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Case Presentation

Identifying Loss of Function Caused by CervicalSpondylotic Myelopathy in Young Adults WithNonathetoid Spastic Cerebral Palsy

Jill R. Meilahn, DO

INTRODUCTION

Cervical spondylotic myelopathy (CSM) is the most common cause of nontraumatic spasticparaparesis and quadriparesis [1]. Symptom onset usually occurs after the age of 40 years2]; however, conditions such as cerebral palsy (CP) may increase the risk for the occurrencef spondylotic or stenotic disease at an earlier age [2]. CP is a static, nondegenerativeondition, yet functional deterioration upon aging frequently is ignored or attributed to CPithout further exploration [3]. Cervical stenosis is 1 of 4 common musculoskeletal

onditions frequently found to contribute to functional decline in adults with CP [3]. Thether common conditions that may contribute to decline include patella alta, degenerativerthritis caused by hip dysplasia, and lumbar spondylosis [3]. However, diagnosis often iselayed, highlighting the difficulties in transition from pediatric to adult care for patientsith CP. The literature includes several reports of patients with athetoid CP experiencing

unctional decline as a result of CSM [4-9]. Reports of CSM in patients with nonathetoid,pastic CP are much rarer [10]. This report describes 2 cases of CSM in patients withonathetoid, spastic, diplegic CP that resulted in a loss of function that was not entirelyeversible with surgery.

CASE PRESENTATION

Case 1

A 32-year-old man with spastic CP and mental retardation was referred to pediatricphysiatry by his primary care provider for treatment of spasticity. He was accompanied byhis mother and a caregiver from his group home. He was verbal but needed assistance topresent his history, which was pertinent for long-standing intermittent urinary inconti-nence, a loss of ambulation, loss of ability to use his hands to feed himself, and increasingstiffness. An occupational therapist (OT) had suggested botulinum toxin injections andmade hand splints. He had been seen several months before by his primary care provider fordecreased ambulation and spasticity that was noted by a physical therapist. Records indicatethat the patient was able to walk with the assistance of a walker at this time. The patient wasstarted on 10 mg baclofen 3 times daily with no improvement.

The patient’s mother stated that until the age of 20 years he had been capable ofindependent ambulation without an assistive device. Between the ages of 20 and 31 years hebegan using progressively more elaborate walkers and aides, until about 6 months beforethis visit, when he could no longer walk at all, even with assistance. The patient’s motheralso noted curling of the fingers of his left, dominant hand. He could no longer hold utensils.The right nondominant hand was becoming contracted. His examination showed briskreflexes and clonus in all extremities. Past neurologic examinations were available only 3months before presentation. Some deterioration was evident during this time, particularlyin a decrease in shoulder abduction from 80° to 30°. During previous visits to his primarycare provider, physical therapist, and OT, all symptoms were attributed to CP, and nofurther testing was performed.

Laboratory results were normal for complete blood cell count with differential, basic

metabolic panel, glycohemoglobin, and thyroid-stimulating hormone. Magnetic resonance

PM&R © 2012 by the American Academy of Physical Me1934-1482/12/$36.00

Printed in U.S.A. http://dx.doi.o

J.R.M. Department of Physical Medicine andRehabilitation, Marshfield Clinic, 1000 NorthOak Ave, Marshfield, WI 54449. Address corre-spondence to: J.R.M.; e-mail: meilahn.jill@marshfieldclinic.orgDisclosure: nothing to disclose

Disclosure Key can be found on the Table ofContents and at www.pmrjournal.org

Submitted for publication January 19, 2012;accepted April 23.

dicine and RehabilitationVol. 4, 783-786, October 2012

rg/10.1016/j.pmrj.2012.04.015783

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784 Meilahn LOSS OF FUNCTION DUE TO CSM IN CP PATIENTS

imaging (MRI) of the brain was normal with the exception ofa slight dilation of the fourth ventricle where the shuntcatheter entered. No previous films were available for com-parison, so a shunt series was performed, which revealed agrossly intact shunt in terms of continuity. A radiograph ofthe cervical spine showed extensive laminectomy starting atC2 and extending to C7 as a result of ventricular peritonealshunt revision and infection at age 3 years.

