Athetoid Cerebral Palsy Research Articles

Cervical Myelopathy in Patients With Athetoid Cerebral Palsy

Retrospective clinical study. To report the surgical outcomes of patients with cervical myelopathy associated with athetoid cerebral palsy and to assess whether a halo vest is necessary for postoperative external immobilization. Although a halo vest has remained the first choice for postoperative external immobilization of patients with cervical myelopathy associated with cerebral palsy, simplification of this method has been attempted in recent years. Studies focusing on postoperative external immobilization are rare. Since 2001, 20 patients underwent surgery with posterior instrumented fusion or posterior fixation and anterior decompression with fusion with a year or longer follow-up. Before 2004, all patients were given a halo vest for postoperative external immobilization. After 2004, halo vests were not used, and when abnormal involuntary neck movements were severe, an intramuscular injection of botulinum toxin was administered before and after surgery. Surgical outcomes, surgical methods and complications were compared between the group that used a halo vest and the group that did not use a halo vest. In the halo vest group, the average Japanese Orthopedic Association score was 6.9 points before surgery and 9.3 points at 1-year follow-up. The average recovery rate was 25.0%. In the group without halo vest use, the average Japanese Orthopedic Association score was 5.8 points before surgery and 9.9 points at 1-year follow-up. The average recovery rate was 35.7%. The group without halo vest use achieved outcomes equal to those achieved in the group with halo vest use. The frequency of complications was less without halo vest use than with halo vest use. No inferiority in clinical outcomes was seen if postoperative halo vest use was omitted. Progress in surgical instrumentation and injection of botulinum toxin may explain this result.

Microendoscopic laminotomy for the cervical myelopathy with athetoid cerebral palsy. Case report

Microendoscopic laminotomy for the cervical myelopathy with athetoid cerebral palsy. Case report

Anterior cruciate ligament reconstruction in a patient with Athetoid cerebral palsy: a case report

Recent years have seen ACL reconstruction performed in a broad range of patients, regardless of age, sex or occupation, thanks to great advances in surgical techniques, instrumentation and the basic research. Favorable results have been reported; however, we have not been able to locate any reports describing ACL reconstruction in patients with athetoid cerebral palsy.We present herein a previously unreported anterior cruciate ligament (ACL) reconstruction performed in a patient with athetoid cerebral palsy. The patient was a 25-year-old woman with level II athetoid cerebral palsy according to the Gross Motor Function Classification System. She initially injured her right knee after falling off a bicycle. Two years later, she again experienced right-knee pain and a feeling of instability. A right-knee ACL tear and avulsion fracture was diagnosed upon physical examination and confirmed with magnetic resonance imaging (MRI) and X-ray examination at that time. An ACL reconstruction using an autologous hamstring double-bundle graft was performed for recurrent instability nine years after the initial injury. Cast immobilization was provided for 3 weeks following surgery and knee extension was restricted for 3 months with the functional ACL brace to prevent hyperextension due to involuntary movement. Partial weight-bearing was started 1 week postoperatively, with full weight-bearing after 4 weeks. The anterior drawer stress radiography showed a 63% anterior displacement of the involved tibia on the femur six months following the surgery, while the contralateral knee demonstrated a 60% anterior displacement of the tibia. The functional ACL functional brace was then removed. A second-look arthroscopy was performed 13 months after the ACL reconstruction, and both the anteromedial and posterolateral bundles were in excellent position as per Kondo’s criteria. The Lachman and pivot shift test performed under anesthesia were also negative. An anterior drawer stress radiography of the involved knee at 36 months following surgery showed a 61% anterior translation of the tibia. The preoperative symptoms of instability resolved and the patient expressed a high degree of satisfaction with the result of her surgery.

Neonatal Transcutaneous Bilirubin Measurements: An Opportunity to Enhance Laboratory Utilization and Improve Patient Care

By far the most common clinical problem addressed by family practitioners and pediatricians in the first weeks of an infant's life is hyperbilirubinemia. With the exception of the standard metabolic screen, the measurement of a total serum bilirubin (TSB)2 concentration is the most common laboratory test ordered for a newborn infant. That is not surprising because the clinical sign, jaundice, is seen in approximately 80% of infants in a well-baby nursery during the first few days (1). The process of observation, clinical monitoring, and laboratory testing that continues throughout the infant's first week of life has a single objective—to prevent extreme hyperbilirubinemia [a TSB concentration >25–30 mg/dL (>428–513 μmol/L)] and its rare but dreaded consequence, bilirubin encephalopathy. Data from most of the Western world suggest that chronic bilirubin encephalopathy, or kernicterus, is currently occurring at an incidence of approximately 0.5–2 per 100 000 live births (2). Thus, of approximately 4 × 106 infants born annually in the US, perhaps 20–40 will develop kernicterus, a permanently disabling condition characterized by athetoid cerebral palsy, severe sensorineural hearing loss, paralysis of upward gaze, and dental dysplasia. For decades practitioners have relied on the appearance and intensity of jaundice as a means of deciding when to obtain a TSB measurement, but recognizing that estimating TSB by eye is difficult and on occasion grossly misleading (3) has prompted the development of transcutaneous bilirubinometry. The instruments that measure the transcutaneous bilirubin (TcB) concentration operate by transmitting light that penetrates the blanched skin and transilluminates the subcutaneous tissues. The …

Identifying Loss of Function Caused by Cervical Spondylotic Myelopathy in Young Adults With Nonathetoid Spastic Cerebral Palsy

Cervical spondylotic myelopathy (CSM) is the most common cause of nontraumatic spastic paraparesis and quadriparesis [1]. Symptom onset usually occurs after the age of 40 years 2]; however, conditions such as cerebral palsy (CP) may increase the risk for the occurrence f spondylotic or stenotic disease at an earlier age [2]. CP is a static, nondegenerative ondition, yet functional deterioration upon aging frequently is ignored or attributed to CP ithout further exploration [3]. Cervical stenosis is 1 of 4 common musculoskeletal onditions frequently found to contribute to functional decline in adults with CP [3]. The ther common conditions that may contribute to decline include patella alta, degenerative rthritis caused by hip dysplasia, and lumbar spondylosis [3]. However, diagnosis often is elayed, highlighting the difficulties in transition from pediatric to adult care for patients ith CP. The literature includes several reports of patients with athetoid CP experiencing unctional decline as a result of CSM [4-9]. Reports of CSM in patients with nonathetoid, pastic CP are much rarer [10]. This report describes 2 cases of CSM in patients with onathetoid, spastic, diplegic CP that resulted in a loss of function that was not entirely eversible with surgery.

Multi-disciplinary therapeutic intervention programmes for athetoid cerebral palsy child in clinical settings: a case report

Cerebral palsy (CP) is a disorder of movement and posture with additional potential to affect cognitive status. Thus, the goals for management of the child with CP include the following: to promote optimal function; to maintain general health; to foster acquisition of new skills; to assist and educate parents and caregivers, and to anticipate, prevent, and treat the complications of this disorder. Single case study, combined with efforts to develop measures specifically for child with athetoid cerebral palsy may make more valuable contributions to the scientific justification of therapeutic interventions in clinical settings. This case report intends to address the importance of multi-disciplinary therapeutic intervention programmes for children with athetoid cerebral palsy. Settings: CRC for persons with Disabilities, Sundernagar, Himachal Pradesh, India.

Intraoperative Vertebral Artery Angiography to Guide C1-2 Transarticular Screw Fixation in a Patient with Athetoid Cerebral Palsy

We present a case of an athetoid cerebral palsy with quadriparesis caused by kyphotic deformity of the cervical spine, severe spinal stenosis at the cervicomedullary junction, and atlantoaxial instability. The patient improved after the first surgery, which included a C1 total laminectomy and C-arm guided righ side unilateral C1-2 transarticular screw fixation. C1-2 fixation was not performed on the other side because of an aberrant and dominant vertebral artery (VA). Eight months after the first operation, the patient required revision surgery for persistent neck pain and screw malposition. We used intraoperative VA angiography with simultaneous fluoroscopy for precise image guidance during bilateral C1-2 transarticular screw fixation. Intraoperative VA angiography allowed the accurate insertion of screws, and can therefore be used to avoid VA injury during C1-2 transarticular screw fixation in comorbid patients with atlantoaxial deformities.

