THE UPPER SIGNIFICANCE AND OF LOWER JOHN M. LIMBS MAZUR, Front SPASTICITY ANNE ChilcIi’en’s the thoracic level, 26% at the upper lumbar level, Fifty-four per cent of these patients demonstrated upper limbs; limbs; 9% were flaccid in the lower casts, and were less likely less likely to be independent to walk than in activities patients with normal upper important in the evaluation, The purpose of this paper neurological involvement bifida cystica, the paralysis. the various is to level of goals types in of which and The may spastic patterns has with with time because system anomalies lesions no previous versus flaccid and the effect activities of daily involvement of is often complex and been thought to be of and the 1963a,b; J. M. Mazur. MD. Associate Southern Illinois University 62708, USA. A. Stillwell. B App Sci (Phty). M. B. Menelaus. MD. FRCS. Royal Children’s Australia. Requests Hospital. for reprints No. the as resultant Smith para1965). should 2. MARCH associated central the Arnold-Chiari syri ngomyelia, of the cord. of the degree Professor School Deputy FRACS, Flemington be sent 1986 British Editorial Society 0301 620X/86/2038 S2.00 68-B, of such hydrocephalus. and tethering reports 1986 in the upper; orthopaedic of to Mr M. B. Menelaus. and Joint spacecould in late Springfield. Chief Physiotherapist ChiefOrthopaedic Road. Parkville. of Bone We of spasticity Medicine. Surgery 24% procedures, were more at the sacral level. limbs with normal spastic days in the lower in hospital and with spastic upper limbs to require special schools of the lesion, patients. childhood or logical pattern to the level paralysed at the degree of spasticity adolescence, and the effect upon function and prognosis, of in were than is the neuro- spina present paper (MBM) recognised that either a or a flaccid paralysis could be present (Menelaus Moreover, the neurological involvement can malformation, occupying VOL. occur and Drummond believed that the lesion was frean upper motor neurone condition, basing this on electrical stimulation studies of the neural (Stark and Drummond 1971). The senior author change nervous find correlate to perform neurological with myelomeningocele The principal lesion of this spastic 1980). spastic lumbar level and 13% paralysis in the lower those with flaccid paralysis. Patients of daily living and were more likely the the lower motor neurone type. paresis to be flaccid (Sharrard Stark quently belief plaque but were with functional patients. The incidence of neurological on the ability to walk living are described. were evaluated and classified according Thirty-one per cent of the patients were 30% at the lower the classic flaccid limbs. more MENELAUS !vh’Ihow’ne limbs. In addition to the spinal cord level treatment and prognosis of myelodysplastic lesion and degree of spasticity the treatment of myelodysplastic children confusing. limbs, 13% were spastic in the upper and lower Patients with spastic lower limbs required MALCOLM Ho.s1ntal, One hundred and nine children with myelodysplasia and type of paralysis and its effect on functional ability. THE MYELOMENINGOCELE STILLWELL. Rota! i/it’ IN IN Surgeon Victoria Illinois 3052, MATERIAL AND METHODS This study consisted of a functional analysis of children with spina bifida cystica. All children aged 8 to 18 years. inclusive, who lived close enough to the Royal Children’s Hospital, Melbourne, to be recalled and personally examined were were asked and logically disturbed children were included. The parents I I 2 agreed to participate. adolescent excluded with the examiner. The All examinations patients’ histories might affect the two they left 109 children were were performed obtained This and hospital records. operative procedures was directed toward and because All diagnoses. were recorded, those diagnoses neurological of 125 One mentally could children psychoretarded not co-operate for the project. by from the authors. the parents hospitalisations. and Particular attention and operations that involvement. The physical examination included: measuring the head circumference; examining voluntary muscle power by a manual muscle test; assessing sensation with reference to pain and light reflexes, touch; examining Babinski’s sign, position sense. deep tendon clonus and rapid alternating movement; and the assessment ofintention tremor. All abnormalities were recorded, and the patients were grouped according to the neurological level and degree of spasticity. There were two main groups depending on the presence or absence of spasticity in the lower extremities. Each group was then divided into two subgroups depending upon whether or not the upper limbs were normal. 