sign in sign up
<<
Home , Ataxia

FEATURE

Approach to Acute Ataxia in Childhood: Diagnosis and Evaluation Lalitha Sivaswamy, MD

opsoclonus myoclonus ataxia syndrome, must receive special mention because the underlying disease process may be ame- nable to surgical intervention. In the tod- dler- and school-age groups, certain condi- tions (such as and acute cerebellitis) require immediate recognition and imag- ing, whereas others (such as post-infec- tious ataxia and concussion) require close follow-up. Finally, mention must be made of diseases outside of the central that can present with ataxia, such as Guillain-Barré syndrome.

he word ataxia is derived from the Greek word ataktos, which T means “lack of order.” Ataxia is characterized by disturbances in the voluntary coordination of posture and movement. In children, it is most prominent during walking (the sine qua non being a staggering gait with impaired tandem), but it can also be present during sitting or standing, or © Shutterstock when the child is performing move- Abstract Lalitha Sivaswamy, MD, is Associate Profes- ments of the arms, legs, or eyes. sor of Pediatrics and , Department Ataxia refers to motor incoordination that is This review focuses on the etiol- of Neurology, Wayne State University School of usually most prominent during movement ogy and diagnostic considerations for Medicine; and Medical Director, Headache Clinic, or when a child is attempting to maintain a acute ataxia, which for the purposes of Children’s Hospital of Michigan. sitting posture. The first part of the review this discussion refers to ataxia with a Address correspondence to: Lalitha Sivas- focuses on the anatomic localization of symptom evolution time of less than wamy, MD, Department of Neurology, Wayne ataxia — both within the nervous system 72 hours.1 State University School of Medicine, Children’s and without — using a combination of his- requires sensory Hospital of Michigan, 3901 Beaubien, Detroit, MI torical features and physical findings. The input from muscles and joints. This sen- 48201; email: [email protected]. remainder of the review discusses etio- sory information is transmitted through Disclosure: The author has no relevant finan- logical considerations that vary depending myelinated axons, via the posterior cial relationships to disclose. on the age group under consideration. In columns of the spinal cord, to higher doi: 10.3928/00904481-20140325-13 infancy, certain specific diseases, such as centers in the cortex and basal ganglia.

PEDIATRIC ANNALS 43:4 | APRIL 2014 Healio.com/Pediatrics | 153 FEATURE

tions that may require immediate in- tervention. Presence of confusion, hallucinations, mood disturbances, or somnolence must be noted. Such symp- toms may indicate toxic ingestion, de- myelinating diseases such as acute dis- seminated encephalomyelitis (ADEM), stroke, or meningo-encephalitis. The presence of papilledema, pupillary dila- tion, and lateral rectus palsy indicates elevated intracranial pressure. Motor examination should focus on eliciting in an anatomically significant pattern, such as one-half of the body or both lower extremities. Often, a younger child with or paraparesis will present with ataxia as opposed to focal weakness of the affected body part (ie, “paretic ataxia”). A detailed cerebellar exam should follow. Dysfunction of midline cerebel- lar structures causes and head . Disorders of the lateral aspects of the cause limb Image courtesy of Lalitha Sivaswamy, MD. Image courtesy of Lalitha Sivaswamy, ataxia, which can be evaluated by the Figure 1. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) demonstrating left cerebellar demyelinating in a teenager with acute-onset ataxia. Similar are noted in the finger-nose or finger-finger test, inten- cortex, as well. A final diagnosis of was made on the basis of imaging and investigations. tion tremor, and . In the lower extremity, one can perform These central structures generate their such as musculoskeletal pathology, can the -shin test to look for incoordina- modulating output, which is conveyed lead to clumsy gait. There are no clear tion. Cerebellar incoordination tends to through motor tracts that lay in the data regarding incidence or gender pre- be most prominent when movements stem and spinal cord and then conveyed diction of acute ataxia in the pediatric are performed slowly. Ataxia caused by through peripheral nerves to the rel- age group. pathology of the cerebellum is not ex- evant muscle groups, thereby closing acerbated by eye closure; therefore, a the action loop. The cerebellum exerts CLINICAL EVALUATION Romberg test will be negative. a modulating control at various lev- History Presence of should be not- els of this loop. Furthermore, the ves- Important questions about patient ed with relevant details. Nystagmus may tibular system in the inner ear monitors history that may lead to an etiology arise from pathology in the vestibular angular and linear accelerations of the are summarized in Table 1. As can system (“peripheral” type) or the central head. This information is also conveyed be seen, there is significant overlap nervous system (“central”). Certain spe- to the cerebellum, which utilizes this in symptomatology between different cific types of nystagmus, such as opsoc- feedback to maintain posture.2 Ataxia disease states, so it may be difficult to lonus, may rarely be noted. can, therefore, arise from disturbances distinguish “central” from “peripheral” Finally, a comprehensive sensory in various parts of the nervous system causes by history alone. Symptom on- exam and an examination of deep ten- (eg, the cerebellum, brain stem, spinal set may occur in just a few minutes or don reflexes should be performed to rule cord, and peripheral nerves), as well over the course of hours or days. out diseases of peripheral nerves or roots as the inner ear (Figure 1). To equate (ie, “sensory” ataxia). Plantar responses ataxia with disease of the cerebellum are not affected by diseases of the cer- alone is, therefore, inaccurate. Finally, Physical examination should focus ebellum; hence, a positive Babinski test certain non-neurological conditions, on identifying life-threatening condi- is indicative of disease processes in the

