<<

SPECIAL ARTICLE

A CLASSIFICATION OF By W. L. Minear, M.D., Ph.D.

A REvuw of the literature reveals con- which the olden term, cerebral paral- fusion in nomenclature and classifica- ysis, is only one of the group. It is fre- tion in the field of cerebral palsy. Using as quently a combination of both motor and a basis the majority opinion from question- sensory involvement. It was formerly con- names sent to the members of the American sidered as involving only a motor handicap, Academy for Cerebral Palsy during 1953 and on this basis confusion arose among (all members were polled; 64 per cent re- those treating such cases, since the other plied) by the Nomenclature and Classifica- associated handicaps were not recognized tion Committee, the following classification and difficulties arising from treating these for cerebral palsy is presented. children were attributed to their being A complete diagnosis should include one mentally defective. However, it is very diffi- on more titles from each of the main head- cult to explain how mental deficiency can ings of this nomenclature. The motor classi- be associated with pure on . fication is given precedence. Next, there The is a very complicated organ and should be a statement as to topography of any injury or lack of development in it gives the disease (body pants involved). There rise to unusual disturbances of function, also should be a statement as to the etiology both along the line of motor control and of the disease. A statement of the capabili- sensation. This places cerebral palsy in a ties of the patient should include the pen- group by itself, being made up of more than tinent motor, sensory, intellectual, emo- one entity depending on the area injured, tional, visual, speech, and status, diseased, or defective.” (From lecture notes and should be made under each heading of Winthrop Phelps.) in the supplemental classification. If the In Cerebral Palsy: Its Individual and pathology in the brain has been determined Community Problems by Denhoff, as a accurately at necropsy or by means of standard definition, reference is made to electroencephalograms, pneumoencephalo- Perlstcin who is quoted as stating that cere- grams, or surgical exploration, a statement bral palsy is generally defined as a “condi- of structural disease should be made (neu- lion, characterized by , weakness, roanatomical diagnosis). Finally, a state- incoordination, or any other aberration of ment of the functional and therapeutic motor function due to pathology of the classification of the patient completes the motor control centers of the brain.” A limited list. Thus, a comprehensive diagnosis will definition cited by Denhoff is one wherein it demand a careful consideration of every is conceived as “a condition in which inter- aspect of the patient and will afford a fenences with the control of the motor sys- sound basis for prognosis, neuroanatomical tem arises as a result of lesions occurring correlation, and treatment. from birth trauma.” The practical definition, according to Dcnhoff, warrants serious con- CEREBRAL PALSY DEFINED sideration : “One component of a broader “The term, cerebral palsy, constitutes five syndrome comprised of neu- main classes of handicapped children, of romotor dysfunction, psychological dys-

Dr. Minear is Chairman, Nomenclature and Classification Committee, American Academy for Cere- bra! Palsy. The proposed classification has the approval of the American Academy for Cerebral Palsy. This classification has been reviewed by the Committee on the Handicapped Child of the American Academy of and receives the endorsement of this committee. 841

Downloaded from www.aappublications.org/news by guest on September 27, 2021 842 MINEAR - CLASSIFICATION OF CEREBRAL PALSY function, convulsions, and behavior dis- disturbances should be excluded as well as orders of organic origin. those primarily the result of spinal cord “The characteristics of the cerebral pal- lesions.” sied child are paralysis, weakness, inco- Cruickshank and Raus,6 state : “As a dis- ordination, on any other aberration of motor ease, cerebral palsy is not progressive nor function due to malfunction of the motor is it contagious or epidemic in form. As a centers of the brain. neuro-physical and neuro-psychological “He may also have other symptoms which deviation, however, it is basically a disease. reflect a damaged brain. There may be con- Glidden Brooks has correctly spoken of vulsions, mental retardation on deficiency, cerebral palsy as a ‘long-term, non-fatal, vision, hearing or perceptual problems, as non-curable disease.’ He considers it a dis- well as speech, behavioral and emotional ease in the of the present discussion. disturbances. It is non-curable, but oftentimes amenable “The underlying cause of these symp- to therapy and training. It is non-fatal per toms-brain damage-is not a specific type. Se. Rather similar pathological findings are Cerebral palsy comprises those motor and found in such clinical entities as mental other symptom complexes caused by a non- deficiency, , amid behaviour dis- progressive brain lesion (or lesions). The orders or organic origin. Thus originates characteristic thing about cerebral palsy is the term ‘brain damage’ syndrome which in- that it is a well defined entity with a eludes cerebral palsy along with other con- variety of etiologies and pathologies. When ditions mentioned. clinical syndromes become better deline- “Cerebral palsy is the neunomotor com- ated with respect to known etiologies and ponent of the ‘brain damage’ syndrome. It pathological changes, they too can be re- must always be kept in mind that the cere- moved from the general category of cere- bral palsied child may suffer from any other bral palsy, and placed in their own cate- component or components of the syndrome gonies. For example, it is not unlikely that along with the neuromotor handicap. will someday be “The concept of the ‘brain damage’ syn- removed from the category of cerebral palsy drome has many practical applications, par- and given its own name. In the aging, ticularly from an educational and ‘parent arteriosclerosis or parkinsonian disease can understanding’ viewpoint. It negates the cause a change in the patient who has had propaganda that the cerebral palsied child cerebral palsy since infancy or childhood, presents a problem unique from that of but these are morbid entities superimposed other similarly handicapped children.” on the original lesion causing the cerebral John F. Pohl defines cerebral palsy as palsy. follows : “Cerebral palsy is the term used to The decision of the American Academy designate a group of neuromuscular dis- for Cerebral Palsy to delete neoplastic brain orders in which there is impairment or loss diseases and the progressive neurological of muscular control due to a lesion of the degenerative diseases of the brain has sev- brain.” cral important advantages. This decision C. L. Balf and T. T. S. Ingram, in a reduced the field of cerebral palsy to a more recent article,2 state as follows : “Cerebral logical and manageable unit. The treat- palsy is a descriptive term, applied to a ment of brain is frequently sur- group of motor disorders of young children, gical or radiological and the treatment pro- in whom full function of one on more limbs gram depends on the type of . It is prevented by panesis, involuntary move- is a great mistake to treat a brain neoplasm ment, on incoondination. Tacit agreement as cerebral palsy. Obviously, the correct has been reached that progressive diseases diagnosis should be made and the proper and those characterized by transient motor treatment program carried out.