MRI of the cervical spine showed significant cervical de-compression with removal of posterior elements to the levelof C6. Despite this finding, spinal canal stenosis was presentwith cord compression at the C2-C3 level. Signal changes inthe cord were consistent with cervical myelopathy (Figure1A). A computed tomography myelogram revealed signifi-cant epidural scarring that was severely compressing the cordon the right side at C2-C3 and C3-C4. Deformity of the cordwas seen on the right side with atrophy at C2-C3. The patientwas diagnosed with spastic quadriparesis secondary to pro-gressive CSM from epidural scarring and recurrent stenosis,which was particularly worse from C2 to C4-C5.

Surgery was performed to explore the previous laminec-tomy defect with widening of laminectomies from C2 to C6.An intradural mass consisting of a foreign body granulomaand thickened epidural scar was resected. One month aftersurgery, the patient and his mother agreed that his conditionhad improved. Three months after surgery, OT achievedconsiderable improvement in active flexion of the right wrist

Figure 1. Cervical magnetic resonan

and elbow and increased right elbow flexion strength and

right hand grip strength. However, he did not return to thelevel of abilities he demonstrated during his mid 20s.

Case 2

A 25-year-old man with spastic diplegic CP and a history ofseizures first presented to orthopedics for right hip pain. Apelvic radiograph showed mild hip dysplasia likely related tothe history of CP. The patient continued to have increasedhip pain and declining ambulation. He saw another orthope-dic surgeon, had a gait lab assessment, and was scheduled forsurgery on his right hip. The gait lab showed a classic diplegicgait, although the right leg was more crouched and internallyrotated than the left. He did not use an assistive device andstated he had never used one. He displayed level 1 mobility,as determined by the Gross Motor Function ClassificationSystem for Cerebral Palsy, and independence for all activitiesof daily living without any upper extremity spasticity. He wasemployed at a furniture store.

The patient was seen for the first time by pediatric physia-try before surgery to assist with anticipated postoperativerehabilitation. He arrived in a wheelchair with his significantother expressing concern about his deterioration during theprevious 6 to 8 weeks. He reported problems with his legsgiving out and decreased sensation to temperature in the leftlower extremity. Until 6 months earlier, the patient had beenworking at a store moving items and stocking shelves. The

ges from (A) case 1 and (B) case 2.

patient was now having difficulty caring for himself, was

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unable to walk, and reported bilateral hand weakness,cramps in his legs and arms, and bladder and bowel incon-tinence.

On examination, marked spasticity was noted in all ex-tremities with brisk reflexes and sustained clonus. The find-ings of previous neurologic examinations were sparse, but apattern of increasing hip pain and decreased ability to walkand decreased sensation in the hands and lower extremitieswas apparent. A cervical MRI demonstrated multilevel cervi-cal spondylosis with severe central canal stenosis at theC5-C6 interspace as the result of a ventral bony ridge, as wellas very pronounced posterolateral disk extrusion on the rightresulting in severe central canal stenosis with effacement toboth the ventral as well as the dorsal CSF space (Figure 1B).The sagittal images showed myelopathic signal changeswithin the cord at this level. Urgent surgery, including aC5-C6 anterior diskectomy and fusion, was performed.

The patient was discharged 2.5 weeks after surgery, atwhich time he was walking 300 feet by using a wheeledwalker. He had marked improvement in hand function withimproved strength, coordination, and reaction time. Thepatient could perform activities of daily living independentlywith assistive devices. Three months after discharge, he re-ported overall improvement of the paresthesias, numbness,and weakness. He believed that his walking had improved,and he no longer used a walker in the house.

DISCUSSION

Most reports of CP-associated CSM are in patients withathetoid movements of the neck [4-9]. Only one other reportdescribes CSM in patients with nonathetoid spastic CP. Reeseet al [10] described 3 patients with nonathetoid CP and

rofound mental retardation with CSM and loss of function.his report describes 2 cases of CSM in communicativeatients with nonathetoid, nonquadriparetic spastic CP thatesulted in severe functional deterioration but was not fur-her evaluated by multiple physicians because it was thoughto be attributable to CP.