154 | Healio.com/Pediatrics PEDIATRIC ANNALS 43:4 | APRIL 2014 FEATURE

TABLE 1. Questions about Patient History that May Lead to an Etiology of Ataxia

History Potential Etiology Localization of Disease Headache Basilar migraine Brain stem Frontal/temporal Space-occupying lesion* Cerebellum Occipital

Acute onset in an unsupervised toddler or following Ingestion of toxins a fall Cerebellar concussion Brain

Basilar migraine, stroke† Brain stem, cerebellum Space-occupying lesions* Posterior fossa Concussion Benign paroxysmal Labyrinth ADEM Brain stem

Recent history of fever, rash, gastrointestinal illness, Acute post-infectious Cerebellum infectious contacts Acute cerebellitis† Cerebellum , polyarteritis nodosa, Henoch-Schöenlein purpura

Guillan-Barré syndrome† Spinal roots and nerves Recent immunizations Acute post-infectious cerebellar ataxia

Acute post-infectious cerebellar ataxia Cerebellum Recent history of viral illness Acute cerebellitis† Cerebellum Miller-Fisher syndrome Brain stem

Benign paroxysmal vertigo Peripheral * Sympathetic ganglia Nystagmus Multiple sclerosis Brain stem Drug ingestion Stroke† Brain stem, cerebellum

Head or neck trauma in an adolescent (eg, during ski- Stroke Brain stem ing, diving, martial arts) Cerebellar concussion Cerebellum Post-ictal ataxia Brain Seizures Drug overdose Fluctuating drug levels

ADEM Brain, spinal cord (drowsiness, confusion, aggression, Multiple sclerosis Brain, spinal cord ) Drug ingestion

Benign paroxysmal vertigo Brain stem Dizziness (true vertigo, spinning sensation) Basilar migraine Brain stem Stroke† Cerebellum or brain stem

Previous self-limiting episodes with family history Genetic episodic ataxias Not known

History of emotional trauma Psychogenic Not applicable

*Indicates conditions that are unlikely to present in an acute fashion.

†Indicates conditions that require immediate attention.

ADEM = acute disseminated encephalomyelitis.

