The treatment of severe dystonia in children and adults.

Twenty-three children (aged less than 18 years) and 17 adults with severe widespread dystonia were treated with high doses of benzhexol (up to 130 mg daily introduced slowly over many weeks). Children tolerated higher doses (median 30 mg/day) than adults (median 20 mg/day). 52% of the children gained useful benefit, many (43%) without unwanted side effects. Such an approach was less successful in adults; 41% gained benefit, but only 35% had no side effects. Twelve adults with severe axial dystonia, and two children with life-threatening generalised dystonia were treated with a combination of a low constant dose of tetrabenazine to which were added pimozide and benzhexol as necessary. The dose of tetrabenazine was aimed at 75 mg daily; pimozide was increased (6 to 25 mg/day) until the dystonia was relieved or Parkinsonism and other side-effects prevented further increments; if necessary benzhexol (6 to 30 mg/day) then was added to control side-effects and to provide additional benefit. 75% of the adults with severe axial dystonia, and one of the two children with life threatening generalised dystonia gained useful benefit from this regime. It is concluded that high dose benzhexol is the present first treatment of choice for children with severe dystonia, and is worth a try in adults but with less expectation of success. When benzhexol treatment alone fails in adults with severe disabling axial dystonia, or in children with life-threatening generalised dystonia, combined therapy with tetrabenazine, pimozide and benzhexol may give valuable symptomatic relief.     

BIOFEEDBACK TECHNIQUES AND BEHAVIOUR MODIFICATION IN THE CONSERVATIVE REMEDIATION OF DROOLING BY CHILDREN WITH CEREBRAL PALSY

In an attempt to decrease the rates of drooling of 12 children with cerebral palsy, the authors investigated the effectiveness of EMG auditory feedback training of the orbicularis oris, of making the act of swallowing a conscious one, and of providing an auditory signal to cue swallowing by means of an Accularm interval timer. After biofeedback training there was a significant decrease in drooling rates and a small increase in swallowing rates. After the Accularm was used the children maintained the decreased drooling rates and there was a further small improvement in swallowing rates. One month after treatment stopped there was a non-significant regression in the rates of both drooling and swallowing. The marked decrease in drooling after biofeedback training must be attributed to more effective swallowing as a result of improved oral motor control, rather than to increased rates of swallowing.     

Prevalence of drooling in children with cerebral palsy attending special schools

Prevalence of drooling in children with cerebral palsy (CP) and its severity across dental age (i.e. children with primary, mixed, and permanent dentitions) was investigated. One hundred and sixty children and adolescents (91 males, 69 females; mean age 10 years 10 months, SD 4 years 2 months; range 4 to 18 years) with CP who attended special schools were involved in this study. Sixty eight per cent of participants had spastic CP with the majority (45%) diagnosed as having quadriplegia. Presence or absence of drooling was determined by direct observation of the participants. Other data, such as age, sex, learning disability, and type of CP were collected using questionnaires. Results showed 93 of 160 children (58%) with CP had a drooling condition and of these 53 (33%) had severe drooling. It was found that the degree of drooling decreased as the child's dental age increased. Prevalence of drooling in children with primary dentition was found to be 75% as compared with 43% of those with permanent dentition, although this difference was not statistically significant. It was concluded that any invasive treatment to reduce drooling should, perhaps, be postponed until the child's oromusculature has matured.     

Addition of Kinesio Taping of the orbicularis oris muscles to speech therapy rapidly improves drooling in children with neurological disorders

Objective: To evaluate the effects of Kinesio Taping (KT) of the orbicularis oris muscles as an adjunct to standard therapy for drooling. Methods: Fifteen children with neurological disorders and drooling received speech therapy and twice-weekly KT of the orbicularis muscles over a 30-day period. Drooling was assessed by six parameters: impact on the life of the child and caregiver; severity of drooling; frequency of drooling; drooling volume (estimated by number of bibs used); salivary leak; and interlabial gap. Seven markers of oral motor skills were also assessed. Results: KT of the orbicularis oris region reduced the interlabial gap. All oral motor skills and almost all markers of drooling improved after 15 days of treatment. Conclusion: In this sample of children with neurological disorders, adding KT of the orbicularis oris muscles to speech therapy caused rapid improvement in oral motor skills and drooling.     

