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BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a nonvasculitic inflammatory demyelinating condition that bears a striking clinical and pathological

resemblance to multiple sclerosis (MS). In most instances, ADEM and MS cases occurring in children are readily distinguishable on the basis of clinical features and findings on laboratory investigations. Neither condition (in reality family of conditions) has a currently identified biological marker rendering a reliable laboratory diagnosis for either condition. MS is typically a chronic relapsing and remitting disease of young adults, while ADEM is typically a monophasic disease of prepubertal children. Abnormalities of findings on cerebrospinal fluid (CSF) immunoglobulin studies are likely in MS but are much less common in ADEM. The onset of ADEM usually occurs in the wake of a clearly identifiable febrile prodromal illness or immunization and in association with prominent constitutional signs and encephalopathy of varied degree. However, the division between these processes is indistinct, which is suggestive of a clinical continuum. Moreover, other conditions along the suggested continuum include optic neuritis, transverse myelitis, and Devic syndrome, clinical entities that may occur as manifestations of either MS or ADEM. Other boundaries of ADEM merge indistinctly with a wide variety of inflammatory encephalitic and vasculitic illnesses as well as monosymptomatic postinfectious illnesses that should remain distinct from ADEM, such as acute cerebellar ataxia (ACA). A further indistinct boundary is shared by ADEM and Guillain-Barré syndrome and that entity is manifested in cases of Miller-Fisher syndrome and encephalomyeloradiculoneuropathy (EMRN). Susceptibility to either condition is likely the product of multiple factors, including a complex interrelationship of genetics and exposure to infectious agents and possibly other environmental factors. Of particular interest are the indications that susceptibility to either condition is in part age related. Most cases of either MS or of ADEM possibly occur as the result of an inflammatory response provoked by prepubertal infection with a virus, viral vaccine, or other infectious agent. Typically, the manifestations of ADEM occur quickly after this prepubertal febrile systemic illness and are monophasic. In a minority of cases, patients with ADEM experience 1 or 2 prepubertal recurrences followed by remission.

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MS plaques are known to exhibit organization features. demyelinative bouts. Immuno-dysregulation in MS or ADEM may consist of responses that are inadequate. or both and that the degree of susceptibility may describe a gradient with regard to severity and risk for recurrence. If a pathophysiological continuum between MS and ADEM exists. Cases with characteristics that fall in the indeterminate area of this continuum. but patchy demyelination with preservation of axon cylinders and the prominence of microglial cells in the inflammatory exudate are not. each resembling the pathology of experimental allergic encephalomyelitis (EAE). especially in the margins of active plaques. 2|Page . Few biopsies have been obtained or submitted to postmortem analysis. fraught as that is with psychosocial consequences. appropriately crediting the immune system with tardy but permanent compensation may be important. The prominence of perivenular round cell inflammation in either illness is a feature that is shared with many forms of encephalitis. In some of these cases. achieving better understanding of the manner in which susceptible individuals with ADEM are able to bring a monophasic or temporarily recurrent immuno-dysregulative response under permanent control is of obvious importance. the likelihood of recurrence may be determined by the fertility of that constitution for persistence of immuno-dysregulation. or possibly a few. that are not found in cases of ADEM. The pathology of various developmental stages of the MS plaque is more fully characterized than the pathology of the lesions of ADEM. represent an important challenge for accurate classification. On the other hand. The threshold for an initial bout of demyelinative illness may be determined by the combination of this immunologic constitution and the nature of a given antigenic stimulus. MS. or the combination of both. too exuberant. thus avoiding inappropriate diagnosis of MS.PATHOPHYSIOLOGY Multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) bear a close pathological resemblance. This is because most patients with ADEM recover completely and without apparent pathological residua. such as those that might be labeled recurrent ADEM. The pathophysiological similarities of these illnesses suggest that the immunologic constitution of susceptible individuals is in some fashion permissive of ADEM. the general pathological similarities suggest but do not confirm the possibility that ADEM is a forme fruste of MS that is somehow effectively and permanently controlled after one.