PEDIATRIC ANNALS 43:4 | APRIL 2014 Healio.com/Pediatrics | 155 FEATURE upper motor neuron pathway, outside of initial presentation, with relatively rapid differs from APCA by the presence, in the cerebellum. resolution over the next few days. The some cases, of systemic symptoms such mental status usually remains clear as fever and neck stiffness, symptoms ETIOLOGY ON THE BASIS OF AGE throughout the course of the illness. The and signs of raised intracranial pressure Infancy presence of extreme irritability should due to rapid compression of the fourth Opsoclonus Myoclonus Ataxia Syndrome raise suspicion about the diagnosis. ventricle, and risk of death; there- Opsoclonus myoclonus ataxia Examination reveals a pure cerebel- fore, there is a pressing need for early (OMA) syndrome can present as early lar syndrome with marked involvement therapy. There is considerable overlap as age 6 months. OMA syndrome is a of gait and significant truncal ataxia. between APCA and acute cerebellitis, paraneoplastic autoimmune phenom- so it may be difficult to distinguish be- enon characterized by chaotic conjugate tween the two conditions. high-amplitude eye movements, body Accidental ingestion of drugs jerks, and truncal/limb ataxia, along with in children in the preschool Toxic Ingestion developmental regression and irritability. Accidental ingestion of drugs in chil- An underlying neuroblastoma or gan- years may account for up to dren in the preschool years may account glioneuroblastoma is often recognized, 30% of cases of acute ataxia. for up to 30% of cases of acute ataxia. although not universally present. Rec- The Purkinje cells of the cerebellum are ognition is facilitated when the triad of especially susceptible to toxic injury. symptoms manifest in close proximity to The average child recovers in less than Accidental ingestion of , each other. However, ataxia alone may 2 weeks after disease onset. It is a self- lead, eucalyptus oil, insecticides such as precede the eye findings, leading to di- limited condition that requires no spe- paraquat and phosphine, dextrometho- agnostic confusion and delays of months cific intervention or investigations. Ce- rphan, and shellfish poisoning may cause to years in initiating investigations.3 rebrospinal fluid (CSF) analysis can be prominent cerebellar symptoms.11 Clini- Nuclear scanning with metaiodobenzyl- performed with no risk of herniation, cal features include depressed menta- guanidine scintigraphy (MIBG scan) has and it usually reveals a mild pleocyto- tion or agitation, seizures, and cerebellar moderately high sensitivity, but children sis with negative viral and bacterial cul- signs. The latter may be masked by the whose scans are negative should receive tures. MRI tends to be normal or shows overall acuity of the situation. Parents high-resolution computerized tomogra- mild, non-specific changes. should be asked to bring in all prescrip- phy (CT) or magnetic resonance imaging tion medication for household members, (MRI) of the chest and abdomen.4 Acute Cerebellitis and a urine/serum drug screen is essen- Acute cerebellitis may occur after a tial in the initial battery of tests. Children Age 1 to 4 Years (Preschool) systemic illness or be the direct result Acute Post-Infectious Cerebellar Ataxia of infection in the cerebellum. Com- Benign Paroxysmal Vertigo Acute post-infectious cerebellar mon agents reported to cause acute cer- Benign paroxysmal vertigo (BPV) ataxia (APCA), which accounts for up to ebellitis are rotavirus, mycoplasma, and must be differentiated from benign par- 40% of cases of acute cerebellar ataxia human herpesvirus 6. Clinical features oxysmal positional vertigo, which is the in certain case series, typically occurs af- include altered sensorium and mani- most common cause of vertigo in adults. ter a febrile illness or immunizations.5 A festations of raised intracranial pres- BPV is characterized by brief spells of prior history of varicella was reported in sure, in addition to features of a pure vertigo and ataxia. An otherwise healthy up to 26% of patients by some authors.1 cerebellar syndrome. CSF analysis is child suddenly looks frightened, pale, A large number of other viruses have likely to reveal pleocytosis, and in rare and wants to hold on to a parent for sup- been implicated, including coxsackie B, cases, antibodies against an infectious port during episodes of BPV.12 The du- echoviruses, mumps, Epstein-Barr, and agent have been demonstrated.8 It must ration of symptoms is a few minutes at influenza A and B. The pathology is be- be mentioned that performing a spinal most, after which the child experiences lieved to be acute demyelination caused tap in the face of significant cerebellar no confusion or sleepiness.13 The typical by cross-reacting antibodies to epitopes edema can be life-threatening. Imag- age of onset is age 1 to 4 years, and reso- in the cerebellum.6 The disease onset ing can reveal abnormalities suggestive lution occurs around the age of 7 to 10 can be up to 3 weeks after the systemic of edema in the cerebellum. Fatalities years. Examination is invariably normal illness has subsided.7 Symptoms evolve have been reported from acute cereb- between spells, as are imaging studies.14 over hours and are most prominent at ellitis.9,10 In essence, acute cerebellitis A family history of migraine is found in

