R

StfefidotItdtfrpoapsfrhar

Nc3

I

lti

FI

©d

eview Article

Ischemic Spinal Cord Infarction in ChildrenWithout Vertebral Fracture

Jessica R. Nance, MSc and Meredith R. Golomb, MD, MSc

latrtird

M

P

odiswrnif

L

Eti

R

C

hbvt

CD0

pinal cord infarction in children is a rare conditionhat is becoming more widely recognized. There areew reports in the pediatric literature characterizingtiology, diagnosis, treatment, and prognosis. The riskactors for pediatric ischemic spinal cord infarctionnclude obstruction of blood flow associated with car-iovascular compromise or malformation, iatrogenicr traumatic vascular injury, cerebellar herniation,hrombotic or embolic disease, infection, and vasculitis.n many children, the cause of spinal cord ischemia inhe absence of vertebral fracture is unknown. Imagingiagnosis of spinal cord ischemia is often difficult, dueo the small transverse area of the cord, cerebrospinalluid artifact, and inadequate resolution of magneticesonance imaging. Physical therapy is the most im-ortant treatment option. The prognosis is dependentn the level of spinal cord damage, early identificationnd reversal of ischemia, and follow-up with intensivehysical therapy and medical support. In addition toummarizing the literature regarding spinal cord in-arction in children without vertebral fracture, thiseview article adds two cases to the literature thatighlight the difficulties and controversies in the man-gement of this condition. © 2007 by Elsevier Inc. Allights reserved.

ance JR, Golomb MR. Ischemic spinal cord infarction inhildren without vertebral fracture. Pediatr Neurol 2007;6:209-216.

ntroduction

Spinal cord infarctions in children can have devastatingong-term consequences, but there are few descriptions ofhe etiologies, treatments, and outcomes of pediatric cordnfarction in the literature. Spinal cord infarction occurs

rom the Division of Pediatric Neurology, Department of Neurology;ndiana University School of Medicine, Indianapolis, Indiana.

ER

2007 by Elsevier Inc. All rights reserved.oi:10.1016/j.pediatrneurol.2007.01.006 ● 0887-8994/07/$—see front matter

ess frequently in children than does cerebral infarctionnd it can be difficult to distinguish from other myelopa-hies [1-4]. Cord infarction may be ischemic or hemor-hagic; the etiologies, with some exceptions, are similar tohose in cerebral infarction. Here, we present two cases ofschemic spinal cord infarction in children and provide aeview of the literature, with discussion of pathogenesis,iagnosis, treatment, and outcome.

ethods

atient Identification

Patient cases at our institution were identified by survey of membersf our pediatric neurology division and by ICD-9 search of the patientatabase between the years 1997 and 2005, using the following codes todentify patients with spinal cord infarction: 336.1 (acute infarction ofpinal cord) and 336.9 (unspecified disease of spinal cord) [5]. Patientsere included if they had focal hyperintensity on T2-weighted magnetic

esonance imaging of the spinal cord consistent with infarction and hado evidence of vertebral fracture on spinal X-ray or magnetic resonancemaging. Of 65 patients initially identified by the search, 2 met criteriaor inclusion.

iterature Review

Ovid MEDLINE and Ovid OLDMEDLINE were used to search thenglish-language literature using keywords “spinal cord” and “infarc-

ion,” and limiting the search to “all child 0-18.” Additional reports weredentified in the reference lists of relevant articles.

eport of Cases

ase 1

The patient was a previously healthy 14-year-old female who beganaving difficulty breathing at school immediately after experiencing aurning sensation down her neck and back. In the nurse’s office, sheomited, then rapidly became unable to move her arms and legs. She wasaken to the emergency room, where she had progressive difficulty

ommunications should be addressed to:r. Golomb; Indiana University School of Medicine; Building XE40; 575 West Dr.; Indianapolis, IN 46202.

-mail: mgolomb@iupui.edueceived September 5, 2006; accepted January 8, 2007.

209Nance and Golomb: Pediatric Spinal Cord Stroke

biaat

Sscw

uTwumiaictw

racbtwhSv

C

bc

irrsOtStiN

adst

ww

ddnbhTdSn

Ctfitds

C

das

E

o1iRtrpto1

cwiw

F(hlt

2

reathing. After losing her gag reflex and ability to vocalize, she wasntubated. Neurologic examination revealed weakness of facial musclesnd absent cough. She was flaccid and areflexic in all extremities,lthough she did exhibit minimal withdrawal in her legs and respondedo light touch throughout.

Magnetic resonance imaging of the brain on admission was normal.pinal magnetic resonance imaging performed 48 hours after admissionhowed an irregular T2 hyperintensity in the lower medulla and anteriorord from C1 to C7 and at T3. There was no enhancement. Diffusion-eighted imaging was not done.The patient spent the next 4 weeks in the intensive care unit. She

nderwent an extensive infectious disease work-up, with negative results.here was no information in her medical record regarding thromboticork-up, history of birth control pills or injections. Spinal fluid wasnremarkable, but she was given a presumptive diagnosis of transverseyelitis and was treated with steroids, plasmapheresis, and intravenous

mmunoglobulin, without clinical response. She required tracheotomynd gastric feeding tube placement, and spent the next 6 weeks inntensive inpatient rehabilitation, with only minimal, inconsistent in-rease in the movement of her right arm. In the months after the event,he patient experienced severe anxiety and had difficulty sleeping. Thereas no history of any illness prior to the onset of the event.Magnetic resonance imaging of the spine 6 months after the event (Fig 1)

evealed linear T2 hyperintensity in the anterior cord from C2 to C5 andt T3. All intervertebral disks and vertebral bodies were normal. Herlinical course and magnetic resonance imaging at 6 months were felt toe more consistent with spinal cord infarction in the anterior spinal arteryerritory than with transverse myelitis. At 18 months after the event, sheas able to turn her head and talk, had minimal movement of her rightand, and decreased pinprick sensation in the C3 dermatome and below.till incontinent of urine and stool, she remained wheelchair-bound andentilator-dependent, with continued anxiety and difficulty sleeping.

