloid deposits in veins, which makes 17% of the total group of patients with ICH. In this group, the age ranged from 54 to 97 (mean age 80.18 ± 8.15 years). A group of 33 (27 women and 6 men) patients comprised 15 (45%) patients with severe CAA, 13 (40%) with moderate and 5 (15%) with mild CAA classified according to the Vonsattel scale. According to the Mountjoy scale 28 (85%) patients had a score of 4, which indicated the total involvement of the vessel. CONCLUSIONS: ß-amyloid deposits in veins were found in 78% of patients with CAA and ICH, which makes 17% of the total group of patients with ICH. Interestingly, ß-amyloid deposits in veins are not so rare in patients with CAA who died due to intracerebral haemorrhage. Cerebral amyloid angiopathy localization in the veins of the brain was observed more frequently than previously suspected. Veins may play a role in the elimination of ß-amyloid from the brain.

Cerebral amyloid angiopathy manifested as a brain tumour. Clinical and neuropathological characteristics of two cases.

We present two cases (female and male patients, aged 64 and 38, respectively) of focal mass lesions mimicking a brain tumour: one with cognitive function deficit, memory troubles, behavioral changes and left hemiparesis, the other with difficulty in orientation and right hemiparesis. General physical and neurological examinations, laboratory tests and neuroimaging were used to diagnose the cases. Both of them showed nonspecific changes in the brain tissue and the brain tumour was suspected. In the first case MRI scan revealed two pathological masses in the right frontal region and hemorrhagical focus with destructions inside lesions. Second patient's MRI scan revealed a pathological mass at the interface of the left temporal and occipital regions. The neurosurgical procedure was performed. The final diagnosis was established on the basis of neuropathological examination of postoperative material. On light microscopy examination a severe cerebral amyloid angiopathy (CAA) was revealed. Amyloidoma was excluded due to the absence of amorphous material and eosynophylic masses. Tumefactive CAA is a rare condition. These two cases of focal, tumefactive, masslike lesions of diffuse cerebral amyloid angiopathy are reported because of diagnostic dilemmas. In patients with history of memory disfunction, neurological deterioration and different multiple changes observed in CT and MRI scans, such as hemorrhagic infarcts and ischemic cerebral lesions, CAA should be suspected. The imaging findings make a distinction between tumefactive CAA and brain tumours like gliomas difficult. A differential diagnosis of CAA and amyloidoma plays a significant role in a neuropathological examination.

Inflammatory cerebral amyloid angiopathy: the overlap of perivascular (PAN-like) with vasculitic (Aß-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. She was admitted with recurrence of symptoms of transient aphasia and central, right-side facial paresis that occurred an hour before her arrival at the hospital. In the admission unit, she was only slightly confused, with no other neurological deficits. An urgent CT scan revealed a recent haemorrhagic stroke in the left frontal lobe. In an hour her condition suddenly deteriorated. After a generalized seizure she presented with right-side hemiparesis with signs of uncal herniation and remained unconscious. A control CT scan showed a large haemorrhagic expansion that comprised the whole left brain hemisphere with 2 cm midline shift. She died about 10 hours after the onset of symptoms. At autopsy chronic inflammation of the thyroid gland, bronchopneumonia, fibrous and fatty heart degeneration and kidney haemorrhagic infarcts were documented. Amyloid deposition and systemic immune disorders in the inner organs were not demonstrated. In neuropathological examination we diagnosed inflammatory form of CAA with coexistence (the overlap) of two, perivascular and vascular, subtypes of CAA-related inflammation.

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. An autopsy study revealed an extensive brain lobar hemorrhage and a dorsal-ventral compression of the cervical spinal cord. Alzheimer's disease-type degenerative changes with concomitant CAA were seen in light microscope examination. Extensive foci of demyelination were found especially in dorsal funiculi of the cervical spinal cord. Smaller foci of demyelination were present in anterior funiculi due to the stenosis of vertebral canal.

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. He died after 53 days due to pneumonia. On macroscopic examination, the brain showed general cortical atrophy and ventricular dilatation. Frontal lobar haemorrhage and focal subarachnoid haemorrhage were seen on the brain autopsy. Microscopic observation demonstrated neuronal loss and microspongiosis in the hippocampus, severe neuronal loss and depigmentation in the substantia nigra pars compacta and locus coeruleus. Lewy bodies were visible in the substantia nigra and amyloid angiopathy, predominantly severe CAA according to the Vonsattel scale, in the meningeal and cortical vessels. In the presented case, the microscopic findings were typical for DLB with concomitant severe CAA. In progressive dementia, neurological deterioration, presence of lobar hemorrhagic infarcts and ischaemic lesions suggest CAA coexistent with DLB and/or AD.