156 | Healio.com/Pediatrics PEDIATRIC ANNALS 43:4 | APRIL 2014 FEATURE many cases, and BPV itself is believed been noted to have cerebellar concussion Multiple Sclerosis to be a migraine precursor.15 following relatively minor head trauma.21 Multiple sclerosis (MS) is an autoim- The pathophysiology is believed to be mune that affects Acute Disseminated Encephalomyelitis damage to the connections between the different parts of the nervous system ADEM is an immune-mediated phe- cerebellum and the cortex, especially the over time. About 50% of the children nomenon that presents after a viral ill- superior cerebellar peduncle, following younger than the age of 5 years who ness or immunization and is character- severe . Transient vasospasm have MS and 5% to 15% of adolescents ized by encephalopathy (confusion, is the purported mechanism behind ataxia who have MS present with acute ataxia, irritability, somnolence, personality following mild injuries.22,23 No specific making it a relatively frequent disease changes) and acute onset of multifocal intervention is required and resolution is manifestation.27,28 Children with MS neurological deficits — most commonly usually complete. have more frequent disease relapses than ataxia.16 Seizures, cranial nerve palsies, their adult counterparts, and they expe- hemiparesis, fever, and meningismus are Stroke rience significant cognitive decline and frequently present.17 Imaging reveals in the region of the cerebel- severe residual in their early bright lesions in the subcortical white lum (posterior circulation) are easily adult years. Early imaging in the form matter, cerebellum, and basal ganglia. missed, as strokes are uncommon in of MRI with administration of contrast CSF is abnormal about half the time, childhood and posterior circulation material and referral to a neurologist for with elevated white cells and protein. strokes present with subtle symptoms initiation of therapy is essential. ADEM is usually a monophasic illness. that are often non-specific, with no clear-cut motor or sensory disturbances. Prescription Drug Use, Substance Abuse, Inner Ear Disease Posterior circulation strokes present and Poisoning One of the rare complications of with dizziness about three-quarters of Adolescents who present with acute acute otitis media is labyrinthitis, which the time, /vomiting or gait distur- ataxia should be questioned regarding is an extension of the middle ear bac- bances about one-half of the time, and drugs of abuse as well as therapeutic terial infection to the inner ear or laby- headache about a one-third of the time.24 agents they may be using for medical rinth, causing sensorineural hearing Onset of symptoms is abrupt. Examina- purposes. , , loss, tinnitus, nystagmus, and vertigo.18 tion may reveal ataxia with tendency to and carbamazepine are the anticonvul- The vertigo leads to ataxia. Fever, full- fall to the affected side, as well as verti- sants most likely to cause ataxia follow- ness and pain in the ear, and mastoiditis cal nystagmus, in addition to other clas- ing acute intoxication. Free as well as may serve as clues to the diagnosis. The sic cerebellar signs. Presence of altered total levels of drugs must be measured presence of nystagmus in a child with level of consciousness, hemiplegia, and in these situations. Antineoplastics, such acute otitis media is often a reliable clue cranial nerve weakness, when present, as fluorouracil (5-FU), (ara- to the presence of impending labyrinthi- may quickly guide the clinician to an C), and methotrexate, may induce acute tis, so it should be treated expeditiously. appropriate diagnosis. MRI is the diag- cerebellar damage. Drugs of abuse that The presence of contrast enhancement nostic test of choice because CT scan may cause ataxia are toluene, cocaine, of the labyrinth on MRI is a sensitive can miss a stroke in the cerebellum and , and . Toluene in- indicator of disease process in this area. brain stem. The majority of cerebellar toxication can occur due to glue-sniffing CT is less reliable.19 strokes are caused by arteriopathy that or acute exposure in areas that are poorly may follow infection (such as varicella), ventilated.29 Cocaine use predisposes to Children Age 5 to 16 years vasculitis (such as Kawasaki disease), cerebellar infarctions, as does envenom- (School Age) or dissection. Edema following a stroke ation due to a scorpion sting.11 Concussion is most prominent in the first 24 hours, Cerebellar concussion is a clinical syn- and if it is in the region of the posterior Basilar Migraine drome wherein a head injury is followed circulation, then it is particularly omi- Children with basilar migraine pres- by transient deficits in functioning of the nous.25 Rapid swelling may compress ent with an aura consisting of ataxia, cerebellum, with intact consciousness the brain stem. The vast majority of diplopia, tinnitus, tingling in their ex- throughout. Patients typically present such patients will die without surgical tremities, or alteration in the level of with a wide-based gait, truncal instability, intervention.26 Therefore, all cerebellar their consciousness that lasts from 5 and . Most cases follow severe strokes must be closely observed in an minutes to 1 hour. A severe, pounding injuries;20 however, a few children have intensive care setting. headache follows within 1 hour of these

PEDIATRIC ANNALS 43:4 | APRIL 2014 Healio.com/Pediatrics | 157 FEATURE

Previous episodes of similar nature New onset acute ataxia

No HISTORY Yes Fever Lethargy/confusion/irritability Basilar migraine Ear pain/discharge Labyrinthitis, ADEM, Seizures MS, stroke, cerebellitis, Benign paroxysmal vertigo Head injury cerebellar concussion

Physical Exam Papilledema No Sixth nerve palsy Yes Stroke, cerebellitis, Poor gag/cough/voice change MS, ADEM, GBS, Facial weakness Miller-Fisher syndrome Hemiparesis Absent tendon reflexes

