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hronic Sequelae of Acute Disseminatedncephalomyelitis in a Child

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NTRODUCTION

cute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the centralervous system. Although uncommon, it is the most common demyelinating disease inhildren and adolescents [1]. Typically, it presents with multifocal neurologic symptoms,uch as long tract signs, hemiparesis, ataxia, cranial neuropathies, spinal cord dysfunction,phasia, movement disorders, optic neuritis, and sensory deficits [1-3]. The major criteriaor the diagnosis are a first clinical event of the central nervous system, demyelinatingisease with acute or subacute onset, polysymptomatic neurologic features, encephalopa-hy, and abnormalities on imaging studies (Table 1) [1-4]. The most useful imaging study isagnetic resonance imaging (MRI) of the brain and spinal cord. Findings may includeultiple, bilateral, and asymmetric deep and subcortical lesions of the white and grayatter, brainstem, and the spinal cord [2-5].The most common form of ADEM is the monophasic type, which occurs 1 to 2 weeks

fter a viral infection or vaccination, with complete resolution of neurologic symptomsithin 6 weeks of the patient’s initial presentation [2,3,6]. Less commonly, a second episodef ADEM can occur and may be defined as either recurrent or multiphasic ADEM (Table 1)4,6]. Immunosuppressive therapy is recommended for children with ADEM, and high-ose intravenous glucocorticoids are the first line of treatment. Patients who are refractoryo steroid therapy may be treated with intravenous immunoglobulin. Furthermore, patientsho do not respond to these modalities may be treated with plasmapheresis [2,3,5].Most children with ADEM make a full recovery in 4 to 6 weeks, except for severe cases

hat require extensive rehabilitation as the result of persistent functional deficits. Theong-term prognosis and rehabilitation issues in the pediatric population are not wellescribed in the literature. We present a rare pediatric case of recurrent ADEM withersistent severe functional deficits.

ASE PRESENTATION

12-year-old boy was admitted to our inpatient rehabilitation facility with severe functionaloss resulting from recurrent ADEM. The patient had a medical history of uncomplicatedronchial asthma until 10 months before admission, when he presented with fever, alteredental status, and generalized weakness. Initial brain MRI, performed at another institu-

ion, reportedly showed multiple lesions in the brain parenchyma, localized in the right andeft hemisphere, mostly in the frontal and temporal lobes, and the posterior aspect of therainstem, specifically at the pons. A diagnosis of ADEM was established, and the patientas treated with a short course of intravenous corticosteroid therapy. After symptomatic

mprovement he was discharged home.One month later, the patient developed acute right hemiplegia, motor aphasia, left facial

erve paralysis, and bilateral internuclear ophthalmoplegia. Compared with the previousadiographic study, follow-up MRI of the brain (Figure 1) showed a larger area of hyperin-ensity involving the brainstem at the level of the pons that extended into the upper medullablongata and superiorly into the midbrain towards the left side. Patchy hyperintensityesions along the basal ganglia, external capsule, and medial temporal lobes bilaterally, right

ore pronounced than left, also were noted.

The patient was admitted with recurrence of ADEM and treated with corticosteroids

S2

PM&R © 2010 by the American Academy of P1934-1482/10/$36.00

Printed in U.S.A.68

.M. Department of Physical Medicine, Reha-ilitation and Sports Medicine, University ofuerto Rico, School of Medicine, Medicalciences Campus, P. O. Box 365067, Sanuan, Puerto Rico 00936-5067. Addressorrespondence to G.M.; e-mail: gerardo.iranda1@upr.edu.†

islcosure: nothing to disclose

.R. Department of Physical Medicine, Reha-ilitation and Sports Medicine, University ofuerto Rico, School of Medicine, Medicalciences Campus, San Juan, Puerto Rico‡

islcosure: nothing to disclose

ubmitted for publication November 17,009; accepted June 2, 2010.

hysical Medicine and RehabilitationVol. 2, 868-871, September 2010

DOI: 10.1016/j.pmrj.2010.06.003

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or 5 days, without clinical progress. Therefore, treatmentith intravenous immunoglobulin was administered for 5ays, and symptomatic improvement was noted. The pa-ient was discharged to a skilled nursing facility instead ofn inpatient rehabilitation unit because of a lack of insur-nce coverage at the time. He received 4 weeks of physicalnd occupational therapy at that facility and then contin-

able 1. Diagnostic criteria for ADEM

Type of ADEM

onophasic ADEM ● First clinical attack of inflammato● Affects multifocal areas of the C● Encephalopathy: behavioral cha

consciousness● Imaging studies abnormalities● No other etiologies can explain t● ADEM relapses (with new or fluct

of the inciting ADEM episode are● ADEM relapses that occur during

completing a tapering of steroidecurrent ADEM ● Recurrence of neurologic sympto

completing steroid therapy● No new lesions on imaging studie

ultiphasic ADEM ● New clinical event that meets AD● New anatomical involvement wit● Encephalopathy may present sim● New imaging lesions, and eviden

dapted from Krupp et al [4].ADEM � acute disseminated encephalomyelitis; CNS � central nervous sy

igure 1. T1-weighted image shows large area of hyperinten-

ity involving the brainstem at the level of the pons. m

ed therapy at home. Two months later, the patient waseadmitted at the acute care general pediatrics hospitalith altered mental status, right spastic hemiparesis, acuteysphagia, and dysarthria. Therapy with plasmapheresisas started for 6 days, with clinical improvement noted.ollow-up MRI of the brain (Figure 2) showed resolutionf most lesions, except a small focal lesion on the left pons.

