Subcortical neglect: intentional or attentional?
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Biomedical subjects
Publications and source records attributed to F Viader.
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The dementia associated with progressive supranuclear palsy (PSP) is considered to be subcortical because the cerebral cortex, unlike the subcortical structures, is usually free from major neuropathological lesions; the characteristic symptoms point to a dysfunction of the prefrontal lobe. The regional cerebral metabolic rate of glucose (rCMR Glu) was studied by positron emission tomography and 18F-fluoro-2-deoxyglucose18FDG in 6 patients presumed to have PSP and was compared with values found in 8 control subjects of similar age. The results obtained showed a highly significant rCMR Glu decrease in the prefrontal cortex of our patients. The loss of several subcortical afferents to prefrontal cortex may be responsible for the frontal cortical hypometabolism present in PSP.
A 32 year old obese, diabetic and hypertensive woman presented with signs of increasing intracranial pressure: diplopia, partial lateralised Jacksonian seizures followed by alternating post-ictal hemiplegia. She was also anaemic. Investigation revealed lead poisoning and thrombosis of the superior sagittal sinus. She recovered rapidly with heparin and calcium EDTA therapy, except for persistent optic atrophy due to the papilloedema. The authors discuss the relation of lead encephalopathy and dural sinus thrombosis, and the contribution of each condition to this patient's clinical symptoms.
Neuropsychological findings in 10 clinical cases of progressive supranuclear palsy are presented. Patients were aged 52 to 80 and the duration of their illness was 1 to 5 years. Severe psychological disturbances e.g. depression or outbursts of irritability were prominent features in 5 patients. Formal neuropsychological testing disclosed mental slowing, impaired attention, reduced verbal fluency and elaborated linguistic abilities, poor abstract thinking and reasoning, mild to moderate memory loss, dynamic apraxia, grasping, motor impersistence, imitation and utilization behaviour. The clinical similarities between these neuropsychological changes and frontal lobe syndromes, together with the lack of cortical involvement, suggest that the "dementia" of progressive supranuclear palsy could be explained by deactivation of the frontal cortex by subcortical lesions. The special importance of pallidal and mesencephalic reticular involvement in the syndrome is hypothetized.
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Four years after the first sign of panarteritis nodosa, a 60 years old woman presented severe pain in the lower thoracic spine followed by motor, sensory and sphincter paralysis. With corticosteroid therapy, condition improved 24 hours later, but myelography revealed an incomplete block from T9 to L1, by an epidural hemorrhage. In the course of panarteritis nodosa, one case of epidural hemorrhage, and a few cases of subarachnoid hemorrhage of the spinal canal have been described.
A 84-year-old diabetic man had a complete right-sided hemiplegia with ipsilateral hypoesthesia and vertical diplopia, but no aphasia or hemianopia. The CT scan showed a low-density area in the posterior limb of the left internal capsule, in the territory of the anterior choroidal artery. The mechanism of diplopia is discussed and is attributed to the involvement of the mesencephalic territory of the anterior choroidal artery.
Periodic bouts of laryngeal dyspnea in a 77-year-old man were observed early in the course of a syndrome due to bilateral opercular infarcts. Laryngoscopy showed alternate slow opening and closure of the glottis superimposed on respiratory vocal cord movements. The disorder could result from bilateral removal of cortical control of bulbar centers, its periodicity suggesting analogies with Cheyne-Stokes' dyspnea.
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An ischemic lesion limited to the anterior limb of the internal capsule provoked a left motor neglect in a 64-year-old man. There was reduction in spontaneous mobility of the left side of the body, reversible during verbal incitations and accompanied by motor extinction. There were no perceptive disorders, or visual, somatesthetic, or auditive neglect. The importance of the motor extinction phenomenon (described by Valenstein and Heilman in a case with a lesion of similar site) is emphasized: it demonstrates the role in motor neglect of a disequilibrium in intentional activation of the hemispheres. Pathological and clinical correlations are discussed in relation to cases reported in the literature and the results of animal studies. It is suggested that motor neglect may result from interruption of connections between the median thalamic nuclei, the striatum, and the frontal cortex. Relationships between motor neglect and perceptive (attentional) neglect syndromes are discussed.
A right-handed 77-year-old man presented with a left homonymous lateral hemianopsia involving the macula. Consciousness, memory, and oral language were normal, but the patient experienced great difficulties in identifying objects and images by sight, even though he recognized their geometrical shapes. He also had prosopagnosia, and alexia without agraphia but no agnosia for colours. The patient died suddenly 6 weeks after the onset of the disorders. Pathological examination demonstrated the presence of a bilateral occipital infarct involving on the right, the inferior border of the calcarine fissure, and the lingual and fusiform gyri, and on the left the fusiform gyrus. This supports the role in man of the inferior occipitotemporal convolutions in the perceptual integration of visual informations, and suggests that this region of the cortex is analogous with the inferotemporal cortex of monkeys which has been shown to be a determinant for the elaboration of visual objects.