IL-10. both MS and ADEM are likely to be mediated by stimulated clones of T-helper cells sensitized to autoantigens such as myelin proteins. CCL17. microglial cells. CSF concentrations of the chemokine CCL11 is lower in adults with MS than in the CSF from adults with ADEM or in normal controls. phagocytes). B cells. Elevations of IFN-gamma. or in normal healthy controls. ADEM-associated concentrations of certain of these neutrophils (CXL7 neutrophil activator and the CL1. IL-6. On the other hand. Basic studies have shown that. Il-2. CCL17. monocytes (CCL3 and CCL5). interferon (IFN)–gamma. microglia.[2] Moreover. However. and CCL22 Th2 activators) are higher in the CSF from individuals with ADEM than those with MS. Interleukin (IL)–1beta. Additionally. IL-8. tumor necrosis factor-alpha. Granulocyte colony-stimulating factor shows a particularly striking elevation at as much as 38-fold greater concentration than is found in the CSF from control subjects. and Th2 cells (CCL1. IL-5. those with ADEM. No significant differences in serum concentrations of cytokines or chemokines were noted in the 3 adult groups. pathological. and IL-8 have been significantly correlated with CSF cell counts and protein concentration in individuals with ADEM. Franciotta et al demonstrated that adults with ADEM have higher CSF concentrations of chemokines that recruit or activate neutrophils (CXL1 and CXL7). in the earliest stages of inflammation. other T cell lines. The pattern of cytokine elevation suggests that ADEM involves activation of macrophages. IL-4. and CCL22) than healthy normal controls. Th1 cells (CXCL10). the possibility of provoking spontaneously recurrent demyelination with this model further supports the concept that ADEM and MS represent a continuum. CSF Th1/Th2 cytokine concentrations were not significantly different in adults with MS.The mechanisms of these demyelinative illnesses remain incompletely understood despite the extraordinary richness and complexity of immunologic abnormalities that have been identified after more than a century of clinical. The complex ensuing inflammatory cascade entails the local action of cytokines and chemokines as well as lymphokine-induced chemotaxis of other cellular mediators of inflammation (eg. Pathogenic differences of MS and ADEM are likely to arise in part because of differences in details concerning proinflammatory and anti-inflammatory cytokines and chemokines. and macrophage inflammatory protein-1beta are significantly elevated in CSF compared with the CSF of controls. in 2006. and laboratory studies. These findings raise 3|Page . and various Th (T helper)–1 and Th2 cells. IL-6.

involving cases with fulminant cervical transverse myelitis or brain swelling. may participate in the progression of MS. Discerning how these inflammatory changes differ in MS or ADEM and how the reactions in either illness are distinguishable from those in other inflammatory and infectious illnesses are among the important subjects of current research. Gray matter involvement also occurs in ADEM. techniques for intensive care were comparatively primitive. EPIDEMIOLOGY Frequency Little is known about occurrence throughout the world. degree of skin pigmentation. Of particular importance is immunization because immunization to pathogens known to provoke ADEM has reduced the incidence of severe forms of ADEM such as those that may follow cases of measles and other viral illnesses. immunization status. a severe ADEM variant. especially of descending subcortical fibers. Current acute mortality rates are probably less than 2%. Disturbance of the blood-brain barrier is likely to be an important event. Genetic factors. and other factors may influence risk. Children younger than 2 years are particularly subject to such severe presentations. diet. prevalence of infectious pathogens. 4|Page . deaths occurred in patients with AHLE. and anti-inflammatory therapies were inadequate. which has become less common since children have received immunization to many common childhood illnesses. the data upon which such estimates were based were obtained in epochs during which measles was prevalent. Recent evidence in studies of the brains of individuals with MS suggest that gray matter degeneration. Formerly. except that cases are likely to occur in all regions of the world. Mortality/Morbidity Although older studies suggest a 10% mortality rate.the possibility that elevated chemokine concentrations might serve as biomarkers for ADEM and that they may provide keys to understanding of the nature of and differences in the pathogenesis of ADEM and MS. The elaboration of antibodies occurs but remains of uncertain significance.