Tuning Algorithms for Control Interfaces for Users with Upper-Limb Impairments

Approximately 40% of Americans with disabilities cannot operate wheeled mobility devices and computers adequately because of diminished upper-limb motor control, sensory limitations, and cognitive impairments. We developed tuning software that can customize control interfaces for individuals with upper-limb impairments. This study compared the differences in each parameter among different diagnostic groups. The age of the subjects ranged from 18 to 80 yrs. The participants were classified into the following groups: athetoid cerebral palsy, spastic cerebral palsy, multiple sclerosis, upper-limb spasticity, and control. We used a validated tuning software protocol to customize an isometric joystick before a virtual tracing or driving task. Tuning parameters were then compared across groups. Seventy-five subjects were included. Gain, the parameter responsible for force-to-output ratios, in each directional axis (leftward gain: P = 0.018; rightward gain: P = 0.003; reverse gain: P = 0.007; forward gain: P = 0.014) was significantly different across the diagnostic groups. Post hoc analyses showed that the control group required smaller leftward gain than spastic cerebral palsy, multiple sclerosis and upper-limb spasticity groups and smaller gain in all other directions compared with spastic cerebral palsy. Gain may be a useful parameter in tuning by clinicians, and efforts aimed at gain customization may aid the development of commercially available tuning software packages.

Proton Magnetic Resonance Spectroscopic Images in Preterm Infants with Bilirubin Encephalopathy

Two preterm infants with athetoid cerebral palsy due to bilirubin encephalopathy were examined by magnetic resonance spectroscopic imaging at age 3 years. An increased glutamate/glutamine complex/creatine ratio was found in the basal ganglia. Chemical metabolic abnormalities of the basal ganglia were clearly demonstrated by color-coded metabolite images.

Mechanical implant failure in posterior cervical spine fusion

The aim of this study was to determine whether the recent refinement and downsizing of the implants for posterior cervical fusion increase the occurrence of implant failure. One hundred forty-two consecutive cases of cervical fusion, using either cannulated Magerl screws or a multiaxial pedicle screw-rod system, were reviewed retrospectively after an average follow-up period of more than 3 years, and the rate and characteristics of the failure of these implants were evaluated. Implant failure occurred in six (4.2%) patients: five with rheumatoid arthritis and one with athetoid cerebral palsy. Occipital plate fracture occurred in two patients, Magerl screw breakage in one patient, cervical pedicle screw fracture in two patients, and disassembly of the pedicle screw and rod in two patients (one with an occipital plate fracture). There was no rod fracture. The implant failures were asymptomatic, except in one patient. Disassembly of the pedicle screw and rod was observed immediately after another surgical procedure under general anesthesia in two patients. The failure rate of 4.2% was similar to the rates reported in the literature for posterior lumbar spinal fusion, confirming the reliability of the recent cervical screw-rod system.

アテトーゼ型脳性麻痺の頚椎症

アテトーゼ型脳性麻痺の頚椎症

Drawing Assist System for Reducing Effects of Involuntary Movements

Creative activities, such as painting and playing instruments, are sources of satisfaction and fulfillment for people with disabilities. However, some individuals with disabilities cannot satisfactorily enjoy such activities because of involuntary movements. In this study, we developed a drawing assist system for users with athetoid cerebral palsy and muscle tension, who experience involuntary movement. To date, assist systems have had difficulty in distinguishing involuntary movement from voluntary movement, and thus we designed a position correction filter to attenuate involuntary movement on the basis of the characteristics of synthesized velocity with respect to involuntary movement. Our system enabled drawing based on the user's own senses and motor control, even when involuntary movement occurred.

Surgical Outcome of Cervical Spondylotic Myelopathy with Athetoid Cerebral Palsy

Repetitive exaggerated neck movements in ACP may result in early degeneration of cervical spine requires operative managements. Moreover, these abnormal involuntary movements give rise to earlier instrument failure requires additional operative treatment and increase the incidence of recurrence of myelopathy. Understanding the outcome of operative management can aid in this decision-making process for the treatment of CSM in ACP.

THE INFLUENCE OF SHORT TERM, INTENSIVE HIPPOTHERAPY ON GAIT IN CHILDREN WITH CEREBRAL PALSY

The purpose of this study was to investigate the effects of short term hippotherapy on functional gait changes in children with cerebral palsy (CP). Participants were two boys (ages 12 and 13) with spastic diplegia and a girl (14 yrs) with athetoid CP. Single-subject (ABA) design was used to determine quantitative changes in functional gait parameters (velocity, stride length, and cadence) following three weeks of hippotherapy. The two boys demonstrated improvement in stride length and gait velocity without sustained improvement in cadence. There was increase in stride length and cadence, but most noticeable improvements in velocity for the girl. The results indicated that it is possible that short-term hippotherapy may improve functional gait in children with cerebral palsy.

