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Biomedical subjects

J Cambier

Publications and source records attributed to J Cambier.

At least 73 records · Page 4Linked to original sources

[Eales' disease with neurologic disorders].

Two cases of Eale's disease, with neurological involvement have been studied with magnetic resonance imaging (MRI). In the first case the ophthalmological disease had been diagnosed ten years before the onset of a cerebellar ataxia. In the second case the characteristic ocular changes were followed eight years later by a myelopathy. MRI in both cases showed multifocal white matter abnormalities. The nosological interpretation of such cases remains uncertain: association of Eale's disease with multiple sclerosis or vasculopathy involving the central nervous system and the retina?

Adult↗

[Epilepsy with bilateral cortical calcifications. Discussion of a durable post-critical deficit].

The epilepsy-bilateral cortical calcifications syndrome includes epilepsy with onset in childhood or adolescence and symmetrical calcifications of cerebral cortex with a predilection for the occipital cortex. The calcifications are sometimes visible on plain radiograph images but may be detected only by CT. Literature perusal revealed reports of 16 cases. We report a case with an aperceptive visual agnosia and marked but resolutive CT anomalies developed during a partial status epilepticus episode.

Adolescent↗

[Distal infarction of the left superior cerebellar artery presenting with dysarthria and unsteadiness].

Small cerebellar infarction with a benign outcome occurs more frequently than a massive infarction with brainstem compression. Retrospective CT studies have shown it. These infarcts are more often localized in the superior hemisphere of the cerebellum which semiology is not well known. Some reports pointed out that an acute cerebellar infarction in the PICA territory may mimic labyrinthine lesion. The authors report a case of an acute small cerebellar infarction in the SCA territory presenting as a dysarthria and unsteadiness, involving the left lobulus semilunaris superior, in a paravermal zone, on CT scan.

Aged↗

[Graphomania. Compulsive graphic activity as a manifestation of fronto-callosal glioma].

The presenting symptomatology in a case of fronto-callosal glioma were affective indifference, severe disorders of attention and dynamic aphasia with marked reduction in spoken expression. Spontaneous and induced writing were abundant and incoercible. Their meticulous production and formal correction contrasted with its semantic incoherence. This behavior is comparable with that of the compulsive activity that may result from pallidal lesions or bilateral frontal lesions. The term graphomania is proposed to distinguish this behavior from echographia and hypergraphism.

Aged↗

MRI evaluation of tuberous sclerosis.

The authors present four cases of tuberous sclerosis examined with MRI. The patho-anatomic aspects are reviewed and analysed with respect to MRI data. MRI appears superior to the CT particularly for imaging of cortical tubers, cystic lesions, and heterotopic clusters; these last two features were never described with MRI before. Here is also presented the second progressive case of giant intracranial aneurysm associated with tuberous sclerosis.

Adult↗

Long-term treatment of epilepsy: open multicenter trial with progabide in epileptic patients.

A long-term open multicenter trial was carried out in 15 European centers with therapy-resistant epileptics to evaluate the efficacy and safety of progabide, a new antiepileptic GABA receptor agonist; 187 patients, suffering from partial epilepsy (57%), primary generalized epilepsy (20%), secondary generalized epilepsy (21%), and unclassified generalized epilepsy (2%), participated in the study. All patients had a total seizure frequency higher than one per month in spite of standard antiepileptic medication; 46% had a mean partial seizure frequency from daily to weekly. Progabide was administered at a mean daily dose of 30.5 mg/kg/day as an add-on to the standard antiepileptic drugs up to one year in 115 patients; 37 patients (19.8%) dropped out because of reasons which were not drug-related (bad compliance, lost to follow-up); in 12 patients (6.5%) progabide was withdrawn for side effects and in 20 (10.7%) for lack of efficacy. 71.3% of patients treated for one year (62% considering the 'cumulative' number of patients) experienced more than a 50% reduction in seizure frequency. This reduction was equally present in patients with partial epilepsy (63.9%) and with generalized epilepsy (62.2% of patients with primary and 57.1% with secondary generalized epilepsy). No signs of tolerance phenomena to the antiepileptic effect of progabide were observed. No side effects were reported in 56.7% of the patients. Clinical side effects were mild and transient, leading to progabide discontinuation in 6.5% of the patients only; an increase in SGPT was observed in 5.7% of the patients, these increases were transient and without any clinical symptom.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Study of the cerebral blood flow by intravenous injection of xenon-133 in thalamic and juxtathalamic lesions].