ED referral Postinfectious Neurology consult cerebellitis Intensive care consult

Close outpatient MRI brain – rule out stroke/MS/ADEM/cerebellitis follow-up/brief period of MRI inner ear – rule out labyrinthitis hospital observation. Drug screen If infant, obtain MIBG scan and/or CT abdomen and ECG chest – rule out neuroblastoma Therapeutic drug levels Figure courtesy of Lalitha Sivaswamy, MD. courtesy of Lalitha Sivaswamy, Figure Figure 2. Algorithm for investigating acute ataxia. ADEM = acute disseminated encephalomyelitis; CT = computed tomography; ECG = electrocardiogram; ED = emergency department; GBS = Guillain-Barré syndrome; MIBG = metaiodobenzylguanidine scintigraphy; MS = multiple sclerosis; MRI = magnetic resonance imaging. symptoms and is typically accompanied eral nerves that do not allow appropri- may include imaging of the spinal cord by nausea/vomiting and photophobia/ ate sensory signals to reach the higher to rule out myelitis and spinal fluid phonophobia. The headache may be coordinating centers. A classic exam- analysis, although in most instances unilateral or bilateral and is relieved by ple of this is Guillain-Barré syndrome. the diagnosis remains a clinical one sleep. The average duration of the head- A preceding viral infection or gastro- that can be made with assurance at ache is 30 minutes to 3 days. Examina- enteritis is noted in at least half of all the bedside due to the triad of ataxia, tion is unremarkable, with no clear cer- cases. Children may experience severe areflexia, and motor weakness. Nerve ebellar signs other than ataxia. Initial pain in the lower extremities or back conduction studies may be performed episodes warrant an MRI. prior to the onset of weakness.30 Weak- in atypical cases to confirm the diag- ness and ataxia reach a nadir within 4 nosis. Coordination with a neurologist Guillain-Barré Syndrome weeks. Rare instances may progress to and intensivist is vital in children be- Children may present with ataxia involvement of the respiratory muscles ing prepared for treatment of Guillain- due to disease processes in the periph- and quadriparesis. Diagnostic testing Barré syndrome.

