1/64. White matter dementia in cadasil.Cerebral white matter disorders may be associated with profound neurobehavioral dysfunction. We report a 62-year-old man who had a slowly progressive 25-year history of personality change, psychosis, mood disorder, and dementia. neurologic examination disclosed abulia, impaired memory retrieval, and preserved language, with only minimal motor impairment. Neuropsychological testing found a sustained attention deficit, cognitive slowing, impaired learning with intact recognition, and perseveration. magnetic resonance imaging of the brain revealed extensive leukoencephalopathy. Right frontal brain biopsy showed ill-defined white matter pallor with hyaline narrowing of white matter arterioles. Granular osmiophilic material adjacent to vascular smooth muscle cells on electron microscopy of a skin biopsy, and an arginine for cysteine replacement at position 169 in the 4 EGF motif of the notch 3 region on chromosome 19q12 established the diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil). This case illustrates that cadasil can manifest as an isolated neurobehavioral disorder over an extended time period. The dementia associated with cadasil closely resembles that which may occur with other white matter disorders, and represents an example of white matter dementia.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/64. Assessment of outcome by EC/IC bypass with 123I-iomazenil brain SPECT.We report two patients with occlusive cerebrovascular disease who were examined by means of benzodiazepine receptor SPECT(BZR-SPECT) with 123I-iomazenil (IMZ) before extracranial-intracranial bypass surgery (EC/IC bypass). Preoperative low perfusion areas detected by cerebral blood flow SPECT (CBF-SPECT) were divided into two parts on BZR-SPECT images. In the low perfusion areas where the BZR were preserved, regional cerebral blood flow (rCBF) increased on postoperative CBF-SPECT, but where the BZR were not preserved, rCBF did not increase on postoperative CBF-SPECT. On visual inspection, the SPECT images of postoperative CBF-SPECT appeared similar to those of preoperative BZR-SPECT. For evaluation of the ischemic brain condition itself, instead of the cerebral metabolism, the distribution and activity of cerebral neurons indicated by BZR-SPECT with IMZ might be utilized.- - - - - - - - - - ranking = 2.5keywords = brain (Clic here for more details about this article) |
3/64. Ruptured intracranial mycotic aneurysm presenting as cerebral haemorrhage in an infant: case report and review of the literature.A 2-month-old male infant presented with intracranial haemorrhage caused by ruptured intracranial mycotic aneurysm. Computed tomography and cerebral selective angiography revealed a large haematoma in the left sylvian fissure and a mycotic aneurysm of a peripheral branch of the middle cerebral artery. Despite the successful surgical removal, the child did not recover from the initial brain injury and died 2 months later. There have been fewer than 10 reported cases of infantile mycotic aneurysms and its occurrence in the absence of infectious endocarditis is exceptionally rare.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
4/64. Irreversible maternal brain injury during pregnancy: a case report and review of the literature.Maternal brain death or massive injury leading to persistent vegetative state during pregnancy is a rare event. Since 1979, 11 cases, including the current one, of irreversible maternal brain damage in pregnancy have been reported. In all but one, the pregnancies were prolonged with a goal of achieving delivery of a viable infant. Current advances in medicine and critical care enable today's physician to offer prolonged life-support to maximize the chances for survival in the neonate whose mother is technically brain dead. We present a case at our institution and review all previously published cases in the English literature for comparison as well as make management recommendations.- - - - - - - - - - ranking = 3.5keywords = brain (Clic here for more details about this article) |
5/64. Pulmonary barotrauma-induced cerebral arterial gas embolism with spontaneous recovery: commentary on the rationale for therapeutic compression.Pulmonary barotrauma-induced cerebral arterial gas embolism (CAGE) continues to complicate compressed gas diving activities. Inadequate lung ventilation secondary to inadvertent breath holding or rapid buoyant ascent can quickly generate a critical state of lung over-pressure. Pulmonary over-pressurization may also occur as a consequence of acute and chronic pulmonary pathologies. Resulting barotrauma frequently causes structural failure within the terminal distal airway. Respiratory gases are then free to embolize the systemic circulation via the pulmonary vasculature and the left heart. The brain is a common target organ. Bubbles that enter the cerebral arteries coalesce to form columns of gas as the vascular network narrows. Small amounts of gas frequently pass directly through the cerebral circulation without occlusion. Larger columns of gas occlude regional brain blood flow, either transiently or permanently, producing a stroke-like clinical picture. In cases of spontaneous redistribution, a period of apparent recovery is frequently followed by relapse. The etiology of relapse appears to be multifactoral, and chiefly the consequence of a failure of reperfusion. Prediction of who will relapse is not possible, and any such relapse is of ominous prognostic significance. It is advisable, therefore, that CAGE patients who undergo spontaneous recovery be promptly recompressed while breathing oxygen. Therapeutic compression will serve to antagonize leukocyte-mediated ischemia-reperfusion injury; limit potential re-embolization of brain blood flow, secondary to further leakage from the original pulmonary lesion or recirculation of gas from the initial occlusive event; protect against embolic injury to other organs; aid in the resolution of component cerebral edema; reduce the likelihood of late brain infarction reported in patients who have undergone spontaneous clinical recovery; and prophylax against decompression sickness in high gas loading dives that precede accelerated ascents and omitted stage decompression.