Neurological outcomes of congenital Zika syndrome in toddlers and preschoolers: a case series.

BACKGROUND: Congenital Zika syndrome causes a spectrum of neurological symptoms with varying effects on function that require different therapeutic strategies. To date, this spectrum of effects and its clinical implications have not been completely described. We describe the neurological examination findings in toddlers and preschoolers, including predominant symptom complexes and comorbidities. METHODS: This study is a case-series neurological evaluation of 75 children with congenital Zika syndrome in Campina Grande, Brazil. The study is part of a cohort of children with congenital Zika syndrome that started in 2015 and is still ongoing. Children with Zika virus infection detected during pregnancy (mothers exhibited rash and were followed and diagnosed by fetal ultrasound abnormalities or RT-PCR) or through microcephaly screening after birth, using Intergrowth 21 guidelines, were selected by laboratory and radiological criteria. Children were examined during a 10-day period in September, 2018, and underwent neurological interview, examination, and assessment of functional outcomes and comorbidities. Children were divided in groups of predominant corticospinal or neuromuscular clinical signs and the associations between these groups and clinical comorbidities were assessed. FINDINGS: All of the children recruited to the study from Nov 29, 2015 to Nov 30, 2017 had imaging correlates of congenital Zika syndrome. Children were assigned to groups depending on the signs exhibited, either corticospinal or neuromuscular, with or without dyskinetic signs. 75 children completed the evaluation, 38 (51%) girls and 37 (49%) boys. Median age was 33 months (range 26-40 months; IQR 29-34). Microcephaly was present at birth in 56 (75%) children, and 19 (25%) children were born with normal head circumference, 15 of whom later developed microcephaly. Neurological examination grouped four children as having isolated dyskinetic signs, 48 children were assigned to the corticospinal group and 23 into the neuromuscular group. Dyskinetic findings were present in 30 (40%) children, either alone (four [5%]) or combined with corticospinal (19 [40%] of 48) or neuromuscular (seven [30%] of 23) findings. Comorbidities were highly prevalent, and the neuromuscular group had worse functional outcomes, evaluated by gross motor function (p=0·026), manual abilities (p=0·0013), and communication function (p<0·0005) classification scales, than the corticospinal group, whereas pneumonia (p<0·0005) and urinary tract infections (p<0·0005) were more frequent in the corticospinal group. Cortical hyperexcitability was supported by several clinical correlates, such as early onset epilepsy, persistence of primitive reflexes, and dystonia. INTERPRETATION: We describe distinct neurological profiles in the congenital Zika syndrome spectrum, with functional outcomes tending to correlate with these groups. The clinical division of children based on the disease signs proposed here is supported by the literature on central and peripheral nervous system pathology in congenital Zika syndrome. The high prevalence of dyskinetic symptoms merits special attention. FUNDING: Brazilian National Council for Scientific and Technological Development and by the Coordination for the Improvement of Higher Education Personnel.

Acute and Chronic Pain in Children and Adolescents With Cerebral Palsy: Prevalence, Interference, and Management.

OBJECTIVE: To determine the prevalence, interference, and management of acute and chronic pain among youth with cerebral palsy (CP) aged 5-18 years attending outpatient rehabilitation services. DESIGN: A cross-sectional study using the Faces Pain Scale-Revised, Patient Reporting Outcomes Measurement Information System Pediatric Pain Interference Scale, and the Cerebral Palsy Quality of Life questionnaire. Where children were unable to self-report, parent or caregiver proxy was obtained. SETTING: Outpatient rehabilitation. PARTICIPANTS: Participants (N=280) with CP aged 5-18 years and their parent or caregiver. Self-report was obtained by 45.7% (n=128) and proxy-report was obtained by 54.3% (n=152) of the cohort. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Presence or absence of acute pain and chronic pain. Secondary measures were pain intensity, pain interference, pain management, and quality of life. RESULTS: Acute pain and chronic pain were reported by 67.1% and 31.4% of participants, respectively. Of those reporting acute pain, 42% also experienced chronic pain. Factors that increased the odds of chronic pain were: predominately dyskinesia (odds ratio [OR]=3.52; 95% confidence interval [CI], 1.64-7.55); mixed spasticity-dyskinesia (OR=1.93; 95% CI, 1.07-3.47); bilateral involvement (OR=3.22; 95% CI, 1.844-5.61) and Gross Motor Function Classification System level IV (OR=2.32; 95% CI, 1.02-5.25), and V (OR=3.73; 95% CI, 1.70-8.20). Pain frequently interferes with sleep, attention, ability to have fun, and quality of life. Short-acting pharmacologic analgesics, thermotherapy, hydrotherapy, and massage were commonly used for pain management. CONCLUSIONS: Routine screening for pain is critical for early identification and intervention. Multimodal interventions are needed to address the biopsychosocial model of pain, and should be tailored for all abilities across the CP spectrum.

