Epidemiology of spastic tetraplegic cerebral palsy in Sweden. II. Prevalence, birth data and origin

The prevalence and origin of spastic tetraplegic cerebral palsy (TPL) was investigated in a population-based study from 15 Swedish counties and the city of Gothenburg. The series comprised 96 children and adolescents born in 1959-1978. The prevalence at the ages of 5-24 years was 8 per 100,000. The etiological analysis was based on the 91 TPL subjects, born in Sweden. The preterm rate was 7%. There were 46 males and 45 females. Excluding postnatal cases, the mean birth weight was 2949 g and the proportion of SGA 21%. An obvious prenatal origin was found in 21 (mainly microcephaly, other CNS maldevelopment, intrauterine CMV-infection), an obvious perinatal origin in nine (mainly cerebral hemorrhage), and an obvious postnatal origin in 16 (mainly CNS infection). A potential prenatal origin was considered in six, a combined pre- and perinatal in 15, a perinatal in 13 and in 11 the origin of TPL was untraceable. An optimality analysis showed that reduced optimality in the partum and postpartum periods discriminated between pre- and perinatal etiology of TPL. Cases with obvious perinatally derived TPL had a high load of complications in the partum and postpartum periods, whereas these periods had been fairly uncomplicated in cases with an obviously prenatal origin of TPL. - It was indicated that some 50-55% of TPL was prenatally, around 30% perinatally and some 15-20% postnatally derived. The low preterm rate as well as the proportion of perinatal brain damage in TPL cases has probably increased in recent birth year periods due to the appearance of very preterm, severely multi-impaired children in the CP panorama.     

A comparison of spastic diplegic and tetraplegic cerebral palsy

The aim of this study was to compare spastic diplegic and tetraplegic cerebral palsy. Thirty-eight children had spastic diplegic cerebral palsy and 48 spastic tetraplegic cerebral palsy. Risk factors of cerebral palsy, seizures, severity of cerebral palsy, electroencephalogram, and magnetic resonance imaging findings were analyzed. Gestational history, low birth weight, and perinatal pathologies were present in similar percentages in both groups. Lower values of the Apgar score were recorded more often in the tetraplegic cerebral palsy group than the diplegic group. The children with spastic diplegia were classified more frequently into levels I and II of the Gross Motor Function Classification System, but patients with spastic tetraplegia were classified more frequently into levels IV and V. Similarly, mental retardation was observed more frequently in the patients with spastic tetraplegia. In magnetic resonance imaging, periventricular leukomalacia was detected in a higher proportion of children with spastic diplegia than in patients with tetraplegia. Cerebral atrophy occurred more frequently in the tetraplegic group compared with diplegic patients. Twenty-four (50.0%) children with spastic tetraplegia had epilepsy compared with six children with spastic diplegia. The incidence of intractable epilepsy was higher in the tetraplegic patients than in the children with spastic diplegia.     

Nosology of spastic tetraplegic cerebral palsy: clinical review of fifty cases

The aim of this study is to contribute to the definition of tetraplegic cerebral palsy (TCP) and to verify the classification criteria currently used by reviewing the clinical and neurological aspects of 50 children having non-progressive encephalopathy with neurological involvement of 4 limbs (symmetric 4-limb type, side-asymmetric type, upper-limb dominated type with or without dystonic traits). All severe diplegic patients, i.e. less upper than lower limb involvement and patients with dystonic hyperkinetic syndrome without spastic features were excluded. The data were supplied by the hospital records, evolution of motor performance and the presence or absence of epilepsy, visual problems, language disorders, intellectual impairment. All the children underwent: neurological examination, functional assessment, cognitive evaluation. Severe motor impairment was found in 80% of the patients, whose clinical characteristics led to a diagnosis of severe TCP or “true TCP” according to Hagberg, whereas 20% of them showed mild to moderate impairment with a more favorable prognosis. This group of patients complied with the classification of Michaelis who defined the quadrispastic CP types more broadly and delineated five subgroups also including cases with milder involvement (side-dominated, three-limb dominated tetraparesis). The intellectual assessment showed that, in the severely affected patients, only a small percentage had severe intellectual impairment (IQ<50, 37,5%), which contrasts with published data on this pathology, while the majority of the subjects showed moderate (25%) or mild intellectual impairment (32,5%) or normal cognitive function (5%). The presence or absence of dystonic traits did not influence the impairments considered in this study, except for the IQ data, which show a slight prevalence of severe intellectual impairment in the spastic group.