213 214 J, M. MAZUR, Classification. limbs) included the spinal to the Group cord, level no motor, ties were IA lesions (flaccid involving Neurological of the sensory normal. but or reflex Group the of the long area of interruption paralysis activity. central nervous there manifested by or pain-withdrawal was down there was IA except that spastiThis group contained system abnormalineurone signs in the paralysis of the lower segment ofthe cord above with interrup- system an intact lesion The upper extremilower limbs, spastic upper limbs) was similar to Group city was present in the upper limbs. those with associated central nervous ties responsible for the upper motor upper limbs, In Group hA (spastic limbs). the lesion spared the terminal which was, however, cut off from tion was below activity. (flaccid lB tracts. upper spasticity. reflex. Below motor was well below The neurological exaggerated It was believed the cervical spine. level of the lesion was the reflex that as deteralso was recorded pin-prick by the most caudal dermatome with The percentages to determine Fig. Distribution of patients within ofthe spinal cord lesion. Group flaccid paralysis in lower limbs. limbs: IIB. spastic I neurological groups. showing the level IA. flaccid paralysis in lower limbs: lB. spastic upper limbs: lIA. spastic lower upper and lower limbs. classified according to the most caudal intact nerve root mined by motor function. The sensory function and light-touch sensation. each group were calculated I neurone the spasticity was caused by an isolated segment of spinal cord functioning below the myelomeningocele plaque. Group JIB (spasticity in upper and lower limbs) was similar to Group IIA with the addition of spasticity being present in the upper limbs even though the spina bifida defect M. MENELAUS paralysis of the lower the terminal portion of function lesion, A. STILLWELL, ofpatients the prevalence the lower sensory the non-dominant Group in cord IA lesion level side. on the (complete flaccid and normal upper of 59 patients 30 girls (54%), dominant side, paralysis limbs). and and 5 on below the spinal There were a total 29 boys, in this group. ofeach type oflesion. Any asymmetry between the sides of the body was recorded for both the motor and sensory examination. Functional ability. Intellectual ability was assumed to be normal if the child attended a normal school and was within two grades of that expected for the chronological The ages ranged from 8 years I month to I 8 years II months (average 14 years 3 months). The level of the motor lesion was thoracic in 13 patients, upper lumbar in 12. lower lumbar in 20 and sacral in 14 (Fig. I). Within Group IA there were 51 (86%) community walkers, I household walker and 7 non-walkers (Table I). The age. patients in this perform themselves daily activity skills, all but one and maintain their personal Independence in feeding, personal hygiene and dressing was assessed to determine the functional level in activities of daily living. Patients were classified according to their mobility. as community walkers, household walkers and non-walkers. Independence and walking were compared between patients in the four group scored very well in their abilities groups. 1OC a ae C 0 RESULTS The age range months (average was from 14 years 8 years I month 2 months). There and 54 boys. The level of the motor lesion (31%) was thoracic, in 28 (26#{176}/o)upper (30%) lower and sensory patients (58%); motor (4%). level There in 10 patients: lumbar, and in 14 (13%) to 18 years 7 were 55 girls in 34 patients lumbar, in 33 sacral. lesions were at corresponding the sensory level was in 42 patients was right-left The right side levels in 63 lower than the was 70 0 ec C 0 40 70 50 ! at a lower level than the non-dominant left; in the other 2 patients, the lower motor level was on the non-dominant left side. There were 20 patients with sensory asymmetry: I 5 had 112929 S a. 20 Hflfl motor (38%), and higher in 4 patients side asymmetry in motor level in 8 the dominant ec 0 F H IA 0 F H D H 0 F hA lB Fig. F HO PB 2 Relationship between independence in skills of daily living (F, feeding: H. hygiene: D. dressing) and neurological group. Patients with normal upper limbs (Groups IA and IlA) were more likely to be independent than those with spastic upper limbs (Groups lB and IIB). THE JOURNAL OF BONE to being able to feed hygiene (Fig. 2). AND JOINT SURGERY THE (flaccid Group lB pnised only SIGNIFICANCE lower OF SPASTLCITY limbs, spastic (9% of the 10 patients were 6 boys and 4 girls. The months to 18 years 7 months All the pin-prick patients and in this position upper total limbs) series). ages ranged from (average 14 years group sensation patients had an intention strated abnormal rapid the spasticity. The level IN THE had UPPER ing, com- feed sonal hygiene Group JIB 9 years I month). 