Effectiveness of the Innsbruck Sensorimotor Activator and Regulator in improving saliva control in children with cerebral palsy

The aim of this study was to determine the effectiveness of an intraoral appliance, the Innsbruck Sensorimotor Activator and Regulator (ISMAR), in improving drooling and eating skills in a group of children with cerebral palsy, and to determine which factors might indicate good candidates for this type of treatment. Eighteen children (13 males, five females; mean age 7 years 10 months, range 4 to 13 years) were selected. Measures of drooling and feeding skills were taken at baseline, at the completion of a 6-month control phase, and at two more 6-monthly time points after the ISMAR was fitted. Children varied greatly in both the length of time taken to tolerate wearing the ISMAR and duration for which the appliance was worn. Only six children (four females, two males) completed the full study. Their motor disabilities were athetosis (n=3), spastic quadriplegia (n=2), and hypotonia (n=1); four of the six children used a wheelchair for locomotion. None had epilepsy and none had greater than mild cognitive impairment. For these children drooling severity scores and eating and drinking skills improved significantly over the treatment period in comparison with the control phase. We conclude that the ISMAR remains a valid option in improving drooling in children and merits further study.     

Botulinum toxin A as a treatment for excessive drooling in children

Drooling is problematic for some neurologically impaired children. Botulinum toxin A injection to salivary glands has effectively reduced drooling in adults but has only recently been used to treat children. This was a preliminary study to determine the efficacy and safety of botulinum toxin in children. Children identified as having severe daily drooling were enrolled. The preinjection assessment included measurement of the amount and frequency of drool. Each parotid gland was injected with 5 U of botulinum toxin A. Follow-up was for a minimum of 16 weeks. Nine children were enrolled, 4-17 years of age. All children had moderate or severe mental retardation. At week 4, all patients had a reduced drooling frequency and eight of nine patients had a reduction in the weight of saliva. Overall, five of nine parents (55%) deemed the treatment successful. This preliminary study demonstrates that botulinum toxin A is a relatively effective treatment for some children with significant drooling without serious side effects.     

Traditional Chinese medicine (tongue acupuncture) in children with drooling problems

Tongue acupuncture is an innovative technique in traditional Chinese medicine. We have demonstrated that specific tongue acupoints are related to various functional domains. This study aimed to assess the efficacy of tongue acupuncture in children with neurologic disability who had severe drooling problems. We conducted an intent-to-treat study in a cohort of 10 children. A continuous course of tongue acupuncture was performed daily to five acupoints in the tongue for a total of 30 sessions. Standardized outcome measures of drooling were evaluated by a blinded assessor to study the efficacy at baseline and after a course of treatment. Statistically significant improvement was noted in the following outcome measures: (1) mean visual analog scale (VAS) decreased from 6.6 (pre-TAC) to 4.67 (post-TAC) (P = 0.002); (2) mean drooling quotient (DQ) decreased from 14.016% (pre-TAC) to 8.335% (post-TAC) (P = 0.0078); and (3) mean drooling score (DS) decreased from 7.4 (pre-TAC) to 4.4 (post-TAC) (P = 0.002). This study demonstrated the efficacy of tongue acupuncture as an adjunctive or alternative treatment for patients with drooling problems and can be integrated as part of the oromotor stimulation program, drooling program, and behavioral modification program before subjecting the patient to invasive surgical procedures on the salivary glands.     