ase 2

The patient was a 17-year-old male football player with a history ofack pain and palpitations. He was started on beta-blockers by hisardiologist. At home he became dizzy, fell down the stairs, and was

igure 1. Patient 1. Digitally enhanced magnetic resonance imagingTR/TE � 3650/104 ms) at 6 months after the event, showing T2yperintensity (arrows) in the anterior medulla and along the anterior

aength of the cord from C2 to C5 and at T3 consistent with infarction ofhe anterior spinal artery territory.

10 PEDIATRIC NEUROLOGY Vol. 36 No. 4

mmediately quadriparetic, with severe neck pain and numbness of hisight face, left arm, and both legs. He was brought to the emergencyoom, where he regained movement of his upper extremities, and showedome movement of his proximal lower extremities within several hours.n examination several hours after admission, he had minimal flexion of

he hips and knees. There was no voluntary movement of the feet or toes.ensory exam revealed absence of all sensory modalities in the feet up to

he shin. Reflexes were 2�/5 throughout the lower extremities. Abdom-nal reflexes were brisk. The patient retained bowel and bladder function.umbness of his face and arms resolved over the next few days.The initial magnetic resonance imaging, performed on the first day of

dmission, was read as normal. Diffusion-weighted imaging was notone. A second magnetic resonance imaging, 1 month after the event,howed a vague area of T2 hyperintensity in the thoracic cord, which washought to be artifactual. T2-weighted axial images were not obtained.

The patient continued to have lower extremity weakness and numbnessith very slow improvement. He was discharged home 4 days later in aheelchair with outpatient physical therapy three times weekly.Magnetic resonance imaging performed 3 months after the event

emonstrated multiple Schmorl’s nodes in the thoracic spine, withesiccation of intervertebral disks. Subtle T2 hyperintensity was alsooted within the central region of the cord extending from T5 to T9, onoth axial and sagittal T2 imaging (Fig 2). Another more subtle T2

yperintensity was seen within the upper cervical cord at the C2 level.hese lesions were seen on the high-resolution screens in the neurora-iology department. Retrospective viewing of previous films showedchmorl’s nodes and similar T2 hyperintensity on T2 sagittal imaging, buto confirmation on T2 axial view was available.Thrombotic work-up revealed compound heterozygosity for the

677T and A1298C mutations of the methylenetetrahydrofolate reduc-ase gene (MTHFR), and the patient was treated with daily aspirin andolic acid. The patient benefited from intensive physical therapy, whichncluded 6 weeks at a rehabilitation hospital. At 7 months after the event,he patient was able to ambulate with a cane. He continued to haveecreased proprioceptive sense in his toes and decreased vibratoryensation to the knee.

omment

Spinal cord infarction in children is rare, and difficult toiagnose. The etiologies of spinal cord stroke in childrenre diverse (Table 1), and differ from those commonlyeen in adult patients.

pidemiology

There is no comprehensive epidemiological descriptionf ischemic spinal cord infarction in adults or children. In988, Sandson and Friedman [6] reported spinal cordnfarction as 1.2% of cases admitted for stroke at theoger Williams General Hospital over a 4-year span. In

he adult population, spinal cord stroke is most commonlyeported in the context of aortic surgery. Reported rates oferioperative and post-operative spinal cord “complica-ions,” many or most of which appear to be of vascularrigin, range from 0.41% in aortic coarctation repair [7] to2% in repair of type IIIb dissecting aortic aneurysms [8].There are few large studies of spinal cord ischemia in

hildren. Of 900 infants autopsied after death before 4eeks of life, 21 demonstrated evidence of hypoxic/

schemic spinal cord damage [9]. Spinal cord injuryithout radiographic abnormality (SCIWORA) represents

mean incidence of 34.8% of traumatic pediatric spinal

cpcinc

SR

apmigfcs

hS9a[pubp

smp

dshtccwas

D

sBddstwcf[ab

tTami

Fc largedf n to T

ord injuries [10]. Spinal cord ischemia is one of fourroposed mechanisms for SCIWORA [11]. The number ofases of SCIWORA actually attributable to spinal cordschemia is difficult to determine, because magnetic reso-ance imaging was not used to define the nature of spinalord parenchymal damage in many children studied [11].

pinal Cord Infarctions Are More Commonlyeported in Adults Than Children: Why?

Spinal cord infarction is more commonly reported indults than in children. This might be due to age-relatedrocesses affecting perfusion of the cord. The most com-on causes of spinal cord infarction in adults are vascular,

ncluding atherosclerosis, abdominal aortic disease/sur-ery, and hypotension [6,12-17]. Compromise of bloodlow to the adult cord has also been associated withervical spondylosis [18]. With the exception of hypoten-ion, these are diseases that take years to develop.