Neural Mechanism and Clinical Significance of the Plantar Grasp Reflex in Infants

The plantar grasp reflex can be elicited in all normal infants from 25 weeks of postconceptional age until the end of 6 months of corrected age according to the expected birth date. This reflex in human infants can be regarded as a rudiment of responses that were once essential for ape infants in arboreal life. The spinal center for this reflex is probably located at the L(5)-S(2) levels, which, however, are controlled by higher brain structures. Nonprimary motor areas may exert regulatory control of the spinal reflex mechanism through interneurons. In infants, this reflex can be elicited as the result of insufficient control of the spinal mechanism by the immature brain. In adults, lesions in nonprimary motor areas may cause a release of inhibitory control by spinal interneurons, leading to a reappearance of the reflex. The plantar grasp reflex in infants is of high clinical significance. A negative or diminished reflex during early infancy is often a sensitive indicator of spasticity. Infants with athetoid type cerebral palsy exhibit an extremely strong retention of the reflex, and infants with mental retardation also exhibit a tendency toward prolonged retention of the reflex.

Perforation Rates of Cervical Pedicle Screw Insertion by Disease and Vertebral Level

Background:Different perforation rates for cervical pedicle screws by disease are expected in relation to bone quality and pedicle morphology; however, no report comparing pedicle screw perforation rate by disease had previously been published. This study investigated the perforation rates of pedicle screws inserted to cervical pedicle by disease and vertebral level using a CT-based navigation system.Materials/Methods:Fifty-three patients who underwent cervical pedicle screw insertion using CT based navigation system were studied. Diseases included rheumatoid arthritis (RA) (24 cases), destructive spondyloarthropathy (DSA) (10), cervical spondylotic myelopathy (CSM) (9), spine tumor (6), and cervical spondylotic myelopathy associated with athetoid cerebral palsy (CP) (4). Screw perforation rates for cervical pedicle screws were studied. Major perforation was defined as perforation 50% of screw diameter or more.Results:Major perforation rate by disease from C3 to C7 was as follows: spine tumor (0/24, 0%), RA (2/59, 3.4%), DSA (3/65, 4.6%), CP (2/20, 10.0%), and CSM (6/40, 15.0%). There were no clinically important complications such as vertebra arterial injury, spinal cord injury, or nerve root injury caused by any screw perforation. Major perforation rate by vertebral level was: C2(2/30, 6.7%), C3(4/49, 8.2%), C4(6/43, 14.0%), C5(1/32, 3.1%), C6(1/41, 2.4%), and C7(1/45, 2.2%), showing highest rate for C4, followed by C3.Conclusions:Cervical pedicle screw perforation rate by disease was higher in CSM compared to RA and DSA. The perforation rate by vertebral level was higher for C4 and C3, in this order.

Cervical myelopathy in athetoid and dystonic cerebral palsy: retrospective study and literature review

The early onset of degenerative cervical lesions has been well described in patients suffering from athetoid or dystonic cerebral palsy. Myelopathy can occur and aggravate of their unstable neurological status. Diagnosis and treatment are delayed and disrupted by the abnormal movements. This retrospective study was implemented to evaluate the symptoms, the anatomical findings, and the surgical management of seven patients from 20 to 56 years old suffering from cervical myelopathy and athetoid or dystonic cerebral palsy. The mean delay in diagnosis was 15 months and the mean follow-up was 33 months. The initial symptoms were spasticity, limbs weakness, paresthesias and vesico-sphinteric dysfunction. In addition to abnormal movements, imaging demonstrated disc herniation, spinal stenosis and instability. All patients were managed surgically by performing simultaneous spinal cord decompression and fusion. Two patients benefited from preoperative botulinum toxin injections, which facilitated postoperative care and immobilization. Strict postoperative immobilization was achieved for 3 months by a Philadelphia collar or a cervico-thoracic orthosis. All patients improved functionally with a mean Japanese Orthopaedic Association score gain of 1.5 points, in spite of the permanent disabilities of the myelopathy. Complications occurred with wound infection, metal failure and relapse of cervical myelopathy at an adjacent level in one case each. All the previous authors advised against isolated laminectomy but no consensus emerged from the literature analysis. Spinal fusion is usually recommended but can be complicated by degenerative adjacent deterioration. Surgical management provides good outcomes but requires a long-term follow-up.

