1/66. adult-type metachromatic leukodystrophy with a compound heterozygote mutation showing character change and dementia.A 26-year-old Japanese woman slowly developed a change of character such as hypospontaneity and blunted affect, followed by obvious mental deterioration. She was diagnosed as having a disorganized type of schizophrenia at the first examination. brain magnetic resonance imaging demonstrated diffuse high intensity in the cerebral white matter, particularly in the frontal lobes. The single photon emission computed tomography images using 123I-IMP disclosed diffuse cerebral hypofusion, especially in the frontal lobes. Electroencephalogram showed a moderate amount of 5-6Hz theta waves on the background of alpha activity. Nerve conduction velocities in the extremities were delayed. The level of leucocyte arylsulphatase was low. In the arylsulphatase A gene analysis, a compound heterozygote having the 99Gly-->Asp and 409Thr-->Ile mutations was confirmed. The patient was diagnosed as having metachromatic leukodystrophy. She gradually showed obvious dementing symptoms such as memory disturbance and disorientation. The characteristics of the psychiatric symptoms in the leukodystrophy are discussed.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/66. Recurring short delirium with postpartum onset in two sisters.We report on a 30-year-old woman who twice developed a short postpartum psychosis with organic signs, but without obvious organic cause. Extensive investigations only yielded a state of moderate hypercoagulability. Her sister had developed similar signs and symptoms during her second puerperium and died 5 days after her delivery. We discuss the combination of various precipitating factors for postpartum psychosis, the possible impact of the findings on its cause and its classification. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/66. Late hemorrhage from persistent pseudoaneurysm in vertebral artery dissection presenting with ischemia: case report.BACKGROUND: vertebral artery dissection lesions tend to resolve spontaneously, but abnormal findings such as aneurysmal-dilatation occasionally persist. However, the clinical features and pathological findings in such cases have never been verified. CASE DESCRIPTION: A 62-year-old man presented with left cerebellar infarction. angiography showed the "pearl and string sign" in the left vertebral artery, and he was diagnosed as having left vertebral artery dissection. Repeated angiography showed persistent aneurysmal dilatation with irregular stenosis. Eleven years after the cerebellar infarction, the patient presented with a subarachnoid hemorrhage from an aneurysm of the left vertebral artery, and the lesion was explored via the left suboccipital approach. The vertebral artery was firm, making the placement of a clip impossible, so the lesion was treated by coating of the bleeding point. The patient died of pneumonia and hyperglycemia on postoperative day 15. Postmortem examination revealed an organized intramural hematoma, thickening of the intima, and fibrous degeneration of the media of the vertebral artery, a fusiform, distended thin arterial wall with intimal disruption at the aneurysmal dilatation, and arteriosclerosis of all cerebral arteries. CONCLUSION: This case indicates that persistent aneurysmal dilatation of a dissection is a pseudoaneurysm prone to rupture, and that healing of the affected vessels might be severely compromised in the presence of pathological conditions such as arteriosclerosis and disturbed intraluminal blood flow in the dissected lesions.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/66. Homonymous visual field defects in patients without corresponding structural lesions on neuroimaging.Homonymous visual field defects usually occur with structural processes affecting retrochiasmal visual pathways. The responsible lesion is usually evident on magnetic resonance imaging or on other neuroimaging studies. When results of neuroimaging are normal, functional illness is often suspected. The authors report four patients with homonymous visual field defects who presented with no evident corresponding lesion on magnetic resonance or computed tomography imaging. Etiologies for the visual field defects included the Heidenhain variant of Creutzfeldt-Jacob disease, degenerative dementia, subtle occipital ischemia demonstrated only on positron-emission tomography scanning, and nonketotic hyperglycemia. Clinicians should be aware of the alternative etiologies of organic homonymous visual field loss in patients with normal neuroimaging.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/66. Severe bradyarrhythmia in a patient with Alzheimer's disease and a patient with cerebral ischaemia, both induced by acute distension of the bladder.Two case reports are presented of bradycardia in two elderly patients, which was resistant to atropine but which resolved immediately the acute bladder distension was treated. Generally, a sympathetic cardiac response is expected, and this paradoxical response may be due not only to lesions of the reflex loop involving both the vagal and the sympathetic nerves, but also to endocrinological factors. As acute urinary distension is not uncommon on geriatric wards, bradyarrhythmia due to bladder distension should be considered in elderly patients.- - - - - - - - - - ranking = 24.830795748348keywords = nerve (Clic here for more details about this article) |