There are subgroups of children who are predisposed toypotension and bony compromise of the spinal cord.ladky and Rorke [9] discovered spinal cord infarction inof 900 asphyxiated premature neonates who underwent

utopsy after death before 4 weeks of life, and Singer et al.19] described spinal cord infarction in an additional threeremature neonates with paraplegia. Both groups attrib-ted the ischemia to poor autoregulation of spinal cordlood flow during systemic hypotension secondary torematurity.Spinal cord infarction has been associated with cervical

pine changes in two children with I-cell disease afterinor trauma [20] and in three children with achondro-

igure 2. Patient 2. Digitally enhanced magnetic resonance imaging (Tentral T2 hyperintensity is seen in axial view at mid T6-level and (B) enrom T5 to T8 (arrows) and Schmorl’s node (arrowhead). Infarction dow

lasia, two of them under general anesthesia [21] and one f

uring gym class [22]. In general, however, the youngpinal cord is less vulnerable to infarction. The spinal cordas extensive collateral blood flow [23]. It may be easiero overlook the diagnosis of spinal cord infarction inhildren, because of the relatively smaller volume andross-sectional area of the pediatric spinal cord, comparedith both the adult spinal cord and the pediatric brain; the

bility of radiographic imaging to detect infarctions in amall cord is limited [24].

iagnosing Spinal Cord Infarction

Magnetic resonance imaging allows for increased sen-itivity in detecting infarction within the spinal cord.efore magnetic resonance imaging was available, theiagnosis of spinal cord infarction relied upon autopsy orocumented vascular occlusion by angiography. Diffu-ion-weighted imaging, although not commonly used onhe spinal cord, now allows identification of cord ischemiaithin hours of the insult [25]. The limitations of spinal

ord magnetic resonance imaging include increased arti-act due to cord movement and cerebrospinal fluid flow26]. Expectation of this artifact may lead to dismissal ofctual lesions, as in Patient 2. Some small infarctions cane appreciated only on high-resolution viewing monitors.Correct diagnosis of spinal cord infarction requires that

he entire spine be imaged in suspicious cases, and that

2-weighted axial and sagittal views are obtained, as wells diffusion-weighted images early in the course to docu-ent ischemia. In case 2, the lesions were difficult to see

n the initial magnetic resonance imaging images on film

4616.7/101.2 ms) at 3 months after the event, showing (A) ill-definedsagittal image of thoracic cord demonstrating central T2 hyperintensity10 can be seen on other cuts.

R/TE �

ormat. When the patient was re-imaged, we included

211Nance and Golomb: Pediatric Spinal Cord Stroke

Tm

DO

odcwdm

csa

aabpcla

[naovIfwmvnma

C

ivbe

itLvtp[

cUioDha

italac

Tw

C

A

T

SI

C

MFC

A

I

M

C

T

2

2-weighted axial views, which revealed the infarction inore detail.

ifferentiating Spinal Cord Infarction Fromther Myelopathy

Magnetic resonance imaging and clinical presentationf spinal cord infarction can be similar to other spinal cordiseases, particularly transverse myelitis. In addition toase 1, at least six other children have been described forhom the diagnosis of ischemic spinal cord infarction wasifficult to distinguish from transverse myelitis [3,4]. The

able 1. Possible risk factors for spinal cord ischemia in childrenithout vertebral fracture

Risk FactorsNo. of

Patients References

ardiacAortic dissection 1 [40]Aortic coarctation repair 4 [27,39]Hypotension

Cardiac arrest 6 [29]Cardiac tamponade 1 [30]

rteriovenous malformationVascular steal 1 [32]Mechanism unknown 5 [31]

hrombotic disordersProthrombin variant 2 [70,71]Protein S deficiency 1 [75]Primary antiphospholipid syndrome 1 [72]

ystemic lupus erythematosus 3 [17,77]nfectious disease

Bacterial meningitis 15 [53,57-67]Amebic menigoencephalitis 1 [69]Viral encephalitis 1 [68]

erebellar herniationMetabolic encephalopathy 3 [50]inor trauma 18 [41-48]

ibrocartilaginous embolism 10 [1-3,85-89]ancerAnterior spinal artery thrombosis 1 [76]

tlanto-axial instabilityAchondroplasia 1 [22]I-cell disease 1 [20]

atrogenicSclerotherapy of esophageal varices 1 [56]Prolonged neck flexion during surgery in

sitting position1 [49]

Umbilical arterial catheters 8 [33-36]Thoracic tumor resection 2 [37]ultifactorialAchondroplasia, surgery 2 [21]Meningitis, Factor V Leiden, Chiari I

malformation1 [74]

Meningitis, lumbar puncture, cardiac arrest 6 [51-55]Weight-lifting, MTHFR homozygosity 1 [73]Umbilical artery catheter, arterial switch

operation1 [92]

Protein S deficiency, spine surgery,epidural catheter

1 [71]

ause unknown/unclearNot reported 2 [93]Unclear 6 [4,91]

otal 108

agnetic resonance imaging lesions seen in all of the these 1

12 PEDIATRIC NEUROLOGY Vol. 36 No. 4

ases were in the anterior two-thirds of the spinal cord,uggesting that anterior spinal artery syndrome was thectual diagnosis.

Anterior spinal artery syndrome is characterized by thebrupt onset of symmetric paralysis, impaired temperaturend pain sensation, and loss of bowel and bladder functionelow the level of occlusion; vibration, light touch, androprioceptive sensations are preserved [27]. Patient 1learly presented with these symptoms below the cervicalevel, which correlated with linear T2 hyperintensity of thenterior portion of the cervical spinal cord.

The Transverse Myelitis Consortium Working Group28] defines inclusion and exclusion criteria for the diag-osis of acute idiopathic transverse myelitis, requiring thatll inclusion criteria and no exclusion criteria be met inrder to confirm diagnosis. Diagnostic criteria for trans-erse myelitis include cerebrospinal fluid pleocytosis orgG index, or gadolinium enhancement representing in-lammation of the spinal cord; progression of symptomsithin 4 hours to 21 days following onset; and findings onagnetic resonance imaging that do not correspond to a

ascular territory. Patient 1 had unremarkable cerebrospi-al fluid findings, progression of symptoms within 30inutes, and magnetic resonance imaging findings in the

nterior spinal artery territory.

auses of Spinal Cord Infarction in Children

As in adults, ischemic spinal cord infarction in childrens caused by decreased blood flow due to hypotension,ascular injury, compression, other impairment, or throm-osis or embolization to spinal arteries. The underlyingtiologies, however, can be quite different.

