1/8. Langerhans cell histiocytosis infiltration in cerebrospinal fluid: a case report.Langerhans cell histiocytosis (LCH) is a disease of unknown etiology characterized by a proliferation of histiocytic cells resembling the integumentary cells bearing the name of langerhans cells. LCH can be unifocal or multifocal, with one- or many-organ involvement. We present a case of LCH diagnosed in the cerebrospinal fluid of a patient with generalized lymphadenopathy and central nervous system involvement.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/8. Hyperfamiliarity for unknown faces after left lateral temporo-occipital venous infarction: a double dissociation with prosopagnosia.Right hemisphere dominance in face processing is well established and unilateral right inferior temporo-occipital damage can result in prosopagnosia. Here, we describe a 21-year-old right-handed woman with acute impairment in face recognition that selectively concerned unfamiliar faces, following a focal left lateral temporo-occipital venous infarct. She was severely impaired in discerning that unknown people seen in everyday life were unfamiliar, although she had no difficulty recognizing familiar people. Thus, she had no prosopagnosia, but abnormal 'hyperfamiliarity' for unknown faces. Her difficulty was not accompanied by delusions or deficits in discrimination, identification or memory for faces. Standard neuropsychological testing showed that her recognition of familiar faces was entirely normal. By contrast, her sense of personally knowing faces was severely impaired when unknown faces evoked weak signals of familiarity based on spurious cues, to the extent that she would misattribute fame to faces that were unknown but to which she had been incidentally exposed on a prior occasion. Priming experiments also revealed that, unlike normal subjects, she made familiarity judgements without accessing semantic identity representations. Moreover, in face recognition tests, she generally showed bias in that she relied more on right-hemisphere strategies to identify global traits and less on left-hemisphere processes compared with healthy subjects. This case provides novel evidence for a differential contribution of the two hemispheres to face recognition. Hyperfamiliarity for unknown faces might arise from an imbalance between reciprocal hemispheric functions in face recognition, with relative hypoactivation of left hemisphere processes but hyperactivation of right-hemisphere processes for retrieving stored associations about people, linking seen faces to representations of affective and personal relevance. Hence, abnormal bias in attributing some personal meaning to unknown faces could be evoked by spurious signals of familiarity based on irrelevant affective associations in the right hemisphere.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/8. 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 = 1keywords = life (Clic here for more details about this article) |
4/8. Aortic arch branch variations--case report and arteriographic analysis.OBJECTIVES: Non-recognition of a critical aortic arch branch variation at surgery, with fatal consequences, prompted a retrospective analysis of the prevalence of aortic arch branch variations in the South African population. methods: Three hundred and twenty aortic arch angiograms were analysed from existing records (January 1988--March 1998) of the vascular unit, Department of Surgery, University of Natal. RESULTS: Aortic arch branch variations were observed in 17 cases (5.3%). These variations were as follows: (i) 11 cases with two primary branches (3.4%); (ii) 1 case with three primary branches (0.3%); and (iii) 5 cases with four primary branches (1.6%). CONCLUSION: The clinical presentations which these variations give rise to are well known and may present during the first days of life or later in adulthood, or remain clinically silent. Non-recognition of the latter situation in the presence of vascular trauma may have fatal consequences.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/8. Successful cardic surgery 24 hours after craniotomy in a patient with infective endocarditis and embolic cerebellar infarction: case report.Follow up management in a patient already treated with decompressive craniotomy for a space-occupying endocarditic stroke is difficult. While immediate valve replacement eliminates the focus and therefore the high risk of re-embolization, a neurosurgical intervention is considered a contraindication to early cardiosurgery. Herein, the first report is presented of a critically ill patient with bacterial endocarditis and a space-occupying cerebellar infarction with imminent herniation, who successfully underwent mitral valve replacement only 24 h after decompressive craniotomy. To prevent rebleeding, maximal hemostasis was ensured during the neurosurgical intervention. For cardiosurgery, the patient was cooled to 21 degrees C, mildly hyperventilated, and maintained at an adequate perfusion pressure during cardiopulmonary bypass. A bioprosthesis was used to reduce the time of anticoagulation. The patient did not develop new infarcts after either intervention, and there was only a very small hemorrhagic transformation without a relevant mass effect. At five months after surgery the patient had minimal neurological abnormalities and was able to conduct his daily life without help.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/8. Acute bilateral inferior cerebellar infarction in a patient with neurosyphilis.BACKGROUND: Bilateral simultaneous infarction in the territories of the posterior inferior cerebellar arteries (PICAs) is rare but was recently reported with increasing frequency, probably because of the wider availability of magnetic resonance imaging. The cause of these infarcts is believed to be atherosclerotic or embolic occlusion of a dominant pica, which perfused the territories of the medial branches of both PICAs. RESULTS: We encountered a patient with simultaneous infarction in the territories of the medial branches of both PICAs. The clinical course, imaging results, and laboratory findings are presented. The patient was diagnosed with neurosyphilis based on a history of chancre, positive serum and cerebrospinal serologies, cerebrospinal pleocytosis, and increased intrathecal immunoglobulin synthesis. We believe that meningovascular syphilis caused the bilateral cerebellar infarct via presumed thromboangiitis of a dominant pica perfusing both cerebellar hemispheres. The patient was treated with intravenous high doses of penicillin. CONCLUSIONS: This case reminds us that meningovascular syphilis should be considered in younger patients with stroke. patients with bilateral cerebellar infarction may solely have symptoms of vertigo and ataxia but can develop life-threatening complications because of edema of the infarcted tissue with resultant hydrocephalus and pressure on the brainstem.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/8. True hemicranial decompression for severe pediatric cranial trauma: a short series of 4 cases and literature review.BACKGROUND: Traumatic acute SDH in pediatric patients is a life-threatening situation. There is a severe increase in ICP caused by acute SDH or diffuse brain swelling or secondary to ischemic brain damage. In certain situations, conventional measures may fail to control such a rapid increase in ICP. CASE DESCRIPTION: The cases of 4 pediatric patients with cranial trauma with raised ICP, in whom hemicranial decompression was performed, are described. All patients had acute SDH with diffuse brain injury; in addition, 2 of them had associated massive infarcts. Three of them survived and had a favorable outcome. CONCLUSIONS: In certain situations, pediatric patients with cranial trauma may be offered hemicranial decompression as a surgical option. These children may have a better long-term outcome despite massive infarcts.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/8. vertebral artery dissection in a child. Is "spontaneous" still an appropriate definition?Though a rare cause of stroke in the general population, in almost one quarter of young patients affected by stroke cervical artery dissection (CAD) is the underlying cause. Among these cases "spontaneous" dissections, intended as non-traumatic, represent about 34% of posterior circulation arterial dissection in patients aged less than 18 years. We here describe the case of a seven-year-old boy who developed a spontaneous vertebral artery dissection (VAD) leading to occipito-mesial, thalamo-capsular and cerebellar infarction. Once a traumatic origin was excluded, clinical history and laboratory findings were further analysed: fever associated with tonsillitis during the previous week, raised inflammatory indices, a throat culture positive for beta haemolysing streptococcus group A and high titres of streptococcal antibodies were found. This case suggests that patients with CAD referred as spontaneous deserve extensive analysis. Subjects presenting with a dissection and an underlying infection are likely to have a hyperinflammatory response (although further experience is needed). In these patients immediate start of antibiotic therapy, treatment with anti-inflammatory drugs and further a lifelong prophylaxis with antibiotics before any invasive procedure are strongly recommended.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |