Cerebral Palsy/complications* ; Child ; Deglutition Disorders/etiology* ; Humans

OBJECTIVE: This study estimated the effects of weekend and off-hour childbirth and the size of perinatal medical care center on the incidence of cerebral palsy. METHODS: The cases were all children with severe cerebral palsy born in Japan from 2009 to 2012 whose data were stored at the Japan Obstetric Compensation System for Cerebral Palsy database, a nationally representative database. The inclusion criteria were the following: neonates born between January 2009 and December 2012 who had a birth weight of at least 2000 g and gestational age of at least 33 weeks and who had severe disability resulting from cerebral palsy independent of congenital causes or factors during the neonatal period or thereafter. Study participants were restricted to singletons and controls without report of death, scheduled cesarean section, or ambulance transportation. The controls were newborns, randomly selected by year and type of delivery (normal spontaneous delivery without cesarean section and emergency cesarean section) using a 1:10 case to control ratio sampled from the nationwide Japan Society of Obstetrics and Gynecology database. RESULTS: A total of 90 cerebral palsy cases and 900 controls having normal spontaneous delivery without cesarean section were selected, as were 92 cerebral palsy cases and 920 controls with emergent cesarean section. A significantly higher risk for cerebral palsy was found among cases that underwent emergent cesarean section on weekends (odds ratio [OR] 1.72, 95% confidence interval [CI] 1.06-2.81) and during the night shift (OR 2.29, 95% CI 1.30-4.02). No significant risk was found among normal spontaneous deliveries on weekends (OR 1.63, 95% CI 0.97-2.73) or during the quasi-night shift (OR 1.26, 95% CI 0.70-2.27). Regional perinatal care centers showed significantly higher risk for cerebral palsy in both emergent cesarean section (OR 2.35, 95% CI 1.47-3.77) and normal spontaneous delivery (OR 2.92, 95% CI 1.76-4.84). CONCLUSION Labor on weekends, during the night shift, and at regional perinatal medical care centers was associated with significantly elevated risk for cerebral palsy in emergency cesarean section.
Case-Control Studies ; Cerebral Palsy ; epidemiology ; etiology ; Delivery, Obstetric ; statistics & numerical data ; Health Facilities ; statistics & numerical data ; Humans ; Incidence ; Infant, Newborn ; Japan ; epidemiology ; Parturition ; Perinatal Care ; statistics & numerical data ; Retrospective Studies ; Time Factors

In this study, we reported a case of progressive pseudorheumatoid dysplasia in Peking University Third Hospital. A 56-year-old male patient presented with hip joint pain for more than 40 years and multiple joints pain with limitation of movements of these joints for 28 years. This patient suffered from joint pain and impaired range of motion of the hip, knee, elbow and shoulder gradually, associated with difficulty in walking and inability to take care of himself. He was diagnosed with "femoral head necrosis" or "ankylosing spondylitis" in local hospitals, but the treatment of nonsteroidal antiinflammatory drugs (NSAIDs) and sulfasalazine was not effective. Up to the age of 14, the patient displayed normal physical development, with the highest height was about 158 cm, according to the patient recall. However, his height was 153 cm at present. There was no history of similar illness in any family member. Physical examinations descried limitation of movement of almost all joints. Enlargement and flexion deformity of the proximal interphalangeal (PIP) joints of the hands resulted in the claw hand appearance. Limited abduction and internal and external rotation of the shoulder and hip could be find. He had normal laboratory findings for blood routine test, biochemical indexes and acute phase reactants such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Furthermore, HLA-B27 and autoimmune antibodies such as rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibody and antinuclear antibody (ANA) were all negative. X-ray of the hip showed loss of the joint space and irregularities of the femoral head, both femoral head were flattened, it could be see hyperplasia, osteophytes, bilateral femoral neck thicken, neck dry angle turned smaller. The radiological findings of the spinal vertebra indicated kyphosis deformity, narrowing of the intervertebral discs, vertebral syndesmophytes and flattening of the vertebra. However, there was no clues of bone marrow edema in the lumbar MRI. At last, genetic testing for the Wnt1-inducible signaling pathway protein 3 (WISP3) gene was done and indicated compound heterozygous mutations: 756C>G and c.866dupA. These two mutations were derived from the patient's mother and father (the patient's parents each had a heterozygous mutation). Two exons of the WISP3 gene had nucleotide changes leading to amino acid mutations. According to the patient's history, symptoms, physical examinations, radiological findings and genetic testing, the final definitive diagnosis was progressive pseudorheumatic dysplasia.
Cerebral Palsy ; Heterozygote ; Hip/pathology* ; Humans ; Joint Diseases/etiology* ; Male ; Microcephaly ; Middle Aged ; Spondylitis, Ankylosing/diagnosis*

Cerebral palsy is a group of syndromes caused by non-progressive brain injury in the fetus or infant and can cause disabilities in childhood. Etiology of cerebral palsy has always been a hot topic for clinical scientists. More and more studies have shown that genetic factors are closely associated with the development of cerebral palsy. With the development and application of various molecular and biological techniques such as chromosome microarray analysis, genome-wide association study, and whole exome sequencing, new achievements have been made in the genetic research of cerebral palsy. Chromosome abnormalities, copy number variations, susceptibility genes, and single gene mutation associated with the development of cerebral palsy have been identified, which provides new opportunities for the research on the pathogenesis of cerebral palsy. This article reviews the advances in the genetic research on cerebral palsy in recent years.
Cerebral Palsy ; etiology ; genetics ; Chromosome Aberrations ; Cytokines ; genetics ; DNA Copy Number Variations ; Genetic Predisposition to Disease ; Genome-Wide Association Study ; Humans ; Mutation

PURPOSE: To investigate the effect of intramuscular Botulinum toxin type A (BoNT-A) injection on gait and dynamic foot pressure distribution in children with spastic cerebral palsy (CP) with dynamic equinovarus foot. MATERIALS AND METHODS: Twenty-five legs of 25 children with CP were investigated in this study. BoNT-A was injected into the gastrocnemius (GCM) and tibialis posterior (TP) muscles under the guidance of ultrasonography. The effects of the toxin were clinically assessed using the modified Ashworth scale (MAS) and modified Tardieu scale (MTS), and a computerized gait analysis and dynamic foot pressure measurements using the F-scan system were also performed before injection and at 1 and 4 months after injection. RESULTS: Spasticity of the ankle plantar-flexor in both the MAS and MTS was significantly reduced at both 1 and 4 months after injection. On dynamic foot pressure measurements, the center of pressure index and coronal index, which represent the asymmetrical weight-bearing of the medial and lateral columns of the foot, significantly improved at both 1 and 4 months after injection. The dynamic foot pressure index, total contact area, contact length and hind foot contact width all increased at 1 month after injection, suggesting better heel contact. Ankle kinematic data were significantly improved at both 1 and 4 months after injection, and ankle power generation was significantly increased at 4 months after injection compared to baseline data. CONCLUSION: Using a computerized gait analysis and foot scan, this study revealed significant benefits of BoNT-A injection into the GCM and TP muscles for dynamic equinovarus foot in children with spastic CP.
Adolescent ; Ankle Joint ; Botulinum Toxins, Type A/administration & dosage/*pharmacology ; Cerebral Palsy/*complications/drug therapy ; Child ; Child, Preschool ; Clubfoot/*drug therapy/*etiology/physiopathology ; Female ; Foot ; Gait/*drug effects/physiology ; Humans ; Injections, Intramuscular ; Male ; Muscle Spasticity/drug therapy ; Muscle, Skeletal/diagnostic imaging ; Neuromuscular Agents/administration & dosage/*pharmacology ; Pressure ; Prospective Studies ; Treatment Outcome ; Weight-Bearing

Acupuncture Points ; Acupuncture Therapy ; Aged ; Bulbar Palsy, Progressive ; etiology ; physiopathology ; therapy ; Cerebral Infarction ; complications ; Female ; Humans ; Male ; Middle Aged ; Pharynx ; physiopathology ; Treatment Outcome

PURPOSE: This study investigated the correlation between isometric muscle strength, gross motor function, and gait parameters in patients with spastic cerebral palsy and to find which muscle groups play an important role for gait pattern in a flexed knee gait. MATERIALS AND METHODS: Twenty-four ambulatory patients (mean age, 10.0 years) with spastic cerebral palsy who were scheduled for single event multilevel surgery, including distal hamstring lengthening, were included. Preoperatively, peak isometric muscle strength was measured for the hip flexor, hip extensor, knee flexor, and knee extensor muscle groups using a handheld dynamometer, and three-dimensional (3D) gait analysis and gross motor function measure (GMFM) scoring were also performed. Correlations between peak isometric strength and GMFM, gait kinematics, and gait kinetics were analyzed. RESULTS: Peak isometric muscle strength of all muscle groups was not related to the GMFM score and the gross motor function classification system level. Peak isometric strength of the hip extensor and knee extensor was significantly correlated with the mean pelvic tilt (r=-0.588, p=0.003 and r=-0.436, p=0.033) and maximum pelvic obliquity (r=-0.450, p=0.031 and r=-0.419, p=0.041). There were significant correlations between peak isometric strength of the knee extensor and peak knee extensor moment in early stance (r=0.467, p=0.021) and in terminal stance (r=0.416, p=0.043). CONCLUSION: There is no correlation between muscle strength and gross motor function. However, this study showed that muscle strength, especially of the extensor muscle group of the hip and knee joints, might play a critical role in gait by stabilizing pelvic motion and decreasing energy consumption in a flexed knee gait.
Biomechanical Phenomena ; Cerebral Palsy/*physiopathology/surgery ; Female ; Gait/*physiology ; Humans ; Isometric Contraction/physiology ; Knee/physiopathology ; Knee Joint/surgery ; Male ; Muscle Spasticity/etiology/physiopathology ; Muscle Strength/*physiology ; Muscle Strength Dynamometer ; Muscle, Skeletal/*physiopathology ; Pelvis ; Postural Balance/physiology ; Tenotomy

BACKGROUND: Adult patients with cerebral palsy (CP), who have advanced degenerative arthritis of the hip, have been treated with resection arthroplasty and arthrodesis. Although total hip arthroplasty (THA) has also been used as one of the alternative options, there are few studies on contemporary bearings used in THA. Therefore, we evaluated the results of the contemporary ceramic-on-ceramic THA in adult patients with CP. METHODS: From January 2005 to December 2007, five adult CP patients (5 hips) underwent THA using contemporary ceramic-on-ceramic bearings. All patients were able to stand or ambulate with intermittent use of assistive devices at home. We retrospectively reviewed the series to determine the results of THA in terms of pain relief, improved function, and durability of prosthesis. RESULTS: There were 3 men and 2 women with a mean age of 35.9 years. All patients had pain relief without decline in mobility postoperatively. One hip was dislocated, which was treated successfully with closed reduction and an abduction brace for 2 months. There was no ceramic fracture, loosening, or osteolysis during the mean follow-up of 6.8 years (range, 5.8 to 8.3 years). CONCLUSIONS: Cementless THA using contemporary ceramic-on-ceramic bearings is a useful option for the treatment of advanced degenerative arthritis of the hip in ambulatory adults with CP.
Adult ; Arthroplasty, Replacement, Hip/*methods ; Biocompatible Materials ; Ceramics ; Cerebral Palsy/*complications ; Female ; Hip Joint/*surgery ; Hip Prosthesis ; Humans ; Male ; Osteoarthritis, Hip/etiology/radiography/*surgery ; Prosthesis Design ; Retrospective Studies ; Treatment Outcome