Systemic hypotension leading to spinal cord infarctionn children can be caused by cardiac arrest [29] or cardiacamponade [30], both of which can also occur in adults.ocally decreased perfusion can be caused by arterio-enous malformations. Spinal arteriovenous malforma-ions are rare in children, but have been associated witharaplegia in five patients with thoracic malformations31] and vascular steal in one patient [32].

Instrumentation during procedures or surgery, or trauma,an lead to vascular injury that precipitates cord infarction.mbilical artery catheters can cause spinal cord infarction

n neonates [33-36], usually due to disruption of the arteryf Adamkiewicz, a major artery of the thoracic cord.amage to this artery with resulting spinal cord infarctionas also been reported in one child during the removal ofthoracic neuroblastoma [37].Surgery on the aorta is a known cause of spinal cord

nfarction in adults [38]; children undergo aortic opera-ions less frequently, and they are usually confined to therch. Four children developed spinal cord infarction fol-owing repair of aortic coarctation [27,39]. Diseases of thebdominal aorta are rare in children, although there is onease of spinal cord ischemia with aortic dissection in an

8-year-old [40].

iuciisaac

te2adt

aiiosttrrnao

sci[h1serobap

isoamrdc[frv

pini

PlSttcsa

ir[euse

T

hopiwhcpiwmiPt

O

wotuaTriod

ii

Minor trauma has also been associated with spinal cordnfarction [41-48], but the exact etiology is not wellnderstood. Pang and Wilberger [11] propose that, inhildren, the relatively less elastic spinal cord is strainednside the more flexible spinal column during hyperflexionnjuries, and that this may result in reactive vasospasm ofpinal cord arteries leading to ischemia. The same mech-nism is proposed in a case of cord infarction occurring inchild following operative removal of a pineal choriocar-

inoma in the sitting position [49].Cerebellar herniation was associated with cervical and

horacic cord infarction in three children with metabolicncephalopathy; two underwent lumbar puncture within4 hours prior to herniation [50]. Cerebellar herniationssociated with cord infarction is also suggested in chil-ren with meningitis who have undergone lumbar punc-ure [51-55].

There are many ways that emboli and thrombi can formnd impair cord perfusion. Embolus formation can beatrogenic: embolic material to the spinal cord vasculatures reported in a pediatric patient undergoing sclerotherapyf esophageal varices [56]. Thrombotic vasculitis, occlu-ive arachnoiditis, or both were implicated in cord infarc-ion of many children with central nervous system infec-ions [53,57-69]. Several thrombotic disorders have beeneported in cases of spinal cord infarction [70-75]. Ante-ior spinal artery thrombosis is described in a child witheuroblastoma [76]. Systemic lupus erythematosus wasssociated with vasculitic ischemia of the spinal cord inne patient [17] and thrombosis in another [77].Fibrocartilaginous embolism as a cause of childhood

pinal cord infarction is becoming increasingly well-haracterized. This condition has been well characterizedn animals, especially dogs [78,79], but also in cats80,81], horses [82], swine [83], and turkeys [84]. The firstuman condition was described in a 15-year-old male in961; nucleus pulposis emboli were found within thepinal cord arteries at autopsy [85]. Patients usuallyxperience sudden back pain with subsequent onset ofapid neurologic deterioration following a “free interval”f minutes to 2 days [1]. Fibrocartilaginous embolism haseen demonstrated, most often affecting the anterior spinalrtery territory, at autopsy in 28 adults and 4 pediatricatients [1,86,87].Magnetic resonance imaging has allowed fibrocartilag-

nous embolism to become better characterized noninva-ively in surviving patients [1-3,88,89]. There are reportsf fibrocartilaginous embolism with varying presentationsnd magnetic resonance imaging findings [2,3]. In ani-als, fibrocartilaginous embolism can be suspected when

adiography and myelography demonstrate intervertebralisk changes and cord swelling at a level correlating withlinical features of spinal cord ischemia [90]. Tosi et al.1] propose that similar criteria may be used to diagnoseibrocartilaginous embolism in humans and that magneticesonance imaging findings may include collapse of inter-

ertebral disk space or Schmorl’s nodes. It has been a

roposed that the vertical herniation of nucleus pulposisnto vertebral bodies, as in the formation of Schmorl’sodes, may allow the entry of fibrocartilaginous materialnto the vasculature of the spinal cord [1-3].

We believe that the pathogenesis of cord infarction inatient 2 is most likely due to fibrocartilaginous embo-

ism, because his magnetic resonance imaging showedchmorl’s nodes and disk changes adjacent to the area of

horacic T2 hyperintensity; presence of the MTHFR muta-ions may also have been contributory. This diagnosis isomplicated by the immediate neurologic deficit, unusualensory distribution, and lack of neurological deteriorationfter onset.