Seventy five patients referred with a diagnosis of pneumonia underwent transtracheal puncture. In 76% of cases this examination led to discovery of an organism in infected patients. In 50% of cases, the bacteriological diagnosis was sufficiently accurately oriented by direct examination to permit rapid and effective treatment. There was virtually perfect agreement with the results of blood cultures when the latter were positive. The organisms most often responsible were Gram positive and above all the pneumococcus. These results led to narrow spectrum antibiotic treatment appropriate for the organism. This early treatment, based upon the results of tracheal puncture, was associated with a favourable course in 85% of cases, in particular in severely ill patients requiring intubation. No notable complications occurred.
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We report here for the first time positive anti-dystrophin labelling of inclusions in three cases belonging to the same family affected by familial cytoplasmic body myopathy (CBM). Inclusions are also stained, as reported previously, by anti-actin antibodies. The anti-desmin reaction was negative in the centre of cytoplasmic bodies (CB) but showed an enhancement of staining in the peripheral part. Abnormal sarcoplasmic staining of fibres with CB was also observed with that antibody. Anti-vimentin antibody labelling was negative. At present, the significance of this labelling by anti-dystrophin antibodies is unknown, but will open new fields for further investigations in an attempt to understand CB pathogenesis.
BACKGROUND AND PURPOSE: Combined perfusion and oxygen metabolism PET imaging is highly predictive of spontaneous outcome after middle cerebral artery (MCA) stroke, independent of clinical scores, but whether the assessment of perfusion alone by SPECT provides similar information remains unclear. We have assessed the prognostic value of (99m)Tc-HMPAO-SPECT at the acute stage of stroke. METHODS: Twenty-seven first-ever nonhemorrhagic MCA territory stroke patients were prospectively studied 4-20 h (mean: 12 h) after onset with (99m)Tc-HMPAO-SPECT. None was part of a therapeutic trial or received thrombolysis. Neurological deficits were quantified at admission and 2 months later with Orgogozo's MCA scale. SPECT images were visually classified by 3 independent observers into one of three patterns, as follows: pattern I = marked and extensive tracer hypofixation; pattern II = moderate and/or focal hypofixation with or without combined hyperfixation, and pattern III = normal or increased uptake without hypofixation. In addition to this visual analysis, we also calculated a voxel-based 'hypoperfusion score', an index of severity x extent of hypofixation according to Mountz's method. RESULTS: There was a good intra- and interobserver agreement. After consensus, 9, 14 and 4 patients were classified in patterns I, II and III, respectively. These patterns had a significant predictive value for raw outcome but not for percentage recovery (p = 0.008 and p = 0.127, respectively). Thus, all patients in pattern III had a good outcome, while most (but not all) patients in pattern I had a poor or intermediate outcome; pattern II patients were more evenly distributed among outcomes. Hypoperfusion scores were highly significantly positively correlated with both 2-month outcomes and percentage recoveries, even after controlling the predictive value of day 0 MCA scores by partial correlations. COMMENTS: We found that SPECT had a significant added predictive value even when compared to admission neurological scores. Although less accurate than PET, (99m)Tc-HMPAO-SPECT may help to predict spontaneous individual neurological evolution, especially whenever perfusion images are normal or show an increased tracer uptake without associated hypofixation.
Although 'intrahemispheric diaschisis' (i.e. a hypometabolism affecting the cerebral hemisphere ipsilateral to but remote from the infarct) may classically exacerbate acute-stage neurological deficit and influence early recovery, it has been studied only rarely. Out of a series of 30 patients with first-ever middle cerebral artery (MCA) territory stroke, we analyzed the data from 19 survivors investigated by (15)O positron emission tomography (PET) both in the acute (within 5-18 h of clinical onset) and subacute (approximately 3 weeks later) stage, and for whom chronic-stage CT coregistered with PET was available to assess infarct topography and size. Orgogozo's MCA scale was used to assess neurological deficits at the time of, and recovery between, the PET studies. Oxygen consumption was obtained for both PET sessions for the whole ipsilateral hemisphere (excluding ventricles and infarct), as well as for the thalamus and the occipital and mesial-prefrontal cortex (i.e. potentially deafferented tissue outside the MCA territory). In all regions except the occipital cortex, the oxygen consumption significantly decreased between the first and the second session, without significant correlation with the concomitant changes in MCA scores. However, acute-stage mesial-prefrontal metabolism was significantly correlated with neurological recovery. Also, both the hemisphere and the mesial-prefrontal metabolism at the second session were significantly correlated with both infarct size and concomitant MCA scores, but the latter relationship became insignificant when infarct size was taken into account. This study reveals no evidence of acute intrahemispheric diaschisis after MCA territory stroke in man. However, it documents for the first time a phenomenon of delayed intrahemispheric remote hypometabolism developing while the patients clinically recover. Because the degree of this secondary phenomenon is a function of infarct size, a mechanism of degeneration of the damaged neuron terminals is likely. Finally, contrary to other reports, neurological recovery was not a function of thalamic hypometabolism, but appeared to be influenced by acute-stage mesial-frontal metabolism, perhaps because this region is part of a network that has an important compensatory role in motor recovery.