Downloaded from www.aappublications.org/news by guest on September 27, 2021 AMERICAN ACADEMY OF PEDIATRICS - SPECIAL ARTICLE 843

After the tumor is removed and there is often sensory involvement in the areas of no danger of recurrence, the patient may proprioception to point discrimination and form perception. Aphasias appear more fre- then be accepted to the ranks of those with quently in right than in left hemiplegias and cerebral palsy to be treated symptomati- are much more common in the acquired than cally for residuals of the lesion. The same in the congenital cerebral palsy. is true for a child with , D. traumatic on infectious damage to the brain. Involves 3 extremities, usually both legs and Under the new definition, the patient is one arm, usually spastic. This may represent hemiplegia plus , or incomplete not accepted as cerebral palsy until a pro- quadriplegia. In the latter case, both arms gnessive nature of the etiology has been will be equal or nearly equal in length. In eliminated. This does not mean that treat- the former, the involved arm will be shorter. ment technique suitable for cerebral palsy E. Quadnplegia cannot be used on patients with progressive () Involvement of all 4 extremities. Patients with the greatest involvement of brain lesions, but rather that attention the legs are usually spastic, and patients with should be concentrated on the treatment greatest involvement of the arms are usually and elimination of progressive damage to the dyskinetics, including athetoids. the brain as long as this is a possibility. F. This term is seldom used. “Paralysis affecting CLAssIFICATIoN OF CEREBRAL PALSY#{176} like parts on either side of the body; bilateral paralysis.” (The American Illustrated Mcdi- I. Physiological (motor) cal Dictionary, Dorland, 21st Edition.) A. Spastic C. Double Hemiplegia B. Athetotic This term is seldom used. “. . . implies those Types of athetosis accept- cases in which the arms are more involved able to the American than the legs. These are usually spastic in Academy for Cerebral type.” (Cerebral Palsy-Its Individual & 1 . Tension Palsy to date. For outline 2. Non-tension Community Problems, edited by William M. of 12-types of athetosis 3. Dystonic Cruickshank, Ph.D. and George M. Raus, described by Winthrop 4. M.D., Syracuse University Press, 1955.) Phelps, see “Types of Cerebral Palsy Defined” III. Etiological herein. A. Prenatal C. Rigidity 1. Hereditary-Genetically transmitted and D. Ataxic may involve racial or familial predilections E. Tremor and often sex-linked. These are often F. Atonic (rare) classified as “cerebral agenesis.” The C. Mixed symptoms are often present at birth and H. Unclassified generally do not progress. Examples : Her- editary athetosis, familial tremor, familial II. Topographical spastic paraplegia. A. 2. Acquired in utero Involves one limb; condition is rare; should a. Prenatal infection () be checked closely to determine if you are rubella, or other maternal infection. not dealing with a paraplegia or hemiplegia. b. Prenatal anoxia-carbon monoxide, or B. Paraplegia strangulation of mother, maternal Involves the legs only and practically always anemia, hypotension, e.g., following of the spastic or rigidity type. spinal anesthesia, placental infarcts, or C. Hemiplegia abruptio, kinking, knots or The lateralized one-half of the body is af- prolapse of the cord. fected and it is usually spastic, although pure c. Prenatal cerebral hemorrhage-ma- athetoid hemiplegias are occasionally seen, ternal toxemia, direct trauma, ma- as are pure rigidity hemiplegias. There is temal diathesis. d. Rh factor, due to Rh factor 0 The form of the following classification, in gen- (conoversial). era!, is patterned after that which has been sue- e. Metabolic disturbances, diabetes. cessfully used for some years by the American f. Gonadal irradiation, harmful exposure Heart Association. to x-ray.