158 | Healio.com/Pediatrics PEDIATRIC ANNALS 43:4 | APRIL 2014 FEATURE

Episodic Ataxia myoclonus syndrome. J Child Neurol. 18. Kitsko DJ, Dohar JE. Inner ear and facial Hereditary episodic ataxia (EA) re- 2012;27(11):1482-1488. nerve complications of acute otitis media, 4. Gorman MP. Update on diagnosis, treat- including vertigo. Curr Allergy Asthma Rep. fers to a group of dominantly inherited ment, and prognosis in opsoclonus-myclo- 2007;7(6):444-450. conditions characterized by periods of nus-ataxia syndrome. Curr Opin Pediatr. 19. Pelligrini S, Gonzalez Macchi ME, Sommer- cerebellar dysfunction that lead to acute 2010;22(6):745-750. fleck PA, Bernaldez PC. Intratemporal com- 5. Gieron-Korthals MA, Westberry KR, Emman- plications from acute otitis media in children: ataxia. Seven different subtypes are uel PJ. J Child Neurol. 1994;9(4):381-384. 17 cases in two years. Acta Otorrinolaryngol recognized, of which EA2 is the most 6. Adams C, Diadori P, Schoenroth L, Frit- Esp. 2012;63(1):21-25. common. Attacks are precipitated by zler M. Autoantibodies in childhood post 20. Fumeya H, Hideshima H. Cerebellar concus- varicella cerebellar ataxia. Can J Neurol Sci. sion- three case reports. Neurol Med Chir. fatigue or strong emotion (such as an- 2000;27(4):316-320. 1994;34(9):612-615. ger or sadness) and may last for hours. 7. Nussinovitch M, Prais D, Volovitz D, Sha- 21. Gohil JR, Munshi SS. Post concussion ataxia In addition to ataxia, patients may ex- piro R, Amir J. Post-infectious acute cer- following minor head injury. Indian Pediatr. perience vertigo, nausea, vomiting, and ebellar ataxia in children. Clin Pediatr. 2006;43(9):829. 2003;42(7):581-584. 22. Haggard P, Miall RC, Wade D, et al. Dam- 31 (rarely) seizures. Patients may have 8. Gayatri N, Tyagi A, Mahadevan U. Acute hy- age to cerebellocortical pathways after closed migranous headaches and downbeat drocephalus in a child with mycoplasma cer- head injury: a behavioral and magnetic reso- nystagmus between spells. The muta- ebellitis. Brain Dev. 2009;31(8):618-621. nance imaging study. J Neurol Neurosurg 9. Kamate M, Chetal V, Hattiholi V. Fulminant . 1995;58(4):433-438. tion responsible for EA2 is located on cerebellitis: a fatal clinically isolated syn- 23. Yeoh HK, Lind CR, Law AJ. Acute transient the CACNA1A gene. drome. Pediatr Neurol. 2009;41(3):220-222. cerebellar dysfunction and stuttering follow- 10. Hacohen Y, Niotakis G, Aujla A, et al. Acute ing mild closed head injury. Childs Nerv Syst. life threatening cerebellitis presenting with no 2006;22(3):310-313. CONCLUSION apparent cerebellar signs. Clin Neurol Neuro- 24. Edlow JA, Newman-Toker DE, Savitz An algorithm for work-up of acute surg. 2011;113(10):928-930. SI. Diagnosis and initial management ataxia is presented in Figure 2. Ataxia 11. Manto M. Toxic agents causing cerebellar of cerebellar infarction. Lancet Neurol. can cause significant anxiety for the ataxias. Handb Clin Neurol. 2012;103:201- 2008;7(10):951-964. 213. doi: 10.1016/B978-0-444-51892- 25. Mackay MT, Prabhu SP, Coleman L. Child- child and family due to the dramatic na- 7.00012-7. hood posterior circulation in arterial ischemic ture of the symptoms. The fact that most 12. Basser LS. Benign paroxysmal vertigo of stroke. Stroke. 2010;41(10):2201-2209. children with acute onset ataxia will childhood (a variant of vestibular neuronitis). 26. Heros SC. Surgical treatment of cerebellar in- Brain. 1964;87:141-152. farction. Stroke. 1992;23(7):937-938. have rapid resolution of their symptoms 13. Marcelli V, Piazza F, Pisani F, Marciano E. 27. Bigi S, Banwell B. Pediatric multiple sclero- must be emphasized to parents and care- Neuro-otological features of benign parox- sis. J Child Neurol. 2012;27(11):1378-1383. givers. Collaboration with a child neu- ysmal vertigo and benign paroxysmal posi- 28. Banwell B, Ghezzi A, Bar-Or A, Mikael- tioning vertigo in children: a follow up study. off Y, Tardieu M. Multiple sclerosis in chil- rologist, radiologist, and intensive care Brain Dev. 2006;28(2):80-84. dren: clinical diagnosis, therapeutic strate- physician may be of benefit in cases 14. Cuvellier JC, Lepine A. Childhood periodic gies, and future directions. Lancet Neurol. where serious intracranial pathology is syndromes. Pediatr Neurol. 2010; 42(1):1-11. 2007;6(10):887-902. a consideration. 15. Lanzi G, Balottin U, Fazzi E, Mira E, Pia- 29. King MD. Neurological sequelae of toluene centino G. Benign paroxysmal vertigo in abuse. Hum Toxicol. 1982;1(3):281-287. childhood: a longitudinal study. Headache. 30. Bradshaw B, Jones H Jr. Guillain-Barre syn- REFERENCES 1986;26(10):494-497. drome in childhood: clinical course, elec- 1. Ryan MM, Engle EC. Acute ataxia in child- 16. Hynson JL, Kornberg AJ, Coleman LT, et al. trodiagnosis and prognosis. Muscle Nerve. hood. J Child Neurol. 2003;18(5):309-316. Clinical and radiological features of acute 1992;15(4):500-506. 2. Brazis PW, Masdeau JC, Biller J. Localiza- disseminated encephalomyelitis in childhood. 31. Winchester S, Mikati MA. Ataxia. In Dulac tion in Clinical Neurology. 4th ed. Philadel- Neurology. 2001;56(10):1308-1312. O, Lassonde M, Sarnat HB, eds. Handbook of phia, PA: Lippincott Williams & Wilkins; 17. Tenembaum S, Chamoles N, Fejerman N. Clinical Neurology. Amsterdam, Netherlands: 2001:309-327. Acute dissmeniated encephalomyelitis: a Elsevier; 2013:1213-1217. 3. Desai J, Mitchell WG. Acute cerebellar long- term follow- up study of 84 pediatric ataxia, acute cerebellitis, and opsclonus- patients. Neurology. 2002;59(8):1224-1231.

PEDIATRIC ANNALS 43:4 | APRIL 2014 Healio.com/Pediatrics | 159