Instrument-based assessment of motor function yields no evidence of dyskinesia in adult first-degree biological relatives of individuals with schizophrenia and schizoaffective disorder.

There is accruing evidence of spontaneous dyskinesia in individuals with schizophrenia that is independent of medication exposure. Dyskinetic motor behavior is also present in individuals who are at high risk of schizophrenia and appears to have prognostic value for the development of psychosis. Nonetheless, it remains unclear whether dyskinesia is present in first-degree relatives of individuals with schizophrenia and thus associated with genetic liability for schizophrenia (i.e., an endophenotype), or whether the motor abnormality is a biomarker specific to the disease state spectrum. There is also limited information about links between dyskinesia and clinically relevant phenomena such as symptoms and cognition. Because dyskinesia marking genetic liability is likely to be subtle, we used sensitive instrument-based measurement of handwriting fluency to quantify dyskinesia in medicated individuals with schizophrenia or schizoaffective disorder, unaffected first-degree biological relatives of individuals with schizophrenia and schizoaffective disorder, and control participants. Results indicated that medicated individuals with schizophrenia or schizoaffective disorder exhibited more dyskinesia than both relatives and controls, with no difference between relatives and controls. Dyskinesia in individuals with schizophrenia or schizoaffective disorder was unrelated to current antipsychotic medication dosage, but associated with worse working memory function and greater positive formal thought disorder. These results provide evidence that dyskinesia is not associated with unexpressed genetic liability for schizophrenia.

The Influence of Test Positions on Clinical Assessment for Scapular Dyskinesis.

BACKGROUND: Appropriate evaluation of scapular dyskinesis is essential for therapeutic strategies. Although the current visual-based assessment is rapid and practical, the reliability of this method is unsatisfactory. It is necessary to adequately understand the conditions of assessment to maximize the benefit of therapeutic interventions. OBJECTIVE: To explore the influence of different test positions on clinical assessment for scapular dyskinesis. DESIGN: Observational study. SETTING: University rehabilitation department. PATIENTS: A total of 102 subjects diagnosed with unilateral shoulder disorder were recruited from among rehabilitation outpatients from November 2015 to February 2016. METHODS: Two experienced raters categorized the subjects' scapular movement pattern according to Kibler et al classification by the vision-palpation method at 4 test positions (at rest, and the end range of elevation in the sagittal, scapular, and coronal planes). MAIN OUTCOME MEASUREMENTS: The overall prevalence of scapular dyskinesis, the distribution of types, and the reproducibility of types at the 4 test positions were analyzed. RESULTS: The overall prevalence of scapular dyskinesis was 90.08%, and the highest frequency was found at the resting position. Type III was the most common type in our sample. In reproducibility analysis, 21.57% of subjects presented with the same type at any position, and 75.49% of subjects presented with 2 types. CONCLUSIONS: Scapular dyskinesis in individuals with shoulder disorder showed a high prevalence, especially at the resting position. More than 1 type of scapular pattern would be present if assessed at different positions. This study indicates that test positions can affect the results of scapular dyskinesis assessment, and that the resting position should primarily be applied. LEVEL OF EVIDENCE: III.

[Dyskinesia in Parkinson's disease--major clinical features, aetiology, therapy]. / Dyskinesien bei Morbus Parkinson--Klinik, Atiologie, Therapie.

Parkinson's disease (PD), a slowly, progressive degenerative disorder of the central nervous system, which affects about ten million people world-wide, is currently treated symptomatically. Current treatment aim i. e. to balance the decreased dopamine turnover in striatal neurons. Chronic exposure to dopaminergic agents, however, supports onset of motor complications and dyskinesia in the long term. Dyskinesia appear mainly as chorea, athetosis, dystonia, stereotypia, ballism or a combination. Sometimes excessive abnormal facial, body and limb movements depend on the overall dosage of dopaminergic substitution. This is why the main therapy is based on reducing the total dosage of dopaminergic substances. Either alternative or additional well-tried substances like apomorphine, amantadine or clozapine are used. New possibilities in treatment emerge from substances like sarizotan, istradefylline, fipampezol or talampanel. Even so disability and reduced quality of life in PD patients and their caregivers may exist. This survey describes the major clinical features, aetiology and demographics of treatment-associated dyskinesia in PD.