[Klippel-Trenaunay syndrome associated with antithrombin III deficiency]. / Syndrome de Klippel-Trenaunay et déficit en antithrombine III A propos d'une observation.

Klippel-Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the case of a patient presenting a KTS with stroke. The biological assessment revealed antithrombin III deficiency. Although rare, antithrombin III deficiency should be kept in mind in KTS patients with neurological involvement.

[Epileptic seizures in non-ketotic hyperglycemia]. / Crises épileptiques et hyperglycémie sans cétose.

Seizures are common in hyperglycemia and are often the first manifestation particularly in non-ketotic hyperglycemia (NKH). Published reports emphasize partial motor seizures almost exclusively. Here we present the clinical, biological and electrophysiological features of 22 consecutive patients with NKH who developed focal seizures and were treated in our department. Neurological exam was normal in 15 cases. When abnormal, it showed postictal obnubilation or diabetic polyneuropathy. Interictal EEG (obtained in 17 patients) showed focal or generalised slowing in 65% of cases, and ictal EEG (six patients) showed rapid spikes most often unilateral. CTs were normal in 77% of cases, and showed age-compatible cortico-subcortical atrophy in the others. All patients were alert, with glucose values between 13.6 and 55 mmoles/l and osmolarity values increased in all cases to a mild or moderate extent (266-309.20 mosm/l). Three out of 22 patients (14%) presented with motor epilepsy partialis continua. In 11/22 cases (50%), diabetes mellitus had not been diagnosed previously. Seizures associated with NKH were resistant to anticonvulsant treatment but responded well to insulin therapy and rehydration. They subsided completely in an average of 4 days, and only one patient had to be transiently transferred to ICU. We conclude on the importance of an early diagnosis of this condition to prevent malignant evolution of the epileptic syndrome into a state of hyperosmolarity and coma associated with a much higher mortality.

[Epilepsy and myasthenia: a case report]. / L'association myasthénie et épilepsie: à propos d'une observation.

Epilepsy is 5 to 7 times more frequent among patients with myasthenia than in the general population. Myasthenia gravis is often associated with other affectsion, most of immunological origin. However, hypotheses explaining the association are still uncertain. We report a case of association in a woman who had epilepsy since the age of 9 years and developed myasthenia at the age of 23 years after having discontinued here anti-epileptic treatment for two years.

Influence of thoracoabdominal pattern of breathing on respiratory resistance.

The purpose of this study was to test the hypothesis that voluntary changes in thoracoabdominal pattern of breathing may increase total respiratory resistance. Thirty-one normal subjects were asked to control their thoracoabdominal pattern of breathing by using a visual feedback. Thoracic and abdominal volume changes were measured by inductance plethysmography. Respiratory resistance and elastance were measured by forced oscillometry. The mean (+/-SD) percent thoracic contributions to tidal volume during thoracic or abdominal breathing were 75 (+/-11) and 25% (+/-9), respectively. These changes induced small but significant increases in resistance (P < 0.005) and elastance (P < 0.002). The increased resistance was observed in 22 subjects for thoracic breathing (P < 0.016) and in 21 subjects for abdominal breathing (P < 0.043). The mean value (+/-SD) of individual increases in resistance during thoracic or abdominal breathing, compared with normal breathing, were 9.2 +/- 17.5 and 9.4 +/- 19.9%, respectively. The fact that departing from spontaneous pattern increases respiratory resistance is consistent with the notion that breathing pattern is optimally adjusted on the basis of mechanical criteria.

Ventilatory conditioning by self-stimulation in rats: a pilot study.

This article describes an experimental attempt to condition breathing pattern in rats. In this experiment, a freely moving rat was first rewarded by an electrical stimulation of the medial forebrain bundle whenever inspiratory duration (TI) exceeded 300 ms. A bidirectional control was then used: TIs longer than 400 ms were rewarded, and then TIs shorter than 300 ms were rewarded. The frequency of TIs longer than 300 ms increased when this event was rewarded, further increased when TIs above 400 ms were rewarded, and decreased during reversal conditioning (TI < 300 ms). At the beginning of the experiment, stimulation caused increased arousal and motor activity, but after prolonged conditioning, the brain stimulation was associated with quiet wakefulness. Although the general procedure appears to be well-suited to the experimental study of voluntary breathing, some possible improvements are suggested for further, more extensive investigations.

[Graphomania. Compulsive graphic activity as a manifestation of fronto-callosal glioma]. / La graphomanie. Activité graphique compulsive Manifestation d'un gliome fronto-calleux.

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.