COMPUTED TOMOGRAPHY FINDINGS IN CEREBRAL PALSY IN
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COMPUTED TOMOGRAPHY FINDINGS IN CEREBRAL PALSY IN
*!+"* COMPUTED TOMOGRAPHY FINDINGS IN CEREBRAL PALSY IN YAOUNDE – CAMEROON Moifo B(1,2*), Nguefack S(3), Zeh OF(1,2), Obi FA(2), Tambe J(2), Mah E(4), Mbonda E(3), Gonsu Fotsin J(1,2) (1) Radiology Department; (3)Neuropediatric Unit ; (4)Neonatalogy Unit : Yaounde Gynaeco-Obstetric and Pediatric Hospital – PO Box : 4362 Yaounde - Cameroon (2) : Department of Radiology and Radiation Oncology, Faculty of Medicine and Biomedical Sciences, University of Yaounde I *Corresponding author Dr Boniface MOIFO, MD Radiology Department, Yaounde Gynaeco-Obstetric and Pediatric Hospital PO Box: 4362 Yaounde - Cameroon Email: [email protected] Phone: 00 237 77805999 ABSTRACT: Background: Cerebral palsy is a neuro-developmental condition beginning in early childhood and persisting throughout life. It is the leading cause of childhood disability affecting function and development. Neuro-imaging, with magnetic resonance imaging being the chief cornerstone, plays a vital role in determining the presence and extent of brain injury, with the possibility of determining the age of the lesions, the prognosis and also depicting concurrent or other mimicking lesions. Aim: To determine and describe common brain lesions of cerebral palsy patients on CT in Yaoundé (Cameroon) where magnetic resonance imaging is still not widely available and accessible. Materials and methods: It was a cross-sectional descriptive study carried out at the Radiology and Pediatric units of a university-affiliated hospital in Yaounde. Consecutive and complete records of all patients with a clinical diagnosis of cerebral palsy who had a head CT scan performed between September 2009 and February 2012 were reviewed. Results: One hundred and twenty patients’ records were reviewed for the study, of which there were 66 females (sex-ratio 0.8). The mean age of the study population was 42 months with 77.4% aged 0 to 60 months. The most frequent associated clinical signs were language impairment (45.6%), mental retardation (40.2%) and epilepsy (37.8%). Spastic quadriplegia was the most frequent (26.67%) clinical subtype followed by spastic hemiplegia (15.83%). CT findings were observed in 90 percent of all the scans performed. Diffuse brain atrophy (52.7%) and cerebral hypodensities (45.4%) were the most frequent imaging findings. Atrophy was mostly cortical and sub-cortical (49.1%) while hypodensities affected the middle cerebral artery territory in 55.1% of cases. Parenchymal calcifications were seen in 11 cases (10.2%) of which two cases of confirmed CMV infections. Brain malformations were founded in eighth patients. Conclusion:. Spastic quadriplegia and infantile cerebral hemiplegia were the most frequent cerebral palsy types. Diffuse brain atrophy, and parenchymal hypodense areas constitute frequent CT findings in patients with CP. Keywords: Cerebral palsy, Computed tomography, Diffuse brain atrophy, Cerebral hypodensities, Spastic quadriplegia, Infantile cerebral hemiplegia, Cameroon. B Moifo et al RÉSUMÉ : Objectif. En l’absence d’IRM, décrire au scanner les principales anomalies cérébrales dans une population d’enfants atteints de paralysie cérébrale à l’Hôpital Gynéco-Obstétrique et Pédiatrique de Yaoundé (Cameroun). Matériel et méthodes. Étude transversale descriptive incluant tous les enfants avec un diagnostic clinique de paralysie cérébrale et ayant effectué un scanner cérébral entre septembre 2009 et février 2012à HGOPY. Résultats. Cent-vingt enfants étaient inclus dont 66 filles. L'âge moyen était de 42 mois avec77,4% d’enfants âgés de 0 à 5ans. Les troubles de langage (45,6%), le retard mental (40,2%) et l’épilepsie (37,8%) étaient les principaux signes cliniques associés. La tétraparésie spastique était la forme la plus fréquente (26,67%) suivie de l’hémiplégie cérébrale infantile (15,83%). Le scanner était pathologique dans 108cas (90%). L’atrophie cérébrale (52,7%) et les hypodensités parenchymateuses (45,4%) étaient les anomalies les plus fréquentes. L’atrophie était à prédominance cortico-sous-corticale (49,1%) alors que les hypodensités intéressaient le territoire de l’artère cérébrale moyenne dans 55,1%. Les calcifications parenchymateuses étaient observées dans 11 cas (9,1%) dont deux cas confirmés d’infection à CMV. Les malformations étaient associées dans huit cas (6,6%). Conclusion. La tétraparésie spastique et l’hémiplégie cérébrale infantile étaient les formes de paralysie cérébrale les plus fréquentes. L’atrophie cérébrale diffuse et les hypodensités constituaient les principales anomalies scanographiques. Mots-clés: Paralysie cérébrale, Scanner, Hémiplégie cérébrale infantile, Cameroun. BACKGROUND: Cerebral palsy (CP) is defined as “a persisting but not unchanging disorder of movement and posture, appearing in the early years of life and due to a non progressive disorder of the brain, the result of interference during its development” [1]. It describes a group of developmental disorders of movement and posture, causing activity restriction or disabilities that are attributed to disturbances occurring in the fetal or infant brain. The motor impairment may be accompanied by a seizure disorder and by impairment of sensation, cognition communication and or behavior [2]. These definitions rely essentially on clinical aspects, thus making the diagnosis of CP clinical. There are four main clinically forms of CP: bilateral spastic form (52%) with spastic tetraparesis and spastic diplegia also known as Little’s syndrome, unilateral spastic form or infantile cerebral hemiplegia (26%), dyskinetic or athetosic form (14%), and ataxic form (4 %) Hypodensités cérébrales, Tétraparésie spastique, [3]. The global prevalence of cerebral palsy is estimated at 1.5 to 2.5 per 1000, and this has remained stable despite a marked improvement in obstetrical methods, follow up of pregnancies and a drop in maternal and infantile mortality and morbidity [4-6]. In Cameroon CP represents 20.39% of Pediatric neurology consultations [7]. It is the leading cause of childhood disability affecting function and development [8]. The brain lesions of cerebral palsy occur from the fetal or neonatal period to up to age 2 years. Imaging in CP is aimed at looking for the presence and extent of brain injury, with the possibility of determining the age of the lesions, the prognosis and also depicting concurrent or other mimicking lesions. While cranial ultrasound (US) and brain computed tomography (CT) scans can be used in CP imaging, magnetic resonance imaging (MRI) is the gold standard imaging modality in the B Moifo et al diagnosis and in the determination of the prognosis of patients suffering from CP.US is the first-line imaging modality for newborns and infant before the closure of the fontanels. Despite radiation, CT-scan is the modality of choice in emergency situations and in poorly equipped areas where magnetic resonance imaging is not available. Some studies have been carried out in Cameroon on etiological, clinical and therapeutic aspects of CP, without any entirely focusing on the CT features [9, 10]. With MRI still not very accessible and available in Cameroon, this study insists therefore on the CT aspects of CP, with main objective to determine and describe the most common lesions on CT and to assess any association between clinical type of CP and findings on CT. MATERIALS AND METHODS: It was a cross-sectional descriptive study carried out at the Radiology and Pediatric units of the Yaounde Gynaeco-obstetric and Pediatric Hospital (YGOPH) from November 2011 to February 2012. Complete consecutive records of all patients with a clinical diagnosis of cerebral palsy, who had at least a head CT performed between September 2009 and February 2012 were reviewed. Authorization for the study was