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Sommario Scopo di questo studio è di contribuire alla definizione del concetto di tetraparesi spastica e di verificare i criteri di classificazione.esistenti. è stata effettuata una revisione degli aspetti clinici di un gruppo di 50 bambini affetti da encefalopatia non evolutiva con compromissione grave ai 4 arti, con o senza tratti distonici. Sono stati esclusi dallo studio i pazienti affetti da grave diplegia e quelli affetti da sindrome distonicoipercinetica senza segni di spasticità. I dati, ricavati dalla revisione delle cartelle cliniche, sono relativi all'acquisizione delle tappe di sviluppo motorio e alla presenza di deficit associati (epilessia, disturbi visivi e del linguaggio, compromissione intellettiva). Tutti i pazienti sono stati sottoposti a: a) esame neurologico, b) valutazione neurofunzionale, c) valutazione intellettiva. Dai dati risulta che l'80% dei pazienti presenta un grave deficit motorio le cui caratteristiche cliniche rispondono alla definizione di “severe tetraplegia” secondo Hagberg, mentre il restante 20% presenta und deficit motorio lievemoderato, le cui caratteristiche cliniche corrispondono meglio alla classificazione proposta da Michaelis che prevede anche quadri clinici di minore gravità. La valutazione intellettuale ha dimostrato che, nei pazienti con maggiore compromissione motoria, solo una piccola percentuale presenta un deficit intellettivo severo (Q.I.<50, 37,5%) in contrasto con i dati della letteratura, mentre la maggior parte dei pazienti ha un deficit intellettivo moderato (25%) o lieve (32,5%) o uno sviluppo cognitivo adeguato (5%). La presenza o assenza di tratti distonici non sembra influire sulla incidenza dei deficit associati considerati, eccetto per il deficit intellettivo che risulta più severo nel gruppo dei soggetti spastici.

     

Use of modafinil in spastic cerebral palsy

After an initial patient with cerebral palsy had an apparent dramatic reduction in spasticity when placed on modafinil, a pilot study was undertaken in 10 pediatric patients to confirm or refute the benefit of modafinil in cerebral palsy. Nine of 10 patients completed the 1-month treatment period. The study patients were treated with 50 or 100 mg of modafinil once daily in the morning. An assessment was made at baseline and at 1 month on treatment. All patients had a clinical examination, Modified Ashworth Scale scores (spasticity) determined by a physical therapist, and videotaping of ambulation. In comparing visit 1 (baseline) and visit 2 (on treatment), statistically significant improvement in the modified Ashworth Scale scores was noted in seven of the nine patients completing the study (P = .0080). A blinded review of the videotapes did not show statistically significant differences in ambulation, but the speed (ft/sec) of gait improved in six of the nine patients (P = .0192). In this study, modafinil, a newly released central stimulant for narcolepsy, showed benefit in treating spasticity in patients with cerebral palsy. A second larger, placebo-controlled, double-blinded trial is planned to confirm these initial results and observations. Modafinil appears to benefit spastic cerebral palsy by a yet to be determined mechanism; however, a primary effect of modafinil on brainstem structures is hypothesized to reduce spasticity of central origin.     

Lower-Extremity strength profiles in spastic cerebral palsy

Although weakness has been identified in cerebral palsy (CP) in isolated muscle groups, the magnitude of weakness in multiple muscles and the patterns of weakness across joints have not been documented. The maximum voluntary contraction of eight muscle groups in the lower extremities of 15 children with spastic diplegia, 15 with spastic hemiplegia, and 16 age-matched peers was determined using a hand-held dynamometer. Children with spastic diplegia were shown to be weaker than age-matched peers in all muscles tested, as were the children with hemiplegia on the involved side, with strength differences also noted on the uninvolved side. Weakness was more pronounced distally in the groups with CP, and the hip flexors and ankle plantarflexors in spastic CP tended to be relatively stronger than their antagonists as compared with the strength ratios of the comparison group. In conclusion, children with spastic CP demonstrate quantifiable lower-extremity weakness and muscle imbalance across joints.     