6 normal light touch, in the upper limbs; 6 tremor, and 7 patients demonalternating movement as well as of the motor lesion was thoracic Table I. and Summary dressing ofall (Fig. 2). CW HW NW Total CW HW NW 6 1 6 13 1 1 Upperlumbar 11 0 1 12 0 Lowerlumbar 20 0 0 20 Sacral 14 0 0 51 1 7 Thoracic Total = CW HW NW Community = Household = Non-walker Group ankle knee reflexes, and lower reflex, reflexes, 8 patients Although there sony awareness Total CW HW in 10, and Group walkers, Group limbs daily VOL. 5 7 0 1 0 1 2 0 0 14 0 0 59 3 2 limbs). This group of 4 3 had had had a positive was no voluntary below the spinal contractures were thoracic lower Babinski’s movement cord lesion, lumbar in two or more in 7 patients, in 9 (Fig. IA, patients in Group IIA and demonstrated good skills living (Fig. 2). All the patients No. per- in all four total series) limbs). There in this group, were 14 6 boys Group IhB CW HW NW Total 1 6 7 0 0 7 7 3 3 4 10 1 2 2 5 2 7 2 0 9 0 2 0 2 0 0 0 0 0 0 0 0 0 0 5 10 10 6 10 26 4 9 14 26 alternating movements as well as spasticity. In this group only one patient was a community walker, 4 were household walkers and 9 could not walk at all. They had poor daily activity skills, only 7 being able to feed themselves ankle clonus, 4 had abnormal hamstring reflex. and no senstimulation I). muscles. The upper lumbar Ten patients IIA were community walkers, 6 were household and 10 were non-walkers (Table I). Like those 6k-B. own NW muscles in 12, in the tibialis anterior in 5, in the gastrocnemius and soleus in 12, in the tibialis posterior in 6, and in the long toe flexor muscles in 5 patients. Ten had lesions their reflexes, and 8 had a positive Babinski’s reflex. All 14 patients showed spasticity or hyper-reflexia in the upper limbs. All the patients had normal light touch, pin-prick and position sensation in the upper limbs. Eight patients had an intention tremor, and 10 had abnormal rapid hA ofthe leg evoked a flexion withdrawal reflex in 8 ofthe 26 patients. Because of the spasticity, contractures developed in the hamstrings in 8 patients, in the peroneal patients motor after themselves. upper lumbar in 5, and lower lumbar in 2. Six patients had increased ankle reflexes, 4 had ankle clonus, 3 had increased knee reflexes, 3 had pathological hamstring Group patients (24% of the total series) comprised I 3 boys and 13 girls. The ages ranged from 8 years 9 months to 18 years 6 months (average 14 years 4 months). All of the patients in this group demonstrated increased deep tendon reflexes indicating an upper motor neurone paralysis below the spinal cord lesion. Twelve patients had an increased increased (spasticity (13% of the look dress Total walker walker (spastic hA 18 could and 8 girls. The ages ranged from 8 years 6 months to I 7 years I 1 months (average 14 years I month). The motor lesions within Group IIB were thoracic in 7 patients, GrouplB IA lesion 21 could and 215 IN MYELOMENINGOCELE the patients Group Spinal LIMBS independently, patients lB did not perform as well as Group IA in daily functions but 6 were independent regarding eat- hygiene LOWER There in 7 patients, upper lumbar in I, and lower lumbar in 2 (Fig. I); three patients were community walkers, 2 were household walkers and 5 were non-walkers (Table I). Group activity AND 2. MARCH 1986 in (Fig. 2). Walking ability. The relationship between spasticity and walking was carefully analysed. In the patients with thoracic lesions, the number ofcommunity walkers decreased from 6 out of 13 in Group IA to I out of7 in Group lB (both groups having flaccid lower limbs), to none in Group upper walkers hA and none lumbar lesions, in Group IA, Group hA and I out of5 in Group IIB. Similarly, in the patients with lower lumbar lesions, all were community walkers in Groups IA and lB. 7 out of 9 in Group IIA and none in Group JIB. All of the patients with sacral motor lesions were in Group IA and all were community walkers. Hydrocephalus. Spasticity in the upper limbs was related to the number lus. Patients in had normal upper in the activities of except one could in Group IIB. In patients with I I out of 12 were community none in Group lB. 3 out of 10 in of shunt in Groups operations IA and shunt procedures each while IIB had an average of four each respectively. shunts; both were Two patients patients with done hA had for hydrocepha- an