Are Some Cases of Spina Bifida Combined With Cerebral Palsy? A Study of 28 Cases

By Ozaras, N; Yalcin, S; Ofluoglu, D; Gureri, B; Et al

Aim. This study is a description of a group of children with spina bifida who present with neurological impairments resembling cerebral palsy. Spina bifida is a complex congenital spinal anomaly causing paraparesis. Some children with spina bifida have neurological impairments, which fit into the definition of cerebral palsy. Extensive spasticity discordant with the level of the spina bifida lesion, upper extremity dysfunction and cognitive impairment are suggestive of concomitant cerebral palsy in these cases. The probable etiology for this problem may be neglected hydrocephalus, meningitis or other brain lesions common in spina bifida.

Methods. In this study we have reviewed our cohort of 365 patients and found 28 cases with the abovementioned findings. Main evaluation parameters used were the Ashworth scale for spasticity, Green and Banks modified classification for hand function, cognitive function, mental status, ambulation and lesion level.

Results. Twenty-eight out of 365 children with spina bifida had neurological impairments resembling cerebral palsy. Their mean age was 59.941.3 (range, 16.8-31.2) months. Seventy percent of the patients were nonambulatory and therapeutic ambulation only was present in 30% of patients. Seventy percent of the children had spasticity of Ashworth grade 2 or higher. Upper extremity dysfunction, and cognitive impairment were also observed in 80% of the patients involved in this study. The spinal lesion was 60% thoracal and 20% upper lumbar, and none of the cases had signs of spinal tethering.

Conclusion. We believe that this group of children with spina bifida may be regarded as having concomitant cerebral palsy. This fact implies that the management of this group of patients having mixed findings must be changed accordingly.

Key words: Spina bifida * Cerebral palsy * Hydrocephalus.

Cerebral palsy (CP) is characterized by disorders of movement and posture caused by a non-progressive injury to the immature brain.1 The distinctive characteristic of these syndromes is the change in muscle tone and posture, both at rest and with voluntary activity. The underlying pathological process in the brain does not progress and occurs in the prenatal, perinatal and early postnatal periods. This period is termed the early formation of brain and includes the first 2 years of life in most definitions. However, some patients up to age of 7 years have been reported.2

CP is one of the most common disabilities affecting children. The reported incidence varies from approximately 2 to 3/1 000 live births in the United States 2 to 8/1 000 live births in our country.3 Recent improvements in neonatal care have not decreased the incidence of CP since the survival rates of small gestational age and premature babies have increased.1

Spina bifida (SB) is the term used to describe the failure of fusion of the neural folds during the neurulation phase of embryologie development. The etiology is multifactorial. The open form of SB known as SB aperta can be seen as meningocele, myelomeningocele or myeloschisis. Children with meningomyelocele are especially prone to various forms of neurological impairment.

Both geographical and racial variations in incidence of SB have been reported. The incidence was reported to be 0.1-0.2% in the United States.4 Ninety percent of children with meningomyelocele will develop clinically significant hydrocephalus. Hydrocephalus is often present at birth or discovered prenatally by ultrasound examination. If progressive hydrocephalus is not treated survival and intellectual performance of children will be influenced negatively.4,5

Ventriculo-peritoneal shunting is the treatment of chioce for hydrocephalus. The 2 most common late complications associated with shunts are obstruction and infection, which also effect survival and intellectual performance.4

The purpose of this study is to investigate if hydrocephalus and other cerebral injury may cause CP in children with meningomyelocele. In addition, whether these children with meningomyelocele can also be called children with CP.

Materials and methods

There were 365 children with SB aperta in our pdiatrie rehabilitation out-patient clinic cohort. The whole cohort was collected over a period of 5 years with the age range of the population being from O months to 22 years. Mental status, muscle strength and tone, superficial and deep sensation, deep tendon and pathological reflexes, level of lesion, head circumference, hand function, mobility and ambulation level, presence of shunt and shunt function were assessed in all children. Cranial and thoraco-lumbar magnetic resonance imaging (MRI) were performed in children with suspected upper motor neuron findings or progressive hydrocephalus.