Race The scientifically imprecise concept of race does not lend itself readily to discussions of ADEM. these problems persist after the first few weeks of illness in only about 35% of cases. PHYSICAL Signs and symptoms found in cases of ADEM : o Alteration in personality o Abnormal consciousness (65-75%) o Ataxia (appendicular more than axial or gait) o Cranial nerve palsies (35-40%) 5|Page . accounting for less than 3% of the reported cases. Overall. referral bias complicates any assessment of relative prevalence. Incidence in adulthood is unclear. and the combination Devic syndrome.Morbidity chiefly includes visual. the ratio of boys to girls is 1.3:1. and intellectual deficits and epilepsy. No other substantial data are available. Visual and motor deficits and problems with bowel or bladder function may persist for varying periods of time (months to permanently) in some cases. particularly in those with transverse myelitis. Age More than 80% of childhood cases occur in patients younger than 10 years. and in most of these patients. motor. In the former group. the ratio of light-skinned to dark-skinned individuals who have some contribution of genetic material from individuals who have left Africa in the past 5 centuries is approximately 6:1. Sex In the authors' series of more than 150 cases. optic neuritis. ADEM is found in all ethnic groups and races. somewhat less than 20% of cases occur in the second decade of life. autonomic. the element of African heritage from the past 5 centuries is presumed small but is in fact unknown. Intellectual deficits (varying from attention problems to mental retardation) and epilepsy arise most often in children whose bout of ADEM occurs before the second birthday. In the authors' series of more than 150 cases. the deficits resolve within 1 year of onset.

the two phases of illness are typically separated by a phase of recovery from fever and other constitutional manifestations of the initial infectious phase of illness. and mycoplasma.o Headache o Language disturbances (10%) o Meningeal signs o Nystagmus o Psychiatric abnormalities o Optic neuritis (10-30%) o Ophthalmoparesis o Seizures. cytomegalovirus. measles was the most common associated illness. focal or generalized (25%) o Sensory loss/dysesthesia o Visual field deficits o Vomiting CAUSES Acute disseminated encephalomyelitis (ADEM) may develop in the wake of a wide variety of infectious illnesses or immunizations. the less certain the etiologic association. The longer the interval between presumed prodrome and ADEM. Among the agents most commonly identified by titer rise suggesting responsibility for the prodromal phase are Ebstein-Barr virus. herpes simplex virus (HSV). especially those associated with large envelopebearing viruses. ADEM is somewhat more common in the colder months of the year. a particular agent is identified only in a minority of ADEM cases. Establishing the etiologic role of immunizations has proven controversial. specific viral agents are seldom identified. during which these various viral illnesses are more prevalent. A minority of cases lack a prodromal phase. However. Prior to widespread immunization programs. Now. The hiatus between onset of viral symptoms and onset of ADEM may range from 2-20 days. ADEM may possibly arise after intervals as long as 30 or more days after an infectious prodrome. 6|Page . most cases occur in the wake of respiratory or gastrointestinal illnesses that are presumed to be of viral etiology. ADEM occurred in approximately 1 out of 800 cases.

Sedimentation rates are occasionally mildly elevated.  Many of the diseases that constitute the differential diagnosis of ADEM produce MRI abnormalities that emulate various ADEM-associated lesions. CSF:serum immunoglobulin G [IgG] index. greater elevation suggests the possibility of vasculitis or infection. Such lesions are found in more than 90% of children with ADEM. CSF myelin basic protein concentration level. especially in recurrent herpes encephalitis. CNS IgG synthetic rate. Elevated CSF HSV or Lyme titers do not exclude the possibility of associated ADEM. Some cases of encephalitis result in the development of multiple tiny or small patches of bright signal 7|Page . proton-density. Red blood cells may be due to modest degrees of AHLE. but this technique is far less sensitive than MRI for the disclosure of extent and number of lesions. IMAGING STUDIES The CT scan low-density abnormalities are found in more than half of childhood or adolescent ADEM cases. Modest-to-moderate elevation of CSF white and red blood cell counts may be found in childhood ADEM. or echo-planar trace diffusion MRI techniques disclose characteristic high-signal lesions in more than 80-90% of cases of ADEM. T2/FLAIR images are particularly important in the evaluation of young individuals presenting with inflammatory CNS demyelinative illnesses that may be ADEM or MS.LABORATORY STUDIES Platelet counts are elevated in a substantial number of children with ADEM. sarcoid. neuromyelitis optica. oligoclonality) employing age-appropriate normative data are positive in fewer than 10% of prepubertal children with ADEM. In such instances it remains incumbent on those evaluating such individuals to exclude non-MS illnesses with specific biological markers such as systemic lupus. They are found in less than 40% of adults initially diagnosed as having ADEM. many of whom are later diagnosed as having MS. Results of CSF immune profile testing (eg. Positivity of studies for CSF oligoclonal bands and immunoglobulin elevation favors the diagnosis of MS in individuals younger than 20 years with first or recurrent bouts of acute CNS demyelinating illness. reflecting demyelination. is frequently elevated in ADEM. T2-weighted. Apparent diffusion coefficient maps show high-signal changes consistent with vasogenic edema. and so forth. ADEM lesions are characteristically centrifugal at the junction of the deep cortical gray and subcortical white matter.

