Reoperation Rates According to Surgical Approach After Operation for Degenerative Cervical Pathology in Patients With Athetoid Cerebral Palsy: A Nationwide Cohort Study.

STUDY DESIGN: National population-based cohort study. OBJECTIVE: The overall complication rate for patients with athetoid cerebral palsy (CP) undergoing cervical surgery is significantly higher than that of patients without CP. The study was conducted to compare the reoperation and complication rates of anterior fusion, posterior fusion, combined fusion, and laminoplasty for degenerative cervical myelopathy/radiculopathy in patients with athetoid cerebral palsy. METHODS: The Korean Health Insurance Review and Assessment Service national database was used for analysis. Data from patients diagnosed with athetoid CP who underwent cervical spine operations for degenerative causes between 2002 and 2020 were reviewed. Patients were categorized into four groups for comparison: anterior fusion, posterior fusion, combined fusion, and laminoplasty. RESULTS: A total of 672 patients were included in the study. The overall revision rate was 21.0% (141/672). The revision rate was highest in the anterior fusion group (42.7%). The revision rates of combined fusion (11.1%; hazard ratio [HR], .335; P = .002), posterior fusion (13.8%; HR, .533; P = .030) were significantly lower than that of anterior fusion. Revision rate of laminoplasty (13.1%; HR, .541; P = .240) was also lower than anterior fusion although the result did not demonstrate statistical significance. CONCLUSION: Anterior fusion presented the highest reoperation risk after cervical spine surgery reaching 42.7% in patients with athetoid CP. Therefore, anterior-only fusion in patients with athetoid CP should be avoided or reserved for strictly selected patients. Combined fusion, with the lowest revision risk at 11.1%, could be safely applied to patients with athetoid CP.

Postoperative Complications of Surgery for Cervical Spondylotic Myelopathy with and Without Athetoid Cerebral Palsy.

STUDY DESIGN: Retrospective cohort study. OBJECTIVES: With the aging of the Japanese population, patients with athetoid cerebral palsy (ACP) are getting older, and the rate of surgery for CSM is increasing in ACP patients. However, postoperative complications of such surgery among adult patients with ACP have not been reported yet. We investigated postoperative complications of surgery for CSM with ACP and compared them with those of surgery for CSM without ACP using a national inpatient database of Japan. METHODS: Using the Diagnosis Procedure Combination database, we identified 61382 patients who underwent surgery for CSM from July 2010 to March 2018. We examined patient backgrounds, surgical procedures, and type of hospital, and a 4:1 propensity score matching was performed to compare the outcomes between the non-ACP and ACP groups. RESULTS: There were 60 847 patients without ACP and 535 patients with ACP. The mean age was 68.5 years in the non-ACP group and 55 years in the ACP group. The percentages of patients who underwent fusion surgery were 21.6% and 68.8% in the non-ACP and ACP groups, respectively. The 4:1 propensity score matching selected 1858 in the non-ACP group and 465 in the ACP group. The ACP group was more likely to have postoperative urinary tract infection (.4% vs 2.8%, P < .001), postoperative pneumonia (.4% vs 2.4%, P < .001), and 90-day readmission for reoperation (1.9% vs 4.3%, P = .003). CONCLUSIONS: We found that ACP patients were more vulnerable to postoperative complications and reoperation after CSM than non-ACP patients.

A Case of General Anaesthesia Using an I-gel Airway for MRI of an Adult Patient With Athetoid Cerebral Palsy.

Patients with athetoid cerebral palsy may develop cervical myelopathy owing to repetitive involuntary motion. In these patients, MRI evaluation is required; involuntary motion is problematic, and general anaesthesia and immobilisation may be necessary. However, MRI studies requiring muscle relaxation and general anesthesia in adults are rare. A 65-year-old man with a history of athetoid cerebral palsy required an MRI of the cervical spine under general anaesthesia. General anaesthesia was administered with 5 mg of midazolam and 50 mg of rocuronium in a room adjacent to the MRI room. The airway was secured using an i-gel airway, and the patient was ventilated using a Jackson-Rees circuit. As the only MRI-compatible monitoring method available at our institution was SpO2 monitoring, blood pressure was monitored by palpation of the dorsal pedal artery, and ventilation was monitored visually by an anaesthesiologist in the MRI room. The MRI was uneventful. After scanning, the patient awoke promptly and was returned to the ward. An MRI scan under general anaesthesia requires monitoring of the patient, securing of the airway and ventilation, and careful selection of suitable anaesthetic agents. Although MRI scans requiring general anaesthesia are rare, anaesthesiologists should be prepared for this eventuality.

A Novel Technique for Occipitocervical Fusion with Triple Rod Connection to Prevent Implant Failure.

Occipitocervical fusion is an effective surgical method for treating various upper cervical disorders. However, complications such as implant failure due to rod breakage have been reported. Therefore, we devised a surgical technique for occipitocervical fusion with a triple rod connection to prevent implant failure. Occipitocervical fusion with triple rod connection was performed in two cases with a high risk of instability such as athetoid cerebral palsy and rheumatoid arthritis. A multiaxial screw (diameter: 4.5 mm) was inserted into the screw hole in the middle of the occipital plate, and subsequently, an additional rod was attached. It was connected to the main rod using an offset connector at the caudal side. The connection of the additional rod was simple and did not interfere with the fusion bed for bone graft between the occipital bone and axis. The head of the screw was crimped to the occipital plate, and the plate was firmly fixed. Moreover, since the head of the screw did not protrude to the dorsal side, the tension of the soft tissue and skin did not increase. No complications occurred after surgery in both cases. In addition, no special instruments were required to connect the additional rod to the main rod in this procedure. Therefore, our technique may be useful as an option to prevent implant failure due to rod breakage at the craniocervical junction.

Combined Anterior-Posterior Fusion Versus Posterior Alone Fusion for Cervical Myelopathy in Athetoid-Cerebral Palsy.

STUDY DESIGN: Retrospective comparative study. OBJECTIVES: Although some studies have discussed the use of lateral mass screws (LMSs) in patients with cerebral palsy (CP), it is unclear whether posterior LMS fixation alone is a suitable method. We aimed to compare the clinical, radiological, and surgical outcomes of 2 surgical modalities, namely, combined anterior-posterior (A-P) instrumented fusion and posterior fusion alone, in athetoid-type CP patients with cervical myelopathy (CM). METHODS: We analyzed 63 patients with athetoid-CP and CM who underwent posterior fusion only with LMS (group A, 35 patients) and A-P fusion (group B, 28 patients). The primary outcome was the 1- and 3-year fusion rates for the surgical segments. The secondary outcomes included the clinical outcomes based on pain intensity determined using the visual analog scale score, neck disability index, and 17-point Japanese Orthopedic Association score, radiological, and surgical outcomes. RESULTS: Fusion was achieved at 3 years postoperatively in 22 of 35 patients (63%) in group A and in 26 of 28 patients (93%) in group B (P = 0.02). The posterior neck pain intensity was also significantly lower in group B than in group A 2 and 3 years postoperatively (P = 0.02 and 0.01, respectively). The incidence of screw loosening and implant-related problems was higher in group A (60%) than in group B (21%) (P = 0.01). The other clinical and radiological parameters were similar between the groups. CONCLUSIONS: For athetoid CP-induced CM, combined A-P fusion would result in superior clinical and radiological outcomes compared to posterior fusion alone.

Surgical outcomes of cervical myelopathy in patients with athetoid cerebral palsy.

