Congenital insensitivity to pain and naloxone.
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Biomedical subjects
Publications and source records attributed to J Cambier.
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Congenital insensitivity to pain remains without a satisfactory physiopathological explanation. In an electrophysiological study on a nociceptive flexion reflex of the lower limb, the effects of naloxone and of placebo were compared in 8 normal subjects and in a patient with congenital insensitivity to pain. In normal subjects, no significant change in the reflex threshold was observed with naloxone or with placebo. In contrast, two electrophysiological abnormalities characterized the patient: (1) spontaneous elevation in the nociceptive reflex threshold of 350% as compared to control, and (2) a large (67%) and rapid (2--3 min) fall of this threshold for about 10 min following the administration of naloxone. These results raise the problem of the relationship between congenital insensitivity to pain and an hyperactivity of a naturally occurring "morphine-like" pain-inhibitory system.
The authors report a case of non-familial amyloid neuropathy in which there was sensory-motor neuropathy of the 4 limbs, multiple lesions in the cranial nerves, and large subcutaneous amyloid deposits. Biopsy samples showed the presence of amyloid deposits in the interstitial tissues, the vessel walls in the muscle hypodermis, and in the bone marrow. A light monoclonal lambda chain was present in the serum. Immunofluorescent studies of the biopsy specimens showed the presence of elective fluorescence with an anti-lambda immunoserum in the amyloid substance.
The authors report the anatomical and clinical findings in a 65 year old patient with a lesion in the hypothalamus presenting mainly as disorders in heat and water regulation. Disorders in heat regulation dominated the clinical picture, in relation to a poikilothermia. Conservation of reactivity to pyrogenic bacteria is debatable. Inappropriate secretion of antidiuretic hormone (ISADH) by a direct effect on the supraoptico-hypophyseal aixs can account for the water and electrolyte disturbances. The anatomical lesions, due to hypothalamic changes, can be included in the circumscribed proliferative reticuloses of the CNS having a pseudo-inflammatory histological appearance.
Clinical observation of two cases with a particular type of Foville's syndrome appears to confirm physiological data from many experimental studies on the paramedian pontine reticular formation (PPRF). The nature of the oculomotor syndrome, which was confirmed by electrooculographic recordings, together with the analysis of pathognomonic features, suggested the existence of limited unilateral pontine lesions involving the formation. Rather than relying on subtle differences in ocular reflex signs it should be noted that each PPRF produces all homolateral saccades and controls pursuit eye movements in both directions, though only in the homolateral hemifield of eye movements.
The authors report on four patients with vascular lesions of the thalamus, three on the left side and one on the right, two of which were due to haemorrhage and two to infarcts. Language difficulties were observed in those cases with left thalamic lesions only. Speech was either slower or faster than normal, and there were verbal perseveration, semantic paraphasia, and more especially, reduction in verbal fluency with loss of control of logic, causing a true jargon in some tests in certain cases. Articulation, and phonemic programming were unaffected, as were comprehension, reading and writing. A comparison is made between these findings and those reported in the literature. Among the hypo-theses suggested, the authors subscribe to that which accepts the predominant role played by the interruption of the activating system of the left hemisphere by the thalamic lesion. The relative inactivation resulting from this could produce modifications in language dynamics and disturbances in attention in relation to language. The semantic paraphasia could be related to the intrusion of the right hemisphere apparatus into language, because of the lack of balance between the activation of each of the two hemispheres. The fact that thalamic lesions are characterized by language disorders would tend to show that sub-cortical dynamic factors constantly regulate the activation of the hemispheres.
Electrophysiological studies of the nociceptive reflex (R3) were carried out in eight normal subjects, and one patient with congenital insensitivity to pain, in order to compare the effects of naloxone and a placebo. No significant variation in the reflex threshold was seen in the normal subjects. Two electrophysiological abnormalities were observed in the patient: a 350 per cent spontaneous elevation in the R3 threshold compared to the control group; a rapid and large drop (67 per cent) in the threshold lasting for about ten minutes after the injection of Naloxone. These results suggest that in this particular case the insensitivity to pain could be related to the permanent hyperactivity of a morphine-like inhibitory system.
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Immitation synkinesia is usually associated with thalamic or parietal lesions. Analysis of three cases shows that the lesions may affect the posterior columns of the spinal cord or even the peripheral nervous system. In our cases, two facts stand out, a large measure of integrity of motor activity and predominant disturbances of lemniscal sensation. Imitation synkinesia should therefore not be seen as due to a lesion of a particular structure but to the disorganization of the lemniscal system at any level. Functional interconnection between the lemniscal and motor systems occurs only in the cerebral cortex. In the light of this fact, imitation synkinesia can be interpreted as the loss of particular functions in the principal cortical motor neurons.
The authors report a case of centropontine myelinosis associated with demyelinising lesions of the tectum mesencephali, the cerebellum, the central grey nuclei and the white matter of the hemispheres. This case is compared with similar observations already studied in the literature. From an aetiological standpoint, attention is drawn to the apparent causative role of diuretic therapy which had resulted in severe water and electrolyte disturbances.
We have reviewed the aetiology, symptomatology, biology and clinical course of 61 cases of the Gayet-Wernicke encephalopathy. Our results do not differ fundamentally from those of Victor et al. (1971). Nevertheless, our study shows fewer oculomotor palsies in the acute stages, lower mortality and a lower incidence of residual anmesic syndromes. These results demonstrate the importance of early diagnosis and of starting the specific therapy in the initial stage of reversible biochemical lesions.
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An electrophysiological study of the blink reflex was undertaken in 20 normal subjects and in 28 patients complaining of central facial palsy caused by unilateral hemispheral damage. In normal subjects, the latency, amplitude and organization of R1 and R2 responses are well known. Habituation of R2 responses occurred between 1 and 2 c/sec stimulation rate. R1 responses habituated at a higher stimulation rate (5 c/sec). In patients with unilateral hemispheral lesion, our results showed that changes in the blink reflex responses were bilateral. On the hemiplegic side the responses showed a decreased amplitude, while they were facilitated on the "normal" side. However, there was no change in latency of the two components of the reflex, on both sides. On the other hand, habituation of the late component occurred on the hemiplegic side for low stimulation rates: (0.5--1 c/sec), while on the "normal" side there was less habituation (3--4 c/sec), as compared with normal subjects. These results agree with those of experimental studies on cortical modulatory influences on brain-stem nuclei. They suggest a tactile origin of the two components of the blink reflex.
A comparative electromyographic study was carried out in normal subjects (group I) and in alcoholics without clinical evidence of polyneuropathy (group II). -The H reflex of the extensor digitorum brevis muscle (E.D.B.), the electromyogram of EDB, the conduction velocity of the fasted motor fibers of the peroneal nerve (PN), the sensory conduction velocity and amplitude of the evoked potential of the cutaneous fibers of PN, the H reflex of the soleus muscle. -Two kinds of changes were observed in group II: --a significant increase in the latent period of H respones in EDB, in particular the latent period of responses provoked by distal stimulation of PN (+25 PER CENT); --A SIGNIFICANT DECREASE IN THE AMPLITUDe of the sensory potential of the PN(--46 per cent). The other parameters studied in group II did not show any significant difference in comparison with the control group. These results indicate that PN is involved early in a complex fashion in latent alcoholic neuropathies. They confirm that the distinction between axonal neuropathy and segmental demyelination is rarely absolute.
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