No abstract

Fisher's syndrome was studied in six patients, one of whom was an 11-year-old girl, and the nosological position of this neuro-ophthalmological disorder was reviewed. The clinical features of the ophthalmoplegia in these cases were reported as secondary to a transient inflammatory lesion in the mid-brain; this was demonstrated in one of the cases by an enhancing area on CT scan. This mid-brain localization could explain not only the classical Fisher's triad but also many variations of the syndrome. The overlap of our cases of Fisher's syndrome with so-called brain-stem encephalitis of Bickerstaff has been emphasized. Many cases of Fisher's syndrome could therefore correspond to an inflammatory process at brain-stem level with or without nerve-root involvement. Clinical and neurophysiological findings show, however, that there is peripheral damage in some cases, so that a neutral attitude needs to be maintained on the nosological position of Fisher's syndrome.

No abstract

The Tolosa-Hunt syndrome (THS) is characterized by remittent and sometimes recurring episodes of painful ophthalmoplegia. The etiopathogenesis is still unclear and is an object of controversy. A non-specific granulomatous process of the wall of the cavernous sinus is claimed by many authors as the possible cause, on the basis of a few pathological studies. Other authors suggest the possible role of autoimmune or specific inflammatory processes localized in the retroorbital perineural tissues. The clinical, laboratory and radiological findings of the eight cases reported in the agreement with those previously described in the literature. However, the visual evoked potentials (VEP) were delayed in three of the four cases in which they had been studied. This finding, together with the observation that some analogies exist between THS and other well known neuritic processes of the cranial nerves, may suggest that at least in some cases THS may be related to an ocular polyneuritis.

A 56 year old woman with acute intermittent porphyria presented mainly motor polyneuropathy, ophthalmoparesis of probable supranuclear origin and a mild organic brain syndrome. The neurological complications gradually cleared during a course of plasmapheresis. The fairly rapid improvement could conceivably have been fortuitous but it may well have been due to plasmapheresis.