PURPOSE: Surgical treatment for cervical myelopathy with athetoid cerebral palsy remains unestablished. Instrumented fusion is reported to have good clinical results; however, there are no data of decompression surgery for this pathology in recent years. This study aimed to assess the surgical outcomes of laminoplasty with or without posterior instrumented fusion for cervical myelopathy in patients with athetoid cerebral palsy. METHODS: A multi-centre surgical series of patients with cervical myelopathy and athetoid cerebral palsy were enrolled in this study. All patients showed symptoms and signs suggestive of cervical myelopathy and underwent laminoplasty with or without instrumented fusion. The Japanese Orthopaedic Association (JOA) score, Barthel index (BI), and changes in the C2-C7 sagittal Cobb angle in the lateral plain radiograph were analysed. RESULTS: There were 25 patients (16 men and 9 women; mean age, 54.4 ± 10.8 years) with cervical myelopathy and athetoid cerebral palsy who underwent surgical treatment. The mean follow-up period was 41.9 ± 35.6 months. Overall, the BI significantly improved after surgery, whereas the JOA score and C2-C7 angle did not improve postoperatively. The recovery rate of the JOA score in the laminoplasty group was significantly higher than that of the fusion group (P = 0.02). CONCLUSIONS: Cervical laminoplasty with or without instrumented fusion for treating cervical myelopathy due to athetoid cerebral palsy is effective in improving activities of daily living. Cervical laminoplasty may be an effective and less invasive surgical method for selective patients, especially for those with small involuntary movements and no remarkable cervical kyphosis nor instability.

Wrist Arthrodesis in Athetoid Type Cerebral Palsy: A Case Study of 2 Patients.

Wrist arthrodesis has been used successfully for the management of severe wrist flexion deformity when soft tissue procedures would not provide adequate correction. However, in athetoid type cerebral palsy which has a component of involuntary movement, the outcome of wrist arthrodesis has not been discussed much. We present our experience in 2 athetoid type cerebral palsy patients who underwent wrist arthrodesis due to severe involuntary movement of the wrist. One patient had a nonunion and both patients had unexpected aggravation of involuntary movement in the adjacent joints. Secure fixation using a pre-contoured plate is necessary and preparation for iliac bone grafting should be considered as proximal row carpectomy is usually not necessary in these patients. In addition, although single-event, multi-level surgery is advocated for patients with cerebral palsy, potential additional procedures for the adjacent joints should be discussed preoperatively because unexpected aggravation of involuntary movement of adjacent joints can occur after stabilization of the wrist.

What is the best treatment option for cervical spinal cord injury by os odontoideum in a patient with athetoid dystonic cerebral palsy?

Context: Atlantoaxial instability in an athetoid dystonic cerebral palsy patient due to os odentoideum represents a rare cause of high-level cervical spinal cord injury. There is no evidence-based treatment protocol for this injury and a number of reports have debated whether nonsurgical or surgical treatment is the best option.Findings: Here, we report the case of a 32-year-old athetoid dystonic cerebral palsy patient with os odontoideum (OO) causing compressive myelopathy on the C1-2 levels. About two weeks after atlantoaxial fusion, the patient started an intensive rehabilitation program and maintained it for four weeks. He showed neurological and functional improvement at discharge after four weeks of training. There were further improvements in function and symptoms four weeks after discharge.Conclusion: Intensive rehabilitation programs are important for the recovery and good prognosis in spinal cord injury patients. However, rehabilitation of CP patients with spinal cord injury is often neglected. We provided intensive rehabilitation therapy to a patient for a cervical spinal cord injury by OO with athetoid dystonic cerebral palsy and achieved neurological and functional recovery.

Development and clinical application of weight－adjustable suit for children with dyskinetic cerebral palsy / 中华实用儿科临床杂志