Sixteen patients with subcortical vascular lesions of one hemisphere, (7 left, including 4 aphasics and 9 right, 4 of which had a left neglect syndrome) and 4 controls without noticeable cerebral pathology underwent cerebral blood flow (CBF) measurements with intra-venous Xe 133, both at rest and during activation. The latter consisted of listening to a text together with eye opening and was considered non specific. At rest, a non significant CBF lowering on the side of the lesion was found in most patients, mainly in right brain damaged ones. Hypoperfusion areas (HA) were noted, predominantly in parieto-temporal cortex in right lesions, and in lower and middle frontal areas as well in left lesions. HA persisted during activation on both sides, except on the right side in left damaged patients. Activation resulted in a bilateral and roughly symmetrical mean CBF increase, which was not significant in left lesions nor in controls, but was significant (p less than .05) in right brain damaged patients without neglect syndrome. Conversely, patients with right lesions resulting in left neglect exhibited conspicuous mean CBF asymmetry during activation with depression on the right side. Right-left difference in mean CBF was significantly higher in this group than in right sided lesions without neglect (p less than .05). These facts are consistent with an activation imbalance between hemispheres as the mechanism of unilateral neglect syndromes, and support the view that right subcortical pathways and especially the thalamus, play a part in the activation of both cerebral hemispheres.

Adult↗

[Amnesia and stiff-man syndrome. Manifestations disclosing paraneoplastic encephalomyelitis].

The authors report a case of hypertonia, the clinical and electrophysiological features of which were identical to those of the Stiff-man syndrome first described by Moersch and Woltman in 1956. This and the five other previously reported cases can be attributed to encephalomyelitis of the anterior horns of the spinal cord. All of these cases had severe but partial involvement of the anterior horns and the probable physiopathological mechanism of the resulting contractures was elective destruction of the small inhibitory neurones. In the present case, the association of a limbic encephalitis and secondary exteriorization of a carcinoma of the pharynx, strongly suggested a paraneoplasic encephalomyelitis. This is the first report indicating an association between the Stiff man syndrome and a paraneoplastic syndrome. Another point of interest was the spectacular response of the hypertonia to steroid therapy suggesting a central neuropharmacological effect quite apart from the non specific anti-inflammatory action on the encephalomyelitis.

Amnesia↗

[Auditory hallucinations in lesions of the brain stem].

Since the publication by Jean Lhermitte in 1922 of his paper on hallucinosis, the peduncular type has been described as a purely visual phenomenon. However, limited brain stem lesions can give rise to analogous manifestations in the auditory field. Five cases of auditory hallucinosis are reviewed, the first four resulting from a lesion of tegmentum of pons responsible for contralateral hemi-anesthesia and homolateral facial palsy with paralysis of laterality. Central type hypoacusis and a severe disorder of localization of sounds revealed a lesion of trapezoid body. The fifth case resulted from a peduncular lesion in region supplied by superior cerebellar artery, the auditory deficit being related to a lesion of inferior corpus quadrigeminum. In one patient, the auditory hallucinosis was followed by a period of visual hallucinations and oneiric delusions. Both auditory and visual hallucinosis can be related to hypnagogic hallucinations. Dream mechanisms (the geniculo-occipital spikes system) escape from normal inhibitory control exerted by the raphe nuclei. Auditory deafferentation could predispose to auditory hallucinosis.

