Spectrum of Movement Disorders and Correlation with Functional Status in Children with Cerebral Palsy.

OBJECTIVES: To detail the spectrum of movement disorders (MD) among children with cerebral palsy (CP) and assess impact on functional status. METHODS: In this cross-sectional study, children with CP were recruited and examined for various MDs. Tone abnormality was assessed using Hypertonia Assessment Tool (HAT), functional status using Gross Motor Function Classification System Expanded and Revised (GMFCS E&R), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS). These scores were classified into mild-moderate (level I-III)/severe (level IV-V) categories. RESULTS: A total of 113 children (mean age 4.9 ± 3.4 y, 66.4% boys) were enrolled. MDs were noted in 52 (46%) children; the most frequent were dystonia (28%), chorea (14%), choreoathetosis (8%). Of 64 children with quadriparetic CP, 27 (42.2%) demonstrated MDs. Of 19 children with hemiparetic CP, 2 (10.5%) had MDs. Of 16 children with dyskinetic CP, 15 (93%) had MDs. Children with dyskinetic CP had significantly higher frequency of MDs (p = 0.001). There was no difference in occurrence of all MDs or dystonia aloneamongst the two categories (mild-moderate/severe) of GMFCS E&R levels, CFCS levels or MACS levels. CONCLUSION: Although diverse MDs occur frequently in CP, these do not correlate with the broad functional status of the child. The study is limited by small sample size.

Teleneurorehabilitation Among Person with Parkinson's Disease in India: The Initial Experience and Barriers to Implementation.

OBJECTIVE: We aimed to assess the feasibility of teleneurorehabilitation (TNR) among persons with Parkinson's disease (PD), considering difficulties imposed by the COVID-19 pandemic in access to healthcare, particularly in low-resource settings. The feasibility of TNR in India has not been formally assessed so far. METHODS: We conducted a single-center, prospective cohort study at a tertiary center in India. Persons with PD with Hoehn & Yahr (H&Y) stages 1-2.5, who were not enrolled into any formal exercise program, were offered TNR as per a predesigned program for 12 weeks. Baseline and post-intervention assessment included Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), part II and III, Parkinson's Disease Questionnaire (PDQ)-8 and Non-Motor Symptoms Scale (NMSS). We assessed adherence to TNR and problems expressed by patients/caregivers by means of open-ended surveys addressing barriers to rehabilitation. RESULTS: We recruited 22 for TNR. Median age (interquartile range [IQR]) was 66.0 (44.0-71.0) years; 66.7% were H&Y stage 2.0. One patient died of COVID-19-related complications. Of the remaining 21, 14 (66.7%) had adherence of ≥75%; 16/21 (76.2%) patients had problems with attending TNR sessions as the family shared a single phone. Slow Internet speed was an issue among 13/21 (61.9%) of the patients. Other issues included lack of rapport, migration to distant hometowns and motor-hand impairment. CONCLUSION: Multiple challenges were faced in implementing a telerehabilitation program among persons with PD, exacerbated by the COVID-19 pandemic. These barriers were present at various levels: recruitment, adherence issues and maintenance. Future TNR programs must address these concerns.

Effect of botulinum toxin type-A in spasticity and functional outcome of upper limbs in cerebral palsy.

INTRODUCTION: Spasticity has been considered to be a main contributor to both the impairment of function as well as posture in children with cerebral palsy (CP). Patterns of upper limb motor involvement in CP vary with resultant limitations in daily independence, participation, and quality of life. Botulinum Toxin-A (BTX-A) is a potent neurotoxin which acts by preventing the release of acetylcholine (Ach) from presynaptic axon at motor end plate reducing focal spasticity. With literature established role of BTX-A available for lower limb spasticity in CP, the purpose of this study was to present an objective analysis of the effect of a single i.m. injection of BTX-A in reduction of spasticity in the upper limb as well as functional outcome in children (4-12yrs) with spastic CP. METHODS: A total of 28 patients (30 upper limbs) of spastic CP with minimum follow up of 6months were included in the study. Modified Ashworth Scale (MAS) and Modified Tardieu Scale (MTS) were used to measure the spasticity. Surface landmarks were used to give I.m. Botox in selected spastic muscles followed by targeted rehabilitation. Functional outcomes were measured by MACS (Manual Ability Classification System) and Canadian Occupational Performance Measure (COPM) before treatment, at 3 and 6 months follow up. RESULTS: Pronator teres was the most frequently injected muscle followed by FCU and Adductor pollicis. MAS scores at all joints and MTS scores at forearm deteriorated between 3 and 6 months. However, MACS and COPM showed sustained improvement at 3months and 6months with statistically significant change. CONCLUSION: I.m. BTX-A injected using anatomical landmarks had significant improvement in both clinical and functional outcome measures. We noticed significant improvement in MACS and COPM at 6 months despite return of local spasticity. It is safe and effective for spasticity of upper limbs in cerebral palsy and capable of improving function without major side effects. MACS & COPM are easy to use, less time consuming & easily adjusted to local needs. Randomized control trials with long follow up are required in future with special focus on dosing and timing, scoring system for functional outcome as per regional needs and issue for antibody formation for repeat injections of BTX-A.