Mental status was grossly assessed on a scale of 3. Patients who had no interest and contact with environment were given 1 point, who had some interest and contact with environment were 2 points, and 3 points were given to those who had more interest and contact with environment. Grade 3 corresponds to normal. Muscle strength in small children was also assessed using a scale of 3 in which O was for no contraction in the muscle, 1 for presence of muscle contraction with movement of the joint, and 2 for full function. Muscle strength has been tested in all 4 extremities. In addition, the present of selective motor control, loss of sensation and abnormal movements are also tested. Muscle tone was evaluated using the modified Ashworth scale (O: no increase in muscle tone, 1: slight increase in muscle tone, 2: more marked increase in muscle tone through most of range of motion, 3: considerable increase in muscle tone, passive movement difficult, 4: affected parts rigid in flexion or extension).5

Level of lesion was assessed using the International Myelodysplasia Study Group Criteria.6 Thereafter, the lesion levels were classified into upper thoracal, lower thoracal, upper lumbar, lower lumbar and sacral levels.

Green and Banks modified classification was used for evaluating hand function (Poor – use of hand only as a paperweight. Fair – use of the hand as an aid but no effectual use of the hand in dressing. Good – use of the hand as help in dressing and eating. Excellent – good use of the hand in dressing, eating, and general activities).3

Ambulation levels were as follows: non-ambulatory, therapeutic ambulation only, household ambulator, functional ambulator. For those children who were non-ambulatory we determined the primary means of mobility as crawling and creeping.

We measured head circumference using a hand tape from frontal to occipital region and also checked for signs of shunt dysfunction.

Results

Cognitive dysfunction, hand dysfunction, increase in muscle tone and pathological reflexes were regarded as possible signs of CP in these children. These symptoms were found in 28 out of 365 children with meningomyelocele. The average age was 59.941.3 (range, 16.8- 31.2) months. Eight children were male and 20 were female. Lower extremity muscle tone was increased and upper extremity dysfunction was present in all children. MRI did not reveal tethered cord findings in any of these patients. All patients had hydrocephalus, which was not treated appropriately or had not responded to treatment. Patients’ findings are shown in Table I.

Discussion

The findings of upper motor neuron syndrome in SB such as spasticity, upper extremity dysfunction and change in mental status are common also to CP. The etiology of these findings is generally attributed to either spinal tethering or hydrocephalus.6 Other central nervous system abnormalities that may lead to a CP like syndrome are meningitis, meningoencephalitis and obvious brain malformations. None of our group of patients had a central nervous system infection. For children who do not have spinal tethering, these signs may also be regarded as part of the syndrome of CP since CP by definition is an insult to the immature brain tissue due to a non-progressive lesion, leading to neuromotor control disorder.7 In isolated hydocephalus, sequela is regarded as CP if seen in the first 2 years of life.

Hydrocephalus is one of the most common symptoms in patients with SB. Ninety percent of children with meningomyelocele will develop clinically significant hydrocephalus.4

The one set of hydrocephalus in some patients does not require treatment although if hydrocephalus is progressive and is not treated appropriately. Survival and intellectual performance of children will be influenced negatively.4,6

Some investigators showed that active signs and symptoms of hydrocephalus were found in 47.7% associated with mental sub- normality in 22.1%.7 In the same study varying degrees of weakness of the muscles of lower limbs were detected in 98.3% cases, spasticity was found in 10.5% and ataxia in 2.9%. Patients with 3 or more shunt operations for correction of hydrocephalus required more time to complete hand function tests and had lower mental status than patients with no shunt operations.8 Mataro etal. suggested that shunt placement improves neuropsychological functioning, especially in verbal and visual memory and attention and cognitive flexibility in patients with SB.9 In this study, 19.1% of 40% was found to be treated appropriately. Mental sub-normalities and upper limb dysfunction were present in 7.9% of patient\s. These symptoms were common also to CP.

It has also been shown that patients with higher level spinal cord deficits (particularly those with thoracic level lesions) required more time to complete hand function tests and had significantly lower mental status than patients with lower level lesions.10 Jansen et al. assessed 25 patients with SB, suggesting that poor hand function correlated strongly with hydrocephalus.11 In this study, most of the patients had thoracic level lesions, poor mental status, fair or poor hand function. Most of them were not independent ambulators. However, 20% of patients had lower lumbar level lesions and also showed poor mental status and hand function. We believe this was a result of unadequately treated hydrocephalus in these patients.