Periventricular lesions (30-45%) and corpus callosum lesions (2025%) are uncommon in childhood ADEM compared with MS. and ADEM are occasionally mistaken for one another. ADEM may produce large unilateral T2 bright lesions. Recognize that no changes on MRI are pathognomonic of ADEM or for that matter of demyelination. even though patients are then showing clinical improvement. that normal findings on a scan do not exclude the 8|Page . some of which appear to have striking central cavitation.  Additional lesions may be found in deeper white matter. or MS. and the spinal cord. posteriorly emphasized white matter changes on T2 weighting suggest leukodystrophy. linear. MS plaques tend to vary in degree of contrast enhancement and may at times enhance quite densely. and few if any are aligned in the Dawson finger orientation. the cerebellum (30-40%). Unusual MRI abnormalities that are found in young individuals suspected of having ADEM may help greatly in distinguishing ADEM from MS or other alternative diagnoses. although encephaloclasia may not be found on follow-up scanning after recovery. Some patients with ADEM have normal findings on MRI on initial presentation that become abnormal and characteristic of ADEM if the study is repeated several weeks later. but response to corticosteroid therapy is poor and follow-up scans may show severe encephaloclasia. cysticercosis). optic nerves. stroke. In rare cases. changes never found in MS plaques. neoplasia. The distribution of ADEM lesions ranges from fairly symmetrical to very asymmetrical. The degree of contrast enhancement of ADEM lesions is typically uniform and usually not very dense. symmetrical. These lesions may suggest neoplasm. Ring enhancement or mass effect sometimes found in ADEM may suggest abscess or tumor.  The indistinct margins of childhood ADEM lesions tend to suggest a "smudged" edge rather than the crisp margin typical of the classic ellipsoid plaques of MS. the brainstem (45-55%). ADEM gives rise to a much wider variety of appearances than MS. This suggests that characteristic features may be missed because of sampling error.on T2-weighted images that have been mislabeled as ADEM. In contrast. abscess. parasitism. Pial enhancement does not occur in ADEM and suggests meningoencephalitis. basal ganglia (30-40%). the thalamus (30-40%). As many as 90% of childhood ADEM lesions enhance with gadolinium. ADEM lesions may contain areas of hemorrhage suggestive of HSV2 encephalitis. Metazoal parasitic diseases of the brain (eg. HSV2 encephalitis or Lyme disease may be difficult to distinguish from ADEM and may involve ADEM mechanisms in pathogenesis.

