1/54. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.A case of 'central deafness' is presented in a 3-year-old male Caucasian child with moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/54. 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 = white (Clic here for more details about this article) |
3/54. Cerebrovascular pathology in Alzheimer's disease and leukoaraiosis.A high percentage of patients with Alzheimer's disease (AD) show evidence of white matter degeneration known as leukoaraiosis (LA), which is due to chronic ischemia. We found that the periventricular veins tend to become occluded by multiple layers of collagen in the vessel walls in the elderly. This collagen deposition is particularly excessive in LA lesions. Therefore, it is present in the brains of many AD patients, along with other ischemia-causing cerebrovascular pathology. We found evidence that there is severe loss of oligodendrocytes in LA, due to extensive apoptosis. No evidence of inflammation was found in the LA lesions. In thick celloidin sections of AD brain, we have obtained detailed 3D views of small (early) deposits of amyloid (stained with beta-amyloid antibody) around capillaries (stained with collagen IV antibody).- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/54. Exceptionally long absence status: multifactorial etiology, drug interactions and complications.To our knowledge, petit mal status lasting longer than 2-3 days has been documented only once [1]. We report a 66 year-old man with well-documented, idiopathic generalized epilepsy who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status. Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/54. Unusual pattern of leukoencephalopathy after morphine sulphate intoxication.We report a 14-year-old girl with an unusual pattern of leukoencephalopathy after intentional intoxication with morphine sulphate tablets. Toxicological analysis showed exceedingly high levels of morphine and its metabolites. MRI disclosed a leukoencephalopathy with high signal from the centrum semiovale, corpus callosum and cerebellar white matter on T2-weighted images. These findings could be only partially explained by a hypoxic-ischaemic event; neurotoxic effects must be considered in this atypical leukoencephalopathy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/54. Hyperacute infarction: early CT findings.The aim of our study is to determine how early and reliably ischemic brain infarcts can be detected on CT scanning. We report two cases of an early CT finding of acute ischemic infarcts where CT examination was obtained within the first 3 hours of onset of neurological symptoms. CT examination showed blurred outlines and decreased attenuation of the left lentiform nucleus, loss of the white-gray matter interface in the left insular cortex and left cortical global hypoattenuation with obliteration of left cortical sulci in one patient. In second patient CT showed the following: hyperdense left middle cerebral artery, loss of clear margins of left lentiform nucleus, subtle focal cortical hypodensity in the left fronto-parietal area with signs of mass effect in the form of ventricular compression. CT is still the first choice in the deferential diagnosis of acute stroke. Although older literature positions have suggested that CT was negative during the first 48 hours, modern CT technology can demonstrate positive findings even in the first 3 hours of onset. This is important in patients with acute stroke, as thrombolytic therapy when elected, has to be given within the first 3-6 hours after onset of symptoms. To determine how early and reliably ischemic brain infarcts can be detected on CT scanning, we report two cases of an early CT finding of acute ischemic infarcts where CT examination was obtained within the first 3 hours of onset of neurological symptoms.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/54. Diffuse white matter changes caused by dural arteriovenous fistula.We present two patients with progressive dementia who showed diffuse white matter changes on magnetic resonance imaging (MRI) associated with dural arteriovenous fistula (DAVF) involving the transverse-sigmoid sinuses. Angiography of both patients revealed that DAVF was associated with multiple occlusive changes in the dural venous sinus. The associated occlusive changes isolated the straight sinus and the DAVF from the other venous sinuses, and concentrated the drainage of the DAVF in the straight sinus. We postulate that the venous hypertension of the straight sinus resulted in the venous ischemia of the white matter, diffuse white matter changes on MRI, and progressive neurological signs including dementia. Treatment of the DAVF reversed white matter changes and neurological signs. Associated with the venous sinus occlusions, the DAVF caused dementia with diffuse white matter changes due to the venous ischemia.- - - - - - - - - - ranking = 9keywords = white (Clic here for more details about this article) |
8/54. Congenital middle fossa arachnoid cysts may cause global brain ischaemia: a study with 99Tc-hexamethylpropyleneamineoxime single photon emission computerised tomography scans.Three children with middle fossa arachnoid cysts, presenting with non-specific symptoms and otherwise well, were investigated before and after surgery with magnetic resonance and 99Tc-hexamethylpropyleneamineoxime single photon emission computerised tomography scans, to assess the effect of the cysts on cerebral blood flow. All patients had evidence of a reduction in cerebral blood flow at presentation, even in the hemisphere contralateral to the middle fossa cyst, implied by perfusion defects seen centrally in the deep white matter of both cerebral hemispheres. After successful surgical excision of the cyst, the perfusion defects disappeared. This was associated with general improvement of pre-existing non-specific symptoms. These findings indicate that middle fossa arachnoid cysts may cause global impairment of brain function by interfering with its blood supply. This does not support the generally held view that such cysts are benign in nature when 'asymptomatic'.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/54. A case of gliosarcoma appearing as ischaemic stroke.OBJECTIVES: Ischaemic stroke attributable to malignant brain tumour is a rarely reported phenomenon and even various imaging techniques including angiography do not necessarily lead to an accurate diagnosis. CASE DESCRIPTION: A 46-year-old, previously healthy man developed apoplectic symptoms with slight right sided hemiparesis and global aphasia. The computed tomography (CT) scan showed lesions of the left temporal lobe and the paraventricular white matter suggestive of left middle cerebral artery (MCA) infarction. Carotid angiography demonstrated compression of the M1 segment of the MCA and occlusion of temporal MCA. The patient initially refused magnetic resonance imaging (MRI) because of claustrophobia. Because of fluctuating symptoms and successive worsening of the condition over weeks an MRI scan was conducted under general anaesthesia. Beneath temporal, opercular, and subcortical infarctions it revealed a left temporal tumour. A tumour biopsy disclosed a gliosarcoma (WHO grade IV). Microscopical examination of the surgical specimen demonstrated invasion of tumour cells into the wall of a greater pre-existing blood vessel. CONCLUSIONS: Malignant brain tumours may cause ischaemic infarction. This is a rare but important differential diagnosis for the origin of strokes. The authors describe the first case with infiltration of intracranial blood vessels by tumour cells of a gliosarcoma.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/54. Intracerebral hemorrhage associated with Sneddon's syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature.Sneddon's syndrome is characterized by livedo reticularis and multiple ischemic infarcts often associated with antiphospholipid antibodies. Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. We report a 38-year-old woman with a history of two miscarriages, Raynaud's phenomenon and livedo reticularis who presented acutely with ICH. Angiography showed prominent leptomeningeal and transdural anastomoses (pseudoangiomatosis). Anticardiolipin antibodies were positive. A right frontal brain biopsy failed to reveal vasculitis and a skin biopsy was nonspecific. MRI showed residual intracerebral hemorrhage (ICH), diffuse atrophy, multiple small white matter infarcts and leptomeningeal enhancement. This is the first report of Sneddon's syndrome presenting with an ICH. It shares features with the Divry-van Bogaert syndrome. We discuss the cause of the pseudoangiomatosis pattern and its role in the genesis of the hemorrhage and suggest that cerebral angiography should be done in every patient with Sneddon's syndrome, as it could impact therapy.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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