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/64. Cerebral arterial dolichoectasia with seizure. Case report.cerebral angiography, performed after a seizure in a patient with a life-long history of typical hemiplegic migraine, disclosed markedly dolichoectatic anterior and middle cerebral arteries. No abnormality of the adjacent capillary or venous structures was present. A positive brain scan was attributed to ischemia induced by vasospasm rather than to the corresponding large tortuous anterior and middle cerebral arteries. There were no permanent sequelae and the patient has been free of seizures on Dilantin and phenobarbital over a 3-year follow-up period. Angiographic demonstration or description of a similar ectatic set of anterior and middle cerebral arteries could not be found in the literature. The concurrence of seizures and hemiplegic migraine adds to the peculiarity of this case.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
7/64. Radical "visual capture" observed in a patient with severe visual agnosia.We report the case of a 79-year-old female with visual agnosia due to brain infarction in the left posterior cerebral artery. She could recognize objects used in daily life rather well by touch (the number of objects correctly identified was 16 out of 20 presented objects), but she could not recognize them as well by vision (6 out of 20). In this case, it was expected that she would recognize them well when permitted to use touch and vision simultaneously. Our patient, however, performed poorly, producing 5 correct answers out of 20 in the Vision-and-touch condition. It would be natural to think that visual capture functions when vision and touch provide contradictory information on concrete positions and shapes. However, in the present case, it functioned in spite of the visual deficit in recognizing objects. This should be called radical visual capture. By presenting detailed descriptions of her symptoms and neuropsychological and neuroradiological data, we clarify the characteristics of this type of capture.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
8/64. Abnormal intracranial vascular networks ("moyamoya" disease), possibly due to occlusion of bilateral internal carotid arteries--a case report with histometrical analysis.An autopsy case of abnormal intracranial vascular networks at the base of the brain corresponding to so-called rete mirabile, associated with occlusion of bilateral internal carotid arteries was reported. This patient was a 62 year-old female who died about two months after sudden onset of subarachnoid hemorrhage. At autopsy, abnormal vascular networks termed as rete mirabile were observed to be collateral blood supplies among the cerebral regions with flow of the anterior, middle, and posterior cerebral arteries, caused by long-standing obstruction of bilateral internal carotid arteries at the syphon level. Morphometrical analysis was done by measuring the length of internal elastic membrane of the internal carotid arteries in cross section, and comparing it with those of controlled persons of the same sex and age without any intracranial disorders and hypertensive histories. The result that no significant difference was observed between the former and the latter values suggested that the unusual cerebro-vascular disorder of this case developed not on the base of congenital anomaly including hypoplasia of internal carotid arteries or arteriovenous malformation but as an acquired lesion established for a long time.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
9/64. cerebral infarction in a child. A case report.Cerebral infarcts in children are rather rare and in most cases no precise etiology is established. The authors describe a case of cryptogenetic cerebral infarction in a 9-year-old boy. The child presented an acute onset of hemiplegia in the right arm and leg, central facial palsy, dysarthria and steppage. The infarction was proved by Computed tomography (CT) and magnetic resonance imaging (MRI). Laboratory and instrumental studies rule out all known causes of brain infarction. The only possible etiopathogenetic hypothesis was a varicella arteritis which occurred 45 days before the clinical manifestation.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
10/64. Impaired dynamic cerebral autoregulation in eclampsia.Eclampsia is frequently associated with brain edema, cerebral infarction or hemorrhage. Its underlying cerebrovascular pathophysiology is still poorly understood. We examined cerebral autoregulation by a non-invasive multimodal assessment in a 28-year-old primaparous woman with postpartal eclampsia. Transcranial Doppler sonography showed considerably increased cerebral blood flow velocity (CBFV) of all basal cerebral vessels. magnetic resonance imaging demonstrated multifocal vasogenic brain edema. Using transfer function analysis, a severely decreased phase shift between respiratory-induced 0.1-Hz oscillations of arterial blood pressure and CBFV was observed, indicating substantial disturbance of dynamic cerebral autoregulation (DCA). In contrast, CO(2)-vasomotor reactivity of the right middle cerebral artery was only slightly reduced. We therefore assume that the cerebral arteriolar dysfunction in eclampsia leads primarily to an impairment of the autoregulatory mechanism that is followed by different degrees of arteriolar vasodilation. Because of its probably high sensitivity to hemodynamic disturbances, assessment of DCA might be of great value in early pre-eclampsia for risk prediction of cerebral arteriopathy and eclampsia.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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