Downloaded from www.aappublications.org/news by guest on September 27, 2021 844 MINEAR - CLASSIFICATION OF CEREBRAL PALSY

g. Maternal . b. Field defects B. Natal 2. Motor 1. Anoxia a. Conjugate deviations (33%) of motor de- a. Mechanical respiratory obstruction. fects) b. Atelectasis. b. Fixation defects C. Narcotism (due to drugs). C. Spasmus fixus (1%) (1. Placenta previa or abruptio. (1. fixus (1%) e. Maternal anoxia or hypotension. C. Esotropia (51%) f. Breech deliveries with delay of the f. (9%) after-coming head. g. Hypertropia g. Bleeding in the first trimester (see h. Hypotropia Eastman). i. Nystagmus C. Postnatal j. Pseudopalsy of the exteriii (22%) 1. Trauma-Subdural , skull frac- C. Auditory status itires, wounds and contusions of the brain 1. Pitch range loss (accidental). 2. Decibel loss 2. Infections-(more common in children H. Speech disturbances than adults) , , brain V. Neuroariatomical (See subheadings under . “Brain,” topographic headings, Standard 3. Toxic causes-Lead, arsenic, coal tar de- Nomenclature of Diseases and Operations.) rivatives, streptomycin, etc. 4. Vascular accidents (more common in adults Failure to discuss the parts of the brain involved than children) congenital aneurysms, circle in the different motor types of cerebral palsy is de- of Willis, hypertensive , liberate. This discussion is reserved until after we emboli due to bacterial endocarditis or correlate the lesions from a sufficient number of fat embolism, cerebrovascular thrombosis, from patients with cerebral palsy with the in debilitated infants, sudden pressure symptoms to gain a scientific understanding of the changes. problem. 5. Anoxia-Carbon monoxide poisoning, The following two headings are added for the strangulation, high altitudes, and deep sake of completeness, and for the use of the dm1- pressure anoxia, . cian studying the cerebral palsied patient. They are 6. Neoplastic, or late development defects- not intended to be used for coding by the medical Brain tumors, brain cysts, internal hydro- record librarian: cephains, hydrocephalus. VI. Functional Capacity (degree of severity)

Iv. Supplemental Class I. Patients with cerebral palsy with no practical limitation of activity. A. Psychological evaluation Class II. Patients with cerebral palsy with slight 1. Degree of mental deficiency, if any. to moderate limitation of activity. B. Physical status Class III. Patients with cerebral palsy with 1. Physical growth evaluation (Wetzel Grid moderate to great limitation of activ- or other) ity. 2. Developmental level (Gesell)’ Class IV. Patients with cerebral palsy unable 3. Bone age to carry on any useful physical activ- 4. ity. C. Convulsive D. Posture and locomotive behaviour patterns VII. Therapeutic E. Eye-hand behavior patterns Class A. Patients with cerebral palsy not requir- 1 . Eye dominance ing treatment. 2. Eye movements Class B. Patients with cerebral palsy who need 3. Eye postures minimal bracing and minimal therapy. 4. Fixation Class C. Patients with cerebral palsy who need 5. Convergence bracing and apparatus, and the serv- 6. Prehensory approach ices of a cerebral palsy treatment team. 7. Grasp Class D. Patients with cerebral palsy limited to 8. Manipulation such a degree that they require long 9. Hand dominance term institutionalization and treatment: F. Visual status 1. Sensory The American Academy for Cerebral a. Amblyopia Palsy also agreed to: (1) Delete neoplastic

Downloaded from www.aappublications.org/news by guest on September 27, 2021 AMERICAN ACADEMY OF PEDIATRICS - SPECIAL ARTICLE 845

and progressive neurological diseases from 3) The decision of the American Academy the cerebral palsy classification. (2) Co-op- for Cerebral Palsy to include the manifesta- crate with the Editor of Standard Nomen- tions of nonprogressive (static) brain lesions clature of Diseases and Operations1 to pre- as cerebral palsy was probably the most pare a classification and coding based on important single decision. The field of cene- the accepted classification along with the bral palsy is now clearly limited to a more following recommendations: (a) Delete the logical and manageable size. Furthermore, term “cerebral spastic infantile paralysis” progressive brain lesions should be classi- and substitute “cerebral palsy.” (b) Assign fled so as to place emphasis on etiology. code numbers for the 6 motor types of cerebral palsy, amid a code number for OF FUTURE CLINICAL WORK OUTLINED “cerebral palsy, type unknown” (Note: The nuxed type may be coded using two on For the time being, the clinician had best more of the type code numbers). be practical and resolve to make a careful symptomatic analysis of patients with cere- POINTS FAVORING THE MOTOR bnal palsy using all clinical tools available CLASSIFICATION AS to him; treat the total patient symptomati- FIRST CHOICE cally and co-operate with the Brain Regis- The consensus giving the motor classifi- try in collecting, studying and correlating cation first choice instead of the etiological the symptoms with lesions. This then is the on netiroanatomical classification is as fol- primary work of the clinician : Let him use lows: a classification that fits his purposes in a 1) The treatment is primarily the treat- practical way-Let him cOncentrate on the ment of symptoms, not the etiology or the phase of cerebral palsy which, by training portion of the brain involved. Etiology fne- and opportunity, he is best able to make, quently cannot be determined, but it is then he will at least be doing his part well. highly important to include it in the diag- It appears that we, as clinicians, are missing nosis when it can be determined (even a lot in our clinical evaluation of the child when determined, the treatment will be with cerebral palsy, especially in the realm symptomatic). Since the treatment of cere- of sensory involvement. Probably this is bnal palsy is symptomatic, a classification true because we are just beginning to work l)ased on major presenting symptoms is use- out techniques to treat the sensory losses. ful and practical. Moreover, if etiology is We have concentrated on the motor and known, the eventual pathology and patho- intellectual phases of cerebral palsy and genesis can better be studied. neglected the sensory phase. If the student 2) The neuroanatomical classification of medicine can grasp and understand the based on position, extent, and character of clinical manifestations of cerebral palsy, the brain lesion, is highly desirable from a he will have broadened his knowledge of scientific point of view, but is presumptive medicine considerably. without necropsy, although electroenceph- alogram and air studies give important in- TYPES OF CEREBRAL PALSY DEFINED formation. When the Brain Registry of the Cerebral palsy comprises those motor and American Academy for Cerebral Palsy has other symptom complexes caused by a non- studied more brains and correlates lesions progressive brain lesion (or lesions). The with primary presenting symptoms, we will various sub-types may be defined as fol- be able to evolve a neuroanatomical diag- lows: nosis for cerebral palsy. Even then, the A. -Chanactenized by a lower diagnosis in the living will be based on threshold of the stretch reflex, an enlarged inference and the treatment will still be the neflexogenic area, augmented responses with treatment of symptoms. , and an abnormal electromyographic

