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The recently proposed definition and classification of cerebral palsy (CP) is still up for debate – see the correspondence in this issue – and additional comments via the Castang website are warmly invited before the final version is completed next month (www.castangfoundation.net/workshops_washington_public.asp). The Castang Foundation funds ‘research into the causes of CP and other neurodevelopmental disorders leading to their prevention’. Rightly, the document does not specify the ‘non-progressive disturbances’ to which CP is attributed, with the exception of cerebral dysplasia. It is unclear why this should be picked out since in developed countries white matter disorders and perinatal vascular events currently appear more common. Data from other parts of the world are less available, but emphasize the continuing importance of post-neonatal causes. As the relative importance of different disturbances changes with medical knowledge, geography, and over time, a non-specific definition is understandable.
Philippi and colleagues' excellent randomized clinical trial (RCT: p 5) led me to reflect on two issues that I believe are worth considering. I hope these remarks may provoke further discussion among both the authors and readers.
The aim of this study was to assess the therapeutic efficacy of osteopathic treatment in infants with postural asymmetry. A randomized clinical trial of efficacy with blinded videoscoring was performed. Sixty-one infants with postural asymmetry aged 6 to 12 weeks (mean 9wks) were recruited. Thirty-two infants (18 males, 14 females) with a gestational age of at least 36 weeks were found to be eligible and randomly assigned to the intervention groups, 16 receiving osteopathic treatment and 16 sham therapy. After a treatment period of 4 weeks the outcome was measured using a standardized scale (4–24 points). With sham therapy, five infants improved (at least 3 points), eight infants were unchanged (within 3 points), and three infants deteriorated (not more than –3 points); the mean improvement was 1.2 points (SD 3.5). In the osteopathic group, 13 infants improved and three remained unchanged; the mean improvement was 5.9 points (SD 3.8). The difference was significant (p=0.001). We conclude that osteopathic treatment in the first months of life improves the degree of asymmetry in infants with postural asymmetry.
Adductor spasticity in children with cerebral palsy (CP) impairs motor function and development. In a placebo-controlled, double-blind, randomized multicentre study, we evaluated the effects of botulinum toxin A(BTX-A) in 61 children (37 males, 24 females; mean age 6 years 1 month [SD 3y 1mo]) with CP (leg-dominated tetraparesis, n=39; tetraparesis, n=22; GMFCS level I, n=3; II, n=6; III, n=17; IV, n=29; V, n=6). Four weeks after treatment, a significant superiority of BTX-A was observed in the primary outcome measure (knee–knee distance ‘fast catch’, p=0.002), the Ashworth scale (p=0.001), and the Goal Attainment Scale (p=0.037).
Fifty-seven children with cerebral palsy (CP) and imaging evidence of vascular thrombosis (study group) and 167 children with CP and other imaging finds (control group)were selected. Sixty-one per cent of the study group were male and 53 (93%) had spastic hemiplegia compared with the control group, of whom 55% were male and 54 (32%) had a diagnosis of spastic hemiplegia. Mean age was 5 years 11 months (SD 5y 1mo) for the study group and 7 years 7 months (SD 4y 7mo) for the control group. Blood spots on Guthrie cards or buccal swabs were used to test both groups and their mothers for the factor V Leiden (fVL) mutation, which predisposes carriers to thrombophilia. Mothers were interviewed to gather antenatal, perinatal, demographic, and socio-economic data. The frequency of the fVL mutation in children with evidence of vascular thrombosis and their mothers was not statistically different from the frequency in children with CP with other imaging findings and their mothers. The frequency of the fVL mutation was significantly higher than the expected population frequency of 4% in the study group (10.5%, p=0.012) and in mothers of the control group (7.2%, p=0.036).