Objective To explore the development and clinical effect of weight_adjustable suit for children with dyskinetic cerebral palsy. Methods Twenty_six cerebral_palsy children with involuntary movement admitted to the Third Hospital Affiliated to Jiamusi University from March to October 2016 were randomly divided into the observa_tion group and the control group,13 cases in each group. The control group was treated with routine rehabilitation trai_ning. The observation group was put on adjustable heavy clothes besides conventional rehabilitation. Before and after treatment,childrenˊs functional independence and gross motor function were assessed and compared by using Wee Punc_tional Independence Measure(Wee_PIM)and Gross Motor Punction Rating Scale(GMPM_88). Results After treat_ment the Wee_PIM score of the control group was(43. 24 ± 5. 58),the GMPM score was(61. 81 ± 9. 46),the Wee_PIM score of the observation group was(50. 21 ± 6. 03),and the GMPM score was(65. 73 ± 10. 17). There were sig_nificant differences between the two groups(P〈0. 05),while the scores of the observation group were significantly higher than those of the control group,and the difference was significant(t﹦2. 582,2. 346,all P〈0. 05). Conclusions The adjustable heavy suit can effectively improve the functional independence and gross motor function of cerebral palsy children with involuntary movement and their comprehensive ability,and it is worth trying clinically.

A unique de novo gain-of-function variant in CAMK4 associated with intellectual disability and hyperkinetic movement disorder.

Calcium/calmodulin-dependent protein kinases (CaMKs) are key mediators of calcium signaling and underpin neuronal health. Although widely studied, the contribution of CaMKs to Mendelian disease is rather enigmatic. Here, we describe an unusual neurodevelopmental phenotype, characterized by milestone delay, intellectual disability, autism, ataxia, and mixed hyperkinetic movement disorder including severe generalized dystonia, in a proband who remained etiologically undiagnosed despite exhaustive testing. We performed trio whole-exome sequencing to identify a de novo essential splice-site variant (c.981+1G>A) in CAMK4, encoding CaMKIV. Through in silico evaluation and cDNA analyses, we demonstrated that c.981+1G>A alters CAMK4 pre-mRNA processing and results in a stable mRNA transcript containing a 77-nt out-of-frame deletion and a premature termination codon within the last exon. The expected protein, p.Lys303Serfs*28, exhibits selective loss of the carboxy-terminal regulatory domain of CaMKIV and bears striking structural resemblance to previously reported synthetic mutants that confer constitutive CaMKIV activity. Biochemical studies in proband-derived cells confirmed an activating effect of c.981+1G>A and indicated that variant-induced excessive CaMKIV signaling is sensitive to pharmacological manipulation. Additionally, we found that variants predicted to cause selective depletion of CaMKIV's regulatory domain are unobserved in diverse catalogs of human variation, thus revealing that c.981+1G>A is a unique molecular event. We propose that our proband's phenotype is explainable by a dominant CAMK4 splice-disrupting mutation that acts through a gain-of-function mechanism. Our findings highlight the importance of CAMK4 in human neurodevelopment, provide a foundation for future clinical research of CAMK4, and suggest the CaMKIV signaling pathway as a potential drug target in neurological disease.

Hemi-Chorea in a Patient with Ketotic Hyperglycemia: An Unusual Presentation.

Chorea has often been associated with lesions in the basal ganglia and in the sub thalamic nucleus. It is possible for a patient with chorea-ballismus to have hyperglycemia at the initial presentation. We hereby present a case of an 81-year-old female, who was on treatment for type 2 diabetes mellitus and presented to us with sub acute onset of abnormal movements of right side of the body. She had semi purposeful, rapid and jerky movements of right upper limb and lower limb along with abnormal tongue movements. Laboratory data showed very high blood glucose levels, urine ketones were positive and pH of arterial blood was normal. MRI brain showed hyperintensities in right basal ganglia. So, hyperglycemia induced hemichorea was considered as a possibility and she was treated with insulin. These abnormal movements decreased subsequently with treatment and patient is doing better in the follow-up visits. This presentation is extremely rare, as review of literature showed similar presentations in patients with non ketotic hyperglycemia but not reported so far in diabetic ketosis.

An Autopsy Case of Respiratory Failure Induced by Repetitive Cervical Spinal Cord Damage due to Abnormal Movement of the Neck in Athetoid Cerebral Palsy.

