Publications by authors named "Degos J"

We report the case of a 53-year old woman who developed a neuromyelitis optica (NMO or Devic's syndrome) after a spinal anaesthesia with bupivacaine. To our knowledge, the use of bupivacaine in human has not been associated to neurological complications like myelitis or NMO. The pathogenesis of this complication will be discussed.

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The objective of this study is to define tremor and cerebellar dysfunction and determine whether kinetic and postural tremor correlate with cerebellar dysfunction in patients with multiple sclerosis (MS). Cerebellar symptoms such as dysmetria often interfere with tremor evaluation in MS. The Stewart-Holmes (SH) manoeuvre, which has been recently quantified, may offer a selective evaluation of cerebellar dysfunction in such patients.

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Three cases of bacterial brain abscesses, in immunocompetent patients, are reported. In all these cases, the diffusion-weighted magnetic resonance (MRI) with apparent diffusion coefficient (ADC) map has permitted an early diagnosis and a rapid treatment. This emergency MRI showed in the three cases a low signal on TI-weighted images, a high signal on T2-weighted and echo-planar images, and a decrease of ADC (0.

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Background: Hepatitis C virus (HCV)-associated neuropathy is usually associated with mixed cryoglobulinemia (MC) and vasculitis. MC may contain viral RNA, and tissues showing vasculitis may contain intracellular HCV. Local HCV replication remains to be evidenced.

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Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II).

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Myelinolysis is characterized by a non inflammatory demyelination, affecting the central portion of the pons. Clinical features usually reflect damage to the descending motor tracts and include spastic tetraparesis, pseudobulbar paralysis, and the locked-in syndrome. We describe the case of a 58-year-old man, who developed a pontine and extrapontine myelinolysis, without hyponatremia, after an anaphylactic shock.

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Unlabelled: Akinetic symptoms or off-period motor signs in patients with Parkinson's disease and motor fluctuations can be associated with subjective symptoms. A systematic interview was conducted in a series of 24 patients. Sixteen out of 24 (67 p.

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We describe a patient, VSB, whose reading was impaired as a consequence of a left temporal-parietal lesion, whereas writing was relatively preserved. At variance with other pure alexic patients described in the literature, VSB claimed to have become unable to mentally visualise letters and words. Indeed, his performance on a series of tests tapping visual mental imagery for orthographic material was severely impaired.

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Macrophagic myofasciitis (MMF), a condition newly recognized in France, is manifested by diffuse myalgias and characterized by highly specific myopathological alterations which have recently been shown to represent an unusually persistent local reaction to intramuscular injections of aluminium-containing vaccines. Among 92 MMF patients recognized so far, eight of them, which included the seven patients reported here, had a symptomatic demyelinating CNS disorder. CNS manifestations included hemisensory or sensorimotor symptoms (four out of seven), bilateral pyramidal signs (six out of seven), cerebellar signs (four out of seven), visual loss (two out of seven), cognitive and behavioural disorders (one out of seven) and bladder dysfunction (one out of seven).

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Objective: To assess the natural progression of cognitive impairment in Huntington's disease (HD) and to reveal factors that may mask this progression.

Background: Although numerous cross-sectional studies reported cognitive deterioration at different stages of the disease, progressive cognitive deterioration has been, up to now, difficult to demonstrate in neuropsychological longitudinal studies.

Methods: The authors assessed 22 patients in early stages of HD at yearly intervals for 2 to 4 years (average, 31.

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A clinical sign is described consisting of a brief extension of the toes after upward tapping of the plantar surface of their terminal phalanges. Electrological data support the hypothesis of a long loop reflex, which we call the "toe phasic posture reflex" (TPPR). In a preliminary study, this sign was observed in patients with central nervous system disease, especially in subcortical brain disorders regardless of etiology.

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Background: Huntington's disease is a neurodegenerative disease of genetic origin that mainly affects the striatum. It has severe motor and cognitive consequences and, up to now, no treatment. Motor and cognitive functions can be restored in experimental animal models by means of intrastriatal transplantation of fetal striatal neuroblasts.