Downloaded from www.aappublications.org/news by guest on September 27, 2021 846 MINEAR - CLASSIFICATION OF CEREBRAL PALSY record (synchronization of discharge rate in C. There is usually little involvement in the hands, fingers, wrists, various parts of the spastic muscle). The or knees. D. Arms and legs describe these flailing motions, pathological stretch reflex must be present usually fully extended, sometimes flexed. to make a diagnosis of spasticity. There is E. Doctor Phelps has never seen flail(ing) athe- a tendency toward greater involvement and toids live beyond maturity. Apparently there contractures, affecting the antigravity mus- is some progression of the disease between des. 10 and 14, with gradual weakening of the muscles of breathing followed by flaccidity B. Athetosis-Chanactenized by an ab- and death. normal amount and type of involuntary mo- Note: This resembles one of the rare progressive tion, normal reflexes, normal clcctromyo- neurological diseases and since it is progressive, graphic findings, uncontrolled, involuntary would be excluded from the new classification. The and inco-ordinate motions with varying de- cause of death in this type of athetosis is not known. gnces of tension. Description of the various Doctor Phelps prefers to retain this entity for the sake of completeness. clinical types by Winthrop Phelps2#{176}follows: VI. Tension I. Rotary A. A state of muscular tension in an anthetoid A. The most common type. (rotary, tremor or dystonic) hiding the char- B. Involves muscles which can take part of acteristic motions of the types. rotary motions. B. When the tension is relieved the true nature C. The rotary motions are usually rather slow, of the athetosis (rotary, tremor or dystonic) sometimes extremely slow. is revealed. D. The feet describe a circular motion; the hands C. Must be distinguished from spasticity, in pronate and supinate; the shoulders internally which a stretch reflex must be present and and externally rotate. from rigidity in which the lead-pipe-like E. There may be varying degrees of tension. resistance of the muscle is found. II. Tremor (tremor-like) D. The state of tension is not constant, and can be shaken out by the examiner. A. Almost as common as the rotary type. E. The athetoid is only classed as tension athe- B. There is an irregular and uneven type of involuntary contraction and relaxation which toid when the tension is the outstanding characteristic and masks the rotary, tremor, involves flexor and extensor, and abductor and adductor mechanisms. or dystonic features. C. Rotary motion is not seen. Involuntary rotary VII. Non-tension motion is not seen. The motion is not a true tremor. A. This is also a transient state and might mask other types of athetosis. III. Dystonic B. In infancy it is often mistaken for the rare A. The extremities assume distored positions atonic type of athetosis. held involuntarily for periods of from a few C. Involuntary motion must be present. D. seconds to a few minutes. Non-tension athetosis is frequently seen in B. The dystonic motions may involve neck, small babies and is usually the first identifi- trunk, arms, and legs. able symptom. As the child grows, one of the C. An entirely different distorted position may first five types mentioned above can be identi- be assumed within the passage of a few fled. minutes. Note: It must be understood that both “tension” and “non-tension” are temporary classifications of Iv. Shudder patients under treatment. A. Closely resembles the shudder that any normal person may experience. VIII. HemiathetosLs B. The shudder may be violent, causing patient A. This is stricily a topographical classification to fall on the floor or simply a mild shudder- and is made when rotary, tremor-like, dy- like motion going through the arms and legs. stonic, or shudder athetosis involve one side of the body. V. Flail(ing) B. Doctor Phelps has not seen a hemi-flail(ing) A. A rare type of athetosis. athetosis. B. Arms and legs are thrown around rather violently from the axial shoulder and hip Ix. Neck and Arm. joints. A. This type is limited to the head, neck, and