Aged↗

BSF1 induces membrane protein phosphorylation but not phosphoinositide metabolism, Ca2+ mobilization, protein kinase C translocation, or membrane depolarization in resting murine B lymphocytes.

The findings presented in this study provide evidence that BSF1 receptors and mIg transmit signals via dissimilar transduction mechanisms that result in a common biologic response, hyper-Ia expression. Specifically, BSF1-containing supernatant does not induce PtdInsP2 hydrolysis as determined by measurement of PtdOH and InsP3. Additionally, BSF1 does not stimulate Ca2+ mobilization, PKC translocation from cytosol to membrane, or membrane depolarization. All of these metabolic events appear to play a central role in hyper-Ia expression mediated by mIg and are initiated after treatment of resting B cells with anti-Ig antibodies. In vitro phosphorylation studies with partially purified plasma membranes from resting B cells revealed that BSF1 interaction with membrane receptors stimulates a membrane-associated protein kinase that phosphorylates an endogenous protein of 44 KDa. Anti-Ig does not stimulate phosphorylation of the 44 KDa protein, suggesting that it does not activate the membrane-associated protein kinase. This observation provides the first evidence of a signal transduction mechanism associated with BSF1-receptor ligation. It indicates that although BSF1 does not modulate events associated with PKC activation, it may function via activation of a membrane-associated protein kinase. This provides a focal point for further studies directed at elucidating signal transduction resulting from BSF1-receptor interaction.

Animals↗

[Idiopathic diffuse polyalgia syndrome].

The term "idiopathic diffuse polyalgia syndrome" is suggested to designate an entity well known to neurologists, rheumatologists and all physicians interested in pain. In this syndrome pain is diffuse but often predominates in a given region. Physical examination shows excessive tenderness in well-defined areas. Radiological and laboratory examinations are negative. Psychological factors are not always obvious. Several arguments are in favour of dysfunction of the pain control mechanisms. The effectiveness of the various treatments prescribed is related to the quality of the doctor-patient relation.

Humans↗

Subcortical neglect.

The unilateral neglect syndrome is defined as a lack of directed attention to one half of the space or the body. It has been reported mainly after cortical lesions of the right parietal or frontal lobe in man. However, there is strong experimental and clinical evidence for the existence of neglect syndromes of subcortical origin. The unilateral neglect syndrome is not the result of elementary sensory deficits. The failure in directed attention is dependent on an imbalance of hemispheric activation.

Aged↗

Infarction in the territory of the anterior choroidal artery. A clinical and computerized tomographic study of 16 cases.

Sixteen cases of the anterior choroidal artery syndrome are reported. In its completed form, this rare syndrome combines the triad of hemiplegia, hemianaesthesia, and homonymous hemianopia. CT examination confirms the diagnosis by revealing an area of reduced density situated in the posterior limb of the internal capsule, sparing the thalamus medially and encroaching upon the tip of the globus pallidus laterally, and corresponding to the distribution of the anterior choroidal artery. Incomplete forms of the syndrome are more frequent. Left-sided spatial neglect may accompany right-sided lesions, as may slight disorders of speech in left-sided lesions. Clinical-anatomical correlations are discussed.

Adult↗

[Cerebrospinal fluid beta-endorphin in congenital insensitivity to pain].

Spontaneously elevated nociceptive threshold levels were markedly diminished after Naloxone injections in 4 patients with congenital insensitivity to pain. This finding suggested the hypothesis of a relation between congenital insensitivity to pain and permanent hyperfunction of an endomorphinic system. Radio-immunoassay of CSF beta-endorphin was performed in all 4 cases. The normal or only slightly elevated levels cannot explain electrophysiologic findings, but as a function of the multiplicity of endogenous opioid systems, hyperactivity of another endomorphinic system cannot be excluded. Other hypotheses may also be proposed.

Endorphins↗