Treatment of blepharospasm with botulinum neurotoxin type A: long-term results.

PURPOSE: To describe the long-term efficacy and side effects of treatment of blepharospasm with botulinum neurotoxin type A (Botox). METHODS: A total of 178 patients with blepharospasm were treated by injections of botulinum toxin in the Eye Clinic of the University of Naples from 1980 to 2001. The severity of spasm for each patient was graded on a four-point scale. Duration of improvement was assessed and reported in months. RESULTS: Of 178 cases, 10 were lost to follow-up; of the remaining patients, 93% reported improvement after treatments. The mean duration of improvement was 3.6 months. Twelve patients (76%) who underwent more than 14 treatments maintained stable relief. Three patients (1.7%) had a total remission of spasms. Side-effects were local; none of the 168 patients experienced any systemic or toxic reaction. CONCLUSIONS: Botulinum toxin therapy for blepharospasm can provide long-lasting relief and reduction of spasms in the majority of patients. This therapy has the advantages of being safe, simple, and repeatable.

Distribution of FMRFamide-like immunoreactivity in the amphibian brain: comparative analysis.

FMRFamide is a small neuropeptide present in particular neurons of the basal forebrain and midbrain of the vertebrate groups studied, especially fishes and mammals. In order to assess interspecies variation, the distribution of FMRFamide-like immunoreactivity was studied in the brains of 13 species of amphibian. Although FMRFamide-immunoreactive (IR) terminals occurred throughout much of the brain, IR cell groups were noted in circumscribed regions of the CNS. In the eight anuran species studied, two major populations of labeled perikarya were observed: one in the septopreoptic area and another one in the caudal portion of the diencephalon. The rostrocaudal extent of both and the number of labeled somata in each neuronal group displayed species-specific differences. In urodeles and gymnophiones, labeled perikarya were located in the diencephalon, but there were remarkable species differences in the number of such cells. It is discussed whether sex or season of collection may account for some of the differences observed. The distribution of FMRFamide-IR perikarya, fibers, and pathways in the brain of anurans, urodeles, and gymnophiones was compared. The existence of FMRFamide perikarya in the anterior preoptic neuropil and medial septum appeared to be a feature common to all anurans; labeled neurons in the dorsal thalamus, however, may be present only in the (viviparous) gymnophione Typhlonectes compressicauda. Cerebrospinal fluid contacting FMRFamide neuronal cell bodies and fibers were observed in each of the three taxonomic orders. The data are compared with those previously obtained for other groups of vertebrates.

A case of Parinaud's syndrome in a boy with delayed puberty.

The authors describe a case of Parinaud's syndrome in a 14-year-old boy with delayed puberty. The neurological examination and the neuroradiological work-up excluded the presence of cerebral pathological processes except for a pituitary microadenoma. As the sole presence of the microadenoma cannot justify gonadotropin deficiency, the authors in this case favor a form of isolated gonadotropin deficiency, and they suggest that the elevation paralysis can be put in the range of median line defects, such as labiopalatoschisis and hypoplasia of the olfactory bulbs, frequently associated with isolated hypogonadotropic hypogonadism.

Friedreich's ataxia: clinical involvement and evoked potentials.

In 15 patients definitely affected by Friedreich's ataxia, precocious SEP abnormalities were constantly recorded independent of the duration of symptoms and the severity of clinical involvement; VEPs were frequently involved, but neither VEP abnormalities, nor visual impairment could be correlated with the severity or duration of Friedreich's ataxia clinical involvement; BAEPs were, to different extents, abnormal. These were completely dissociated from hearing disorders and significantly correlated (P less than 0.001) with Friedreich's ataxia clinical disability. Our observation of increased latency of the VEP P100 component and the early disappearance of BAEP Wave V with persistence of Wave I is in contrast with the hypothesis that changes are related to primary axonal degeneration. For practical purposes SEPs could help in the early diagnosis of Friedreich's ataxia, and BAEPs could be used for the electrophysiological monitoring of its clinical progression.