286 CONTROVERSY Neurology his own classification—particularly the ................................................................................... unsatisfactory nature of the dividing line Arch Dis Child: first published as 10.1136/adc.88.4.286 on 1 April 2003. Downloaded from between diplegia and quadriplegia and the fact that many children change The term diplegia should be categories as they grow older. In 2000, the European Collaboration abandoned SCPE10 did not include diplegia in its classification because of the inherent A F Colver, T Sethumadhavan ambiguities. Not surprisingly, all this confusion is ................................................................................... mirrored in the standard paediatric text- Use of the term has served to confuse classification and books. Forfar and Arniel16 regard diplegia as a obscure interpretation of epidemiological and clinical studies clear entity in which the legs are more affected than the arms; the more affected rom the writings of Freud onwards more a descriptive grid than a classifi- the upper limbs, the lower is intelligence. there is broad agreement on the cation, and for him diplegia was “paraly- It is linked to specific cerebral patholo- Fdefinition of cerebral palsy (CP) but sis affecting like parts on either side of gies, which are themselves linked to pre- attempts to classify it, based on brain the body”. mature delivery or asphyxia. In Nelson,17 711 pathology, timing of postulated insult, In Ingram’s classification of 1955, diplegia is spasticity of just the legs, with aetiology, or clinical syndrome, taking spastic diplegia was described “as a con- good prognosis for intelligence and account of one or more of neurological dition of more or less symmetrical pare- seizures. The lesions in the brain are findings, distribution, and associated sis of cerebral origin more severe in the similar in diplegia and quadriplegia, impairments, have been less successful. lower limbs than the upper and dating except for more extensive necrotic de- Any syndrome must be clearly de- from birth or shortly thereafter”. He generation of white matter which coa- fined, meaningful, reliable, and used qualified the disorder with an associ- lesces into cystic cavities in quadriplegia. consistently by different people. A few ation with prematurity and lesser inci- 18 In Rudolph, diplegia requires the legs to CP syndromes such as choreoathetosis dence of mental retardation, pseudobul- have greater spasticity and weakness with deafness caused by bilirubin en- bar palsy, and seizures compared to than the arms. The children are fre- cephalopathy and ataxia caused by hy- quadriplegics. He also described its clini- quently preterm and the deficit is more drocephalus have stood the test of time cal evolution through the stages of hypo- apparent in wrist extensors and activi- and are reviewed by Ingram.1 However, tonia, dystonia, and rigidity.12 Later in his ties of daily living such as self feeding, we think that diplegia is not a descrip- 1966 review article “The neurology of drawing, or writing. Quadriplegia is tion of a valid category or syndrome and cerebral palsy”, he re-emphasised what determined by symmetric impairment of use of the term has served to confuse he regarded as the unequivocal distinc- classification and obscure interpretation tion between spastic diplegia and other all four extremities; growth retardation, 13 mental retardation, language disorders, of epidemiological and clinical studies. bilateral cerebral palsies. 19 We draw on historical papers and our In 1959 in England,8 The Little Club and seizures are common. Avery de- own new analyses of recent published presented a definition and classification scribes spastic diplegia as restricted to http://adc.bmj.com/ epidemiological papers to argue that the of cerebral palsy: “In diplegia there is bilateral spastic involvement of the lower term diplegia should be abandoned. affection of the muscles of all four limbs. extremities, often associated with nor- The lower limbs are the more affected.” mal cognitive function. Quadriplegia is HISTORICAL OVERVIEW OF USE The classification had an additional spastic involvement of the extremities, OF TERM DIPLEGIA category “atonic diplegia”, but the rea- often with orobuccal musculature ren- Table 1 summarises important classifica- soning presented in the article for this dering swallowing and talking difficult. tions of the past 150 years which we category and other aspects of the classi- shall discuss with respect to their use of fication are difficult to follow. By 1964 THE PRESENT SITUATION on September 25, 2021 by guest. Protected copyright. the term diplegia. the problems were apparent, and an The most recent International Classifi- 20 William Little2 first described the