Cases reported "Dementia, Vascular"

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21/128. Reversible posterior leukoencephalopathy occurring during resection of a posterior fossa tumor: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Our goal was to present a clinically and radiographically documented case of reversible posterior leukoencephalopathy (RPL) that occurred during resection of a posterior fossa tumor. Although RPL has been previously described in multiple nonsurgical settings, we hope that this case description makes RPL more clinically and radiographically recognizable to neurosurgeons. CLINICAL PRESENTATION: RPL is the clinical syndrome of headaches, altered mental status, seizures, and visual loss, with radiographic findings of reversible parieto-occipital changes on cerebral computed tomographic and magnetic resonance imaging scans. It has been previously reported in the settings of malignant hypertension, renal disease, eclampsia, and immunosuppression. To our knowledge, the patient presented represents the first clinically and radiographically documented case of RPL occurring during resection of a posterior fossa tumor. The patient intraoperatively exhibited wide fluctuations in blood pressure and awoke with clinical and radiographic findings consistent with RPL. INTERVENTION: Aggressive intraoperative and postoperative management of the patient's blood pressure, supportive intensive care, rehabilitation, and close radiographic follow-up were performed. CONCLUSION: RPL can occur as a result of intraoperative variations in blood pressure, even among young, previously healthy individuals. With the aforementioned interventions, the patient experienced significant clinical and radiographic recovery. ( info)

22/128. The delusions of Capgras and intermetamorphosis in a patient with right-hemisphere white-matter pathology.

    Previous neuropsychological studies have demonstrated an association between person misidentification and right-hemisphere dysfunction. In the study reported here, we explore the contribution of facial and visual recognition impairments in a patient with right-hemisphere subcortical white-matter pathology in the frontal and parietal lobes and a diagnosis of vascular cognitive impairment. The patient displayed false recognition of unfamiliar faces and deficient retrieval of key biographic detail for famous faces. These results are discussed in the context of the contribution of deficiencies in the visual system and subcortical white-matter lesions to the development of Capgras delusion. ( info)

23/128. Primary antiphospholipid syndrome presenting as pseudobulbar palsy in a young male.

    Antiphospholipid antibody syndrome (APS) is now recognized as an important risk factor for young stroke. Recurrent stroke seems to be common in patients with APS and a first stroke, recurrent stroke and vascular dementia are feared consequences of APS. We are reporting a case of primary APS with recurrent stroke presenting as pseudobulbar palsy in an young man. ( info)

24/128. Creutzfeldt-Jakob disease: the need to widen the differential diagnosis.

    We report the case of a 76 year old woman who presented with a clinical picture initially typical of a vascular dementia. The diagnosis was revised to Creutzfeldt-Jacob disease in the terminal stages. While Creutzfeldt-Jacob disease is rare, one must consider it in the differential diagnosis as it can mimic other dementias particularly in the chronic form which we describe here. ( info)

25/128. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy.

    The clinicopathologic features of two Japanese sisters with cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) are described. Neither patient had a history of hypertension, and both experienced cerebrovascular events before reaching their forties. Severe degenerative changes in the lumbar spine and knee joints were seen on radiographs. MRI showed extensive cerebral white matter lesions, which revealed remarkable arteriosclerotic changes on autopsy. ( info)

26/128. Succinate in dystrophic white matter: a proton magnetic resonance spectroscopy finding characteristic for complex II deficiency.

    A deficiency of succinate dehydrogenase is a rare cause of mitochondrial encephalomyopathy. Three patients, 2 sisters and 1 boy from an unrelated family, presented with symptoms and magnetic resonance imaging signs of leukoencephalopathy. Localized proton magnetic resonance spectroscopy indicated a prominent singlet at 2.40ppm in cerebral and cerebellar white matter not present in gray matter or basal ganglia. The signal was also elevated in cerebrospinal fluid and could be identified as originating from the two equivalent methylene groups of succinate. Subsequently, an isolated deficiency of complex II (succinate:ubiquinone oxidoreductase) was demonstrated in 2 patients in muscle and fibroblasts. One of the sisters died at the age of 18 months. Postmortem examination showed the neuropathological characteristics of Leigh syndrome. Her younger sister, now 12 months old, is also severely affected; the boy, now 6 years old, follows a milder, fluctuating clinical course. magnetic resonance spectroscopy provides a characteristic pattern in succinate dehydrogenase deficiency. ( info)

27/128. methotrexate-induced toxic leukoencephalopathy.

    Drug-induced leukoencephalopathy is a devastating adverse event that can cause significant morbidity and mortality. risk factors include advanced age, administration of certain chemotherapies, presence of an Ommaya device, central nervous system malignancy, and most important, exposure to cranial radiation. A 73-year-old woman developed leukoencephalopathy 2 months after her last dose of intraventricular methotrexate. Although leukoencephalopathy is a severe adverse effect of the agent, her case is different because it occurred without prior exposure to cranial radiation. ( info)

28/128. L-asparaginase-induced reversible posterior leukoencephalopathy syndrome in a child with acute lymphoblastic leukemia.

    Reversible posterior leukoencephalopathy syndrome (RPLS) is being increasingly described with various etiologies even in the absence of hypertension. We present an 11-year-old patient with acute lymphoblastic leukemia who presented with seizures while on treatment with L-asparaginase. MRI showed bilaterally symmetrical nonenhancing occipital lesions characteristic of RPLS. L-asparaginase-induced RPLS is a rare cause of neurological symptoms in patients on induction chemotherapy. ( info)

29/128. Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease.

    patients with severe acute chest syndrome (ACS) requiring endotracheal intubation and erythrocytopheresis are at increased risk for neurologic morbidity. This study examines patients with sickle cell disease who developed severe episodes of ACS, leading to endotracheal intubation, ventilatory support for respiratory failure, and erythrocytapheresis. magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) studies, a neurologic examination by a pediatric neurologist, and cognitive testing were done in all patients. Five consecutive patients, aged 3 to 9 years, were identified with severe ACS. All patients developed neurologic complications resulting from ACS episodes, including seizures (n = 2), silent cerebral infarcts (n = 3), cerebral hemorrhage (n = 2), and reversible posterior leukoencephalopathy syndrome (n = 3). Children with severe ACS should have a magnetic resonance image of the brain, neurologic examination by a neurologist, and cognitive testing to detect the presence of neurologic morbidity. ( info)

30/128. Strategic infarcts of the thalamus in vascular dementia.

    Strategic infarcts or focal hemorrhages involving the paramedian nuclei of the thalamus may alter consciousness and produce complex neuropsychological symptoms such as impairment of memory, attention and motivation. Lesions disrupting the thalamo-prefrontal circuits lead to severe subcortical dementia. We analysed here the clinical, neuropsychological and neuroimaging data of 19 patients with cerebrovascular lesions in the thalamus. In six patients with bilateral paramedian infarcts, and in two with anterior thalamic infarcts, vascular dementia and severe personality changes developed. SPECT findings did not correlate with the neuropsychological symptoms. Transcortical sensory and motor aphasia was observed in four patients with thalamic hemorrhages and infarcts. In these patients SPECT detected hypoperfusion in adjacent cortical areas. Clinical symptoms and outcome of four patients are reported in detail. The clinico-morphological correlations of the thalamo-cortical circuits are reviewed and the possible causes of multiple cognitive and behavioural consequences of vascular thalamic lesions are discussed. ( info)
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