[Hemichorea, hemiballism disclosing non-ketotic hyperglycemia]. / Hémichorée, hémiballisme révélateurs d'une hyperglycémie sans cétose.

Non ketotic hyperglycemia is a metabolic disorder which can induce various hyperkinetic disorders. We report the case of a 74-year old woman admitted to hospital because of choreo-ballic movements of her left hemibody. Laboratory tests revealed non-ketotic hyperglycemia. CT scan showed an increased density in the right putamen and head of caudate. Magnetic resonance imaging disclosed a hyperintense signal involving the right putamen on T1-weighted images. On T2-weighted images, there was a slight hyperintensity in the right putamen and head of caudate. Correction of the underlying hyperglycemia led to complete resolution of abnormal movements. Pathophysiology of this clinicoradiologic syndrome is briefly discussed.

[A case of cerebral giant-cell angiitis associated with cerebral amyloid angiopathy. Favorable evolution with corticosteroid therapy]. / Un cas d'angéite cérébrale à cellules géantes associée à une angiopathie amyloïde cérébrale. Evolution favorable sous traitement corticoïde.

We report a biopsy proven case of cerebral giant cell angiitis associated with cerebral amyloid angiopathy. Clinical history and radiological lesions were rapidly progressive. The patient recovered with corticosteroid treatment. The pathogenesis relationship between angiitis and amyloid angiopathy are discussed.

Magnetic resonance imaging of subacute myelopathy due to cobalamin deficiency.

A 77-year-old woman presented with rapidly ascending sensory ataxia. Magnetic resonance imaging (MRI) showed extensive involvement of the dorsal columns of the spinal cord. Hematological data were normal despite severe cobalamin deficiency. The contribution of magnetic resonance imaging in subacute degeneration of the spinal cord is discussed.

[Motor evoked potentials]. / Potentiels évoqués moteurs.

Due to the motor evoked potentials recorded in limb muscles after transcranial and spinal magnetic stimulation, conduction in the central motor pathways can now be evaluated safely and painlessly in man. The central motor conduction time obtained includes the time required for transmission, along the fast pyramidal fibres, from the cortex to the spinal motoneurons, the synaptic transmission to motoneurons and the conduction on a short segment of the motor nerve root. Lengthening of this time almost always reflects dysfunction of the central motor pathways. The abnormalities observed are not specific of any particular cause, and they must be interpreted in relation to the context. The usefulness of this new electrophysiological technique is being tested by radiological and anatomico-clinical correlations in various diseases of the central nervous system (e.g. disseminated sclerosis, cerebral infarction, spinal cord injury) and the locomotor apparatus (e.g. cervical myelopathy, radiculopathy).

Left tuberothalamic artery territory infarction: neuropsychological and MRI features.

We examined a 29-year-old woman who had language disturbances and memory impairment after a left thalamic infarction. MRI showed injury that was limited to the tuberothalamic artery territory. Beside reduced voice volume and verbal memory trouble, she presented with aspontaneity, loss of psychic self-activation and affective drive. Considering thalamic lesions, this loss of psychic self-activation or 'athymhormie' was found in a left thalamic infarct and could not be considered as an exclusive characteristic of bithalamic infarctions. The disappearance of the neurobehavioral disturbances within 15 days after the onset of the troubles was associated with the decreasing of the mass lesion found by MRI.

Hot-water epilepsy in an adult: ictal EEG, MRI and SPECT features.

Reflex epilepsy constitutes a rare form of epileptic seizures. We observed a 20-year-old man who presented with seizures induced by immersion in hot water. The trigger stimulus was specific. Contrast CT scan and MRI were all normal, not revealing any structural lesion. Ictal EEG recorded during a hot bath showed focal epileptic discharges in the left temporo-occipital area. Interictal SPECT showed a hypometabolism in the same cerebral region. Neuroimaging studies were rarely performed in this uncommon type of epilepsy. Nevertheless, in our case the result of the SPECT suggests a localized functional disturbance in the emergence of the disorder.

[Cerebrospinal fluid beta-endorphin in chronic pain in man]. / Bêta-endorphine du liquide céphalo-rachidien et douleurs chroniques chez l'homme.

Concentrations of beta-endorphin in the cerebrospinal fluid were measured in 21 painless subjects and 37 patients with chronic pain. Statistical analysis of the results showed no significant difference between the two groups. This lack of correlation between beta-endorphin concentrations in the CSF and the presence of chronic pain highlights the difficulties and limitations encountered when trying to determine the role of endogenous opioid systems with this method.

[Improvement of Joseph's disease with sulfamethazole-trimethoprim]. / Amélioration d'une maladie de Joseph par le sulfaméthoxazole-triméthoprime.

