[Familial indifference to pain with somatosensory asymmetry: possible central anomaly]. / Forme familiale d'insensibilité à la douleur avec asymétrie de la somesthésie: anomalie centrale?

A family of seven siblings is described. The mother and six siblings have been examined, the eldest and youngest of whom suffer from congenital indifference to pain , although both were ticklish, and itched. The functions examined included somatosensory perception thresholds and autonomic functions; perception thresholds were greatly raised in the painfree subjects and to a lesser extent in some other family members, asymmetrically in all cases, being higher in the dominant hand. Painfree Subject 1 also underwent cerebrospinal fluid analysis at age 16, which showed normal B-endorphin levels but undetectable enkephalins. Electrophysiological tests when a child demonstrated notably that most (raised) measured values were lowered by naloxone. Light microscopic sural nerve biopsy performed on painfree Subject 1 in childhood did not suggest any abnormalities other than a thickened nerve sheath. Threshold asymmetry has not been observed in large numbers of subjects without neurological deficits. There were no significant autonomic changes in any tested family member, though there was some asymmetry. It is suggested that the findings may imply a congenital anomaly of the central nervous system which accounts for the somatosensory, biochemical, and electrophysiological abnormalities.

Trigeminal neuralgia: a quantitative sensory perception threshold study in patients who had not undergone previous invasive procedures.

The authors investigated 28 patients with "idiopathic" trigeminal neuralgia who had undergone no previous invasive procedures; together these patients had a total of 50 affected trigeminal divisions. Quantitative sensory perception thresholds were measured before operation. Preoperative measurements in the affected divisions indicated raised thresholds for touch (von Frey filaments) and temperature, but not for pinprick or heat pain, in agreement with the findings of Nurmikko. Only the tactile threshold was also significantly affected in the unaffected divisions on the affected side. The authors discuss their findings in relation to the pathophysiology of trigeminal neuralgia, concluding that the origin of the condition is almost certainly central to the gasserian ganglion.

Sensory effects of microvascular decompression in trigeminal neuralgia.

Nineteen patients with "idiopathic" trigeminal neuralgia, who had not undergone any previous interventional procedures, possessed a vessel or vessels compressing the preganglionic nerve root that was demonstrated by magnetic resonance tomographic angiography. Pain was relieved immediately in all of these patients after they underwent microvascular decompression without observed nerve damage. Although preoperative measurement of sensory perception thresholds showed elevations in the thresholds for touch (von Frey filaments) and warmth and coolness sensations, these thresholds normalized during the postoperative period. An apparent deficit in the pinprick (sharpness) sensation appeared postoperatively, but the deficit gradually regressed and completely disappeared by 1 year after surgery; this phenomenon may have been a statistical anomaly. The patients' pain disappeared immediately postoperatively and remained absent throughout the follow-up period. The authors conclude that damage to the nerve or nerve root is not essential for the relief of trigeminal neuralgia.