For some children, the etiology of spinal cord infarctions unclear. Even after extensive work-up, the mechanismemains unclear [4,91] or is found to be multifactorial21,51-55,71,73,74,92], with no clear single cause. Thetiology of the spinal cord infarction in Patient 1 isnknown. She has no history of trauma or changes in herpine that would be consistent with fibrocartilaginousmbolism.

reatment

Medical treatment of childhood spinal cord infarctionas not been widely reported, and it is unclear if it affectsutcome. Anticoagulation with heparin and aspirin ap-eared to improve outcome in patients with spinal cordnfarction and thrombotic disorders [70,71,73]. Patient 2as treated with aspirin and folic acid for MTHFReterozygosity; it is unclear if this has affected his out-ome. Patient 1 and Patient 2 both underwent extensivehysical therapy. Supportive care and rehabilitative phys-cal therapy are key in the long-term treatment of childrenith spinal cord infarction. Both patients had improve-ent in their neurological function, although Patient 1’s

mprovement was minimal. Seven months after his infarct,atient 2 continued to show significant improvement with

herapy.

utcome After Spinal Cord Infarction

Outcomes after spinal cord infarction have been moreidely reported in adults than in children. A review ofutcomes in 199 patients by Cheshire et al. [17] showedhat 43 (22%) cases resulted in death, 47 (24%) cases werenimproved, 70 (35%) cases showed some improvement,nd 39 (19%) cases were considered markedly improved.his review included some children, but results were not

eported with respect to age, cause, or spinal cord regionnvolved. In two series of adults with cord infarction,utcome was poorer in patients with more profoundisability at onset [12,16].One follow-up study of 8 patients with cord infarction

ncluded two children: one had improvement in neurolog-cal function and the other had deterioration [93]. There

re reports of varied outcomes in children with spinal cord

213Nance and Golomb: Pediatric Spinal Cord Stroke

tsvosii

CS

vfPbr[rcrp

p2acb

iacdm

C

cacTarTtlvbadinoan

cm

Tpo

Hg

I

R

tN

ul

cS

mA

D

82

c6

dt

i

c

g

AcC

S

n

cc

c

ts

n

P

2

rauma [10,11,94-98], but there is no confirmation ofpinal cord infarction, and many of the children hadertebral fractures. Moffett and Berkowitz [58] reviewedutcomes in 19 children with bacterial meningitis andpinal cord infarction, finding death in 5 patients, somemprovement in 12 patients, and return to normal functionn 2 patients.

ommon Medical and Psychosocial Complications ofpinal Cord Infarction

The complications of spinal cord infarction includeentilator dependence and decreased bowel or bladderunction, depending on the areas of spinal cord infarction.atients are more susceptible to respiratory infections andladder infections from indwelling catheters or urinaryetention; these infections can have significant morbidity97]. Patient 1 remains ventilator-dependent and long-termeliance on a Foley catheter has led to several urinary tractomplications, including repeated infections and surgicalemoval of a bladder stone. She is monitored by aulmonologist and a urologist.Chronic pain and spastic deformity are additional re-

orted complications of cord infarction [16,19,93]. Patientexperienced chronic neuropathic pain and muscle pain

ssociated with spasticity. He is taking medication toontrol the pain and spasticity. Patient 1 receives regularotulinum injections for spasticity.It is important to recognize and address the psychologic

mpact of spinal cord infarction. Patient 1 has experiencednxiety, depression, and insomnia as a result of herondition. Patient 2 has reported no long-term psychiatricifficulties, which may be related to his steady improve-ent.

onclusion

The understanding of ischemic spinal cord infarction inhildren is an evolving area in pediatric neurology. Evens the causes become better characterized in children, ourases demonstrate the continued difficulty in diagnosis.he outcome of spinal cord infarction is difficult to changefter the insult. Improvement in treatment requires moreapid and exact diagnosis, with both axial and sagittal T1,

2, and diffusion-weighted magnetic resonance imaging ofhe spinal cord to visualize both intrinsic and extrinsicesions in suspected cases. Angiography, to assess forascular occlusion and arteriovenous malformation, maye indicated in some cases. Thrombotic evaluation playsn important role in diagnosis, because thrombotic disor-ers can be medically treated. As magnetic resonancemaging becomes more sensitive, and as new modalities ofeuroimaging become available, we expect the diagnosisf spinal cord infarction to become better characterizednd hopefully easier to manage. It is important to recog-

ize and address the varied physical and psychosocial s

14 PEDIATRIC NEUROLOGY Vol. 36 No. 4

omplications of spinal cord infarction in order to opti-ize outcome.

he authors of this paper take full responsibility for the content of thisaper. We would like to thank Dr. Lawrence Walsh for contribution ofne case to our paper and Ms. Nina Talib, MSc, for technical assistance.M.G. is supported by the following grants: National Institutes of

ealth NINDS grant no. K23 NS048024 and the Clarian Values Fundrant no. VFR-171.This study was approved by the Institutional Review Board of the

ndiana University School of Medicine (Study # 0207-55).

eferences

[1] Tosi L, Rigoli G, Beltramello A. Fibrocartilaginous embolism ofhe spinal cord: a clinical and pathogenetic reconsideration. J Neuroleurosurg Psychiatry 1996;60:55-60.[2] Han JJ, Massagli TL, Jaffe KM. Fibrocartilaginous embolism: an

ncommon cause of spinal cord infarction: case report and review of theiterature. Arch Phys Med Rehabil 2004;85:153-7.

[3] Davis GA, Klug GL. Acute-onset nontraumatic paraplegia inhildhood: fibrocartilaginous embolism or acute myelitis? Childs Nervyst 2000;16:551-4.[4] Wilmshurst JM, Walker MC, Pohl KR. Rapid onset transverseyelitis in adolescence: implications for pathogenesis and prognosis.rch Dis Child 1999;80:137-42.[5] International Classification of Diseases: ICD-9-CM. Denver,

E: American Medical Association, 1999.[6] Sandson TA, Friedman JH. Spinal cord infarction: report ofcases and review of the literature. Medicine (Baltimore) 1989;68:

82-92.[7] Brewer LA 3rd, Fosburg RG, Mulder GA, Verska JJ. Spinal cord

omplications following surgery for coarctation of the aorta: a study of6 cases. J Thorac Cardiovasc Surg 1972;64:368-81.[8] DeBakey ME, McCollum CH, Crawford ES, et al. Dissection and

issecting aneurysms of the aorta: twenty-year follow-up of five hundredwenty-seven patients treated surgically. Surgery 1982;92:1118-34.