The Surgical Management of Drooling

As with all branches of surgery, selection of the appropriate operative procedure for a particular patient involves careful weighing of all the alternatives and full discussion with the patient and carers. Each of the procedures described has its devotees and detractors. For an individual patient, however, the risks of each, the likely postoperative course and the results of the surgery--both in terms of the expected chance of improvement in drooling and the presence or otherwise of residual scarring or taste--must be balanced to determine the optimum plan for treatment. The long-term results of submandibular duct transposition for drooling in the author's own institution have recently been reported. An initial improvement in the drooling of all patients was maintained for at least two years in 17 of 20 patients. Only two patients experienced complications requiring further surgery (ranulas in each case). It is suggested that these very satisfactory results, achieved without external scarring and without compromising the sense of taste, support the contention that submandibular duct transposition is the surgical treatment of choice for children and young people with cerebral palsy who drool excessively.     

Anticholinergic-induced chorea in the treatment of focal dystonia

The occurrence of chorea, induced by trihexyphenidyl (benzhexol hydrochloride) during the treatment of five adult patients who had focal or segmental dystonia, is described. The dose at which chorea appeared ranged from 15 to 60 mg/day (mean 31.7 mg/day). All but one patient had developed common adverse effects of this drug (dry mouth, blurred vision, and confusion) at lower doses (mean 21.8 mg per day). There was an inverse relationship between the age of the patient and the dose of trihexyphenidyl at which chorea developed.     

Management of drooling in individuals with neurodisability: a surgical experience

From 1975 to 1 January 1999, 1103 neurologically involved patients (mean age 13.2 years; 686 males, 417 females) referred with problematic drooling, or sialorrhea, were assessed at a pediatric rehabilitation center by a team consisting of an otolaryngologist, speech pathologist, and a dentist. The initial standard treatment for persistent sialorrhea (in the compliant or aware patient) is oral-motor training. A group of 522 patients with persistent significant drooling after a minimum of 6 months of oral-motor training, or profuse drooling in the presence of a low cognitive level, underwent surgery, usually when over 6 years of age. From 1978 to 1 January 1998, the operation of first choice was submandibular duct relocation (SDR), and was completed in a total of 226 patients. Midway through 1988, sublingual gland excision was also completed at the time of submandibular duct relocation (SDRSGE); 249 of these procedures have been completed to January 1st 1999. Those patients who had SDRSGE had significantly fewer complications that required additional surgery than those that had SDR only. However, the impact of surgery on the drooling as evaluated in subsets of both groups (SDR n=115, SDRSGE n=106) was statistically similar. The study of 11 children with salivary gland radionuclitide scans to determine the effect of submandibular duct surgery on gland function was inconclusive. The pattern of oral-motor function in 26 children studied after SDRSGE surgery suggested that those children with severe impairment of volitional motor function and profuse drooling tended to have a poorer outcome following surgery compared to those with milder impairments.     

Botulinum toxin injections for children with excessive drooling

The objective of this study was to evaluate the feasibility of ultrasonography-guided injections of botulinum toxin A into the parotid glands of children with severe drooling (sialorrhea). Excessive drooling is common in children with chronic neurologic disorders. Preliminary observations in adults suggest that injections of botulinum toxin A into the parotid glands can decrease drooling, but the optimal dose, sites of injection, and concomitant use of imaging during injections and its use for children have not been established. Ultrasonography was used to guide the injection of botulinum toxin (10-25 IU) into both parotid glands of nine children with excessive drooling. Subjective and objective measures of the severity of drooling were collected before and after botulinum toxin A injections. A booster injection was provided if the initial response was inadequate. Injections were well tolerated, and no adverse reactions were observed. Ultrasonography revealed that the parotid gland showed a variable depth, extent, and vascularization. Eight of nine patients needed a booster injection after 1 month. Objective measures of drooling severity were improved in seven of nine patients. However, subjective improvement was reported in only three of nine patients, and this improvement was functionally significant in only one patient. Although intraparotid injection of botulinum toxin A is safe and causes a reduction in saliva production in children, the doses used in this study did not result in functionally significant improvement. Higher doses of botulinum toxin A in the parotid glands or concomitant injections into the submandibular glands can increase the efficacy of these injections. Variability in size, depth, and vascular supply of the parotid gland suggests the importance of ultrasonography guidance for optimizing injections. These results underscore the need for further studies to establish the efficacy of this treatment in children.     