MRI findings in patients with spastic cerebral palsy. II: correlation with type of cerebral palsy

The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. Eighty-one patients had diplegia, 45 had quadriplegia, and 26 had hemiplegia. Of patients with diplegia, 72 had periventricular leukomalacia (PVL) and very few had other types of lesions. In patients with quadriplegia, three main types of brain lesions were observed: PVL in 12 patients, term-type brain injury in 22, and brain anomaly in 10. In the 26 patients with hemiplegia, 17 had a unilateral lesion (rare in patients with diplegia and quadriplegia), and bilateral lesions were seen in seven others.     

儿童痉挛型脑性瘫痪的神经外科治疗

目的 探讨神经外科治疗痉挛型脑性瘫痪的方法和效果.方法 对351例2~14岁(平均4.8岁)痉挛型脑瘫患者施行选择性脊神经后根切断术(SPR)135例,其中颈胸段26例,腰骶段109例;选择性周围神经缩窄术(SPN)216例,其中单神经干92例,多神经干124例.结果 随访期间6-31个月,平均17.8个月.颈胸段和腰骶段SPR痉挛改善率:上下肢分别为82.7%和91.4%,肢体运动功能改善率:上下肢分别为83.7%和72.5%.SPN痉挛改善率:上下肢分别为67.4%和92.7%,运动功能改善率:上下肢分别为54.9%和84.3%.结论 神经外科治疗痉挛型脑瘫为有效的康复训练创造了机会和条件.术前正确的评估,选择恰当的手术方式,掌握手术技巧,方能提高手术效果减少并发症.     

Entrapment neuropathy in patients with spastic cerebral palsy

OBJECTIVES: We performed nerve conduction and needle electromyographic tests in 29 patients with spastic cerebral palsy (SCP) and severe limb deformities. Nerve conduction abnormalities were detected in 32 of 400 sensory or motor nerves, while 11 of 29 patients (37.9%) showed abnormal nerve conduction, indicating one or more entrapment neuropathies. Patients with SCP develop severe joint contractures and deformities due to spastic muscle tone and limited muscle and joint use/flexibility; these contractures and deformities can, in turn, cause nerve damage, possibly as a result of the stretching, angulation or compression mechanisms in the anatomic fibro-osseous passages, where nerves are particularly susceptible.     

Executive functions in youth with spastic cerebral palsy

Dependent on criteria used, between 35% and 53% of the participants with cerebral palsy fulfilled the criteria of clinically relevant executive function problems as defined by Conners' (1994) Continuous Performance Test. Executive function problems were noticed mainly in participants with bilateral brain lesions and who had been born preterm. Findings highlight the need to check for attention problems in children with cerebral palsy.     

MRI findings in patients with spastic cerebral palsy. I: correlation with gestational age at birth

The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. The relation was studied between the patients' gestational age at birth and their MRI findings, including the severity of periventricular leukomalacia (PVL) seen on MRI. In 119 of these patients, the CP was thought to be due to acquired, destructive brain injury. PVL was seen in 90 and posthemorrhagic porencephaly in 11. These preterm-type brain injuries were observed often in patients who had been born preterm but were also seen in those born at term. Full-term-type border-zone infarct, bilateral basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia were seen in 9,14,7, and 3 patients, respectively; these term-type brain injuries were observed only in patients born at or near term. Of the patients with PVL, 90% had been born preterm. Severe PVL was common in the patients whose gestational ages at birth were between 25 and 32 weeks; all patients with PVL who had been born at term had only mild PVL. The authors concluded that MRI findings for patients with spastic CP are closely correlated with gestational age.     

