Cases reported "Brain Diseases"

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1/110. Basal forebrain amnesia: does the nucleus accumbens contribute to human memory?

    OBJECTIVE: To analyse amnesia caused by basal forebrain lesions. methods: A single case study of a patient with amnesia after bleeding into the anterior portion of the left basal ganglia. Neuropsychological examination included tests of attention, executive function, working memory, recall, and recognition of verbal and non-verbal material, and recall from remote semantic and autobiographical memory. The patient's MRI and those of other published cases of basal forebrain amnesia were reviewed to specify which structures within the basal forebrain are crucial for amnesia. RESULTS: attention and executive function were largely intact. There was anterograde amnesia for verbal material which affected free recall and recognition. With both modes of testing the patient produced many false positive responses and intrusions when lists of unrelated words had been memorised. However, he confabulated neither on story recall nor in day to day memory, nor in recall from remote memory. The lesion affected mainly the nucleus accumbens, but encroached on the inferior limb of the capsula interna and the most ventral portion of the nucleus caudatus and globus pallidus, and there was evidence of some atrophy of the head of the caudate nucleus. The lesion spared the nucleus basalis Meynert, the diagnonal band, and the septum, which are the sites of cholinergic cell concentrations. CONCLUSIONS: It seems unlikely that false positive responses were caused by insufficient strategic control of memory retrieval. This speaks against a major role of the capsular lesion which might disconnect the prefrontal cortex from the thalamus. It is proposed that the lesion of the nucleus accumbens caused amnesia.
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keywords = nucleus
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2/110. Isolated superior oblique palsies with brainstem lesions.

    Isolated unilateral superior oblique palsies resulting from brainstem lesions occurred in three patients. MRI documented contralateral tegmental lesions of the trochlear nucleus and adjacent intraaxial trochlear nerve. Lacunar infarct was the cause in two patients and a small hemorrhage in a third.
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ranking = 0.11111111111111
keywords = nucleus
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3/110. Hypertrophic olivary degeneration following surgical excision of brainstem cavernous hemangioma: a case report.

    Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubo-olivary pathway. It is distinguished from other types of neuronal degeneration in that hypertrophy, rather than atrophy, takes place in the neurons in the inferior olivary nucleus. Prior to the invention of magnetic resonance imaging (MRI), HOD was difficult to be detected, and a firm diagnosis could only be made at autopsy. We present a case of bilateral HOD following surgical excision of a cavernous hemangioma in the brainstem. The literature and imaging findings of this uncommon condition are reviewed.
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ranking = 0.11111111111111
keywords = nucleus
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4/110. kleine-levin syndrome and psychosis: observation from an unusual case.

    OBJECTIVE: This study evaluated the possible pathologic relation between kleine-levin syndrome (KLS) and mood disorders. BACKGROUND: A 28-year-old man with a remote history of KLS had the sudden onset of a manic episode with psychotic features after the end of hypersomnolence. METHOD: The patient received an extensive laboratory examination, including single photon emission computed tomography and magnetic resonance imaging. RESULTS: Single photon emission computed tomography showed decreased tracer perfusion in the basal ganglion, hypothalamus, and right frontotemporal region. magnetic resonance imaging revealed a cystic lesion in the pineal region. CONCLUSIONS: Hypothalamic dysfunction has been described in KLS and mood disorders, but pineal gland dysfunction has been mentioned only rarely. The clinical and neuroimaging findings suggest the need for further study of KLS.
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ranking = 5.0060062295581
keywords = basal ganglion, ganglion
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5/110. Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report.

    46-year-old acromegalic women presenting high level of growth hormone (32 ng/ml) in the serum underwent surgery. The intrasellar tumor, 16 mm in diameter, has been removed. The biopsy material consisted of two types of closely adjacent and intermingled tissues, one of which was growth hormone positive acidophilic adenoma, the second component were haphazardly oriented ganglion cells of various size and shape, also multinuclear, with bundles of unmyelinated fibers. The cytoplasm and processes of ganglion cells were immunopositive for neurofilaments and for synaptophysin on cellular membranes and processes. There were none glial fibrillary acidic protein positive cellular elements. The authors discuss commonly used name of choristoma for this type of tumor and the problem of possible neurosecretory stimulation of pituitary adenoma by neuronal hamartoma.
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ranking = 2.8850343000766
keywords = ganglion
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6/110. The auditory pathology of anoxia.

    A case of anoxic encephalopathy is reported, with study on a whole-auditory-pathways basis, and the method of processing tissues is outlined. Pathologic changes are found throughout the central part of the auditory pathway. The importance of including the superior ventral cochlear nucleus (SVCN) with cochlear structures in correlating findings with audiometric data is supported. The previously advanced tonotopic frequency pattern, with zonal vulnerability, of the spheroid cells of SVCN is supported.
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ranking = 0.11111111111111
keywords = nucleus
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7/110. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.

    The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.
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ranking = 5.0060062295581
keywords = basal ganglion, ganglion
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8/110. Neuropathology of Raine syndrome.

    We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.
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ranking = 0.11111111111111
keywords = nucleus
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9/110. Geniculate hemianopias: incongruous visual defects from partial involvement of the lateral geniculate nucleus.

    Quantitative perimetric studies in 4 patients with involvement of a lateral geniculate nucleus revealed strikingly incongruous defects in the corresponding homonymous fields of vision. The patterns of these hemianopias are analysed and correlated anatomically with established retinotopic projections on the six cellular laminae of the geniculate nucleus. Incongruous wedge-shaped field defects appear to be pathognomonic of focal disease in the dorsal crest of the geniculate nucleus. Other patterns typify lesions of the medical or lateral horns of the nucleus. On theoretical grounds monocular hemianopic defects should result from unilaminar geniculate lesions, but this perimetric sign awaits confirmation. In each case of geniculate disease where the retinal nerve fibre layer has been examined specifically for efidence of retrograde homonymous atrophy, typical hemiretinal signs were found to be present.
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ranking = 0.88888888888889
keywords = nucleus
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10/110. Menkes' kinky hair disease: a light and electron microscopic study of the eye.

    light and electron microscopic studies of the ocular tissue of a case of Menkes' kinky hair disease are described. The copper deficiency responsible for this systemic and neurologic disease appears to cause a progressive degeneration of retinal ganglion cells, loss of nerve fibers, and optic atrophy. The pigment epithelium is also abnormal with only small and irregular melanin granules present among electron-dense inclusion bodies. Abnormal elastica is present in Bruch's membrane.
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ranking = 1.4425171500383
keywords = ganglion
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