Familial hemifacial spasm and determinants of late onset.

The role of hypertension in the late onset of hemifacial spasm (HFS) is evaluated in a family, spanning four generations. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed a variable anatomical relationship between nervous and vascular structures in the symptomatic cerebello-pontine angle. In one case, showing neurovascular conflict (NVC), microvascular surgical decompression was followed by clinical resolution of HFS. Neuroimaging suggesting NVC was found in all symptomatic patients of the last two generations and in three younger subjects not affected by HFS. As a determinant for the late development of clinical expression is reviewed the role of arterial hypertension, detected few years before HFS appearing in all symptomatic subjects. The distribution of NVC in several members of the same family suggests a genetic susceptibility towards vascular anomaly.

Long-lasting benefits of botulinum toxin type B in Parkinson's disease-related drooling.

PURPOSE: To investigate the safety, efficacy and effectiveness of botulinum toxin type-B (BTX-B) injections into the parotid glands to reduce drooling in Parkinson's disease (PD) subjects. METHODS: A double-blind, randomised, placebo-controlled study enrolled 36 advanced phase PD subjects who complained of disabling drooling. Patients received either 4000U BTX-B or placebo. Anatomically guided injections were performed. Outcome measures were chosen to assess both the subjective feeling of improvement (i. e. the Drooling Severity and Frequency Scale, DSFS, visuo-analogic ratings of familial distress, VAS-FD, and social distress, VAS-SD) and objective saliva reduction (saliva production over five minutes was checked by weighing dental rolls). The Global Impression Score (GIS) was also applied, rating improvement from 0 to 3. RESULTS: One month after injections, BTX-B patients showed a meaningful improvement in almost all subjective outcomes. Two-way analysis of variance gave a significant time x treatment effect, F-value being 52.5 (p < 0.0001) for DS-FS, 23.2 (p < 0.0001) for VAS-FD, 29 (p < 0.0001) for VAS-SD, and 28.9 (p < 0.0001) for UPDRSADL drooling item score. All BTX-B subjects declared sialorrhea reduction of any kind (moderate for 44.4% cases, and dramatic for 33.3% subjects), at variance with 61.1% controls who denied any benefit from treatment. (Chi-square = 22.9; p < 0.0001). When present, benefits lasted on average 19.2 +/- 6.3 weeks in the BTX-B group compared to 6.7 +/- 1.4 weeks in controls (T-value: 26.4; p < 0.0001). CONCLUSIONS: BTXB represents a safe and efficacious tool for the management of PD-related drooling, ensuring a longlasting waning of this disabling symptom.

Botulinum toxin type A for drooling in Parkinson's disease: a double-blind, randomized, placebo-controlled study.

To investigate the safety and efficacy of botulinum toxin type A (BoNTX) treatment to reduce sialorrhea in Parkinson's disease (PD), a double-blind, randomized, placebo-controlled study enrolled 32 PD patients complaining of excessive drooling. Patients received either 50 U Botox in each parotid gland or placebo without using ultrasound guidance. Subjects treated with BoNTX experienced a reduction in both drooling frequency and familial and social disability (TimexGroup effect: P<0.01), as well as in saliva production (Time x Group effect: P<0.0001). No adverse events were recorded. BoNTX injections are safe and effective treatment for the management of PD-related drooling.

Quality-of-life assessment in patients with hyperhidrosis before and after treatment with botulinum toxin: results of an open-label study.

BACKGROUND: Patients with hyperhidrosis, a disorder characterized by increased sweat production, experience substantial functional and emotional problems. Botulinum toxin type A (BTX-A) has been shown to be useful in the treatment of hyperhidrosis; however, few studies have considered the effects of treatment on patients' quality of life (QOL). OBJECTIVES: The objectives of this study were to assess QOL in patients with focal hyperhidrosis; to investigate whether the impairment in QOL in these patients is related to the type of hyperhidrosis or the number of sites involved; and to compare the changes in QOL and the response to BTX-A treatment in patients with axillary and palmar hyperhidrosis. METHODS: Patients with focal primary hyperhidrosis of the axillae, palms, and soles who had experienced decreased QOL and whose condition had not responded to conventional topical and physical therapies were included in this open-label study. Patients completed a self-administered Dermatology Life Quality Index (DLQI) questionnaire before and 2 weeks after treatment with BTX-A. RESULTS: All 41 patients had experienced a decrease in QOL as measured by the DLQI. The impairement in QOL was not dependent on the number or types of sites involved. Treatment with BTX-A led to improvement in QOL in all patients, with the median DLQI score decreasing (ie, improving) significantly from pretreatment level (P < 0.001). The improvement in QOL and response to treatment were similar in patients with axillary and palmar hyperhidrosis. CONCLUSIONS: Further studies with a longer follow-up period are needed to assess the long-term effects of BTX-A; however, preliminary data from the present study suggest that BTX-A improves QOL in patients with focal hyperhidrosis, independent of the presenting clinical picture.