Attentional and executive impairments in children with spastic cerebral palsy

Aim Children with cerebral palsy (CP) are reported to have learning and social problems. The aim of the present study was to examine whether children with CP have impairments in attention or executive function. Method We examined attention and executive function with standardized neuropsychological measures in a group of children with unilateral (n=15) or bilateral (n=18) spastic CP (14 females, 19 males, mean age 11y 4mo, SD 1y 1mo, range 9y 1mo–13y 7mo; Gross Motor Function Classification System level I n=22, II n=3, III n=6, and IV n=2). Performance was compared with test norms. Results Verbal cognitive functioning fell within the normal range, whereas sustained (p=0.001) and divided attention (p<0.001) were found to be impaired. Greater impairment was observed in executive function in general (p<0.001) and in inhibition (p=0.038) and shifting (p<0.001) in particular. No significant difference was found between types of CP (unilateral and bilateral). Performance of all timed tasks was slower than the test norm (p<0.00). Interpretation The finding of slower performances across tasks may indicate a general impairment in efficiency of information processing in relation to white‐matter lesions. Impairments in attention and executive functions are present in children with CP and may help to explain why these children have increased social and learning problems.     

Trunk control and functionality in children with spastic cerebral palsy

ABSTRACT

Purpose: To investigate the relationship between trunk control in sitting and functionality in children with spastic cerebral palsy (CP).

Methods: Fifty-eight children with spastic CP were included in the study. Functional abilities were evaluated with the Gross Motor Function Measurement-88 (GMFM-88) and the Pediatric Evaluation of Disability Inventory Functional Skills domain (PEDI-FSD). Trunk control in sitting was tested with the Trunk Control Measurement Scale (TCMS) and the Trunk Impairment Scale (TIS). The scores of the TCMS and TIS were compared with GMFM-88 and PEDI-FSD with Spearman correlation analysis.

Results: The total score of GMFM-88 was signi?cantly correlated with the total score of TCMS (r_ho = 0.90, p < 0.01) and TIS (r_ho = 0.78, p < 0.01). The total score of PEDI-FSD was also signi?cantly correlated with the total score of TCMS (r_ho = 0.76, p < 0.01) and TIS (r_ho = 0.72, p < 0.01).

Conclusions: The evaluation of trunk control can provide valuable information for functional abilities of children with spastic CP.     

Neuropsychologic differences between bilateral dyskinetic and spastic cerebral palsy

This study compared the neuropsychologic performance of 30 adolescents and adults with bilateral dyskinetic, mixed, and spastic cerebral palsy aged between 16 and 38 years. The sample was relatively homogeneous in terms of motor severity; no patients were able to walk unaided. In all subjects, we evaluated the general function of nonverbal reasoning and the following specific neuropsychologic areas: language, visual perception, memory, praxis, and frontal functions. Individuals with dyskinetic cerebral palsy had better auditory comprehension, visuospatial abilities, immediate visual memory, and working verbal memory than those with spastic cerebral palsy. Frontal function was the only cognitive function on which subjects with dyskinetic cerebral palsy had lower scores. We conclude that the neuropsychologic profiles of dyskinetic and spastic cerebral palsy are different.     

Lower limb muscle volumes in bilateral spastic cerebral palsy

Aim: Muscle weakness is a feature of individuals with spastic cerebral palsy (SCP) but there are few reports in the literature of muscle volume in this group. This study compares muscle volumes in adolescents and young adults with SCP with those of their typically developing (TD) peers. Design: Measurements of the volumes of nine major lower limb muscles in 19 independently ambulant subjects with SCP (mean age 14.2 years (sd 2.7), 11 male, GMFCS I (n = 5); GMFCS II (n = 14)), 19 TD subjects (mean age 16.5 years (sd 3.0), 11 male) were made using magnetic resonance imaging. Results: Lower limb muscles were smaller in the SCP group (p ? 0.023 in all muscles) than the TD group with the exception of the vastii (lateralis + intermedius; p = 0.868) and gluteus maximus (p = 0.056). Average muscle volume deficit was 27.9%. Muscle volume deficits were significantly greater for distal muscles than proximal muscles (p < 0.001). Conclusions: Reduced muscle size in adolescence and the natural history of sarcopenia in adulthood may contribute to the early loss of mobility of adults with SCP.