Prevalence and predictors of drooling in 7‐ to 14‐year‐old children with cerebral palsy: a population study

Aim To establish a prevalence estimate for drooling and explore factors associated with drooling in a population sample of children with cerebral palsy (CP) aged 7 to 14 years living in Victoria, Australia. Method A self‐report questionnaire was used to collect data on drooling from parents of children born between 1996 and 2001, and registered with the Victorian Cerebral Palsy Register. Results A total of 385 children (231 males, 154 females; mean age 10y 9mo [SD 1y 7mo], range 8–14y) were studied. The clinical type and distribution of CP were spastic (341), ataxic (16), dyskinetic (17), hypotonic (10), and unknown (1). Distribution in Gross Motor Function Classification System (GMFCS) levels was I (103), II (98), III (52), IV (63), V (61), and unknown (8). After adjustment for topographical pattern of motor impairment and GMFCS level, 40% were reported to have experienced drooling between 4 years of age and the time of completing the questionnaire. A significantly higher prevalence of drooling was found in children with poor gross motor function and in those with more severe presentations of CP, including poor head control, difficulty with eating, and inability to sustain lip closure (p<0.001 for each). Drooling was shown to be significantly associated with both intellectual disability and epilepsy in this group of children (p<0.001 for both). Interpretation With a prevalence of 40%, drooling is an important comorbidity in CP. It was considered severe in 15% of children. Poor oromotor function was associated with drooling and could be the target of interventions for this under‐researched problem.     

Secondary non-response due to antibody formation in a child after three injections of botulinum toxin B into the salivary glands

Botulinum toxin (BTX) offers a new treatment option to reduce drooling in adults and children. Antibody formation against BTX is known to be one reason for clinical secondary non-response to this treatment. This is a case report on the development of secondary non-response to BTX type B (BTX-B) in a 15-year-old male, with bilateral dyskinetic cerebral palsy (Gross Motor Function Classification System Level IV) with additional learning disability* and microcephaly, treated for the indication of drooling. After three successful treatment sessions, the fourth and fifth injections showed no clinical response. This was associated with the presence of antibodies against BTX-B as determined using the mouse diaphragm assay. Thus, formation of neutralizing antibodies against BTX-B appears to be an important issue, not only in patients treated for cervical dystonia but also in children treated for drooling. Subsequent injections with an adequate dose of BTX type A (BTX-A) did not show any clinical response either, although no antibodies to BTX-A were detected. Besides the unanswered questions of dosing and distribution, a second possible explanation could be that BTX-B gave rise to non-neutralizing antibodies that cross-react with BTX-A. The resulting immune complexes could be taken up by phagocytes and, thereby, impede clinical response.     

Treatment of sialorrhea in children with cerebral palsy: a double-blind placebo controlled trial

Objectives

To prospectively study the efficacy and safety of intraparotid gland injection of Botulinum neurotoxin serotype A (Dysport^®) for the treatment of sialorrhea (drooling) in children with cerebral palsy (CP).

Patients and methods

Twenty-four children, ages 21 months to 7 years, were recruited and randomized to receive either treatment with 100 U Botulinum toxin or placebo. Rating scales for the frequency and severity of drooling were performed at the time of injection, at 1 month, and at baseline prior to the second injection. A second set of injections of either 140 U of drug or placebo was given 4 months later, and the same rating scales were used. Eight patients declined the second injection. Due to high dropouts in the placebo group in second set of injections, statistical analysis was performed for the results of the initial injection only.

Results

Scores of the median frequency (p = 0.034) and severity (p = 0.026) of drooling were reduced in the treatment group. Median total score also declined in the treatment group (p = 0.027). After the second injection, five out of nine patients injected with the drug showed a decline in the total score; including three patients who did not respond to the first injection. Only two patients experienced transient increase in drooling after the treatment with the drug.