syn- annotation14 recommended that descrip- cation of Diseases has a category “spas- drome complex of cerebral palsy in 1862. tion should be based on clinical features tic diplegia” which it does not further His paper proposed a link between and that attempts to define certain define and another category “diplegia of abnormal parturition, difficult labour, syndromes combining clinical aetiologi- upper limbs”! However the common premature birth, asphyxia neonatorum, cal and pathological features should be ground in the papers we have just and physical deformities, which he de- avoided. In particular the idea of a diple- discussed appears to be that diplegia is a scribed lucidly. He did not use the term gic syndrome should be avoided. spastic form of cerebral palsy with lower diplegia. In their first epidemiological report in limbs more affected than upper limbs. In 1890, Sachs and Peterson proposed 1975, the Hagbergs9 used a classification It is however unclear whether pres- a classification which linked clinical syn- with a definition of diplegia similar to ence or absence of prematurity, seizures, drome to timing of the insult,3 and intro- Ingram’s. Hypertonic cases were diplegic or mental retardation is relevant to the duced diplegia and paraplegia as sepa- where the lower limbs were more af- definition or just an association. For rate categories. fected than the upper ones. Cases were example, if a child has severe four limb In 1893, Freud considered cerebral regarded as diplegic even when they involvement with upper limbs slightly palsy to be caused not just at parturition exhibited severe generalised damage and less affected than lower limbs, does pres- but also sooner in pregnancy because of “might have been classified as tetraple- ence or absence of the above features “deeper effects that influenced the devel- gia by other investigators”. Their classifi- such as mental retardation determine opment of the foetus”.4 Freud was the cation had an additional category of whether this is classified as diplegia or first to use the term “cerebral diplegia”, “ataxic diplegia” where the children had quadriplegia? which covered all bilateral cerebral pal- diplegia with ataxic traits, especially Even more importantly, what does sies, including non-spastic types. dysynergia and intention tremor in the “more affected” mean and should the Further classifications appeared in the upper limbs. However by 1989, comparison be based on clinical signs or 1950s.56As table 1 shows, Minear’s6 was Hagberg15 appreciated the limitations of function? If comparison is based on www.archdischild.com CONTROVERSY 287 Table 1 Classifications of cerebral palsy Arch Dis Child: first published as 10.1136/adc.88.4.286 on 1 April 2003. Downloaded from Reference Year Classification Little2 1862 Hemiplegic rigidity Paraplegic rigidity Generalised rigidity Disordered movements without rigidity Sachs and Petersen3 1890 Paralysis of intrauterine origin Diplegia Paraplegia Hemiplegia Birth palsies Diplegia Paraplegia Hemiplegia Diataxia (ataxia) Acute acquired palsies Hemiplegia Paraplegia Diplegia Choreo-athetoid Freud4 1893 Unilateral disorders—hemiplegia Right or left Bilateral disorders—diplegia Generalised rigidity Paraplegic rigidity Bilateral hemiplegia Choreo-athetosis Others Wyllie5 1951 Congenital symmetric diplegia Congenital paraplegia Quadriplegia or bilateral hemiplegia Hemiplegia with additional qualifications Choreo-athetoid cerebral palsy referring to all categories Mixed forms of cerebral palsy Ataxic cerebral palsy Atonic diplegia Minear(6) 1956 A. Physiological Spasticity, athetosis, rigidity, ataxia, tremor, atonia, mixed, unclassified B. Topographical Monoplegia, diplegia, paraplegia, hemiplegia, triplegia, quadriplegia C. Aetiological Prenatal, natal anoxia, postnatal, cause described D. Trauma Cause described E. Supplemental Psychological evaluation Physical status, convulsive seizures, posture and locomotive behaviour pattern, eye-hand behaviour pattern, visual status, auditory status, speech disturbances F. Neuroanatomical G. Functional capacity Class I–IV H. Therapeutic Class I–IV http://adc.bmj.com/ Neurology Extent Severity Ingram7 1955 Hemiplegia Right or left Mild Moderate Severe Double hemiplegia Mild Moderate Severe Diplegia on September 25, 2021 by guest. Protected copyright. Hypotonic Paraplegia Mild Dystonic Triplegia Moderate Rigid or Spastic Tetraplegia Severe Ataxia Cerebellar Unilateral Mild Vestibular Bilateral Moderate Severe Ataxic diplegia Hypotonic Paraplegia Mild Spastic Triplegia Moderate Tetraplegia Severe Dyskinesia Dystonic Monoplegia Mild Choreoid Hemiplegia Moderate Athetoid Triplegia Severe Tension Tetraplegia Tremor Other Little Club8 1959 Spastic cerebral palsy Hemiplegia Diplegia Double hemiplegia Dystonic cerebral