Cases reported "Cerebrovascular Disorders"

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1/68. Vascular changes in tuberculous meningoencephalitis.

    Our report refers two cases of tuberculous encephalomeningitis which differ in the course and pathological changes. In case 1 blood vessels showed features of peri, endo-, or panvasculites. In some vessels endothelium proliferation leading to the stenosis or obliteration of the vascular lumen was observed. necrosis was an effect of vessels occlusion. In case 2 many fewer vessel were involved in onflammation process. Vascular changes were also less extensive and were observed more rarely. Tuberculous infection often caused less tissue lesions than vascular changes. Different pathological changes probably depend on the type and virulence of Myobacterium tuberculosis and on the host immune response to the infection.
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2/68. The experience of transition for a daughter caregiver of a stroke survivor.

    The purpose of this study was to describe the experience of transition for a daughter caregiver of a stroke survivor. A phenomenologic, longitudinal case study of a woman in her late twenties provided the means by which the lived experience of transition could be studied and understood. The eleven unstructured, audiotaped interviews took place approximately every two weeks over a six and a half month period. They began six weeks after her mother's stroke and lasted until three months after her mother returned home, when life became more organized and predictable for a time. The interviews were transcribed verbatim and analyzed according to a modified version of Colaizzi's descriptive phenomenologic methodology. Since there was a temporal consideration, a time-ordered matrix was used to identify themes over time. The themes that emerged over time included: (a) changing relationships, (b) becoming a caregiver then stepping back, (c) enduring emotional turbulence, (d) taking one day at a time and (e) struggling to hang onto hope. Integration of the themes provided an exhaustive description. The experience of transition was a process that involved disruptions in close relations and daily living. The connection to a network of relationships changed to disconnection, and the fabric of interrelated lives began to unravel. The many sensations experienced were different and conflicting, revealing a rapidly changing perception of the world. An unknown future threatened to become overwhelming. Focusing on the present helped to quell the turbulent sensations experienced. An unknown future temporarily provided the opportunity to hope that daily life could once again become familiar and comfortable. The extent and object of hope changed over time. A pattern of chaos exemplified the process of transition. The passage of time revealed that life as it was known before the transition had changed. A new way of being in the world was experienced.
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3/68. Aicardi-Goutieres syndrome: a genetic microangiopathy?

    Aicardi-Goutieres syndrome (AGS) (McKusick 225750) is an autosomal recessive disease with onset in the 1st year of life, resulting in progressive microcephaly, calcification of cerebral white matter, thalamus and basal ganglia, generalized cerebral demyelination and a chronic low-grade CSF lymphocytosis, without evidence of infection. We report the autopsy of a patient who died with this disorder at the age of 17 years. Findings were severe microencephaly, diffuse but inhomogeneous cerebral white matter loss with associated astrocytosis, calcific deposits in the white matter, thalami and basal ganglia. neocortex and cerebellar cortex were affected by wedge-shaped microinfarctions. Small vessels showed calcification in the media, adventitia and perivascular spaces. These findings are similar to some previous publications that in retrospect may have been AGS, but this is the first reported cerebral microangiopathy in which the diagnosis AGS was made during lifetime. This report provides evidence that microangiopathy plays a significant role in the pathogenesis of AGS.
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4/68. depression in late life, cognitive decline and white matter pathology in two clinico-pathologically investigated cases.

    case reports: We report two cases of late life depression who became progressively more resistant to treatment, developed cognitive impairment, and began to exhibit neurological abnormalities and evidence of vascular disease. A discussion of the clinical features of the cases is accompanied by reports of neuropathology and neuroimaging findings. Extensive white matter lesions were present on computed tomography in both patients, and basal ganglia infarcts were seen in one. Neuropathology revealed evidence of cerebral atrophy, demyelination and white matter lesions in addition to cerebrovascular and generalised vascular disease. Neither patient exhibited Alzheimer pathology outwith the norm for their age. We believe this to be the first report of neuropathological findings in depression with white matter changes. literature review: The pathological basis of white matter lesions and their relationship to depression, its age of onset and clinical features is addressed in relation to the cases described. Pathological investigation of white matter lesions has not previously been carried out in depression and hypotheses regarding their nature in this illness are based on extrapolation from research in a variety of other disorders. The association of depression with vascular risk factors is considered, as is the relationship between depression and cognitive deficits. There is a need for further investigation in this area.
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5/68. Presence of delayed myelination and macrocephaly in the sister of a patient with vacuolating leukoencephalopathy with subcortical cysts.