Conclusion

Botulinum toxin is an effective and safe treatment option for drooling in children with CP.     

Drooling in children with cerebral palsy: effect of salivary flow reduction on daily life and care

The purpose of this study was to investigate the effect of salivary flow reduction on daily life and provision of care in children with cerebral palsy (CP). Parents of children with CP were asked to fill in a questionnaire on the impact of drooling on the daily life of their children and their families and the data were then analyzed. Forty-five children with severe drooling (28 males, 17 females; mean age 9y 5mo [SD 3y 7mo]; range 3 to 16y) were monitored before and after receiving medication (scopolamine and botulinum toxin) to reduce salivary flow. Type of CP included hypotonia (n = 1), spastic paresis (n = 27), and mixed motor disorders with spastic and dyskinetic paresis (n = 17). Eight children were independently ambulant and 37 children were wheelchair users. Thirty-four children had learning disability with a developmental age of below 6 years. Six participants dropped out of the study; data on 39 children were analyzed. Results showed that anticholinergic agents effectively reduced salivary flow. Drooling diminished substantially and this was accompanied by a significant reduction in care needs, making daily care less demanding. The amount of reported damage to communication devices and computers decreased. In addition to the evaluation of primary variables, such as the salivary flow rate, investigation of impact of drooling on daily life provides useful information about the outcome of treatment for reduction in drooling.     

Randomized trial of botulinum toxin injections into the salivary glands to reduce drooling in children with neurological disorders

The primary aim of this randomized, controlled trial was to assess the effectiveness of botulinum toxin A (BoNT-A) injections into the submandibular and parotid glands on drooling in children with cerebral palsy (CP) and other neurological disorders. Secondary aims were to ascertain the duration of any such effect and the timing of maximal response. Of the 48 participants (27 males, 21 females; mean age 11y 4mo [SD 3y 3mo], range 6-18y), 31 had a diagnosis of CP and 15 had a primary intellectual disability; 27 children were non-ambulant. Twenty-four children randomized to the treatment group received 25 units of BoNT-A into each parotid and submandibular gland. Those randomized to the control group received no treatment. The degree and impact of drooling was assessed by carers using the Drooling Impact Scale questionnaire at baseline and at monthly intervals up to 6 months postinjection/baseline, and again at 1 year. Maximal response was at 1 month at which time there was a highly significant difference in the mean scores between the groups. This difference remained statistically significant at 6 months. Four children failed to respond to the injections, four had mediocre results, and 16 had good results. While the use of BoNT-A can help to manage drooling in many children with neurological disorders, further research is needed to fully understand the range of responses.     

Thickened saliva after effective management of drooling with botulinum toxin A

Aim The aim of this study was to evaluate the rheological properties of saliva after submandibular botulinum toxin type A (BoNT‐A) injections. Method We enrolled 15 children (11 males and six females; age range 3–17y, mean age 9y 10mo) diagnosed with spastic (n=9) or dyskinetic (n=6) quadriplegic cerebral palsy (CP); Gross Motor Function Classification System level IV or V; and two children with intellectual disability (IQ <70) who experienced moderate to severe drooling. Salivary flow rate and drooling quotient were measured at baseline and at different times after BoNT‐A injections up to 24 weeks. The mucin concentration of saliva was analysed before and after BoNT‐A treatment. Results Both submandibular salivary flow rate (baseline 0.38mL/min; 24wks after injection 0.26mL/min) and drooling quotient (baseline 42.5%; 24wks 28.80%) were substantially reduced, with a concomitant increase in mucin concentration within 8 weeks after BoNT‐A injection (from 0.612 to 1.830U/mL). The parents of nine children observed thickened saliva. Swallowing and chewing were problematic in seven children. Two of these children needed treatment with mucolytics because of pooling of thickened saliva in the throat. Interpretation When making decisions about the use of BoNT‐A, the risk of problems with masticatory and swallowing functions as a result of thickening of saliva after BoNT‐A treatment should be taken into account.     