[Apolipoprotein E (APOE) gene polymorphism and risk and prognosis in cerebral amyloid angiopathy-related haemorrhage]. / Polimorfizm genu apolipoproteiny E (APOE) a ryzyko i rokowanie w krwotokach mózgowych spowodowanych przez mózgowa angiopatie amyloidowa.

The authors present current opinions about the role of APOE (apolipoprotein E gene) genotype as a factor modifying risk, course and prognosis in haemorrhagic stroke of cerebral amyloid origin. The search for the role of genetics in haemorrhagic stroke has been ongoing for more than 15 years. One of the most frequently investigated genotypes in the context of intracerebral haemorrhages is the APOE genotype. Alleles APOE e2 and e4 have been established as risk factors for cerebral amyloid angiopathy (CAA), as well as for cerebral amyloid angiopathy-related haemorrhage (CAAH). Moreover, APOE genotype seems to determine prognosis in CAAH in terms of early mortality, as well as risk of recurrence. Current findings related to the association between different isoforms of apoE and haemorrhagic stroke due to CAA do not allow us to formulate any clinical recommendations yet.

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. In both diseases, IHC examination showed a reduction or loss of expression of smooth muscle actin (SMA) in tunica media of the cerebral arterioles. Fibrous thickening of the wall of the small meningeal arteries, intracerebral arterioles and numerous capillaries, with amyloid or granular deposits, drew our attention. In these vessels, marked expression of fibrillar collagen type III as well as strong immunoreactivity of the basement membrane (BM) component collagen type IV were found. The most damage was observed in the FAD/CAA double-barrel vessel wall and in some CADASIL arterioles changed by fibrinoid necrosis. The fibrous changes of the small vessels were more distinct in CADASIL t han in FAD/CAA. In FAD,electronmicroscopic examination revealed both amyloid and collagen fibres within the thickened BM of capillaries and the small arterioles. Clusters of collagen fibres between lamellae of BM, frequently in a pericyte position,were observed,and some were seen in the degenerated pericytes as well. Typical changes of the pericytes were accumulation of lipofuscin-like material and their degeneration. The mitochondria of the pericytes and of the endothelium were rare and swollen, with damaged and reduced cristae. The VSMCs of the arteriolar walls exhibited degenerative changes with atrophy of the cellular organelles. The fibrous,collagen-richCADASILsmallcerebralvessels,despite the weakness of the vessel wall due to reduction of VSMCs, appeared to be stronger than in FAD/CAA. These findings may suggest an accelerated process of transformation of the small cerebral vessels in which early onset of VSMCs loss is a predominant feature of the vascular changes in both presented diseases.

["Malignant" middle cerebral artery territory infarction]. / Zlosliwy zespól tetnicy srodkowej mózgu.

The pathology, clinical course, outcome, diagnosis, treatment and prognosis of dramatic malignant middle cerebral artery territory infarction were presented. About 10% of stroke patients suffer from malignant middle cerebral artery territory infarction, mainly due to brain edema and herniation. This syndrome causes high mortality. The newest conservative and surgical treatment was presented.

[Changes in lipoprotein (a) [Lp(a)] level after an ischemic stroke]. / Zmiany poziomu lipoproteiny (a) [LP(a)] po udarze niedokrwiennym mózgu.

The aim of the work was to recognize whether often observed high levels of apolipoprotein (a) [Lp(a)] in patients shortly after an ischemic stroke are a result of the acute phase reaction. In 13 patients Lp(a) was determined within the first 24 hours after the stroke onset, after the next 7 days and after three months i.e. when it could be considered that Lp(a) level was the same as before onset of the disease. In 17 patients only two determinations were performed. Another acute phase indicator: C-reactive protein (CRP), as well as serum lipids were also determined. CRP level was increased in the first determination and increased further after 7 days. After three months it returned to low values. High density lipoprotein (HDL) cholesterol which demonstrates a negative acute phase response changed in the opposite way. No similar fluctuations of Lp(a) level were observed. It can be concluded that during the investigated period Lp(a) had no properties of the acute phase reactant.

[Prognostic factors in the acute phase of haemorrhagic stroke]. / Czynniki prognostyczne w ostrym okresie krwotoku mózgowego.

The aim of the study was establishing which parameters play a significant prognostic role in acute haemorrhagic stroke in its acute phases (during 30 days from stroke onset). The material included 110 patients with haemorrhagic stroke (HS) treated in the years 1997-1999 at the II Neurology Department, Institute of Psychiatry and Neurology in Warsaw or at the Neurology Department, Hospital in Pila. The analysis of risk factors, fitness before stroke and on the first day of the disease was based on history data. On the first day of the disease the assessment included state of consciousness, brain stem signs, vomiting, stroke type on the basis of clinical condition (according to Oxford classification, OCSP), blood pressure, body temperature measurements, intensity of neurological signs according to Scandinavian scale (SSS). In the blood glucose level, fibrinogen, leucocyte count and ESR were determined. In the first week consciousness state, blood pressure and miction were checked repeatedly. It was found that serious consciousness disturbances, stroke type TACS, POCS and severe not classified stroke, presence of brain stem involvement, low SSS score, high body temperature, high leucocyte count and glucose level were more frequent in patients who died within 30 days after stroke onset. Prognostically important were also instability of blood pressure and urinary incontinence in the first week. All clinical observation and laboratory analyses of prognostic importance in the acute phase of HS can be done in typical hospital settings.