viruses. rhythmic delta. o The immune profile is also helpful in distinguishing ADEM from MS. Any 2 of the following criteria could distinguish MS from acute disseminated encephalomyelitis (sensitivity 81%. PROCEDURES The lumbar puncture is an essential aspect of acute disseminated encephalomyelitis (ADEM) workup. single photon emission CT scanning. It assists in distinguishing ADEM from various forms of meningoencephalitis. Diagnosis of ADEM should always rest on clinical grounds in children as in adults. One or more of these studies is positive in no more than 10% of ADEM cases. diagnosing ADEM on the basis of findings on scanning alone is dangerous. IgG synthetic rate. especially upon the basis of titers for the various bacteria. From a retrospective analysis. although the development of a pathognomonic imaging result is unlikely. The IgG index. or other agents that may produce a directly infectious form of meningoencephalitis.  Radiographic studies and other laboratory tests are especially valuable in ruling in or out alternative diagnoses.ADEM diagnosis. or oligoclonal bands are positive in more than two thirds of all first clinically recognized MS bouts and in 90-98% of individuals who have experienced multiple MS bouts.  Magnetization transfer MRI. 9|Page . and (3) presence of 2 or more periventricular lesions. Callen et al propose diagnostic criteria for MRI to distinguish a first MS attack in children from those with acute disseminated encephalomyelitis. specificity 95%): (1) absence of a diffuse bilateral lesion pattern. OTHER TESTS The EEG often exhibits disturbance of normal sleep rhythms. or spikes may be found in the waking state during the early stages of ADEM. Focal or generalized slowing. and that the appearance of new lesions during recuperation from ADEM may not represent recrudescence of disease. sharp waves. (2) presence of black holes.  For these reasons. or nuclear magnetic resonance (NMR) spectroscopy may possibly prove helpful in distinguishing ADEM from alternative diagnoses.

An oral taper for 3 weeks or some other interval is sometimes appended. some brain tumors. sarcoid. brain biopsy is necessary to distinguish ADEM from other diagnostic possibilities. and various forms of acute or chronic bacterial or viral meningoencephalitis. The same argument may hold true for severe cerebral ADEM such as tends to arise in some young children (< 3 y old) who also may have marked permanent neurologic impairments after severe ADEM. The CSF : serum IgG index or synthetic rate formulations may show positive results in neurosyphilis. MEDICAL CARE Acute disseminated encephalomyelitis (ADEM) is often treated with high-dose intravenous corticosteroids. Lyme disease. very high-dose corticosteroids (30-50 mg/kg) administered intravenously at presentation to patients with transverse myelitis may be advantageous from the vantage point of its capacity to close the blood-brain barrier and limit swelling. The diagnosis of ADEM is confirmed when typical perivenular demyelinative changes with axonal sparing are observed. IVIG may be preferable in instances where meningo-encephalitis cannot be excluded based upon the hypothesis that corticosteroids might worsen the course of infection. One common protocol is 20 mg/kg/d of methylprednisolone (maximum dose of 1 g/d) for 3-5 days. Occasionally. although this approach is employed by some clinicians. Marked cord swelling may account for poor outcome in some cases of transverse myelitis because of circulatory impairment and cord infarction. Theoretically. Guillain-Barré syndrome. and a wide variety of bacterial or viral meningoencephalitides or other forms of CNS inflammation. subacute sclerosing panencephalitis (SSPE). stroke.o Note that the findings on immune profile studies may be positive in various infectious conditions such as neurosyphilis. Improvement may be observed within hours but usually requires several days. 10 | P a g e . There is as yet no convincing evidence that treatment with the combination of intravenous corticosteroids and IVIG confers any advantage in such cases. Lyme disease. It is administered as 2 g/kg intravenously for 2-3 days. to which it appears to be responsive. The chief alternative therapy is intravenous immune globulin (IVIG).

with such alternative approaches as :  Combination of intravenous corticosteroids and IVIG  Cyclosporin  Cyclophosphamide  Plasma exchange/plasmapheresis . Non–taper-related recurrences occur in as many as 5% of children with ADEM. Taper-related recurrence occurs in as many as 3-5% of cases and usually responds to prolongation of taper. This rare and interesting subgroup tends to have onset of disease before 6 years of age. Although it has been suggested that IVIG administered in treatment of a single recurrence may prevent further recurrence. and despite recurrence.Severe ADEM has also been treated. IgG synthetic rate. adhesion molecules. The relationship of this group to patients with ADEM or MS or some other form of inflammatory CNS illness remains unclear. A subset of patients manifest repeated recurrences that prevent discontinuation of corticosteroids or necessitate changing to various steroid-sparing treatments such as cyclophosphamide or beta-interferons. although some prepubertal children manifest 2 or even 3 recurrences within a year or two of the initial bout but then manifest no further recurrences for follow-up intervals as long as 18 years. Similar phenomena occur in other postinfectious diseases. IgG index. Greater understanding of trimolecular complex regulation. The polymorphism of the human major histocompatibility complex and apparent heterogeneity of T cell response to autoantigens render this a daunting project. apparently successfully. such as Guillain-Barré syndrome or opsoclonus-myoclonus. the evidence for this remains inconclusive because most children with a single recurrence of ADEM that are treated with corticosteroids also have no further recurrences. although anticytokines represent an intriguing avenue of therapeutic research. and inflammatory cytokines may permit development of more specific and effective ADEM therapies. 11 | P a g e . these children do not manifest evidence for CSF immune profile (ie. oligoclonal bands) abnormality. In such instances most children have just a single recurrence.