To spare more children from painful muscle biopsy, a new non-invasive diagnostic motor performance test is undergoing development. Fifteen functional items were used to measure muscle strength and muscle endurance in 68 patients (47 males, 21 females; mean age 7y 8mo, SD 2y 2mo; range 4 to 11y), who had been referred to our specialist centre in the past 3 years on suspicion of myopathy. All the patients had undergone muscle biopsy. To correct the patients' outcomes for age, sex, and body size, regression prediction equations were obtained from a stratified random sample of 64 normally developing primary-school children aged 4 to 11 years (32 males, 32 females; mean age 8y 1mo, SD 2y 4mo). Feasibility was evaluated on the basis of five criteria. Validity was assessed using logistic regression analysis, receiver operating characteristic analysis, and sensitivity and specificity at a specifically chosen cut-off point. Reproducibility was evaluated by test–retest reliability in a stratified random sample of 40 patients who returned for re-measurements using the intraclass correlation coefficient. Seven items satisfied all five feasibility criteria, had high diagnostic power, and high test–retest reliability. The motor performance test can improve diagnostic procedure in children suspected of having myopathy.
The linguistic abilities of children born preterm at 32 weeks' gestation or earlier at Kuopio University Hospital during 1984 to 1986 were evaluated during successive phases of a prospective study. The study protocol included the Rapid Automatic Naming test and Wechsler Intelligence Scale for Children – Revised at 9 years of age and a modified Stroop Color-Word test and the Wechsler Intelligence Scale – Revised at the age of 16 years. Fifty-one children born preterm (26 males, 25 females) and 51 age-matched and sex-matched term controls (26 males, 25 females) were studied at the age of 9 years. At the age of 16 years, 40 children born preterm (19 males, 21 females) and 31 term controls (14 males, 17 females) participated in the study. The children born preterm scored significantly lower in two naming tasks than the controls at the age of 9 years. However, there was no difference between the study groups in naming skills at the age of 16 years or in verbal IQ in either study phase. Maternal education level was not associated with naming skills. Thus, the consequences of preterm birth seem to be minor in relation to linguistic skills during school age and diminish by adolescence.
The purpose of the study was to determine the prevalence of ‘soft’ motor deficits in school-aged children with either developmental language disorder (DLD), autism (with high IQ [HiAD] or low IQ [LoAD]), or low IQ without autism (LoIQ), and to evaluate the utility of a refined neurological examination to discriminate between these groups. A total of 242 children (74% male), aged 7 or 9 years, were evaluated as part of a longitudinal, multi-institutional study, with a standardized neurological examination that included Denckla's Physical and Neurological Examination for Soft Signs. Most of the scores separated children into two groups defined by nonverbal IQ, with the DLD and HiAD groups performing better than the LoAD and LoIQ groups. Exceptions included motor impersistence and stereotypies, which were more likely in the autistic groups. The neurologists' summary clinical impressions indicated better sensory/motor skills, oromotor skills, and praxis in the HiAD than in the DLD children. Inability/unwillingness to perform tasks was much more frequent in LoAD than LoIQ children.
The rate of autism spectrum disorders (ASDs) and brain abnormalities was analyzed in 31 individuals (15 males, 16 females; age range 1mo to 31y, mean age 8y 11mo) with CHARGE association, as part of a multidisciplinary study. A meticulous neuropsychiatric examination was performed, including standardized autism diagnostic interviews. Judgement regarding ASDs was impossible in three infants and three patients who were deaf and blind. Five individuals met diagnostic criteria for autism, five for an autistic-like condition, and seven for autistic traits. Brain abnormalities were indicated in almost three-quarters of examined individuals, and midline abnormalities of the forebrain in one-third. Awareness of the coexistence of CHARGE and ASDs is important in habilitation care in CHARGE. Moreover, the results indicate that a subgroup of ASDs may be associated with errors in early embryonic brain development.
Language disorders have been reported after severe falciparum malaria but the deficits have not been described in detail. We assessed language outcome in three groups of children aged 6 to 9 years (n=487): those previously admitted to Kilifi District Hospital, Kenya, with cerebral malaria (CM; n=152; mean age 7y 4 mo [SD 1y 1mo]; 77 males, 75 females); or those with malaria and complicated seizures (M/S; n=156; mean age 7y 4mo [SD 1y 2mo]; 72 males, 84 females); and those unexposed to either condition (n=179; mean age 7y 6mo [SD 1y 1mo]; 93 males, 86 females). Median age at hospital admission was 28 months (interquartile range [IQR] 19 to 44 mo) among children with a history of CM and 23 months (IQR 12 to 35mo) among children with a history of M/S. A battery of eight assessments covering the major facets of speech and language was used to measure language performance. Cognitive performance, neurological/motor skills, behaviour, hearing, and vision were also measured. Eighteen (11.8%) of the CM group, 14 (9%) of the M/S group, and four (2.2%) of the unexposed group were found to have a language impairment. CM (odds ratio 3.68, 95% confidence interval 1.09 to 12.4, p=0.04) was associated with significantly increased odds of an impairment-level score relative to the unexposed group. The results suggest that falciparum malaria is one of the most common causes of acquired language disorders in the tropics.