    Leukoencephalopathy with swelling and a discrepantly mild clinical course ("van der Knaap disease") is a recently identified syndrome. It is characterised by macrocephaly occurring during the first year of life, initially normal or nearly normal development, and slowly progressive ataxia and spasticity with initial preservation of intellectual functions. MRI shows diffuse abnormality in signal intensity, as well as swelling of the hemispheral white matter with subcortical cyst-like spaces in the fronto-parietal and anterior temporal areas. It is thought to have an autosomal recessive mode of inheritance, since many patients have consanguineous parents and more than one affected patient is often present within the same family. We report on two sibs: a 5-year old boy affected with "van der Knaap disease" and his macrocephalic sister whose first MRI (2 years 6 months) showed delayed myelination, which led us to suspect the same disease as her brother, however with subsequent normalisation at the second MRI (3 years 6 months).
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6/68. Intracerebral haematomas with agenesis of the internal carotid artery and tetralogy of fallot.

    We report a rare case with tetralogy of fallot (TOF) and agenesis of the internal carotid artery (ICA) who presented serious intracerebral haematomas. In the literature, this is the first documented case having these complications simultaneously. Extreme hypoxic insults followed by recovery were detected by O2 saturation monitor before two bleeds. Chronic brain hypoxia could make the vasculature weak, which was shown in the histological examination. A 2-year-old girl was transferred to us with a general convulsion due to intracerebral haematoma. She had been showing general cyanosis from birth due to TOF. Repeated intracerebral haemorrhages ended her life. Histological study showed dilated vascular channels in the subarachnoid space and necrotizing vasculature obstructed by fibrinous thrombi adjacent to the haematoma. fibrosis of the vessel wall with infiltration of macrophages suggested subacute or chronic lesions rather than acute necrosis due to the multiple haemorrhages. The intracerebral haematomas and agenesis of the ICA were observed as unilateral hemispheric vascular complications of TOF. Chronic brain hypoxia could play an important role in weakening the vessel wall and erythrocytosis caused obstructing thrombi. We speculate these factors generated the intracerebral haematomas.
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7/68. cerebrovascular disorders in children with the factor v Leiden mutation.

    Since 1995, at least 128 children with a cerebrovascular disorder, cerebral palsy, or both and the factor v Leiden mutation have been reported. The majority of these strokes were in the first year of life, many of them in the perinatal period. Two thirds had an additional exogenous risk factor for thrombosis, and 42% had another recognized endogenous prothrombotic risk factor in combination with the mutation. We review the association of the factor v Leiden mutation and a cerebrovascular disorder in children younger than 16 years of age and describe the clinical features of 8 children with cerebral palsy and the Leiden mutation. This mutation should be considered in the evaluation of children with a stroke or its sequelae, including infants with perinatal stroke.
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8/68. COX-2 inhibitor for the treatment of idiopathic stabbing headache secondary to cerebrovascular diseases.

    The idiopathic stabbing headache (ISH) is characterized by a stabbing pain of short duration, variable localization and an errant evolution pattern. As its biological mechanisms are unknown and the treatment options are little effective, this disorder shows a strong impact on the patient's life. Two females and one male, aged 76, 66 and 72 years, respectively, started presenting ISH within 20 days after the onset of a stroke. All the patients were treated for the ISH with celecoxib, a COX-2 specific inhibitor, with full recovery from ISH up to 6 days after it was first administered. The interruption of the drug 60 days after the treatment with celecoxib induced again the appearance of algic symptoms in two patients. We concluded that cerebrovascular diseases (CD) can lead to ISH and that the COX-2 inhibitor can be an effective prophylactic drug for ISH after CD.
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9/68. MR evaluation of crossed and uncrossed cerebral-cerebellar diaschisis.

    In three patients with infantile hemiplegia syndrome, MR imaging done later in life showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum in two and ipsilateral in one. By comparison, the cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemispheres in two patients. These observations provide morphological evidence of the phenomenon of crossed and uncrossed/ipsilateral cerebral cerebellar diaschisis (CCD and ICD). functional neuroimaging studies in support of the concept of CCD and ICD have been critically reviewed in the light of the morphological changes demonstrated in the cases cited herein.
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10/68. Sneddon's syndrome and renal carcinoma. Case report.

    A patient with Sneddon's syndrome in association with renal neoplasm is discussed. The association has not been reported before and raises questions concerning the pathogenesis of vascular proliferation in Sneddon's syndrome.
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