obtained from the institutional authorities. Clinical information was obtained from the documentation of pediatric neurologists. Data was collected using a standardized form divided into three major sections. The first section was on basic demographic information such as age and gender. The second section sought for clinical information such as signs, symptoms, CP type, and also on the most probable etiology. The third section investigated the various CT findings that were depicted. CT protocol comprised unenhanced and contrast enhanced CT scans using a single detector CT scanner (Neusoft, Philips 2007). Image acquisition was spiral from the skull base to the vertex, using 3mm-thick slices. The images were reviewed on a workstation by one radiologist. All data were coded and entered into the statistical software SPSS 17.0 (SPSS Inc, Chicago, USA) and subsequently analyzed. RESULTS: One hundred and twenty patients were reviewed, of which there were 66 females (sexratio 0.8; p = 0.31). The mean age of the study population was 42 months (3.5 years). Table I summarizes the age-group distribution of the study population. Table I. Age-group distribution Age-group (years) [0-1] Frequency 45 Percentage (%) 38.7 ]1-5] 45 38.7 ]5-10] 16 11.7 Above 10 14 10.8 Total 120 100 The clinical types of CP observed are presented in table II. Spastic quadriplegiahad the highest number of cases (32 cases, accounting for 35.16% of specified cases and 26.67% of all cases) B Moifo et al Table II. Cerebral palsy clinical types Cerebralpalsy types Frequency Spastic quadriplegia 32 Spastic hemiplegia 19 15.83 20.88 Spastic diplegia 8 6.67 8.8 Dyskinetic form 4 3.33 4.4 Ataxic form 1 0.83 1.09 Mixed forms 27 22.50 29.67 Unspecified forms 29 24.17 - Total 120 100 - Regarding the period during which the brain assault was supposed to have occurred, they were classified as antenatal (15.7%), perinatal (68.4%) and postnatal (15.7%). Other clinical signs were associated on the CT-scans requests in CP patients: language impairment (45.6%), mental retardation Relative frequency From total of 120 cases From 91 specifiedcases 26.67 35.16 40.2%), epilepsy (37.8%), microcephaly (26.1%), and visual disturbances (19.3%). One hundred and three head CT scans (85.7%) out of 120 were unenhanced. Findings were observed in 108 scans (90%) as presented in table III and Figure 1. Table III. Cerebral palsy clinical types and CT-scans results Cerebralpalsy types Abnormal CT-scan findings Normal CT-scan findings Total Spastic quadriplegia 30 (93,8%) 2 (6,2%) 32 Spastic hemiplegia 17 (89,5%) 2 (10,5%) 19 Spastic diplegia 7 (87,5%) 1 (12,5%) 8 Dyskinetic form 4 (100%) 0 (0%) 4 Ataxic form 0 (0%) 1 (100%) 1 Mixed forms 25 (92.6%) 2 (7.4%) 27 Unspecified forms 25 (86.2%) 4 (13.8%) 29 Total 108 (90%) 12 (10%) 120 Brain atrophy and hypodensities were the most frequent parenchyma lesions. Atrophy was both cortical and sub cortical in 49.1 percent (Tables III and IV and Figure 3). B Moifo et al Table IV. Spectrum of CT-scan findings CT-scan findings Atrophy Anoxic-ischemic-like hypodensities Parenchyma calcifications Malformations Basal ganglia hypodensities Hygroma Tumour Frequency 57 49 11 8 4 2 1 Percentage 52.7 45.4 10.2 7.4 3.7 1,8 0,9 Table V. Brainatrophy types Types of atrophy Cortical and sub cortical Cortical Sub cortical Total Frequency 28 19 10 57 Percentage (n=57) 49.1 33.3 17.6 100 Concerning the distribution of brain hypodensities with respect to arterial territories, hypodensities of the middle cerebral artery territory were present in 34.7 percent of cases (Table V and Figure 4). Parenchyma calcifications were seen in 11 cases of which 36.4 percent (four cases) were ependymal calcifications with two confirmed Table V.: Distribution of brain hypodensities with respect to the vascular territories. Territory Frequency Percentage (n=49) MCA 17 34.7 Diffused 12 24.5 ACA + MCA 10 20.4 ACA 6 12.2 PCA 2 4.1 Watershed 2 4.1 Total 49 100 ACA: anterior cerebral artery, MCA: middle cerebral artery, PCA: posterior cerebral artery B Moifo et al Table VI. Brain malformations in cerebral palsy patients. Types Pachygyria Lissencephaly Corpus callosum agenesis Vermis agenesis Dandy Walker syndrome Total Frequency 2 2 2 1 1 8 Fig1. Spastic quadriplegiain a 7-month-old girl (1a): diffuse cortical and subcortical hypodensities with areas of porencephaly (multicystic encephalopathy). Spastic hemiplegia in . an 18-month-old boy (1b): cortical and subcortical left parietal hypodensities, posterior periventricular hypodensity and ventricular septum agenesis Fig3. Spastic quadriplegia in 4-year-old girl (3a): diffuse cortical and sub-cortical atrophy with ventricular and subarachnoïdian spaces expansion. Spastic hemiplegia in 5-yearold girl (3b): diffuse cortical and sub-cortical right cerebral atrophy with enlarged right ventricle. Spastic diplegic CP in 24-month-old boy (3c): diffuse frontal and parietal cortical atrophy with sub-arachnoïd spaces expansion. Spastic tetraplegic CP in 3.5-year-old boy (3d): diffuse sub-cortical hypodensities and atrophy predominantly on the frontal lobes. Percentage (n=8) 25 25 25 12.5 12.5 100 Fig2. Spastic diplegic CP in 8-month-old boy (2a): diffuse frontal cortical and subcortical homogeneous hypodensities. Mixed form of CP in a 24-month-old girl (2b): Cortical and sub-cortical brain hypodensities predominantly on the left parietal lobe. Fig4.Spastic quadriplegiain 2-month-old boy (4a): diffuse cortical and sub-cortical hypodensities (multicystic encephalopathy) with diffuse brain atrophy. Bilateral frontal porencephalic hypodensities in 8-yearold girl (4b) with CP. Cortical and sub-cortical occipital hypodensities in 11-year-old girl (4c) with CP. Bilateral parietal sub-cortical hypodensities in 10-year-old boy (4d) with CP. B Moifo et al Fig 5.CP in 3-month-old boy (5a) craniosynostosis. Pachygyria in a 4-year-old male (5b) with CP. Cortical, sub-cortical and subependymal calcifications in 1-month-old girl (5c) with CP and proved CMV infection. Frontal atrophy and corpus callosum agenesis in 2-yearold male (5d) with CP. DISCUSSION: The most frequent clinical signs in CP patients were language impairment (45.6%), mental retardation (40.2%) and epilepsy (37.8%). Spastic quadriplegia was the most frequent (26.67%) clinical subtype followed by spastic hemiplegia (15.83%). CT findings were observed in 90% of all the scans performed. Diffuse brain atrophy (52.7%) and cerebral hypodensities (45.4%) were the most frequent imaging findings. Parenchymal calcifications were present in 11 cases and brain malformations in eighth patients. There was no sex predilection in the occurrence of cerebral palsy in this study. However, Johnston and Hagberg [11] suggested that sex hormones seem to protect the female fetal brain from anoxia and ischemia and hence would be less predisposed to cerebral palsy compared to the males. Mbonda et al [7], Karumuna and Mgone [12] found a sex ratio of 1.3 in favour of males, while Ndiaye et al [13] found a sex ratio of 1.44 in favourof males. Stanley et al [14] suggested that lower socioeconomic status and male sex may increase risk factors for cerebral palsy. Most of the patients with clinical suspicion of cerebral palsy in this study were aged between 1 month and 5 years old (77.4%). This is in line with what the literature suggests, on the average period for clinical diagnosis and the establishment of a possible prognosis [13, 15]. At 1 year, the brain structure is well developed to permit more precise clinical manifestations and diagnosis and at 1.5 years a notion on the evolution of the condition can be obtained and consequently on the outcome or prognosis. Most of the brain insult was likely to occur during the perinatal period. Previous studies have shown that neonatal asphyxia and antenatal infections are responsible for most cases of cerebral palsy [7, 13, 16]. The insult that gives