[Treatment for self-injurious behavior in mentally handicapped populations--a report from our practice].

OBJECTIVE: Self-injurious behavior (SIB) is a common problem in individuals with intellectual disabilities, yet its clinical management remains to be established. We aimed to clarify the background factors and profiles of SIB in mentally and physically handicapped subjects and subsequently examined the efficacy of treatments attempted in our practice in order to achieve a better understanding of SIB and appro- priate interventions in these populations. METHODS: We surveyed 92 mentally handicapped subjects with SIB (including 25 females and 67 males, most of whom also had physical disabilities) as outpatients or inpatients of our institution using a retrospective questionnaire com- pleted by the corresponding doctors. RESULTS: Regarding psychobehavioral complications, impulsive mood swings (such as sudden rage) were more frequent than other behaviors. As to probable triggers of SIB, the patient's physical condition (17 cases) and noise or the temperature of the surrounding environment (38 cases) were identified. A total of 81 of 92 patients were given a prescription for SIB. Risperidone was the most commonly prescribed drug (75 cases), found to be effective in 38 (50.6%) cases, whereas phenothiazine antipsychotics were often more effective in patients with profound SIB. SSRIs (selective serotonin reuptake inhibitors) were applied in 20 cases, being effective in only five (25%) patients and discontinued in 11 (55%) patients due to the onset of several side effects, including overexcitement. As an additional agent for mood control, the antiepileptic topiramate was effective in 10 (76.9%) of 13 cases. Aside from medication, equipment for elbow extension was beneficial in some of the patients with profound SIB. CONCLUSIONS: Pharmacological intervention is sometimes beneficial, albeit only partly. Clinicians must therefore also consider triggers and factors in the surrounding environment for the development of SIB in mentally handicapped individuals.

A survey of subcutaneous blood flow in patients with SMID and subcutaneous ceftazidime administration using mentholated warm compresses in healthy subjects.

OBJECTIVES: To investigate subcutaneous blood flow rate (SBFR) in healthy volunteers and patients with severe motor and intellectual disabilities (SMID), and evaluate the effect of mentholated warm compresses (MWCs) on SBFR and subcutaneous ceftazidime absorption in healthy volunteers. METHODS: SBFR at the forearm, chest and abdomen were evaluated in Japanese healthy volunteers and in adults with SMID. The effects of MWCs on blood flow rate and ceftazidime pharmacokinetics were evaluated in healthy volunteers. RESULTS: SBFR was significantly lower in the forearms of female patients with SMID (n = 11) than in the forearms of healthy females (n = 6); it was not significantly lower in the abdomen or chest. There were no significant differences between male patients (n = 18) or controls (n = 12) in SBFR at any site. MWC application increased SBFR 1.3- to 2.0-fold compared with baseline in healthy controls (n = 6). MWC application increased ceftazidime maximum blood concentration, SBFR and time above mutant prevention concentration in a single healthy subject. CONCLUSIONS: Abdominal SBFR in patients with SMID did not differ from that of healthy subjects. MWC application increases SBFR and subcutaneous drug absorption rate in healthy humans.

Absorption Kinetics of Subcutaneously Administered Ceftazidime in Hypoperfused Guinea Pigs.

BACKGROUND: Pneumonia is the most common cause of death in patients with severe motor and intellectual disabilities (SMID), and intravenous ceftazidime (CAZ) is a widely used treatment for such infections. However, intravenous administration in patients with SMID may be difficult because of insufficient vascular development. OBJECTIVES: The aim of our study was to determine the feasibility of subcutaneous drug administration by mentholated warm compresses (WMCs) as an alternative delivery method for ceftazidime in patients with SMID. METHODS: CAZ was subcutaneously administered to the abdominal region of naphazoline-treated hypoperfused guinea pigs, which were used as a hemodynamic model of patients with SMID. MWCs or warm compresses (WCs) were applied to the injection site to increase blood flow. We calculated the cumulative CAZ absorption over time by using the deconvolution method. RESULTS: Application of MWCs or WCs increased blood flow at the administration site and increased CAZ plasma levels. Application of MWCs or WCs after subcutaneous CAZ injection led to higher CAZ plasma levels than the mutant prevention concentration for a longer period than was observed for CAZ administration without the application of MWCs or WCs. CONCLUSIONS: The application of MWCs or WCs enhanced subcutaneous CAZ absorption by increasing blood flow. MWCs and WCs are considered to be safe and routine methods to induce defecation after surgery on the digestive system; thus, the combination of these methods and subcutaneous CAZ administration is a potential method for treating pneumonia in patients with SMID.

[Care continuity for patients with cerebral palsy during transition from childhood to adulthood].

Around adolescence and thereafter, many cases with severe cerebral palsy have worsening of respiration and swallowing due to worsening of deformity and other factors. Appropriate management including prone positioning, naso-pharyngeal air-way, and positive pressure breathing with mask and bag or in-ex sufflator is effective for chronic respiratory disorder. Modification of posture or food texture according to the result of video-fluorographic examination may be effective for dysphagia along with intermittent oral catheterization feeding. Gastro-esophageal reflux and stasis in duodenum get worse around this age. Gastro-jejunal feeding with hand-made catheter set is useful. Cervical myelopathy and radiculopathy may be critical in athetoid type of cerebral palsy. Pseudoseizure as expression of conversion disorder should be considered especially around this age.