We herein report the clinical and autopsy findings of a 48-year-old right-handed man with athetoid cerebral palsy who suffered from cervical myelopathy due to abnormal neck movement, and who died of respiratory failure. Pathologically, the external appearance of the ventral surface of the cervical spinal cord revealed a linear indentation running obliquely at the level between the C4 and C5 segments. In the most severely compressed lesion, the gray matter was predominantly affected and severely atrophic. Microscopically, clusters of oligodendrocytes associated with thinly myelinated axons were also observed in the lateral funiculus. The latter findings are unique, and could be interpreted as regenerative and/or restorative phenomena of the central nervous system following chronic repetitive spinal cord compression.

Evaluation of the efficacy of cervical perivascular sympathectomy on drooling in children with athetoid cerebral palsy.

OBJECTIVE: To evaluate the efficacy of cervical perivascular sympathectomy (CPVS) for drooling in children with athetoid cerebral palsy (ACP). METHODS: The severity and frequency of drooling and the amount of salivation of 32 ACP children with drooling were evaluated before CPVS and at 4th, 12th and 24 weeks postoperatively by the teacher drooling scale (TDS) and salivary flow rate (SFR). RESULTS: Fifteen children exhibited improvements on drooling according to the TDS score at 4th week after surgery (P < 0.05). Later, the number of children decreased to 10 at 12th week (P < 0.05) and to 8 at 24 week after surgery (P < 0.05). SFR was 0.67 mg/min at baseline, which decreased to 0.58 mg/min (P < 0.05) at 4th week after surgery. However, SFR showed a gradual increase at 12th week and 24 week with no significant difference. CONCLUSIONS: Although CPVS was effective in improving drooling in some children with ACP, the results were not satisfactory. Thus, CPVS still needs to be cautiously used. Furthermore, more rigorous clinical studies should be performed to detect the effectiveness and safety of this procedure.

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.

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.

Chronic Pain and Psychosocial Functions in Adults with Athetoid Cerebral Palsy

OBJECTIVE: To evaluate the effect of chronic pain on psychosocial functions in adults with athetoid cerebral palsy. METHOD: Thirty-five adults with athetoid cerebral palsy were recruited as subjects, then the radiologic and electrodi agnostic studies were assessed. The characteristics of pain in severity and disability status with Von Korff's chronic pain grade classification system and psychosocial functional status using affect balance scale, Beck depression inventory, social adjustment scale and satisfaction with life scale were evaluated. RESULTS: Twenty-eight (80.0%) subjects had the pain lasting more than 3 months. The mean score of characteristic pain intensity was 65.5+/-11.5. Eleven subjects (31.4%) showed both abnormal radiologic and electrodiagnostic findings, and these subjects had higher disability score among the subjects who complaint of chronic pain (p<0.05). The pain intensity had significant relationship with negative affects such as depression and dissatisfaction with life style (p<0.05). In addition, social adjustment tended to be lower in subjects with severe chronic pain. CONCLUSION: Chronic pain was commonly observed in adults with athetoid cerebral palsy, and the pain adversely affected psychosocial functions. Therefore, proper management of chronic pain might be helpful for enhancing their psycho social functions in these subjects.

A Study on Acoustic Characteristics of Dysarthria in Athetoid Cerebral Palsy

OBJECTIVE: Athetoid cerebral palsy is a nonprogressive disorder, due to hypoxic injury or jaundice in basal ganglia, characterized by impairment of postural reflexes, arrhythmical involuntary movements, and dysarthria. Dysarthria is a group of motor speech disorder resulting from a disturbance of motor control of the speech mechanism. The purpose of this study is to evaluate the acoustic characteristics of dysarthria in athetoid cerebral palsy. METHOD: We investigated the acoustic characteristics of dysarthria in 8 male patients with athetoid cerebral palsy and 6 males as a control group using Computerized Speech Laboratory (i.e., Multi-Dimensional Voice Program (MDVP), Visi-Pitch, and Electroglottography) and Nasometer. RESULTS: In the MDVP analysis, average fundamental frequency, jitter, and shimmer are significantly increased in patient group as compared to control group (p<0.05). In the Visi-Pitch analysis, maximal phonation and diadochokinetic rate are significantly decreased in patient group (p<0.05). The athetoid cerebral palsied patients have many pitch breaks. Nasalance is not significantly decreased in patient group in comparision with control group. CONCLUSION: We think that average fundamental frequency, jitter and shimmer are increased and maximal phonation time and diadochokinetic rate are decreased in athetoid cerebral palsied patients.

