1/237. Right-to-left interatrial shunt in ARDS: dramatic improvement in prone position. The mechanisms leading to shunting through a patent foramen ovale include high right-sided cardiac pressures and respiratory factors due to mechanical ventilation and also anatomical changes in the right atrium as described in the platypnea-orthodeoxia syndrome. We report a patient with the adult respiratory distress syndrome (ARDS) who had a right-to-left atrial shunt which decreased in the prone position, after which oxygenation improved. The patient was admitted to the intensive care unit because of ARDS due to an invasive fungal infection. He had a history of chronic lymphocytic leukemia and paradoxical embolisms through a patent foramen ovale. Despite mechanical ventilation and antifungal treatment he developed severe ARDS. He was therefore turned to the prone position. Blood gas values improved dramatically (arterial oxygen tension/fractional inspired oxygen ratio increasing from 59 to 278 torr). Transcranial Doppler sonography was performed with bubble study, which confirmed a massive right-to-left shunt in the supine position and which instantaneously decreased in the prone position. This case suggests that a decrease in right-to-left shunt in patients who have a patent foramen ovale could partly explain the improvement in hypoxemia in the prone position. ( info) |
| 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. ( info) |
3/237. Cerebrovascular changes in neurofibromatosis. Vascular changes in neurofibromatosis are most commonly described in the renal arteries. In the present study, two children with neurofibromatosis and cerebral vascular occlusive changes demonstrated by cerebral angiography are reported. Although focal neurological findings in children with neurofibromatosis are often due to tumours, the sudden development of neurological symptoms in such cases should alert paediatricians to the possibility of cerebral vascular disease. ( info) |
| We report a 44-year-old woman with atypical aortic coarctation accompanied by cerebral artery disease. She was hospitalized for vertigo. An extra-anatomic bypass between the ascending aorta and abdominal aorta was performed using partial cardiopulmonary bypass under moderate hypothermia to reduce the after load of the left ventricle and maintain cerebral blood flow and cerebral perfusion pressure. The postoperative course was uneventful and there was no postoperative neurological deficiency. ( info) |
5/237. Vasculopathic changes of cadasil can be focal in skin biopsies. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a newly described cause of vascular dementia. Pathologic examination shows multiple small infarcts in the deep cerebral white matter together with a nonatherosclerotic, nonamyloid angiopathy involving the media of small cerebral arteries. Ultrastructurally, characteristic granular material is present in the basal lamina of vascular smooth muscle cells in cerebral and extracerebral blood vessels. The ultrastructural changes have also been demonstrated in skin biopsies of affected patients; consequently, some investigators have recently recommended skin biopsies for the diagnosis of cadasil. This study describes a 54-year-old male with a family history for strokes who had clinical and radiological features suggestive of cadasil. A skin biopsy was performed to confirm the diagnosis. Initially, the characteristic vasculopathic changes of cadasil were not identified within small blood vessel walls. However, multiple deeper sections in other areas showed electron-dense material associated with vascular smooth muscle cells, characteristic of cadasil. Subsequent genetic testing demonstrated a single nucleotide substitution at position 659 on chromosome 19p13.1 causing an amino-acid change (Cys --> Phe), a finding indicative of cadasil. The involvement of blood vessels within the dermis makes skin biopsy a useful adjunct in the diagnosis of cadasil. However, as illustrated by this case, the findings may be focal, requiring a thorough evaluation of the entire biopsy specimen. ( info) |
6/237. 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. ( info) |
7/237. Multiple major cerebral artery thromboses with profound thrombocytopenia in acute leukaemia. A child with acute lymphoblastic leukaemia complicated by prolonged gastrointestinal and skin haemorrhages due to profound thrombocytopenia finally died of thrombotic occlusions of major cerebral arteries due to mucormycosis. biopsy of any suspect lesion is needed urgently before prolonged therapy with amphotericin b is started. So far there have been no cures in childhood. ( info) |
8/237. 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. ( info) |
| stroke is a well-documented complication of amphetamine abuse. methylphenidate, chemically and pharmacologically similar to amphetamines, is widely used in the treatment of attention deficit disorder in children. The possibility of vasculitis connected to methylphenidate should not be surprising. A case is reported of stroke associated with ingestion of methylphenidate in an 8-year-old boy. family history was negative and other causes of vasculitis were excluded. We draw your attention to the risk of using methylphenidate for a long period of time. ( info) |
10/237. Calcification of intracranial vessels in neurocysticercosis. We report calcification of intracranial vessels in neurocysticercosis. Calcification was observed in the middle cerebral arteries in two patients, and the circle of willis in two others. The patients with middle cerebral artery calcification underwent CT with inhaled stable xenon and an area of mild hypoperfusion was observed in the ipsilateral cerebral hemisphere. ( info) |