The possible exceptions are ADEM-related postinfectious demyelinative syndromes. mumps. 12 | P a g e . and non-Reye syndrome. Pediatric intensivists generally become involved in severe cases for management of airway. ACTIVITY No clear restrictions on activity exist except as indicated by the severity of disease.SURGICAL CARE Surgical treatment for severely elevated intracranial pressure has been undertaken for cases of AHLE. subsequent disappearance in Asia and the Middle East). Because these severe cases often followed measles. This rule may also be true of the relapsing neurobrucellotic illnesses. Although such severe cases were regularly noted in the medical literature from the 1920s until the mid 1970s. Some of these cases were likely examples of hyperacute ADEM. this change in prevalence likely reflects the removal of pathogens that are provocative of such severe forms of ADEM. examples of what have been termed obscure encephalopathies of infancy. and other diseases for which effective vaccines have been developed and because the disappearance of such cases has followed the availability and use of such vaccines (earlier disappearance in the United States and Western Europe. that arise in the wake of illnesses such as brucellosis or malaria. sometimes in association with the development of brain edema. breathing. Prevalence clearly has dramatically decreased. including the types that closely resemble or are examples of ADEM. Outcome of such interventions was mixed. Surgical interventions have ranged from placement of pressure bolts to decompression of the intracranial fossae by unroofing of the cranium. CONSULTATIONS Consultations with infectious disease specialists are occasionally warranted to consider alternative diagnoses. recovery is clearly more rapid and relapse is less likely if patients are treated with enforced bedrest. few examples have been noted since that time. hemorrhagic brain purpura. In the case of acute brucellosis. and circulation.

Although somewhat less clear in the case of cerebral malaria. this form of therapy appears. 13 | P a g e . exist is unknown. blocks complement cascade. In the case of cerebral malaria and in cases of the more severe varieties of neurobrucellosis. including IFN-gamma. little doubt exists that enforced bedrest with appropriate positioning (because of elevation of intracranial pressure) is of importance. Blocks Fc receptors on macrophages. May promote remyelination. The initial dose should be administered under close supervision because rare instances of anaphylaxis after initial dose have been reported. Solu-Medrol. May increase CSF IgG modestly. and whether deleterious effects. however. Anti-inflammatory Agents a) Methylprednisolone (Adlone. bedrest is often necessary because of the low mental status and weakness of such individuals. Depo-Medrol) Considerable experience has accumulated in the use of various corticosteroids in the treatment of ADEM. Gamimune. such as enhancement of tendency to recurrence. Generally. to be safe. MEDICATION SUMMARY The goals of pharmacotherapy are to reduce morbidity and prevent complications. and augments suppressor T cells. suppresses inducer T and B cells. May down-regulate proinflammatory cytokines. No conclusive evidence exists that this form of therapy is effective. The usual approach is administration of methylprednisolone for 3-5 d IV (or the equivalent dose of some other anti-inflammatory corticosteroid). within the considerable limits of present knowledge. Medrol. b) Human immune globulin (Gammagard. IVIg) Believed to treat conditions associated with inflammation and immune dysregulation by neutralizing circulating myelin antibodies through anti-idiotypic antibodies. The weight of evidence at present supports the view that corticosteroids may shorten the time to onset of improvement. Sandoglobulin. Whether this form of therapy shortens time to maximal recovery is unclear.