Downloaded from www.aappublications.org/news by guest on September 27, 2021 AMERICAN ACADEMY OF PEDIATRICS - SPECIAL ARTICLE 847

shoulder girdle, with little or no athetosis XII. Emotional Release

in the legs. . A. Usually the rotary or tremor-like athetosis is B. Some of these learn to typewrite or pick tip present, combined with release of the laugh- objects or write with their toes. ing, crying and anger mechanisms. A very C. There may be or may not be a speech defect. slight stimulation for laughter or crying will D. The motion may be rotary, tremor-like, dys- produce the whole picture of this emotion tonic, or shudder, but most of them are dys- without any particular background of feel- tonic. ing of the emotion. E. Doctor Phelps thinks it advisable to use the B. The patient does not like the emotional dis- separate classification of neck and arm athe- plays, but depending on the tension associ- tosis for determining the best plan for place- ated with the athetosis present, the reaction ment and treatment. may be more or less marked. Note: Not all these types of athetosis have been x. Deaf Athetosis accepted by the Academy, but are listed for the A. Athetosis with etiology of Rh incompatibility, sake of completeness. usually showing some degree of . B. Deafness may be slight enough so as to be C. Rigidity-A disturbance of the ago- of no importance in speech or education. nist-antagonist relations with resistance to C. There is often loss of high pitch or high slow passive motion of both agonist and frequency tones with essentially normal hear- ing in the lower pitch ranges. They often antagonist muscles. If the resistance to hear low pitch sounds very well and give the passive motion is continuous, it is referred parent and casual observer the impression to as the “lead-pipe” rigidity-if discon- that their hearing is keen. tinuous, “cog-wheel” rigidity. The resist- D. Child usually learns to become an automatic ance is greater to slow than to rapid mo- lip reader, especially for consonant sounds. E. The specific pitch range lost is usually above tion, whereas, in spasticity, there is greater 2,048 or, more frequently, between 4,098 resistance to rapid motion. In rigidity, the and above. antagonists to the antigravity muscles are F. There may be no athetosis in the tongue, most involved. Total motion may be de- throat and lips, but they are often diagnosed creased. The main characteristic is hyper- as such because they repeat the words to the examiner without consonants. tonicity, normal or diminished reflexes, no clonus, no stretch reflexes and no involun- Note: Audiometer tests should be done or, in young children, the psychogalvanic skin resistance tary motion. method; then, appropriately tuned hearing aids D. Ataxia-Ataxia is primary inco-ordina- or amplifiers can be supplied to bring up the hear- lion due to disturbance of kinesthetic or ing in that part of the sound spectrum lost. balance sense, or both. Characterized by G. Besides having athetosis in hearing loss, these disturbance in the sense of balance and children have limitation of vertical eye mo- equilibrium, dyssynengias, and the patient tions, either upward or downward, or both, with normal lateral eye motions. often exhibits the “rebound phenomenon” H. Deafness, athetosis, and vertical eye motion with astereognosis and depth perception loss, is the triad of symptoms distinguishing involvement. Atonia and may be the definite athetoid. present.

XI. Balance Release E. Tremor-Which may be intentional, A. A rare type. non-intentional or constant, uncontrollable, B. The motions resemble exactly those which involuntary motions. of a rhythmic, alternat- are seen in an individual walking in a moving ing, or pendular pattern due to alternate train or on a loose piece of rope placed on agonist and antagonist contractions. the floor. Although the child is walking on a perfecily steady floor or sidewalk, the balance F. Atonia-Lack of tone, and failure of release mechanism comes through fully and muscles to respond to volitional stimulation. unnecessarily. These patients seldom fall. The muscle lacks the firmness or turgor of C. The picture is grossly the opposite of the the normal relaxed muscle. Weak stretch ataxic , in which the balance motions are reflex may be obtained as well as increased decreased. Involuntary motions are present in the balance release athetoid, but are absent deep reflexes, but no involuntary motion is in the ataxic. present. This distinguishes it from non-ten-

Downloaded from www.aappublications.org/news by guest on September 27, 2021 848 MINEAR - CLASSIFICATION OF CEREBRAL PALSY