[An estimation chart for the possibility of aspiration in patients with severe motor and intellectual disabilities: its reliability and accuracy].

Although the most common cause of death in patients with severe motor and intellectual disabilities (SMID) is aspiration pneumonia, there are no criteria to detect aspiration. We have been making an evaluation tool to detect aspiration in patients with SMID easily, in cooperation with the project of comprehensive study of disability, social health, and welfare, supported by the Ministry of Health, Labor and Welfare, Japan. Here we studied the reliability and accuracy of the 2001 edition of the "questionnaires for functions of eating and swallowing" and "estimation chart for the possibility of aspiration" in 20 patients with risk of aspiration (13 with cerebral palsy, six with acquired cerebral palsy and one with muscle disease). Family members or nursing staffs taking care of them answered the questionnaires. A pediatrician and an occupational therapist or speech therapist checked the chart. Reliability was checked by the agreement between the estimation of the two professionals, and accuracy was determined by comparing the results of the chart and videofluolography. The results showed good reliability (kappa 0.63) and accuracy (kappa 0.47). Based on these results and additional analysis of the check items, the new edition of the chart was established.

[Effect of intrapulmonary percussive ventilation in a severely disabled patient with persistent pulmonary consolidation].

An intrapulmonary percussive ventilator (IPV) improves airway clearance and lung function, and is useful for wide variety of respiratory disorders, such as cystic fibrosis, chronic obstructive pulmonary disease, aspiration pneumonia, and neuromuscular diseases. However, there are few reports on IPV use in patients with severe neurological impairment, scoliosis and thoracic deformity. They have poor mobility of the rib cage and difficulty in sputum expectoration. The use of IPV significantly improved persistent consolidation shown by chest computed tomography (CT) in one of such patients. The patient was a 33-year-old woman with severe spastic quadriplegia and tracheostomy and she was dependent on mechanical ventilation because of chronic restrictive respiratory failure. After fever and mild hypoxemia for one day, chest CT revealed consolidation of the left lower lobe. An IPV-I ventilator was used for 15 min once a week, with a stroke frequency of 250-300 cycles/min and pressure of 22 PSI. Mechanical ventilation was withheld during the IPV therapy. Chest physiotherapy was also done. According to the worsening of the consolidation on chest CT, the frequency of IPV was changed to once a day at day 23 and then to twice a day. Chest CT at day 44 showed further improvement. In patients with severe motor and intellectual disabilities, it is sometimes difficult to control progressive deterioration of pulmonary function and persistent atelectasis even with tracheostomy, mechanical ventilation, and conventional physiotherapy. Our results indicate that IPV may improve respiratory functio and the quality of life in such patients.

A case of Mobius syndrome--radiological and electrophysiological findings.

Mobius syndrome is characterized by congenital bilateral facial palsy and abducens nerve paralysis, but reports of radiological and electrophysiological findings are scarce. A 4-year-old boy presented with mask-like facies noted at birth after a 34-week pregnancy. Examination revealed bilateral facial and abducens nerve paralysis with no other neurological abnormalities. Computed tomography revealed bilateral absence of facial nerve canal in the middle ear. Brain magnetic resonance imaging indicated a narrow than expected nerve in the internal auditory canal (IAC). Evoked electromyography and blink reflex testing to evaluate facial nerve function yielded no responses bilaterally. Facial palsy thus appears to be caused by facial nerve dysplasia or aplasia.

Sensorineural hearing loss in patients with cerebral palsy after asphyxia and hyperbilirubinemia.

The location of lesions causing hearing loss in patients with cerebral palsy due to asphyxia or neonatal hyperbilirubinemia has remained unclear. We performed behavioral audiometry, distortion product otoacoustic emission (DPOAE) and auditory brainstem evoked response (ABR) in six patients with cerebral palsy due to asphyxia or neonatal hyperbilirubinemia in order to determine the lesion location causing their hearing impairment. In all cases, behavioral audiometry revealed a threshold elevation of 50-75 dB and ABR were no response. DPOAE were totally absent in five patients and normal in one patient. Our study suggests that lesions causing hearing loss potentially include the organ of Corti especially at the outer hair cells and the cochlear nerve.

[Actions to improve daily medical care for children with severe neurological impairment in school and community life].

With an increasing number of children with severe neurological impairment living in their houses, there is growing demand for daily medical care such as tube feeding and sputum suctioning in schools and community life. On the basis of activities of many neuro-pediatricians in many districts. Social Activity and Public Relations Committee made a detailed statement about this problem, and appealed to the government and other related institutions, which promoted governmental activity on this problem. The system of neuro-pediatricians' participation to schools should be improved. Medical support in community life requires further discussion and proposal.

[Medical care and support in school and community life to very severe neurologically-impaired children--advance and problems in medical, educational and social management for improvement of QOL].

With an increasing number of children with severe neurological impairment living in their houses, there is growing demand for medical care and support in school and community life. In such cases, respiratory disorder, gastro-esophageal reflux and dysphagia are closely related. To improve these disorders, appropriate rehabilitation and daily managements, such as posture control, are important as well as medical and surgical treatment. Social and educational support is also necessary for improvement of the QOL of these children and their family. For example, daily medical care such as tube feeding and sputum suctioning should be provided by school staffs. Pediatric neurologists should actively participate in such educational and social activities.