TABLE I.-Patients’ findings.

All our patients had grade 2 or more spasticity according to the modified Ashworth scale. Spasticity is a common finding in SB when spinal tethering is present. However, in our cases, spinal tethering was not found on MRI, moreover, we did not expect to find symptomatic spinal tethering in this age group. This suggested that spasticity resulted from upper motor neuron lesion due to hydrocephalus.

Conclusions

The findings of increased spasticity in the absence of tethering, cognitive impairment and upper extremity dysfunction in these children suggest that this group of SB children may be regarded as having CP as well. This implies that the management plans for this group of patients having mixed findings must be changed accordingly. In SB bracing is started with the highest brace possible, reducing the brace size as the child’s condition allows, however, in CP, the brace is generally limited to an ankle foot orthosis (AFO). Due to upper extremity dysfunction in certain severe diplegics and total body involved children with CP, use of walkers and crutches are limited whereas in SB they are necessary for augmentative mobility. The issue of mental problems, upper extremity dysfunction and spasticity in children with SB must be evaluated in infancy and early childhood so that necessary rehabilitative measures may be taken as early as possible. Therefore, we believe that SB children showing signs of CP must be distinguished early and that this group of children undergoes appropriate rehabilitation interventions.

References

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2. Stempien LM, Gaehler-Spira D. Rehabilitation of children and adults with cerebral palsy. In: Braddom RL. Buschbacher RM, Dumitru D.Johnson EW. Matthews D, Sinaki M. editors. Physical Medicine & Rehabilitation. 1st ed. Philadelphia: WB Saunder; 1996. p.l 133-49.

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4. Hays RM, Massagh TL. Rehabilitation concepts in myelomeningo cele. In: Braddom RL, Buschbacher RM. Dumitru D. Johnson EW, Matthews D. Sinaki M, editors. Physical Medicine & Rehabilitation. 1st ed. Philadelphia: WB Saunder; 1996.p.ll33-9.

5. Bohannon RW. Smith MB. Interrater reliability on a modified Ashworth scale of muscle spasticity. Phys Ther 1987:67:206-7.

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7. Gingold M, Iannaccone ST. Cerebral palsy and developmental disabilities. In: Lazar RB, editor. Principles of Neurologic Rehabilitation. 1st ed. New York: McGraw-Hill; 1998.p.153-73.

8. Singh U, Gogia VS. Rehabilitation of patients with spina bifida. Indian J Pediatr 1997;64 (6 Suppl):77-82.

9. Mataro M, Poca MA, Sahuquillo J, Cuxart A, Iborra J, de la Calzada MD etal. Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocaphalus. J Neurol Neurosurg Psychiatry 2000:68:615-21.

10. Mazur JM, Ayhvard GP, Colliver J, Stacey J1 Menelaus M. Impaired mental capabilities and hand function in myclomeningocele patients. Z Kinderchir 1988;43 Suppl 2:24-7.

11. Jansen J, Taudorf K. Pederson H.Jensen K, SeitzbergA, Smith T. Upper extremity function in spina bifida. Childs Nerv Syst 1991 ;7: 67-71.

N. OZARAS1, S. YALCIN2, D. OFLUOGLU1, B. GURERI1, C. CABUKOGLU2,3, B. EROL2

1 Department of Physical Medicine and Rehabilitation

Marmara University School of Medicine, Istanbul, Turkey

2 Department of Orthopaedic Surgery and Traumatology

Marmara University School of Medicine, Istanbul, Turkey

3 Private Hospital of Pendik Sifa, Istanbul, Turkey

This paper was presented at the 1st ISPRM Congress. July 7-13, 2001. Amsterdam, The Netherlands.

Submitted for publication January 12, 2004.

Accepted July 3, 2005.

Address reprint requests to: N. zaras. MD, Marmara Tniversitesi Tip Fakultesi Hastanesi, Tophanelioglu C. No:15/13 Altunizade, Istanbul, Turkey. E-mail: dufluoglu@hotmail.com

Copyright Edizioni Minerva Medica Sep 2005