Effect of impaired ambulation and anti-epileptic drug intake on vitamin D status of children with cerebral palsy.

BACKGROUND: Children with cerebral palsy (CP) are vulnerable to developing vitamin D deficiency. There is little information on the prevalence and severity of vitamin D deficiency in these patients. OBJECTIVE: To study vitamin D status in children with CP with special reference to their intake of anti-epileptic drugs (AED) and ambulatory status. METHODS: The relative effects of AED use and ambulatory status on the vitamin D status of 120 children with CP aged 2-10 years were examined in this observational study. The patients were classified into four groups (30 in each) on the basis of AED use and ambulatory status: ambulatory (CPA), ambulatory receiving AED (CPAD), non-ambulatory (CPNA) and non-ambulatory receiving AED (CPNAD). A control group of 30 age-matched healthy children was also included. Parameters assessed included dietary calcium intake, sun exposure, serum total and ionised calcium (tCa, iCa), inorganic phosphate (iP), alkaline phosphatase (ALP), parathormone (PTH), 25 hydroxy vitamin D [25(OH)D] levels and a wrist radiograph to detect rickets. Vitamin D status was defined on the basis of serum 25(OH)D levels as normal (>50 nmol/L), mild deficiency (25-50 nmol/L), moderate deficiency (12.5-25 nmol/L), severe deficiency (<12.5 nmol/L). RESULTS: Median (IQR) serum 25 (OH)D levels in patients with CP were 35.6 (26.75-64) nmol/L compared with 60 (37-69.25) nmol/L in controls (p = 0.04). Sixty per cent of children with CP and 36.7% of controls were vitamin D-deficient [25(OH)D < 50 nmol/L]. Children with CP had a significantly lower dietary calcium intake and sun exposure than controls (p < 0.0001 each). Serum tCa and iCa levels were significantly lower (p = 0.01 and p < 0.001, respectively) and PTH and ALP levels significantly higher (p = 0.04 and p = 0.001, respectively) in children with CP than in controls. Patients in the CPNAD group were the worst affected, 83.3% of them being vitamin D-deficient with median (IQR) 25(OH)D levels of 33.5 (12.5-45.25) nmol/L. Also, 53.3% of them had raised ALP and 17.2% raised PTH levels. CONCLUSION: Children with CP are highly vulnerable to vitamin D deficiency. In these patients, AED use and lack of sun exposure contribute towards poor vitamin D status, the effect being more pronounced when they co-exist.

Sprengel's deformity: association with musculoskeletal dysfunctions and tethered cord syndrome.

Sprengel's deformity is a rare and complex congenital deformity of the shoulder girdle. The deformity commonly occurs sporadically, though in combination with other congenital anomalies, such as congenital scoliosis, fusion of cervical vertebrae, and conditions like Klippel-Feil syndrome may coexist. We report a case of a 14-year-old girl with bilateral Sprengel's deformity presenting with a progressive bilateral lower limb weakness and gait abnormality. Radiological investigations demonstrated multiple musculoskeletal abnormalities on x-ray and lumbar spina bifida occulta causing tethering of the cord on MRI. The patient consulted neurosurgeons and orthopaedic surgeons, who recommended no operative intervention and conservative management till the end of growth spurt. Therefore, we prescribed a home-based exercise regimen to strengthen the periscapular and intrinsic foot muscles. Although rare, Sprengel's deformity can be associated with other musculoskeletal abnormalities including lumbar spina bifida and comprehensive neurological examination should not be ignored as it is much more than a cosmetic problem.