sion athetosis (just a matter of degree). This The evaluation of functional capacity of is rare in pure form, but may be the initial the patient with cerebral palsy, as pre- symptom in any of the forms of cerebral sented in this paper, is a rough estimate and palsy. It deserves special mention because it is subject to many individual variations. In may be the outstanding initial symptom. order to make the evaluation more scien- The atonic form of cerebral palsy, in most tific, a careful study should be made similar instances, does not describe a type, but the to the work of McBride on “ Eval- outstanding symptoms of a type. uation.” The details of this type of evalua- G. Mixed-The mixed class need not be tion should be worked out by specialists in used often, as the predominant motor symp- each field in order to insure proper numeri- toms determine the classification. cal evaluations. The majority of members returning ques- STATISTICAL DATA tionnaires wanted to accept a classification TYPES OF ATHETOSIS : The majority of the based on motor symptoms. A few members members of the American Academy of Cere- with a good deal of experience in the field bral Palsy approved of Tension, Non- of cerebral palsy strongly urge a simple tension, Dystonic, and Tremor-like types of classification, e.g., (1) Pyramidal; (2) Extra- athetosis. The others were rejected for van- pyramidal; (3) Ataxic; and (4) Mixed. One ous reasons. Possibly, some of the other supporter of this classification feels that we types would have been accepted if under- are likely to do more harm than good by stood by the members, each type needs accepting one more complicated, and that further study. (See outline of Athetosis, we must not let our classification outrun herein.) our understanding. Many are opposed to DISCUSSION this type on the premise that the clinician The majority of the members of the presumes that he knows the approximate American Academy for Cerebral Palsy ad- site of the lesion. cept a motor classification, listing 6 sep- Most members feel that certain well nec- arate types (7 with the mixed type), fol- ognized neurological syndromes such as the lowed by the topographic involvement and progressive neurological diseases and neo- etiology when this can be determined. The plastic diseases should not be included in a chief advantage of this classification is that cerebral palsy classification, but only the it aids the clinician in planning treatment static (non-progressive) neurological states. of the patient with cerebral palsy. Pro- The field of cerebral palsy is now limited ponents of this classification point out that to a more logical and manageable size. the best a clinician can (10 15 describe the Those who prefer the descriptive or symptoms carefully so that, eventually, symptomatic (motor) classification must anatomical study of post-mortem material accept a stern word of warning that neuro- can be correlated with the clinical symp- logical change as the toms. Despite its obvious faults, the motor nervous system matures and that one must classification is practical for the clinician be extremely cautious in making a final and for those engaged in the various phases descriptive diagnosis in infancy. (Those of treating cerebral palsy. We cannot escape concerned are urged to read the paper by the fact that the treatment of cerebral palsy Byers.3) For example, some infants with is, largely, the tneatment of symptoms, not cerebral palsy may undergo a sequence of the treatment of the etiological factor or the hypotonicity, hypertonicity, and, finally, treatment of some specific area of the brain. athetosis, on some other extnapynamidal The site of brain lesion is, of course, added as the nervous system matures. whenever this can be determined accu- It is only when the symptoms become static, rately, by autopsy, surgery, on other following maturation of the nervous sys- methods. tem, that the descriptive classification is of

Downloaded from www.aappublications.org/news by guest on September 27, 2021 AMERICAN ACADEMY OF PEDIATRICS - SPECIAL ARTICLE 849

value. A further study of changing neuro- to school age or by comparing initial with logical patterns during childhood and in- final neurological descriptions from first to fancy is urgently needed. fifth year of life or later, to determine the As it has been demonstrated that the pattern of changing neurological symptoms. motor classification must be applied with Those who have examined large numbers caution, until there is some degree of of children with cerebral palsy over many maturation of the nervous system, we may years have had the disconcerting expeni- profit by making a survey of children with ence of making a diagnosis of “atonia” only cerebral palsy, followed from early infancy to find, in later years, that the child was

Type of Athetosi8 Approve Do P,’ot Approve No Answer

Rotary 16 7

1abduction-adduction 12 Treiuor . ) flexion-extension

l)ystonic 27 13 5 Shudder 15 23 7 Flail(ing) 19 18 8 Tension so 9 6 Non-tension 2’ 15 8 Ilerniathetosis (topographical) 1 18 6 Neck and arm (topographical) 19 () 6 l)eaf athetoid 18 7 Balance release is s 7 Emotional release 11 24 10

Classification Based on Motor Symptoms 27 of the members preferred listing the types separately, e.g., spastic, athetoid, tremor, rigidity, ataxic, atonic, an(l mixed. 15 of the members preferred the classification listing 3 main types of cerebral palsy: (1) Spastic (a) Atonic; () Dyskinetic (a) Athetosis, (b) Rigidity, (c) Tremor-like; (3) Ataxic; and (4) Mixed. 3 members expressed no preference.

Classification based on Etwlegy 13 members prefer including progressive neurological diseases.

‘g members prefer deleting progressive neurological diseases. 16 members wish to include neoplastic diseases. 26 members wish to delete neoplastic diseases.

Supplemenlal Classification: These were checked as important enough to be included in the diagnosis by the follow- jug number of members (out of 45): 35-iiitelligence quotient 36-Major sensory disturbances 3-I)evelopmental level (Gesell) 20-Physical status (comparative) 38 -Convulsive seizures 4-Posture and locomotive behavior 24 -Eye and hand behavior patterns 17-Additional behaviour ternis 32-Visual status 35-Auditory status

The returned questionnaires indicate the following choices as a basis of classification for cerebral palsy: First choice: Motor Symptoms Second choice : Topographic Involvement Third choice: Etiology Fourth choice: Anatomical Site Fifth choice: Severity of Involvement Sixth choice: Degree of Seventh choice: Supplemental Data