average of two patients in Groups lB and and five shunt procedures in the series had infected thoracic lesions in Group 216 J. M. MAZUR. IIB. Only one patient had a head A. STILLWELL, circumference greater than two standard deviations from normal and she was also a Group [lB patient with a thoracic lesion. Orthopaedic admissions. Patients in Group IA required fewer shorter in orthopaedic admissions periods of immobilisation Groups lB. IIA, or and than IIB. The operations did the number of who had spasticity more dren likely to require in Group lB and special schools In the past. in their upper limbs, bifida is equally important. were the cord below and were I) had complete the myelomeningocele loss were spinal cord of paralysis, Group a flaccid paraparesis below the spinal II patients have a spastic paraparesis. (Group below however, cystica I patients cord The lesion, largest of sensation and plaque. Presumably only lesion. spashave Group group reflexes there no functioning neural elements below the plaque, or lesion involved the terminal portion of the spinal so that function was intact down to a certain level, which there was impairment of motor, sensory, reflex activity. Group II patients had a “gap” in cord function with loss of motor, sensory, distal to which was an intact but cord. In the isolated cord, spasticity and reflex activity, isolated segment of and stretch reflexes may be striking. Tonic reflexes could be elicited: for example. stroking the dorsum of the foot evoked foot or toe extension, Although there was no voluntary movement of the legs and no central response to pin-prick, the slightest stimulation at any point of the leg generally evoked a fiexion-withdrawal reflex. Theoretically, there would be a third group in which patients would have a “skip long normal lesion”. descending cord In these tracts showing and then linking would then expect area there and the of lower patients running no lower up with to find motor there would through an motor an intact voluntary neurone neurone segment movement denervation. infants and we studied and adolescence; the type change with age because be normal area of abfunctions, below. One below the In our series were no patients with a “skip lesion”. Stark and Baker (1967) examined newborn infants presented a study similar to ours. They found 3% of spina bifida patients to be neurologically normal; patients in of neurological of associated as a spacecord. (2) All care of the senior author (MBM); this would exclude neurologically normal or near normal children as they would not have been referred for orthopaedic care. In the present study. each group was divided into subgroup A, those with normal upper limbs, and subgroup B, those with spasticity in the upper limbs. Spasticity shunts in the upper limbs required to control B subgroups those had pendence and 70% activities. schooling: limb as many spasticity to the number of Patients in the shunt operations adversely as affected inde- in activities of daily living: 8 1 % of Group ofGroup IIA were independent in feeding, and dressing Group IIB Upper only 4 of 10 children IIB attended normal and were within their chronological lB and lB three related Group two 3 of thoracic and lumbar All patients with sacral IA. One would not II as the plaque would functioning spinal cord IA per- ofGroup in all also seemed and 77% of in Group schools. Patients with in all groups. were in Group lesions in Group there to be any while only 60% were independent limb spasticity of Group IA 85% attended normal school their expected level for found ever, was related hydrocephalus. twice in A. Upper sonal hygiene and 29% of classified according to the level of the This study demonstrates that the type tic or flaccid, two grades of age. Patients special schools. Six of the 10 chilI I ofthe 18 in Group [lB attended DISCUSSION with spina newborn central nervous system abnormalities such occupying lesion or tethering ofthe terminal our patients were under the orthopaedic for the disabled. patients studied hospital Scholastic ability was correlated with the the upper limbs. Eighty-five per cent of in Group IA and 77% ofpatients in Group hA school and were within for their chronological They late childhood lesion might of attended a normal the expected level 43% similar to our Group I; 50% similar to our Group II; and 3% had “skip lesions”. The differences between our series and theirs may be related to the following. ( I) and patients admissions for orthopaedic procedures averaged 3, 5, 4 and 5 per patient respectively for Groups IA, lB. IIA and IIB. and the number oforthopaedic operations averaged 4, 7, 