Respiratory Patterns Associated with Swallowing in Athetoid Cerebral-Palsied Patients

OBJECTIVE: To evaluate the changes of the respiratory patterns associated with swallowing in athetoid cerebral palsied patients (CP), and to understand its relationship with their dysphagia and aspiration. METHOD: Seven adult athetoid CP and seven control subjects were selected for this study. Each subject performed swallowing tasks in upright sitting position, and submental electromyography and respirography by pneumobelt on mid-abdomen were recorded simultaneously. Swallowing tasks were composed of swallowing 5 cc, 75 cc of water, and chewing a cookie. And athetoid subjects performed videofluoroscopic swallowing study (VFSS) for each food texture. RESULTS: 1) In athetoid CP, respiratory patterns during swallowing were variable and more irregular than the resting tidal breathing. 2) In athetoid CP, completion time of the swallowing task and recovery time to the resting tidal breathing after swallow were significantly prolonged (p<0.01). 4) Athetoid subjects had significantly higher rate of postdeglutitive inspiration than that of the controls (p<0.01). 5) In correlation with the VFSS findings, aspirated group of the athetoid subjects had significantly higher rate of postdeglutitive inspiration than that of non-aspirated group (p<0.05). CONCLUSION: Respiratory-swallowing coordination was disrupted in the athtoid CP, and their higher rate of postdeglutitive inspiration suggested the higher risk for aspiration of the food materials, especially in drinking of large amounts of liquid.

Respiration Patterns and Abdominal Muscle Activities during Speech Production in Athetoid Cerebral-Palsied Patients

OBJECTIVE: To evaluate the relationship of the respiration patterns and the abdominal muscle activities during various speech productions in the adults with athetoid cerebral palsy (CP). METHOD: Subjects were 7 athetoid CPs and 7 healthy controls. Respirography and abdominal muscle activities were recorded by use of the pneumobelt and the surface EMG during their performances of six kinds of non-speech and speech tasks. RESULTS: The followings were observed in the athetoid CP. 1) Respiratory phase was irregular and variable in the intensity and period of revolution. The frequency of the respiratory phase was not different from the control in usual respiration, but higher in deep breathing. The time to persist one vowel phonation was shorter. 2) Inappropriate and frequent inspirations were occurred during speech task and the number of syllables was fewer and irregular for one speech breath. 3) The abdominal muscle activities were abrupt, irregular, inconsistent with the respiratory phase during all kinds of non-speech and speech tasks. CONCLUSION: These findings suggest that the abnormal respiration patterns and abdominal muscle activities contribute to the abnormal speech production of athetoid CP and their incoordination is similar to athetoid movement of the extremities. We should consider the athetoid speech production as well as the dysarthria in the athetoid CP.

Short period Clinical Observations of Cerebral Palsied Children with Athetosis Treated with Madopar / 中国康复理论与实践

@# In this paper,we studied the clinical effects,side effects,doses ,dosing interval,relations between doses and effects of using Madopar in 61 children with athetoid cerebral palsy.Taking ADL Evaluation Scheme for Cerebral Palsied Children as main efficacy value,very marked statistic difference exists when two groups with or without Madopar were assessed on the third evaluation in our study,and it shows the effects of Madopar while its adverse events are mild and transient.The average and maximal initial dose is 9mg/kg/d and 26mg/kg/d respectively,and the average titration interval is one week while the shortest inetrval is a day.It can be titrated to 20mg/kg/d on average and 38mg/kg/d on maximal dose.There was no correlation between doses and effects.Suggestion was made for further investigation.