Downloaded from www.aappublications.org/news by guest on September 27, 2021 850 MINEAR - CLASSIFICATION OF CEREBRAL PALSY

athetoid on spastic. Atonia may be an initial In a letter dated October 20, 1954, Ada- symptom of any one of the major types of line C. Hayden, Associate Editor of Stand- cerebral palsy. One must look carefully for ard Nomenclature of Diseases and Opera-

involuntary motion which, if found, would tions, states,12 “. . . I would like to state that indicate athetosis. Some feel that the atonic we expect to have a complete revision of phase may be an initial symptom of all the the section on for the 5th ecU- major types of cerebral palsy. Some chil- tion. At this time, the conditions which you

dren may go through rigidity with opistho- present would be coded as follows : ... All tonos, flaccidity and athetosis. Therefore, cerebral palsy is coded cerebral spastic in- we must accept the fact that the motor fantile paralysis 933.4-076 with the supple- classification in infancy is no more than a mentary terms.” tentative appraisal. This would indicate that all types of cere- Conditions resembling cerebral palsy in- bral palsy are caused by underdevelopment dude such conditions as the “high cervical of the brain due to prenatal influence (see

syndrome” described by 78 These pages 49-50, Etiologic Classification, Stand- should probably be grouped under “Spinal ard Nomenclature of Diseases and Opera- Palsy” and the entire neurological group tions). Given the following clinical diag- then referred to as “Cerebrospinal Palsy.” noses, the record librarian, using the pres- The term “Cerebrospinal Palsy” is probably ent edition, would code as follows:

better than cerebral palsy for the whole Diagnosis: Cerebral palsy, , grouping, because it has been demonstrated etiology brain hemorrhage at birth. Coding: that spinal lesions exist alone or in combina- 933.4-076; 94x-050 of extrinsic lion with brain lesions in certain types of arteries of the brain, and 969 hemiplegia.

so-called “Cerebral Palsy.” Diagnosis: Cerebral palsy athetoid quadri- The introduction to Standard Nomencla- plegia, dystonic type, etiology unknown. Cod- ture of Diseases and Operations,1 now in ing: 933.4-076; 9211 Athetosis; 96x Quadni- use by medical record librarians, states: plegia, tetraplegia; 9216 Dystonic movements.

“The method of classification is based on Diagnosis: Cerebral palsy, rigidity quadniplegia, two elements : The portion of the body con- etiology cerebral anoxia at birth. Coding: cerned (topographic) and the cause of the dis- 933.4-076; 96x Qudaniplegia; 540 Anoxemia. order (etiologic). These two elements are Diagnosis: Cerebral palsy, ataxic type, etiology designated by code number separated from unknown. Coding: 933.4-076; 271 Atonia. each other by a hyphen. The first three digits Diagnosis: Cerebral palsy, atonic type, etiology describe the topographic site; the last three, unknown. Coding: 933.4-076; 272 Atonia. following the hyphen, describe the etiologic agent. Combined, they form a complete diag- Diagnosis: Cerebral palsy, tremor type, quadri- nostic code number.” plegia. Coding: 933.4-076; 96x Quadniplegia; 9228 Tremor. The above method of coding and classifica- The proposec classification system, includ- tion for cerebral palsy is impractical, and, ing coding, is as follows: in some cases, impossible, since the etiology and the anatomical area of the brain can CEREBRAL Pu..sy “X”#{176} only infrequently be determined accurately. I. Physiological (motor) The present edition of Standard Nomencla- A. Spastic X(S)#{176} ture of Dfiseases and Operations’ can be B. Athetotic X9211 used for the spastic type of cerebral palsy C. Rigidity X(R)#{176} if it is caused by a disease due to prenatal D. Ataxic .X271 influence (see page 392, line 4), however, E. Tremor .X9228 if the spasticity is due to other causes, e.g., F. Atonic X272 postnatal influence, or in other types of 0 Above, indicates that code number is to be cerebral palsy, the coding is unsatisfactory. assigned.

Downloaded from www.aappublications.org/news by guest on September 27, 2021 AMERICAN ACADEMY OF PEDIATRICS - SPECIAL ARTICLE 851

C. Mixed X (plus any combina- lesion left by the removal of a brain tumor, tion above) however, is still considered one of the H. Unclassified .. .XYOO etiological factors of cerebral palsy.

11. Topographical Cerebral palsy comprises the motor and other symptom complexes caused by a non- A. Monoplegia . . . -948 llndicate proper