High early case fatality after ischaemic stroke in Poland: exploration of possible explanations in the International Stroke Trial.

PURPOSE: To determine why Polish patients included in the International Stroke Trial (IST) had a higher early case fatality than patients from other countries. METHODS: Of 19,435 patients randomised to IST, 17,370 had CT or autopsy-confirmed ischaemic stroke. We compared the baseline clinical state and 14-day case fatality of patients randomised in Polish centres with those from all other IST countries. We examined: age, gender, presence of atrial fibrillation (AF), conscious level, neurological deficit, cause of death and aspirin use in the 3 days before stroke. RESULTS: In Poland, the 14-day case fatality rate was significantly higher than in other IST countries (13% vs. 9.6%; odds ratio, OR, 1.5, 95% confidence interval, CI, 1.2-1.9). In Poland, a significantly higher proportion of deaths was attributed to coronary heart disease (2.5% vs. 0.7%) and pulmonary embolism (1.0% vs. 0.4%). Polish patients had higher percentage of AF and drowsiness, but were younger. When these factors were adjusted for, Polish patients still had approximately a 57% higher risk of death within 14 days compared with other countries. A logistic regression analysis revealed that an increased risk of death within 14 days in Poland compared to other IST patients was present among patients younger than 75 years (OR 1.7; 95% CI 1.3-2.4), females (OR 1.8; 95% CI 1.3-2.5), patients who were alert at onset (OR 2.4; 95% CI 1.7-3.4), with partial anterior circulation syndrome (PACS) (OR 2.0; 95% CI 1.4-2.8) or lacunar syndrome (LACS) (OR 2.4; 95% CI 1.1-5.0) and without AF (OR 1.8; 95% CI 1.3-2.4). Polish patients were less likely to have been on aspirin before their stroke than other countries (7.6% vs. 20.8%). CONCLUSION: The 14-day case fatality was significantly higher in Poland than in other countries in IST. Part of the increase was due to greater stroke severity in the Polish patients. However, differences persisted even after adjustment for baseline stroke severity. Several factors may have contributed the excess: poor attention to treatment of cardiovascular disease, less attention to treatment of secondary complications of stroke and some other--as yet unidentified--factor specific to Poland.

Advanced atherosclerosis of the aortic arch is uncommon in ischemic stroke: an autopsy study.

Aortic and carotid atherosclerosis are known risk factors for stroke. The aim of the study was to determine the frequency of atherosclerotic lesions in the aorta and carotid arteries in subjects dying of ischemic and hemorrhagic stroke and to determine whether aortic atherosclerosis was associated with any specific ischemic stroke subtype. Autopsies were performed in 207 patients who died during hospitalization for stroke from 1993 to 1997. Subjects ranged in age from 37 to 98 years, mean 74.45 years (SD +/- 11.84). There were 132 women and 75 men. Stroke was hemorrhagic in 66 and ischemic in 141. Advanced atherosclerotic lesions were less frequent in the aortic arch (1.9%) than in the thoracic aorta (51.7%), abdominal aorta (60.4%), or carotid arteries (23.7%). Moderate atherosclerotic lesions in the aortic arch were observed more frequently in ischemic (75.2%) than hemorrhagic stroke (56.1%, p=0.026). Advanced or moderate atherosclerotic lesions in any part of the aorta did not predict ischemic stroke subtype. Advanced atherosclerosis of the carotids was more common in ischemic stroke (28.4%) than hemorrhagic stroke (13.6%, p < 0.05). Advanced atherosclerosis of the carotids was more common in stroke due to atherothrombosis (51.4%) than in stroke due to cardiac embolism (22.1%) or stroke of unknown etiology (5.6%). The low frequency of advanced atherosclerotic lesions of the aortic arch suggests that this disease process is not a common mechanism of stroke.

[Lateral medullary infraction due to left atrial myxoma--a case report]. / Sluzak lewego przedsionka przyczyna zespolu bocznego opuszki--opis przypadku.

A case of 53 year old women with Wallenberg syndrome due to atrial myxoma is reported. She was operated. Atrial myxoma on 7 x 8 cm was removed. After few months she can walk independently, she needs only a little help in everyday activities.