Because complications are more common in patients with cerebral palsy (CP), surgeons and anesthesiologists must be aware of perioperative morbidity and be prepared to recognize and treat perioperative complications. This study aimed to determine the incidence of and risk factors for perioperative complications of orthopedic surgery on the lower extremities in patients with CP. We reviewed the medical records of consecutive CP patients undergoing orthopedic surgery. Medical history, anesthesia emergence time, intraoperative body temperature, heart rate, blood pressure, immediate postoperative complications, Gross Motor Function Classification System (GMFCS) level, Cormack-Lehane classification, and American Society of Anesthesiologists physical status classification were analyzed. A total of 868 patients was included. Mean age at first surgery was 11.8 (7.6) yr. The incidences of intraoperative hypothermia, absolute hypotension, and absolute bradycardia were 26.2%, 4.4%, and 20.0%, respectively. Twenty (2.3%) patients had major complications, and 35 (4.0%) patients had minor complications postoperatively. The incidences of intraoperative hypothermia, absolute hypotension, and major postoperative complications were significantly higher in patients at GMFCS levels IV and V compared with patients at GMFCS levels I to III (P<0.001). History of pneumonia was associated with intraoperative absolute hypotension and major postoperative complications (P<0.001). These results revealed that GMFCS level, patient age, hip reconstructive surgery, and history of pneumonia are associated with adverse effects on intraoperative body temperature, the cardiovascular system, and immediate postoperative complications.
Adolescent ; Adult ; Cerebral Palsy/*complications ; Child ; Child, Preschool ; Female ; Humans ; Intraoperative Complications/*etiology ; Lower Extremity/*surgery ; Male ; Middle Aged ; Orthopedic Procedures/*adverse effects ; Postoperative Complications/*etiology

PURPOSE: The purpose of our study was to investigate the effect of gait training with rhythmic auditory stimulation (RAS) on both kinematic and temporospatial gait patterns in patients with hemiplegia. MATERIALS AND METHODS: Eighteen hemiplegic patients diagnosed with either cerebral palsy or stroke participated in this study. All participants underwent the 4-week gait training with RAS. The treatment was performed for 30 minutes per each session, three sessions per week. RAS was provided with rhythmic beats using a chord progression on a keyboard. Kinematic and temporospatial data were collected and analyzed using a three-dimensional motion analysis system. RESULTS: Gait training with RAS significantly improved both proximal and distal joint kinematic patterns in hip adduction, knee flexion, and ankle plantar flexion, enhancing the gait deviation index (GDI) as well as ameliorating temporal asymmetry of the stance and swing phases in patients with hemiplegia. Stroke patients with previous walking experience demonstrated significant kinematic improvement in knee flexion in mid-swing and ankle dorsiflexion in terminal stance. Among stroke patients, subacute patients showed a significantly increased GDI score compared with chronic patients. In addition, household ambulators showed a significant effect on reducing anterior tilt of the pelvis with an enhanced GDI score, while community ambulators significantly increased knee flexion in mid-swing phase and ankle dorsiflexion in terminal stance phase. CONCLUSION: Gait training with RAS has beneficial effects on both kinematic and temporospatial patterns in patients with hemiplegia, providing not only clinical implications of locomotor rehabilitation with goal-oriented external feedback using RAS but also differential effects according to ambulatory function.
Acoustic Stimulation/*methods ; Aged ; Ankle Joint/physiopathology ; Biomechanical Phenomena ; Cerebral Palsy/*diagnosis/physiopathology ; Female ; Foot Joints/physiopathology ; *Gait ; Gait Disorders, Neurologic/etiology/physiopathology/*rehabilitation ; Hemiplegia ; Humans ; Knee/physiopathology ; Knee Joint/physiopathology ; Male ; Middle Aged ; Stroke/*diagnosis/physiopathology