Does Motor Performance Matter in Botulinum Toxin Efficacy for Drooling?

The aim of this study was to define factors that influence therapy outcome of submandibular botulinum toxin injections for drooling in children with cerebral palsy or mental disability. We postulated that differences in response may be explained by the variation of dysfunctions in the various cerebral palsy subtypes. Prospectively collected data were evaluated of 80 spastic and 48 dyskinetic children, of whom 70% had an IQ of <70. In addition, the data of 23 fully ambulant children with mental disability only were examined. Flow and Drooling Quotient were assessed at baseline and at 8 weeks after injection. After treatment, both the Drooling Quotient and submandibular flow decreased in all children. Morbidity associated with the procedure was limited. Ninety-three children responded to botulinum. Decrease of submandibular flow in these children was associated with reduction of parotid flow. In those who did not respond to therapy, spread across all 3 diagnostic classifications, parotid flow increased after injection. Response failure is characterized by increased parotid flow after injection; however, the precise role of parotid flow in therapy failure remains unclear. We cannot predict who will respond to botulinum toxin to treat drooling.     

安坦对氯丙嗪血药浓度的影响—─短期和长期服药病人的比较

以短期和长期服用氯丙嗪的精神分裂症病人为对象，比较合并安坦后两组氯丙嗪血药浓度变化的差异。根据以往连续服用氯丙嗪≤１年或≥５年，将样本分为短期服药组（１７例）和长期服药组（２１例），给予口服安坦４ｍｇ／日，分别于合并安坦前、合并后２周、４周测定氯丙嗪血药浓度。结果发现短期服药组氯丙嗪血药浓度有显著下降（Ｆ＝４．０７，Ｐ＜０．０５），长期服药组无显著下降（Ｐ＞０．０５）。在降低幅度＞２０％的病例中，短、长期服药组分别占４１．２％和９．５％（χ２＝５．６１，Ｐ＜０．０１）。相关分析结果，氯丙嗪血药浓度下降幅度与以往服药期呈显著负相关。可见安坦降低氯丙嗪血药浓度的作用仅在以往服药期较短或部分急性病人当中显得较为明显，而长期服药病人合并安坦后氯丙嗪血药浓度无显著变化     

Salivary gland botulinum toxin injections for drooling in children with cerebral palsy and neurodevelopmental disability: a systematic review

Aim The aim of this paper was to systematically review the efficacy and safety of botulinum toxin (BoNT) injections to the salivary glands to treat drooling in children with cerebral palsy and neurodevelopmental disability. Method A systematic search of The Cochrane Central Register of Controlled Trials, PubMed, CINAHL (Cumulative Index to Nursing and Allied Health Literature), EMBASE, and the Physiotherapy Evidence Database (PEDro) was conducted (up to 1 October 2011). Data sources included published randomized controlled trials (RCTs) and prospective studies. Results Sixteen studies met inclusion criteria. Three outcome measures support the effectiveness of BoNT for drooling. One RCT found an almost 30% reduction in the impact of drooling on patients’ lives, as measured by the Drooling Impact Scale (mean difference ?27.45; 95% confidence interval [CI] ?35.28 to ?19.62). There were sufficient data to pool results on one outcome measure, the Drooling Frequency and Severity Scale, which supports this result (mean difference ?2.71; 95% CI ?4.82 to ?0.60; p<0.001). There was a significant reduction in the observed number of bibs required per day. The incidence of adverse events ranged from 2 to 41%, but was inconsistently reported. One trial was terminated early because of adverse events. Interpretation BoNT is an effective, temporary treatment for sialorrhoea in children with cerebral palsy. Benefits need to be weighed against the potential for serious adverse events. More studies are needed to address the safety of BoNT and to compare BoNT with other treatment options for drooling.