[9] Sladky JT, Rorke LB. Perinatal hypoxic/ischemic spinal cordnjury. Pediatr Pathol 1986;6:87-101.

[10] Pang D. Spinal cord injury without radiographic abnormality inhildren, 2 decades later. Neurosurgery 2004;55:1325-42.

[11] Pang D, Wilberger JE Jr. Spinal cord injury without radio-raphic abnormalities in children. J Neurosurg 1982;57:114-29.[12] Salvador de la Barrera S, Barca-Buyo A, Montoto-Marques

, Ferreiro-Velasco ME, Cidoncha-Dans M, Rodriguez-Sotillo A. Spinalord infarction: prognosis and recovery in a series of 36 patients. Spinalord 2001;39:520-5.[13] Satran R. Spinal cord infarction. Stroke 1988;19:529-32.[14] Williams LS, Bruno A, Biller J. Spinal cord infarction. Top

troke Rehabil 1996;3:41-53.[15] Foo D, Rossier AB. Anterior spinal artery syndrome and its

atural history. Paraplegia 1983;21:1-10.[16] Masson C, Pruvo JP, Meder JF, et al. Spinal cord infarction:

linical and magnetic resonance imaging findings and short term out-ome. J Neurol Neurosurg Psychiatry 2004;75:1431-5.

[17] Cheshire WP, Santos CC, Massey EW, Howard JF Jr. Spinalord infarction: etiology and outcome. Neurology 1996;47:321-30.

[18] Ogino H, Tada K, Okada K, et al. Canal diameter, anteropos-erior compression ratio, and spondylotic myelopathy of the cervicalpine. Spine 1983;8:1-15.

[19] Singer R, Joseph K, Gilai AN, Meyer S. Nontraumatic, acuteeonatal paraplegia. J Pediatr Orthop 1991;11:588-93.[20] Goodman ML, Pang D. Spinal cord injury in I-cell disease.

ediatr Neurosci 1988;14:315-8.[21] Mayhew JF, Katz J, Miner M, Leiman BC, Hall ID. Anaesthe-

ia for the achondroplastic dwarf. Can Anaesth Soc J 1986;33:216-21.

p1

slV

c

Di8

ap

AN

d2

t

SR

Ar

v

i

ul

f

Pb

Sn1

a

IR

c

i

W1

wr

iP

w

d

c1

o

cs

tA

g5

i1

d1

g

c9

f9

tr

rcg

a

i

p

t

pP

n

d1

ce

d

rP

rN

t

t

[22] Wieting JM, Krach LE. Spinal cord injury rehabilitation in aediatric achondroplastic patient: case report. Arch Phys Med Rehabil994;75:106-8.[23] Thron AK. Postmortem angiography and microangiography of

pinal cord vessels. In: Vascular anatomy of the spinal cord: neuroradio-ogical investigations and clinical syndromes. New York: Springer-erlag, 1988;13–64.[24] Bammer R, Fazekas F. Diffusion imaging of the human spinal

ord and the vertebral column. Top Magn Reson Imaging 2003;14:461-76.[25] Kuker W, Weller M, Klose U, Krapf H, Dichgans J, Nagele T.

iffusion-weighted MRI of spinal cord infarction: high resolutionmaging and time course of diffusion abnormality. J Neurol 2004;251:18-24.[26] Pierre-Jerome C, Arslan A, Bekkelund SI. MRI of the spine

nd spinal cord: imaging techniques, normal anatomy, artifacts, anditfalls. J Manipulative Physiol Ther 2000;23:470-5.[27] Servais LJ, Rivelli SK, Dachy BA, Christophe CD, Dan B.

nterior spinal artery syndrome after aortic surgery in a child. Pediatreurol 2001;24:310-2.[28] Transverse Myelitis Consortium Working Group. Proposed

iagnostic criteria and nosology of acute transverse myelitis. Neurology002;59:499-505.[29] Gilles FH, Nag D. Vulnerability of the human spinal cord in

ransient cardiac arrest. Neurology 1971;21:833-9.[30] Lin CC, Chen SY, Lan C, Ting-Fang Shih T, Lin MC, Lai JS.

pinal cord infarction caused by cardiac tamponade. Am J Phys Medehabil 2002;81:68-71.[31] Riche MC, Modenesi-Freitas J, Djindjian M, Merland JJ.

rteriovenous malformations (AVM) of the spinal cord in children: aeview of 38 cases. Neuroradiology 1982;22:171-80.

[32] Bandyopadhyay S, Sheth RD. Acute spinal cord infarction:ascular steal in arteriovenous malformation. J Child Neurol 1999;14:685-7.

[33] Aziz EM, Robertson AF. Paraplegia: a complication of umbil-cal artery catheterization. J Pediatr 1973;82:1051-2.

[34] Brown MS, Phibbs RH. Spinal cord injury in newborns fromse of umbilical artery catheters: report of two cases and a review of theiterature. J Perinatol 1988;8:105-10.

[35] Haldeman S, Fowler GW, Ashwal S, Schneider S. Acutelaccid neonatal paraplegia: a case report. Neurology 1983;33:93-5.