6/66. Reorganization of somatic sensory function in the human thalamus after stroke.A patient with thalamic stroke underwent microelectrode-guided stereotactic thalamic exploration during surgery for control of tremor. The results of somatic sensory mapping in this patient were compared with explorations carried out during stereotactic surgery for the control of essential tremor (70 patients). There was evidence both of somatotopic reorganization and of anatomic reorganization of the representation of deep structures in the principal somatic sensory nucleus of the thalamus and the nuclei located anterior to it. This case demonstrates that thalamic reorganization can occur after a thalamic stroke and may play a role in recovery from such a stroke.- - - - - - - - - - ranking = 7keywords = organ (Clic here for more details about this article) |
7/66. A light and electron microscopic study of oedematous human cerebral cortex in two patients with post-traumatic seizures.PRIMARY OBJECTIVE: brain cortical biopsies of two patients with clinical diagnosis of complicated brain trauma who had seizures, were studied by means of light and electron microscopes in order to correlate structural alterations with seizure activity. methods AND PROCEDURES: biopsy samples of left frontal cortex and right parietal cortex were processed by current techniques for light and transmission electron microscopy. RESULTS: The tissue showed severe vasogenic oedema with perivascular and intraparenchymatous haemorrhages. At the capillary wall, increased vesicular and vacuolar transendothelial transport, open endothelial junctions, thickened basement membrane and swollen perivascular astrocytic end-feet were observed. Some pyramidal and non-pyramidal nerve cells appeared dense and shrunken and others exhibited marked intraneuronal enlargement of membrane compartment. The myelinated axons displayed signs of degeneration and a process of axonal sprouting. Numerous swollen asymmetrical axo-dendritic synaptic contacts were observed in the neuropil, which exhibited mostly closely aggregated spheroidal synaptic vesicles toward the presynaptic membrane and numerous exocytotic vesicles sites. The perisynaptic astrocytic ensheathment appeared retracted or absent, whereas the extracellular space appeared notably dilated. Synaptic disassembly was also observed. CONCLUSION: The findings demonstrate, in two patients with post-traumatic seizure activity, brain barrier dysfunction, vasogenic oedema, anoxic-ischaemic neurons, axonal sprouting, numerous altered excitatory synapses and synaptic disassembly. Some considerations on clinical and research applications are discussed.- - - - - - - - - - ranking = 24.830795748348keywords = nerve (Clic here for more details about this article) |
8/66. The clinical and MRI correlate of ischaemia in the ventromedial midbrain: Claude's syndrome.The eponymous syndrome of Claude is caused by a lesion of the red nucleus and adjacent third nerve nucleus, resulting in the combination of an ipsilateral oculomotor palsy and contralateral ataxia. The MRI correlate of this syndrome has only occasionally been described. We present three cases with MRI findings which confirm the association of this clinical syndrome with infarction of the ventromedial midbrain. The coexistence of hypertension and small vessel ischaemia in two cases suggests this type of infarct may arise as a result of small vessel disease.- - - - - - - - - - ranking = 24.830795748348keywords = nerve (Clic here for more details about this article) |
9/66. Cerebral, myocardial and cutaneous ischemic necrosis associated with calcific emboli from aortic and mitral valve calcification in a patient with end-stage renal disease.We report the case of a 57-year-old diabetic male with chronic renal failure who developed secondary hyperparathyroidism and calcification of mitral and aortic valves and interatrial septum. Multiple ischemic lesions developed in the skin of hands, feet and penis, and in the brain, and these were presumed to be due to septic emboli from cardiac valvular infective endocarditis. Multiple blood cultures were negative, however, and despite antibiotic therapy the patient expired. autopsy (limited to trunk) demonstrated multiple calcific emboli in the heart and spleen, apparently derived from the prominent calcific deformities in the aortic and mitral valves. These were associated with acute and organizing myocardial infarcts and acute splenic infarcts, suggesting that the multiple ischemic lesions in the brain were also due to calcific emboli. A possible contributory component of infective endocarditis, however, was indicated by postmortem cultures of aortic and mitral valves positive for enterococcus faecium. Calcific embolism is a rarely recognized but potentially lethal complication of end-stage renal disease, and the clinical diagnosis and the preventive therapeutic options for the control of the product of calcium and phosphate and/or parathyroidectomy should be considered.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/66. Ischemic stroke in a user of thermadrene: a case study in alternative medicine.A case of a young user of Thermadrene (SportPharma USA, Inc, Concord, Calif), an herbal remedy for weight loss, was studied. We believe that Thermadrene can cause serious complications such as ischemic stroke. Because these remedies are available over the counter and are not regulated by the food and Drug Administration or similar organizations, there is a need for critical scrutiny of their content and side effects, as is performed for other pharmaceuticals.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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