Utility of modified transarticular screw in the middle and lower cervical spine as intermediate fixation in posterior long fusion surgery

The use of a pedicle screw (PS) in the cervical spine ensures strong fixation. However, 6.7-29% of such screws appear to be malpositioned using manual insertion techniques, especially at C-3 to C-6 where the pedicle diameter is smaller, potentially causing catastrophic complications such as vertebral artery (VA) and spinal cord or nerve root injuries. To optimize safety, the authors use a new technique: cephalad and/or caudad ends at C-2 and C-7/T-1, respectively, are fixed with PSs, and intermediate points around C3-6 are fixed using a modified transarticular screw technique that captures 3 dorsal cortices and preserves the ventral cortex of the facet in posterior long fusion surgery involving occipitospinal fixation. The purpose of the present study was to demonstrate this technique and evaluate the clinical and radiological outcomes. Thirty-nine patients, 8 men and 31 women, with a mean age of 61.7 +/- 11.0 years at surgery, were included in the study. Twenty-eight occipitospinal fusions and 11 posterior long fusions were performed. Patients were divided into 2 groups: a rheumatoid arthritis (RA) group consisting of 26 patients and a non-RA group of 13 patients including 7 with athetoid cerebral palsy. Clinical outcomes were evaluated according to the Japanese Orthopaedic Association (JOA) score. For radiological evaluation, the Cobb angle on lateral radiographs was measured preoperatively, postoperatively, and at the final follow-up, and the degree of realignment from pre- to postoperation and the loss of correction from postoperation to the follow-up were compared between the 2 patient groups. The recovery rate of the JOA score was 50.6 +/- 20.7% in the RA group and 37.3 +/- 24.3% in the non-RA group. Neither VA injury nor spinal cord or nerve root injury occurred among this series. The degree of realignment was greater in the non-RA group (9.2 +/- 13.9 degrees ) than the RA group (1.4 +/- 12.7 degrees ) as the Cobb angle was more kyphotic preoperatively in the non-RA group (2.9 +/- 18.6 degrees ) than in the RA group (17.4 +/- 15.7 degrees ). However, 38.5% of patients in the non-RA group had a correction loss > 10% compared with 7.7% in the RA group; this difference was statistically significant. The featured transarticular screw technique, which preserves the ventral cortex of the facet, as intermediate fixation in long fusion is a safe and easy procedure with few complications. It ensures acceptable clinical and radiological outcomes, especially in patients with RA.

Poster 84: Isolated Anterior Division Axillary Nerve Entrapment at the Quadrilateral Space in a Patient with Athetoid Cerebral Palsy: A Case Report

Poster 84: Isolated Anterior Division Axillary Nerve Entrapment at the Quadrilateral Space in a Patient with Athetoid Cerebral Palsy: A Case Report

Integration of Metamodel and Acoustic Model for Dysarthric Speech Recognition

We investigated the speech recognition of a person with articulation disorders resulting from athetoid cerebral palsy. The articulation of the first words spoken tends to be unstable due to the strain placed on the speech-related muscles, and this causes degradation of speech recognition. Therefore, we proposed a robust feature extraction method based on PCA (Principal Component Analysis) instead of MFCC, where the main stable utterance element is projected onto low-order features and fluctuation elements of speech style are projected onto high-order features. Therefore, the PCA-based filter will be able to extract stable utterance features only. The fluctuation of speaking style may invoke phone fluctuations, such as substitutions, deletions and insertions. In this paper, we discuss our effort to integrate a Metamodel and an Acoustic model approach. Metamodels have a technique for incorporating a model of a speaker’s confusion matrix into the ASR process in such a way as to increase recognition accuracy. The integration of metamodels and acoustic models enables fluctuation suppression not only in feature extraction but also in recognition. The proposed method resulted in an improvement of 9.9% (from 79.1% to 89%) in the recognition rate compared to the conventional method.