When seizures occur. ataxia. additional management issues for seizures arise during the subsequent course of treatment. low vision. cerebellar)  Bladder or bowel function Recurrence is the chief outpatient complication and is rare. Physical and occupational therapy may be indicated in patients with paresis. Some difficult cases require slow tapers. and other focal neurologic abnormalities that impair function. they usually do so transiently at the onset of disease.Further Inpatient Care After initial evaluation and initiation of therapy. Patients who are placed on tapering doses of oral corticosteroids require follow-up to ascertain the rate and extent of improvement. Urgent return or consultation may be warranted by patients who display relapse during taper of corticosteroids. Further Outpatient Care Outpatient care indications depend on the course of illness and the extent of recovery at the time of discharge. or speech therapists. Some patients require follow-up with urologists or gastroenterologists because of persistent bladder or bowel problems. pyramidal. further inpatient care is dictated by the evolution of disease and rate of recovery exhibited by the patient. occupational. In such instances. COMPLICATIONS Abnormalities of :  Vision  Motor function (ie. In rare instances. extrapyramidal. the relapse is usually controlled by restoration of a higher medication dosage with slower ensuing taper. Provision for feeding and for the treatment of abnormalities of bowel or bladder function may be indicated. 14 | P a g e . It may include the involvement of physical.

and quadriparetic. 15 | P a g e . Disturbances of mood and personality may outlast motor deficits. patients with myelitis. Recovery is poorest in children younger than 2 years.5% of the authors' cases have resulted in mortality due to ADEMrelated complications. comatose. as may sphincter abnormalities in patients with spinal cord disease. although limited experience suggests this possibility.PROGNOSIS The outlook for recovery is generally excellent. and those who have significant edema of the brain or spinal cord. Degree of recovery is unrelated to severity of illness. Although some older series suggest up to a 10% mortality rate. only 1. In other cases of ADEM. but they may also wane over ensuing months. Complete recovery may be observed even in children who become blind. modest visual or motor deficits may persist. Whether ultra–high-dose corticosteroid therapy and other treatments for edema might improve the outcome for these groups is not yet known.

et al. et al. Jun 2009. Callen DJ.67(3A):643-51. Franciotta D. Rust RS. 7.72(3):232-9. Gaspareto EL. 3. Incidence of acquired demyelination of the CNS in Canadian children. Acute disseminated encephalomyelitis: clinical features. Khajuria K. Sadovnick D. Jan 20 2009. et al. Zardini E. et al. Apr 1988. 5. Lotze T. Neurology. et al. 4. Multiple sclerosis and acute disseminated encephalomyelitis diagnosed in children after long-term follow-up: comparison of presenting features.51(6):480-6. Trotter JL. 16 | P a g e . Ishizu T. Ravaglia S. 6. 8. Jan 2009. Role of MRI in the differentiation of ADEM from MS in children. and HLA DPA1*0301 allelic association study. Cytokines and chemokines in cerebrospinal fluid and serum of adult patients with acute disseminated encephalomyelitis.72(11):96873. Heyman R.23(4):406-10.247(2):202-7. Mar 17 2009. HLA DQA1*0102. Alper G. J Neuroimmunol. Veluttini-Pimentel ML. Stephens D. Li DK. Branson HM.46(1):72-4. HLA DRB1*1501. CSF cytokine and chemokine profiles in acute disseminated encephalomyelitis. Chowdhary J. Gouveia ME. Dev Med Child Neurol. Shroff MM. Sep 25 2006. Minohara M. Banwell B. Sep 2009. Ann Neurol. Arnold DL. Wambera K. HLA DQB1*0602. Magalhaes S. Kennedy J. J Neurol Sci. Malfetano FR. Alvarenga MP. Ichiyama T. Ashraf SM. Wang L. 2. Arq Neuropsiquiatr. Jun 2006.175(1-2):52-8. Measles with acute disseminated encephalomyelitis (ADEM).REFERENCES 1. Dodson WE. Indian Pediatr. HLA DRB1*1503. Alves-Leon SV. Cerebrospinal fluid IgG in childhood: the establishment of reference values. Neurology.

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