Neurofibromatosis type 2 (NF2) remains a challenging diagnosis in childhood where there may be no neurological involvement. A 12-month-old male in whom NF2 was suspected because of characteristic ophthalmological and cutaneous lesions is reported. Cranial MRI showed no tumours. A pathogenic mutation was identified on NF2 gene analysis. The child developed hypertension due to renal vascular disease. Although renal vascular disease is a recognized complication of neurofibromatosis type 1 (NF1), it has not been reported in NF2.
A 5-year-old female presented with prolonged afebrile right-sided focal seizures, right brachio-facial paralysis, and dysarthria; consciousness was not altered. Fever appeared 20 hours after onset of neurological symptoms. At admission (day 1) cerebral computerized tomography and cerebrospinal fluid (CSF) analyses were normal including undetectable alpha-interferon (α-IFN) and negative herpes simplex virus (HSV) polymerase chain reaction (PCR). Acyclovir was started at a dosage of 60mg/kg/day for 21 days and neurological symptoms improved. Cerebral magnetic resonance imaging (MRI) showed lesions in the left thalamus and left parietal lobe. On day 8, CSF contained an elevated leukocyte count with a predominance of lymphocytes, but α-IFN and HSV DNA were still undetectable. Delayed intrathecal synthesis of specific anti-HSV antibodies was found on day 26 and confirmed herpes simplex encephalitis (HSE) diagnosis. Twenty months after this episode, the patient presented with a febrile meningeal syndrome. PCR detected HSV DNA in CSF and cerebral imaging showed a new left temporal lesion. At relapse onset, intrathecal synthesis of specific anti-HSV antibodies had disappeared. Acyclovir was started at a dosage of 60mg/kg/day for 21 days and neurological status improved. At discharge, neurological examination showed right hemiparesis and bucco-facial dyspraxia. Diagnostic problems of HSE diagnosis in children are highlighted. It is suggested that the premature disappearance of intrathecal synthesis of a specific anti-HSV antibody might play a permissive role in the resurgence of cerebral viral replication.
This study reviews the instruments used for the clinical assessment of spasticity in children with cerebral palsy, and evaluates their compliance with the concept of spasticity, defined as a velocity-dependent increase in muscle tone to passive stretch. Searches were performed in Medline, Embase, and Cinahl, including the keywords ‘spasticity’, ‘child’, and ‘cerebral palsy’, to identify articles in which a clinical method to measure spasticity was reported. Thirteen clinical spasticity assessment instruments were identified and evaluated using predetermined criteria. This review consists of reports on the standardization applied for assessment at different velocities, testing posture, and quantification of spasticity. Results show that most instruments do not comply with the concept of spasticity; standardization of assessment method is often lacking, and scoring systems of most instruments are ambiguous. Only the Tardieu Scale complies with the concept of spasticity, but this instrument has a comprehensive and time-consuming clinical scoring system.
Nothing in clinical medicine is one hundred per cent certain. Part of a doctor's education involves learning how to cope with the anxiety that uncertainty in decisions affecting life and death inevitably produces. This paper examines: (1) the role of anxiety – both rational and irrational – in the provision of health care; (2) the effects of uncertainty upon the doctor–patient relationship; (3) the threat uncertainty poses to medical authority (and the assumption of infallibility that props it up); (4) the contribution of clinical uncertainty to the rising popularity of alternative therapies; and (5) the clash between the medical and the legal understanding of how certainty should be defined, particularly as it affects the paediatric community. It concludes by suggesting some strategies that might facilitate successful navigation between the opposing and ever-present forces of certainty and uncertainty.