rise to cerebral palsy occurs during immature brain development. According to most references [8], this initiating event can take place anytime between prenatal development and age 2 years. However, children are usually not diagnosed until after age 1 year, with the condition becoming identifiable as children fail to meet developmental milestones. The higher frequency of spastic forms may be due to the fact that the clinical type of CP was not specified in 24.17% (29 of 120 cases). In Mbonda et al study [7], spastic forms accounted for 57.8% while 38.1% of cases were mixed cases, and in Ndiaye et al study, accounted for 49% with 26.5% spastic quadriplegia [13]. In the study carried out by Kolawole et al, the spastic forms were responsible for 83 % positive yields on CT [15]. Spastic cerebral palsy, due to cortex/pyramidal tract lesions, is the most common type and accounts for approximately 80% of cases [8, 14]. This type of cerebral palsy is characterized by spasticity (velocity dependent increase in tone), hyperreflexia, clonus, and an upgoing Babinski reflex. Extrapyramidal or dyskinetic cerebral palsy (athetoid, choreoathetoid, and dystonic) comprises 10-15% of this disorder and is characterized more by abnormal involuntary movements. Ataxic cerebral palsy comprises less than 5% of cerebral palsy. The prevalence of CP subtypes varied from one study to another [3, 7, 8, 11, 13, 14]. According to Bax et al [3], clinical factors correlate with CP are prematureness (45.2%), infections during pregnancy (39.5%) and multiple pregnancy (12%). In our setting, brain assault was supposed to have occurred in perinatal period in 68.4% with asphyxias and antenatal infections identified as leading risk factors, and anoxicischemic-like hypodensities counting for 45.4% of CT-scan anomalies. A study on epilepsy in children with cerebral palsy by Mbonda et al. also showed neonatal asphyxia and infections to be the most frequent risk factors for CP [7]. Gururaj et al. also found neonatal hypoxia responsible for 60% of cerebral palsy and seizures [16]. With respect to CT technique, 86 % of the scans were not contrast-enhanced. Neuroimaging of CP with computed tomography does not usually require contrast enhancement. Contrast can be used more to exclude other entities that are not part of the syndrome that may enhance like tumors and infections. In our study the positive yield of CT was high (90%). This is an encouragement for settings with at best CT as modality for neuroimaging. This high positive yield of CT scans in CP is comparable to that observed by Kolawole et al. who recorded a 72.5 % yield [15]. Diffuse brain atrophy (52.7%) and parenchymal hypodense areas (45.4%) were the most frequent CT findings. Atrophic changes had been earlier reported to be the commonest CT findings in cerebral palsy accounting for 30.8% to 54.4% [7, 13, 15]. This brain parenchyma reduction can be explained by neuronal destruction from anoxo-ischemic lesions and infections. Bax et al [3] with MRIscans observed that white-matter damage of immaturity,including..periventricular leukomalacia was the most common finding (42.5%), followed by basal ganglia lesions (12.8%), cortical/subcortical lesions (9.4%), malformations (9.1%) and focal infarcts (7.4%)and that there are good correlations between the MRI and clinical findings. Brain hypodensities were mostly (55.1%) on the middle and/or anterior cerebral arteries territories. MRI is the modality of choice to differentiate white matter lesions from cortical lesion, and to diagnose basal ganglia lesions and periventricular leukomalacia[3]. Some negative CT cases may be positive on MRI. Importantly also some brain malformations were observed. These could be considered as differential diagnoses for the clinical manifestations that were initially attributed to be due to cerebral palsy. In CP the extent and topography of lesions determine the clinical type of motor impairment (unilateral or bilateral spastic forms, ataxic and dystonic forms) and the frequency and severity of associated impairments [3, 17].The white matter lesions are more frequent, especially with prematureness and bilateral spastic forms, dyskinetic or ataxic forms. In children born at term, cortical lesions and the basal ganglia lesions are more common in unilateral forms or dyskinetic forms. 