A case of Joseph's disease characterized by spasticity in all 4 limbs, dystonia and disorders of ocular motility was considerably improved by cotrimoxazole, which confirms a recently published case. Further studies are required to establish this therapeutic effectiveness.

[Methodology for a controlled trial in Alzheimer's disease]. / Méthodologie d'un essai contrôlé dans la maladie d'Alzheimer.

The various evaluation scales available to study the clinical courses of Alzheimer-like dementias are controverted, and the practical aspects of a controlled trial are being considered. A tentative protocol has been designed, in which multiple individual assessments are combined with several validated scales and tests. This protocol would lend itself to a multicentre study, as it only includes evaluation tools that are easy to handle and do not require too much time. It should make it possible to select a relatively homogeneous group of patients and provide an opportunity to quantify the course of Alzheimer's disease over one year. In the discussion, emphasis is laid upon the foreseeable difficulty to evaluate a therapeutic benefit in our present imperfect knowledge of the natural history and prognostic factors of the disease.

[Cerebrospinal fluid beta-endorphin in congenital insensitivity to pain]. / Béta-endorphine du liquide céphalo-rachidien au cours de l'insensibilité congénitale à la douleur.

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.

[Study of pain thresholds by recording flexor reflexes in thalamic syndromes]. / Etude du seuil de la douleur par enregistrement des réflexes de flexion au cours des syndromes thalamiques.

Both thresholds of nociceptive flexion reflex and pain sensation were studied in 6 normal subjects and in 6 patients with typical thalamic pain. In these patients, on the painful side, these thresholds were found increased (98 p. 100; 89 p. 100 respectively) compared to the normal side. Values obtained in this latter did not significantly differ from those observed in normal subjects. After 8 days of indalpine treatment, the nociceptive reflex threshold was furthered increased in the painful side while the pain threshold was not modified by this drug. In the normal side, changes observed after indalpine were similar to that obtained in normal subjects. All the indalpine-induced modifications were reversed by naloxone in both patients and normals. These results are discussed in the context of the possible mechanisms of thalamic hyperpathia.

[Neurologic manifestations of the acquired immunodeficiency syndrome]. / Les manifestations neurologiques du syndrome d'immuno-dépression acquise (SIDA).

Recognition of the neurological symptoms and signs of the acquired immunodeficiency syndrome (AIDS) since 1982 has demonstrated the involvement of the nervous system in approximately one third of the cases. Certain opportunistic infections or tumors had been previously described in the course of immunodeficiency states of other origins: cerebral toxoplasmosis, cryptococcosis, progressive multifocal leukoencephalopathy, atypical mycobacteriosis and cerebral lymphoma. Other disorders such as subacute encephalitis raise specific etiopathogenic questions. Several of these affections can be associated or succeed each other and this is the natural course in AIDS. The detection of those conditions that are curable, among which toxoplasmosis, is of primary importance.

[Secondary prevention of cerebral infarction caused by atherosclerosis. 3-year comparison of flurbiprofen and an ergot derivative]. / Prévention secondaire des infarctus cérébraux dus à l'athérosclérose. Comparaison à trois ans du flurbiproféne et d'un dérivé de l'ergot de seigle.

A multicentre controlled trial was conducted on 383 patients to compare the effectiveness of flurbiprofen (100 mg/day) and an ergot derivative in preventing cerebral infarction. The end-point of the trial was recurrence within 3 years of treatment with one or the other drug of a fatal or non-fatal cerebral infarction. Only those patients who had already experienced an established and disabling ischaemic accident in the carotid or vertebro-basilar territory were included; 88% of these entered the trial less than 6 weeks after the accident. Sixty-one patients had recurrent cerebral infarction during the 3-year observation period: 22 in the flurbiprofen group and 39 in the ergot derivative group (p less than 0.02 log rank test adjusted by centre). The actuarial recurrence rates in the two groups were 13% and 21% respectively. These results concur with those of other trials involving aspirin to demonstrate the value of antiplatelet aggregation drugs in the secondary prevention of atherothrombotic cerebral infarction.

[Stress-induced analgesia (author's transl)]. / Analgésie provoquée par le stress. Etude électrophysiologuique chez l'homme.

The effects of a repetitive stress (expectation of severe pain) on the threshold of a nociceptive flexion reflex of the lower limb (Rlll, Bi) were investigated in normal subjects. A progressive and significant rise of the Rlll, Bi Threshold was observed as the stress was repeated several times over a 60 to 90 minutes' period. This effect was entirely reversed by naloxone, with fall of the threshold below initial values. In a double-blind test, the injection of a placebo had no significant effect on the reflex threshold when compared with control values. These results are in agreement with those reported in animals. They provide experimental evidence for an involvement of endogenous opiate systems in the mechanism of stress in normal subjects.