[36] Krishnamoorthy KS, Fernandex RJ, Todres ID, De Long GR.araplegia associated with umbilical artery catheterization in the new-orn. Pediatrics 1976;58:443-5.[37] Boglino C, Martins AG, Ciprandi G, Sousinha M, Inserra A.

pinal cord vascular injuries following surgery of advanced thoraciceuroblastoma: an unusual catastrophic complication. Med Pediatr Oncol999;32:349-52.[38] Ross RT. Spinal cord infarction in disease and surgery of the

orta. Can J Neurol Sci 1985;12:289-95.[39] Castro-Vilanova MD, de Toledo M, Mateos F, Simon R.

squemia-infarto medular [Spinal cord infarction/ischemia. In Spanish].ev Neurol 1999;29:977-80.[40] Garland H, Greenberg J, Harriman DG. Infarction of the spinal

ord. Brain 1966;89:645-62.[41] Ahmann PA, Smith SA, Schwartz JF, Clark DB. Spinal cord

nfarction due to minor trauma in children. Neurology 1975;25:301-7.[42] Choi JU, Hoffman HJ, Hendrick EB, Humphreys RP, KeithS. Traumatic infarction of the spinal cord in children. J Neurosurg

986;65:608-10.[43] Ergun A, Oder W. Pediatric care report of spinal cord injury

ithout radiographic abnormality (SCIWORA): case report and literatureeview. Spinal Cord 2003;41:249-53.

[44] Linssen WH, Praamstra P, Gabreels FJ, Rotteveel JJ. Vascularnsufficiency of the cervical cord due to hyperextension of the spine.ediatr Neurol 1990;6:123-5.[45] Cheshire DJ. The paediatric syndrome of traumatic myelopathy

ithout demonstrable vertebral injury. Paraplegia 1977;15:74-85.[46] Horowitz SH, Patel N. Peripheral neurophysiology of acute

istal spinal cord infarction. Pediatr Neurol 2003;28:64-5. 2

[47] Riviello JJ Jr, Marks HG, Faerber EN, Steg NL. Delayedervical central cord syndrome after trivial trauma. Pediatr Emerg Care990;6:113-7.[48] Lenn NJ. Spinal cord infarction due to minor trauma. Neurol-

gy 1977;27:999.[49] Nitta H, Yamashita J, Nomura M, Igarashi N. Cervical spinal

ord infarction after surgery for a pineal region choriocarcinoma in theitting position: case report. Neurosurgery 1997;40:1082-5.

[50] Herrick MK, Agamanolis DP. Displacement of cerebellarissue into spinal canal: a component of the respirator brain syndrome.rch Pathol Lab Med 1975;99:565-71.[51] Phelan M, Manson JI. Spinal cord dysfunction with quadriple-

ia complicating pneumococcal meningitis. Aust Paediatr J 1987;23:7-9.[52] Norman MG. Respiratory arrest and cervical spinal cord

nfarction following lumbar puncture in meningitis. Can J Neurol Sci982;9:443-7.[53] Tal Y, Crichton JU, Dunn HG, Dolman CL. Spinal cord

amage: a rare complication of purulent meningitis. Acta Paediatr Scand980;69:471-4.[54] Swart SS, Pye IF. Spinal cord ischaemia complicating menin-

ococcal meningitis. Postgrad Med J 1980;56:661-2.[55] DeSousa AL, Kleiman MB, Mealey J Jr. Quadriplegia and

ortical blindness in hemophilus influenzae meningitis. J Pediatr 1978;3:253-4.[56] Seidman E, Weber AM, Morin CL, et al. Spinal cord paralysis

ollowing sclerotherapy for esophageal varices. Hepatology 1984;4:50-4.[57] Haupt HM, Kurlinski JP, Barnett NK, Epstein M. Infarction of

he spinal cord as a complication of pneumococcal meningitis: caseeport. J Neurosurg 1981;55:121-3.

[58] Moffett KS, Berkowitz FE. Quadriplegia complicating Esche-ichia coli meningitis in a newborn infant: case report and review of 22ases of spinal cord dysfunction in patients with acute bacterial menin-itis. Clin Infect Dis 1997;25:211-4.[59] Glista GG, Sullivan TD, Brumlik J. Spinal cord involvement in

cute bacterial meningitis. JAMA 1980;243:1362-3.[60] Seay AR. Spinal cord dysfunction complicating bacterial men-

ngitis. Arch Neurol 1984;41:545-6.[61] Verghote M, Rousseau E, Geoffroy G. Quadriplegia after

neumococcal meningitis. Pediatr Infect Dis 1985;4:559.[62] Nadal D, Boltshauser E. Transient paraparesis following bac-

erial meningitis. Helv Paediatr Acta 1987;42:55-7.[63] Boothman BR, Bamford JM, Parsons MR. Paraplegia as a

resenting feature of meningococcal meningitis. J Neurol Neurosurgsychiatry 1988;51:1241.[64] Coker SB, Muraskas JK, Thomas C. Myelopathy secondary to

eonatal bacterial meningitis. Pediatr Neurol 1994;10:259-61.[65] Kirkpatrick M, Brooker RJ, Helms PJ, Cole GF. Spinal cord

ysfunction in neonatal meningococcal meningitis. Eur J Pediatr 1994;53:367-8.[66] Kastenbauer S, Winkler F, Fesl G, et al. Acute severe spinal

ord dysfunction in bacterial meningitis in adults: MRI findings suggestxtensive myelitis. Arch Neurol 2001;58:806-10.

[67] Graus F, Arbizu T, Ruyfi G. Partial Brown-Séquard’s syn-rome and meningococcal meningitis. Arch Neurol 1981;38:602.[68] Caruso JM, Tung GA, Brown WD. Central nervous system and

enal vasculitis associated with primary varicella infection in a child.ediatrics 2001;107:E9.[69] Schumacher JD, Tien RD, Lane K. Neuroimaging findings in

are amebic infections of the central nervous system. AJNR Am Jeuroradiol 1995;16:930-5.[70] Young G, Krohn KA, Packer RJ. Prothrombin g20210a muta-

ion in a child with spinal cord infarction. J Pediatr 1999;134:777-9.[71] Hakimi KN, Massagli TL. Anterior spinal artery syndrome in

wo children with genetic thrombotic disorders. J Spinal Cord Med

005;28:69-73.