Diagnostic Criteria

emyelinating disease in the CNS of acute or subacute onsetpolysymptomatic presentation

uch as confusion, excessive irritability, and/or alteration in

ntsymptoms, signs or MRI findings) occurring within 3 months

dered part of the same acute event.ering of steroid administration or within 4 weeks ofonsidered part of the initial inciting ADEM episode.

or more months after initial insult, and at least 1 month after

may have enlargement of previous lesionsiagnostic criteria

symptoms

resolution of previous lesions

RI � magnetic resonance imaging.

igure 2. T1-weighted image shows a small lesion at the left

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870 Miranda and Ramos CHRONIC SEQUELAE OF ADEM IN A CHILD

Upon admission to our inpatient rehabilitation unit, theatient was found to be cooperative, attentive, and orientedo person, time, and space. He presented with dysarthricpeech, central obesity with a moon face and buffalo hump,nd poor airway secretion management. There was no evi-ence of trauma to the head, no skull deformities, and hisupils were equal but slowly reactive to light. Central nystag-us and right nasolabial flattening were observed. Inten-

ional tremor and poor motor coordination in all extremitiesas present, as well as right hemiparesis, more prominentroximally than distally.

Functional assessment confirmed that the patient wasompletely dependent for mobility and mostly dependent forll self-care activities, except for upper extremity dressingnd eating, which he performed with maximal assistance. Heerformed cognitive skills under the supervision of a care-aker. On admission, he received a Wee Functional Indepen-ence Measure (FIM) score of 40. Rehabilitation was focusedn preventing secondary complications, such as contracturesnd pressure ulcers; improving upper and lower extremitytrength, endurance, and mobility; functional training inelf-care; and cognitive skills.

After 4 weeks of inpatient rehabilitation, which includedhysical, occupational, and speech therapy for at least 3 hoursaily, little functional progress was observed. Different thera-eutic techniques were used, including an aggressive stretchingrogram to avoid contractures, coordination exercises to im-rove fine motor skills, functional electrical stimulation to pro-ote muscle activity, standing frame for weight-bearing activity,

nd multiple memory, problem-solving, and communicationxercises. Upon discharge, no neuromuscular changes on phys-cal examination were observed.

Although he did not present functional improvement inobility, he did show small improvements in performing

elf-care activities, such as eating and dressing his upperxtremities with moderate assistance, and maximal assistanceith bladder and bowel management. He showed improve-ent in all cognitive skills, becoming independent in com-rehension and social interactions adequate for age level,artially independent in expression and memory skills, al-hough no change was observed in problem solving (ie, heequired supervision by his mother and/or therapist duringhis activity). The patient’s functional status improved inelf-care activities and cognition, and he received a total WeeIM score of 50 on discharge. A custom-made wheelchairas prescribed to aid in mobility and proper positioninghile he continued physical, occupational, and speech ther-

pies at home.

ISCUSSION

hree distinct presentations of monophasic, recurrent, andultiphasic ADEM have been established in the literature to

escribe subsequent events after an initial ADEM episode. s

ur patient had symptomatic recurrence and brain changesn MRI within 1 month after the acute event had resolved.ccording to Krupp et al [4], this episode was part of the

nitial insult because it included fluctuating symptoms, signs,r MRI findings within 3 months of the inciting ADEM event.urthermore, our patient presented a new event of ADEMith recurrence of initial symptoms and signs at 4 months

fter initial event without new MRI findings. This event fitshe criteria for recurrent ADEM because it occurred morehan 3 months after initial event, it did not occur while theatient was receiving steroids or within 1 month of complet-

ng therapy, and no new findings were observed on MRI [4].hese distinctions were established to help categorize ADEMnd understand its prognosis because the initial presentationf ADEM may be similar to that of multiple sclerosis (MS).owever, it may be distinguished from MS on the basis of

linical, biochemical, and long-term neuroimaging studies [2].The basis of ADEM treatment is immunotherapy, al-

hough there are no randomized controlled trials to prove thefficacy of treatment options. This case is an example of aatient refractory to intravenous corticosteroid who was laterreated with intravenous immunoglobulin also withoutymptomatic response; consequently, plasmapheresis wassed with clinical improvement, although severe functionaleficits persisted. Further research is needed to evaluate theffectiveness of treatment modalities to prevent recurrentDEM and secondary complications.

Rehabilitation issues in ADEM are not well described inhe literature because most cases have little neurologic se-uelae leading to functional deficits. It was believed thateurologic deficits were the main predictor of functionalutcome. There is evidence that long-term functional out-ome is limited by neurocognitive deficits. Sunnerhagen et al7] studied rehabilitation problems after ADEM and foundhat motor and sensory involvement is restored more quicklynd is less important for long-term functional outcome thanocial and cognitive problems. Hahn et al [8], who studiedeurocognitive outcome after ADEM, found that even thosehildren with mild clinical presentations, show excellentlinical recovery, normalization of their MRI findings, anday exhibit mild cognitive impairment years after their acute

llness. These cognitive problems mainly refer to attentionnd executive functions, including visuospatial and visuo-otor function [8]. Our patient had very limited functionalrogress despite multiple therapeutic interventions becausee persisted with both severe motor and cognitive deficits,robably attributable to recurrent demyelination leading toxonal loss [8].

Functional improvement is not dependent on the resolutionf the imaging abnormalities [8]. However, long-term follow-upith sequential imaging studies should be performed to docu-ent recovery and to confirm the diagnosis of ADEM and rule

ut MS [2,3,6,9,10]. Also, a neuropsychological evaluation

hould be recommended to assess possible neurocognitive def-

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cits. Long-term rehabilitation is recommended to help the pa-ient reintegrate into full-time activities or adjust to residualisabilities, especially in cases with recurrent ADEM.

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alomyelitis: A long-term follow-up study of 84 pediatric patients.Neurology 2002;59:1224-1231.

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