10% of CP are linked to a brain malformation. There are usually subtle lesions especially in the basal ganglia that can be missed on CT-scan and even on MRI[17]. Limitations to the study were largely due to retrospective data collection, as many cases were not classified clinically. CONCLUSION: Spastic quadriplegia and infantile cerebral hemiplegia were the most frequent cerebral palsy types. Diffuse brain atrophy, and parenchymal hypodense areas constitute frequent CT findings in our patients with CP. With a high CT positive yield, CT then remains useful in the screening of patients with cerebral palsy in developing countries where magnetic resonance imaging is still relatively unavailable B Moifo et al and inaccessible. Cranio-cerebral malformations should be given some consideration prior to the workup for cerebral palsy. REFERENCES : 1. MacKeith R, Makenzie I, Polani P. The Little Club Memorandum on terminology and classification of “cerebral palsy.”Cerebral Palsy Bulletin. 1959;1:27–35. 2. World Health Organisation. International Classification of Functioning, Disability and Health (ICF) [Internet]. 2001 [cited 2012 Feb 19];Available from: www.who.int/cassifications/icf/en/ 3. Bax M, Tydeman C, Flodmark O. Clinical and MRI correlates of cerebral palsy. The European cerebral palsy study. Journal of the American Medical Association. 2006;296:1602–8. 4. Nelson K, Ellenberg J. The Asymptomatic Newborn and Risk of Cerebral Palsy. American Journal of Diseases of Childhood. 1987;141:1333–5. 5. Rumeau-Rouquette C, Grandjean H, Cans C, Mazaubrun C du, Verrier A. Prevalence and trends of disabilities in school-age children. International Journal of Epidemiology. 1997;26:137–45. 6. Newman C. Prise en charge des troubles moteurs de l’enfant avec une infirmité motrice cérébrale. Pediatrica. 2006;17:20–3. 7. Mbonda E, Nguefack S, Chiabi A, Djampou NE, Pondy OA, Mbassi AH, Fru F, Mah E, Mbonda Chimi PC, Bogne JP, Tchokoteu PF, Tetanye E. Epilepsie chez les Enfants Atteintes d’Infirmité Motrice Cérébrale: à propos de 412 Observations à Yaoundé. Clinics in Mother and Child Health. 2011;8:1–5. (French). 8. Abbel-Hamid ZH, Zeldin SA, Bazzano TFA, Ratanawongsa B, Kao A, Neumeyer MA, Talavera F. Cerebral palsy. Mescape 2013.[Internet]. [cited 2013 may 22]; Available fromhttp://emedicine.medscape.com/article/117 9555-overview#a0156. 9. Motchie F. Infirmité motrice cérébrale: aspects étiologiques, cliniques et thérapeutiques. University of Yaounde 1; 1992. 10. Bediang G. Aspects cliniques, étiologiques et scanographiques des infirmités motrices cérébrales de l’enfant à Yaoundé. MD thesis, University of Yaounde 1; 2008. 11. Johnston M, Hagberg H. Sex and the pathogenesis of cerebral palsy. Dev Med Child Neurol. 2007;49:74–8. 12. Karumuna JM, Mgone CS. Cerebral Palsy in Dar Es Salaam. Centr Afr J Med 1990; 36:8-10. 13. Ndiaye M, Tall I, Basse AM, Touré K, Seck LB, Sene MS, Sow AD, Diagne SN, Diop AG, Sow HD, Ndiaye MM. Infirmité motrice d’origine cérébrale : une serie sénégalaise. AJNS 2012; 31(1): 15-22. 14. Stanley F, Blair E, Alberman E. Cerebal Palsies: Epidemiology and Causal Pathways.London, United Kingdom: MacKeith Press; 2000. 15. Kolawole T, Patel P, Mahdi A. Computed tomographic (CT) scans in cerebral palsy. Pediatric Radiology. 1989;20:23–7. 16. Gururaj A, Sztriha L, Bener A, Dawodu A, Eapen V. Epilepsy in children with cerebral palsy. Seizure. 2003;12:110–4. 17. Hertz Pannier L, Ferey S. Imagerie et développement neuromoteur chez l’enfant IMC (paralysie cérébrale). J Radiol. 2009;90(10):1337. Doi : 10.1016/S02210363(09)75311-9