215Nance and Golomb: Pediatric Spinal Cord Stroke

Yl

w

c2

Ac

Iw

Gf

N

ce

f

J

Sh

wD

i

e

eP

Vp

Cf

Rn

S

a

aS

S

lJ

S1

ec

1

cc

2

[72] Hasegawa M, Yamashita J, Yamashima T, Ikeda K, Fujishima Y,amazaki M. Spinal cord infarction associated with primary antiphospho-

ipid syndrome in a young child: case report. J Neurosurg 1993;79:446-50.[73] Cooper D, Magilner D, Call J. Spinal cord infarction after

eight lifting. Am J Emerg Med 2006;24:352-5.[74] Almasanu BP, Owensby JR, Pavlakis SG, Edwards JH. Spinal

ord infarction in meningitis: polygenic risk factors. Pediatr Neurol005;32:124-6.[75] Ramelli GP, Wyttenbach R, von der Weid N, Ozdoba C.

nterior spinal artery syndrome in an adolescent with protein s defi-iency. J Child Neurol 2001;16:134-5.

[76] Lewis DW, Packer RJ, Raney B, Rak IW, Belasco J, Lange B.ncidence, presentation, and outcome of spinal cord disease in childrenith systemic cancer. Pediatrics 1986;78:438-43.[77] Falko JM, Williams JC, Harvey DG, Weidman SW, Schonfeld

, Dodson WE. Hyperlipoproteinemia and multifocal neurologic dys-unction in systemic lupus erythematosus. J Pediatr 1979;95:523-9.

[78] Cauzinille L. Fibrocartilaginous embolism in dogs. Vet Clinorth Am Small Anim Pract 2000;30:155-67, vii.[79] Gandini G, Cizinauskas S, Lang J, Fatzer R, Jaggy A. Fibro-

artilaginous embolism in 75 dogs: clinical findings and factors influ-ncing the recovery rate. J Small Anim Pract 2003;44:76-80.

[80] Coradini M, Johnstone I, Filippich LJ, Armit S. Suspectedibrocartilaginous embolism in a cat. Aust Vet J 2005;83:550-1.

[81] Scott HW, O’Leary MT. Fibro-cartilaginous embolism in a cat.Small Anim Pract 1996;37:228-31.[82] Fuentealba IC, Weeks BR, Martin MT, Joyce JR, Wease GS.

pinal cord ischemic necrosis due to fibrocartilaginous embolism in aorse. J Vet Diagn Invest 1991;3:176-9.[83] Benson JE, Schwartz KJ. Ischemic myelomalacia associated

ith fibrocartilaginous embolism in multiple finishing swine. J Vetiagn Invest 1998;10:274-7.[84] Stedman NL, Brown TP, Rowland GN. Intravascular cartilag-

nous emboli in the spinal cord of turkeys. Avian Dis 1998;42:423-8.[85] Naiman J, Donohue WL, Prichard JS. Fatal nucleus pulposis

mbolism of spinal cord after trauma. Neurology 1961;11:83-7. o

16 PEDIATRIC NEUROLOGY Vol. 36 No. 4

[86] Yousef OM, Appenzeller P, Kornfeld M. Fibrocartilagenousmbolism: an unusual cause of spinal cord infarction. Am J Forensic Medathol 1998;19:395-9.[87] Toro G, Roman GC, Navarro-Roman L, Cantillo J, Serrano B,

ergara I. Natural history of spinal cord infarction caused by nucleusulposus embolism. Spine 1994;19:360-6.[88] Raghavan A, Onikul E, Ryan MM, Prelog K, Taranath A,

hennapragada M. Anterior spinal cord infarction owing to possibleibrocartilaginous embolism. Pediatr Radiol 2004;34:503-6.

[89] Furtner M, Felber S, Poewe W. Fibrokartilaginare Embolie desückenmarks [Spinal fibrocartilaginous embolism. In German]. Nerve-arzt 2005;76:1246-9.[90] Cook JR Jr. Fibrocartilaginous embolism. Vet Clin North Am

mall Anim Pract 1988;18:581-92.[91] Vandertop WP, Elderson A, van Gijn J, Valk J. Anterior spinal

rtery syndrome. AJNR Am J Neuroradiol 1991;12:505-6.[92] Lemke RP, Idiong N, al-Saedi S, et al. Spinal cord infarct after

rterial switch associated with an umbilical artery catheter. Ann Thoracurg 1996;62:1532-4.[93] Pelser H, van Gijn J. Spinal infarction: a follow-up study.

troke 1993;24:896-8.[94] Dare AO, Dias MS, Li V. Magnetic resonance imaging corre-

ation in pediatric spinal cord injury without radiographic abnormality.Neurosurg 2002;97:33-9.[95] Davis PC, Reisner A, Hudgins PA, Davis WE, O’Brien MS.

pinal injuries in children: role of MR. AJNR Am J Neuroradiol993;14:607-17.[96] Grabb PA, Pang D. Magnetic resonance imaging in the

valuation of spinal cord injury without radiographic abnormality inhildren. Neurosurgery 1994;35:406-14.

[97] Burke DC. Spinal cord trauma in children. Paraplegia 1971;9:-14.[98] Liao CC, Lui TN, Chen LR, Chuang CC, Huang YC. Spinal

ord injury without radiological abnormality in preschool-aged children:orrelation of magnetic resonance imaging findings with neurological

utcomes. J Neurosurg 2005;103:17-23.