Cervical stenosis is reported to be 1 of 4 common muscu-oskeletal conditions seen in patients with CP, also includingatella alta, degenerative arthritis caused by hip dysplasia,nd lumbar spondylosis; however, neurologic changes toorequently are attributed to the CP without further effort tond a more specific diagnosis [3]. This scenario may be theesult of discomfort or lack of knowledge by physicians whoormally treat adult patients [11]. Difficulties in the transi-

tion from pediatric to adult care in patients with disabilities,including CP, are well documented [11]. Challenges arise asa result of the need to superimpose the complex care neces-sary for people with CP on the fragmented services charac-teristic of the adult health care system [11]. The potential forimprovement in transition from pediatric to adult care lies in

planning ahead, preparing for changes over the life course,

and family involvement [12]. Skills for clinician-patient com-munication should be emphasized in young people with CP,and clinicians should prepare a complete discharge summaryupon transition to allow for continuity of care [11]. In bothcases presented here, communication regarding changes infunction was suboptimal, and unavailability of past test re-sults for comparison in the first case presented may havecontributed to the delay in diagnosis. It is notable that in bothcases presented here, pediatric physiatry raised alarm regard-ing the possibility of CSM in these adult CP patients.

Symptoms of CSM vary widely, and many resemble char-acteristics of CP, including gait disturbance, lower extremitystiffness and jerking, upper and/or lower limb sensory loss orweakness, loss of hand dexterity, urgency of urination and/ordefecation, and occasional urgency incontinence [2], whichmay contribute to new or worsening symptoms being attrib-uted to the underlying CP. In a report of 3 patients withspastic CP and profound mental retardation, Reese et al [10]reported delays in diagnosis from 4 to 8 months because of aninitial attribution of symptoms to CP or cognitive impair-ment. Similarly, McCluer [4] reported substantial delay inthe diagnosis of CSM in 4 patients with athetoid CP. McCluer[4] discussed the challenges of diagnosing neurologic deteri-oration in persons with CP, including difficulty obtaining anaccurate history because of poor verbal skills, failure to noticeor pursue new neurologic symptoms, difficulty documentingaccurate neurologic examinations, disbelief of reports of pre-vious levels of independence in self-care, and difficulty ob-taining good radiographs because of involuntary movementsand body position. Disbelief of patient and parent reports ofpremorbid functioning is thought to be of considerable im-portance here, even though the patients were communica-tive.

The differential diagnosis of increasing spasticity withdeclining function is broad. Spasticity in patients with anunderlying chronic central nervous system disorder can beincreased by most illnesses or noxious stimuli. In the casespresented here, laboratory work was performed to assess forinfection, endocrinopathy, inflammatory myopathy or ar-thropathy, and electrolyte or calcium abnormalities. Theresults of all laboratory tests were normal. In the first casepresented, MRIs of the brain also were performed to assess forhydrocephalus, tumor, stroke, or other new central nervoussystem pathology. In the second case, high suspicion for CSMand clear findings upon cervical spine MRI did not necessi-tate further testing.

Surgical intervention for CSM is considered in patientswith moderate to severe and/or worsening neurological def-icits with the goal of decompressing the spinal cord andstabilization to prevent future spine deformation [2]. Surgicalintervention appears to have been successful for halting theprogression of neurological decline in the cases presentedhere. Both patients regained at least some function, including

the ability to ambulate with an assistive device. In the general

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786 Meilahn LOSS OF FUNCTION DUE TO CSM IN CP PATIENTS

population, improvement in condition is expected in ap-proximately 50%-75% of patients after surgery [2]. Most casereports of surgical intervention in patients with CP and CSMdescribe good outcomes and recovery of some function[5,8,10], with the exception of the report by McCluer in 1982[4]. The experience of the patients described here and thosedescribed by Reese et al [10] suggest that neurologic deteriora-ion can be halted by surgical intervention in patients with CPnd progressive CSM, making a correct diagnosis essential.

CONCLUSION

It is important for clinicians to remember that CP is a static,nondegenerative condition and that deterioration in functionwarrants further attention. Some clinicians have recom-mended screening for cervical stenosis with both clinical andperiodic imaging studies, especially in patients with athetoidor spastic quadriparetic CP [3]. Adult practitioners assumingare of patients with CP should develop a high index ofuspicion for cervical stenosis even in patients without athe-oid movement, as demonstrated here. Rapid diagnosis andurgical correction of spinal compression can lead to regain-ng some or all of the lost function, making awareness andecognition of this condition especially important.

ACKNOWLEDGMENT

I thank the Marshfield Clinic Research Foundation’s Office ofScientific Writing and Publication for assistance in the prep-

aration of this article.

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