B. Paraplegia ... .X . .. -94l physiological progressive brain lesion (or lesions).

C. Hemiplegia .. .X . . . -969 Icode number The nomenclature and classification ques- 1). Triplegia X . . . -978 tionnaires indicate that the members of the E. Quadriplegia . .X ... -96x American Academy for Cerebral Palsy wish Ill. Etiological (Add to diagnosis when determined) to accept a motor classification, listing each A. Prenatal -0 type of cerebral palsy separately : Spastic, B. Natal Anoxia -048 Athetoid, Tremor, Rigidity, Ataxic, Atonic, C. Postnatal and Mixed. 1. Trauma -4 The following choices as a basis for 2. Infection -1 classification of cerebral palsy were made 3. Toxic causes -3 4. Vascular accidents -50 by the American Academy for Cerebral 5. Anoxia -421 Palsy: 6. Neoplastic -8 First choice: Motor Symptoms (When coding etiology, above, indicate agent by Second choice: Topographic Involvement proper digits). Third choice: Etiology Iv. Supplemental (may be coded from ‘supple- Fourth choice: Anatomical Site (of lesion) mentary terms” in nomenclature book) Fifth choice: Severity of Involvement A. Psychological evaluation Sixth choice: Degree of Muscle Tone B. Physical status Seventh choice : Supplemental Data C. Convulsive seizures D. Posture and locomotive behaviour pattern The first 3 choices above should be used E. Eye-hand behaviour pattern by the medical record librarian and by F. Visual status doctors discharging patients with cerebral C. Auditory status palsy from hospitals or institutions so as to H. Speech disturbances establish a common understanding and uni- V. Neuroanatomical (select code number from formity to hospital records. It is under- “Brain,” pp. 38-39-40’) stood that the neuroanatomical classifica- tion (Fourth choice) is to be used when it VI. Functional Capacity (degree of severity) can be proven, but not by presumption. Class I The majority of the members approve of Class II tension, non-tension, dystonic, and tremor- Class III like types of athetosis. The other types were Class IV rejected for various reasons. Probably, some VII. Therapeutic of the other types would have been ac- Class A cepted if understood by the members. Each Class B Class C type is described and defined herein. There is a general lack of agreement on the van- (Note:Class VID and VII, above, need not be coded by ous terms used in cerebral palsy. Defini- librarian, but added to the final diagnosis.) tions of these terms are now being made by a committee for a meeting in 1955. CONCLUSIONS There is a good deal of evidence that The majority of the members of the neurological signs and symptoms change in American Academy for Cerebral Palsy voted the child with cerebral palsy as the nervous to exclude progressive neurological diseases system matures and that one must be cau- and neoplastic diseases of the brain from tious in making a final descriptive or symp- the classification of cerebral palsy. The tomatic diagnosis in infancy. The pattern of

Downloaded from www.aappublications.org/news by guest on September 27, 2021 852 MINEAR - CLASSIFICATION OF CEREBRAL PALSY changing neurological symptoms from in- sophical Method. Oxford, Clarendon, fancy through childhood should be studied. 1933, pp. 26-53. 5. Fay, T. : Cerebral palsy; medical con- The high cervical syndrome described by siderations and classification. Am. J. Fay is being confused with cerebral palsy. Psychiat., 107:180, 1950. This syndrome needs further study. It 6. Cruickshank, W. M., and Raus, G. M., should be determined whether the term editors: Cerebral Palsy; Its Individual cerebrospinal palsy would not be more ap- and Community Problems. Syracuse, Syracuse Univ. Press, 1955. propniate for the entire neurological group. 7. Fay, T. : Problems of rehabilitation in pa- The American Medical Association’s tients with cerebral palsy. Delaware Standard Nomenclature of Diseases and State M. J., 18:57, 1946. Operations (Fourth Edition), commonly 8. Hillman, J. W., Sprofkin, B. E., and Par- used by medical record librarians, is not rish, T. F. : Birth injury of the cervical suitable for the classification of cerebral spine producing a “cerebral palsy” syndrome. Am. Surgeon, 20:900, 1954. palsy. In this edition, all cerebral palsy is 9. Fay, T. : Personal communication. coded “Cerebral spastic infantile paralysis” 10. Gesell, A., and Amatruda, C. : Develop- with supplementary terms added to denote mental Diagnosis, 2nd Ed. New York, various types. Hoeber, 1947. A complete classification for cerebral 11. Cuibor, G. P. : Personal communication. 12. Keats, S. : Cerebral palsy; recent advances palsy is presented, using the majority opin- in diagnosis and classification. J. M. Soc. ion from questionnaires sent to the mem- New Jersey, 45:491, 1948. hers of the American Academy for Cerebral 13. Minear, W. L. : Cerebral palsy; spasticity. Palsy during 1953 as a basis. Rocky Mountain M. J., 48:757, 1951. 14. Ibid.: Cerebral palsy. Southwestern Med., 31:367, 1950. REFERENCES 15. Minear, W. L., Binkley, E., and Snow, W. B. : Report, Nomenclature and Classi- 1. American Medical Association: Standard Nomenclature of Diseases and Opera- fication Committee, presented before American Academy for Cerebral Palsy, tions, 4th Ed., Plunkett, R. J., editor. New York, Blakiston, 1952. November, 1954. 16. McBride, E. : Personal communication. 2. Balf, C. L., and Ingram, T. T. S. : Problems 17. Perlstein, M. A. : Infantile cerebral palsy; in the classification of cerebral palsy in classffication and clinical correlations. childhood. Brit. M. 2:163, 1955. J., J.A.M.A., 149:30, 1952. 3. Byers, R. K. : Evolution of hemiplegias in 18. Perlstein, M. A. : Personal communication. infancy. Am. J. Dis. Child., 61:915, 19. Phelps, W. : Personal communication. 1941. 20. Pohi, J. F. : Cerebral Palsy. St. Paul. Bruce 4. Collingwood, R. C. : An Essay on Philo- Pub. Co.. 1950.

Downloaded from www.aappublications.org/news by guest on September 27, 2021 SPECIAL ARTICLE: A CLASSIFICATION OF CEREBRAL PALSY W. L. Minear Pediatrics 1956;18;841

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/18/5/841 Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on September 27, 2021 SPECIAL ARTICLE: A CLASSIFICATION OF CEREBRAL PALSY W. L. Minear Pediatrics 1956;18;841

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pediatrics.aappublications.org/content/18/5/841

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1956 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

Downloaded from www.aappublications.org/news by guest on September 27, 2021