6 and 7 per patient respectively. Periods in plaster casts averaged 17, 30, 27 and 30 weeks per patient for Groups IA, lB. IIA and IIB respectively. Schooling. function patients M. MENELAUS to hA grades of age. while 14 in Group lesions lesions, expect were howsacral be too caudal for below the level of the lesion. One would expect sacral lesions in Group lB as it would be possible to have a sacral plaque and an associated cerebral anomaly creating spasticity above the plaque; there were, however, no such cases in the series. more Orthopaedically, patients with spasticity difficult to manage. Patients in Groups IIB required 60% more orthopaedic more operations and longer in casts were much lB. hA and hospital admissions, than did patients in Group IA. Literature in the past has related walking ability to the level of the motor lesion. Our patients’ walking ability compares favourably with those reports (Hoffer et a!. 1973; De Souza and Carroll 1976; Feiwell, Sakai and Blatt 1978; Stillwell and Menelaus 1983). Figures comparing walking abilityto neurosegmental meaning. however, unless the presence spasticity that is taken into account, Our level have or absence data clearly little of shows the presence ofspasticity adversely affects walking. Patients in Groups lB and IIB were handicapped abnormal not only upper weaker of crutches Groups IIA limb functions; these spastic but less coordinated, making difficult. and IIB, THE Spasticity caused JOURNAL in the lower impaired overall OF BONE AND by limbs were the control JOINT limbs, in function; SURGERY THE these patients had SIGNIFICANCE persistent OF SPASTICITY contractures IN THE and lower UPPER limb deformities which hampered orthotic fitting and eventual walking. Patients in Group IA (flaccid lower limbs, with normal upper limbs) had the best prognosis, functioned best in activities ofdaily living and were the best walkers. The observation and documentation of the precise neurological is important child’s status for potential activities; ofeach child three reasons: ability (2) to give to realistic with (I) walk and expectations myelomeningocele to determine perform AND LOWER LIMBS created one contracture important. therefore, fled according cid in addition the motor that to whether to grouping their limbs arc them according wish to thank ofthe manuscript. Vicki De Souza U, Carroll N. Ambulation patient. J Bone Joint Surg (Am] altered if spasticity nise the difference voluntary voluntary VOL. 68--B. into control control. No. account of patients management is present. between 1986 evaluation, with may myelomenneed to be The surgeon must recoga spastic muscle under no and an overpowering Transferring a spastic 2. MARCH in the Ann Jones for the muscle under muscle that had of the braced mvelomeningocele 1976:58 A: I I I 2 8. E, Sakai I), Blatt T. The effect of hip reduction patients with myelomeningocele: potential gains surgical treatment. J BoneJoint Su[Ain] 1978:60 Hoffer MM, Feiwell lation in patients 1973:55 A:137 Menelaus MB. T/zt’ orthopat’du ed. Edinburgh etc: Churchill WJW. Spina Smith ED. Spina Springfield, Stark GD, Baker GC. in myelomeningocele. Stark GD, Des in of on function and hazards A: 169 73. F, Perry R, Perry J, Bonnett C. Functional ambuwith myelomeningocele. J Bone Joint .Surg [.4i;z] 48. Sharrard taken and Feiwell cance be Morris REFERENCES to the parents; Sharrard WJW. Meningomyelocele: closure ofthe sac. Proc Soc and prognosis Orthopaedic spastic or flacto the level of the normal pniate surgery. The orthopaedic surgeon. more than any other clinician managing these patients. has the greatest opportunity to observe neurological deterioration. Spasticity is clearly an important factor. Its signifishould may simply lead to another. It is these patients should be classi- lesion. The authors preparation and (3) to decide on the appropriate management. In addition, it is important to observe any deterioration in the neurological status which may, ifdue to syningomyeha or cord tethering. be stabilised or improved by appro- treatment, ingocele. 217 IN MYELOMENINGOCELE hifida Illinois: hifida. 0/ Livingstone. hifida S1fl(1 prognosis i/ic total Thomas, neurological Lb’s Med of immediate 1963h: involvement .Veurol Drummond M. The spinal cord itled Child A/euro! 197 I : 13 suppl operative 12. I : 190 care Of s,;iiial 1965. Child 2nd (v.stica. 1980. i%-Ied 1963a:56:5l() Paraplegia and CC The ?;lciFzag(’nle?lt 9. I?1v(’lolnc!un,gocelc. of the lower limbs 1967:9: 732 44. lesion in myelomeningocele. 25: 1 15. Stillwell A. Menelaus MB. Walking ability in mature spina hifida. .1 Pediatr On/sop 1983:3: 184 90. patients with