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A few neuropsychological studies have suggested the existence of bilateral hemispheric representations for the proximal parts of the limbs in humans. We report the case of a patient who presented with a callosal disconnection syndrome, which at a later stage of disease became restricted to left agraphia, left agraphesthesia and left auditory extinction. The anomic character of the agraphesthesia was demonstrated.

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Using a proprioceptive "straight-ahead" pointing task, we determined the position of the subjective sagittal middle in thirty unselected patients with unilateral vascular lesions in the right hemisphere and twenty-two normal controls. Patients with extensive right parietal damage (n = 16) showed an ipsilesional (rightward) deviation of their egocentric reference, whereas patients with lesions that substantially spared the right parietal lobe (n = 14) showed a contralesional (leftward) deviation. No significant correlation emerged between the position of the egocentric reference and the performance on a neglect battery.

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This study describes issues related to the safety and tolerability of fetal striatal neural allografts as assessed in five patients with Huntington's disease. Huntington's disease (HD) is characterized by motor, cognitive, and behavioral disturbances. The latter include psychological disturbances and, as a consequence, we took particular care to analyze behavioral changes, in addition to the usual "safety" follow-up.

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Background And Purpose: We know remarkably little about the mechanisms underlying cortical activation. Such mechanisms might be better understood by studying the effect of well-localized lesions on the cortical activations in simple paradigms.

Methods: We used H(2)(15)O and positron emission tomography to measure regional cerebral blood flow (rCBF) at rest and during hand vibration in 7 patients with unilateral thalamic lesion involving the ventroposterior (VP) somatosensory thalamic relay nuclei.

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Breakdown of the blood-brain barrier (BBB) and ensuing gliosis are common events following physical trauma to the central nervous system (CNS) or during autoimmune diseases such as experimental allergic encephalomyelitis (EAE). Some studies of EAE in rodents report that peripheral injections of complete Freund's adjuvant (CFA), which contains heat-inactivated Mycobacterium to provoke peripheral inflammation without adversely affecting the CNS, can itself lead to increased BBB permeability to small tracer molecules and certain serum proteins. To study the equivocal relationship between serum protein extravasation and reactive gliosis, we injected C57BL/6 mice with CFA and histologically assessed the permeability of various serum proteins and the reactivity of proximal microglia and astrocytes in the uninjured brainstem and spinal cord enlargements after 1-4 weeks.

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Neurofibromatosis type 1 (NF1) is a genetic disease with a wide range of neurological manifestations. To examine these, and to evaluate neurological morbidity in adulthood of patients with NF1, we studied a hospital-based series of 158 patients that included 138 adult patients aged >18 years and 20 children. NF1 evaluation included a multidisciplinary clinical and a clinically oriented radiological investigation.

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Objective: To investigate whether tactile extinction alters the cortical somatosensory activations induced by hand vibration.

Background: Tactile extinction occurs mainly after right-brain lesions and consists of the inability to perceive a contralesional cutaneous stimulation when a similar stimulus is applied to the mirror region of the ipsilesional hemibody. The pathophysiology of tactile extinction is poorly understood, but it is considered to be a deficit of selective attention of somatosensory stimuli.

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Unilateral neglect--the inability to pay attention to events occurring on one side of space--usually occurs for left-side events after focal right-hemisphere damage. We report a 73-year-old woman with probable AD and no evidence of focal brain lesions who showed signs of right-side neglect and extinction. Neglect was more severe after 1 year.

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Selective impairment in recognition of faces (prosopagnosia) resulting from certain localized cortical lesions has been advanced as an argument for a face specific brain module. The argument is claimed to be strengthened by the discovery of an inversion superiority effect in the recognition of faces by a prosopagnosic patient (Farah et al., Vis Res 1995b;35:2089-2093).

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We describe a disorder similar to body-image agnosia or autotopagnosia characterized by the inability to designate targets situated outside the body. This disorder, which we have termed allotopagnosia, occurs exclusively in subjects with a lesion involving the posterior region of the left parietal lobe. The most common manifestation is the designation of parts of the body of another person as being part of the patient's own body (heterotopagnosia with self-designation).

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