sign in sign up
<<
Home , Intention tremor

Lucia Cavanagh et al., IJCR, 2021; 5:206

Case Report IJCR (2021) 5:206

International Journal of Case Reports (ISSN:2572-8776)

Acute Demyelinating (ADEM) following vaccination

Lucia Cavanagh1*, Adriana M. Strutt2, Paul E. Schulz3

1Department of Psychiatry, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California - Los Angeles, Los Angeles, CA, United States. 2Department of , Baylor College of Medicine, Houston, TX, United States. 3Department of Neurology, McGovern Medical School of UT Health Houston, Houston, TX, United States.

ABSTRACT Acute disseminated encephalomyelitis (ADEM) is an inflamma- *Correspondence to Author: tory of the central (CNS). Lucia Cavanagh, PhD Its diagnosis can be challenging due to having varied clinical 760 Westwood Plaza, C8-226 presentations, including a range of motor, speech, cognitive, and Los Angeles, CA 90095 behavioral changes that can vary in frequency and intensity, and Phone: (310) 794-6223 there being no strictly defined diagnostic criteria for it in adults. Here we present a 58-year-old White male who developed ADEM following an uncommon cause (i.e., rabies vaccination), How to cite this article: which was difficult to diagnose due to atypical manifestations. Lucia Cavanagh, Adriana M. Strutt, Paul E. Schulz. Acute Demyelinatin- Keywords: Acute disseminated encephalomyelitis (ADEM); g Encephalomyelitis (ADEM) follow- rabies; vaccine; ; cognitive dysfunction; neuropsych- ing rabies vaccination. Internation- ology al Journal of Case Reports, 2021; 5:206. Abbreviations: Acute disseminated encephalomyelitis (ADEM); central nervous system (CNS)

eSciPub LLC, Houston, TX USA. Website: http://escipub.com/

IJCR: https://escipub.com/international-journal-of-case-reports/ 1 Lucia Cavanagh et al., IJCR, 2021; 5:206 Introduction MRI criteria have been identified specifically for ADEM in adults. However, diffuse or multifocal Acute disseminated encephalomyelitis (ADEM) white matter are commonly cited, and is a monophasic disease characterized by an gray matter lesions in and basal inflammatory reaction and demyelination of the ganglia have also been reported to a lesser central nervous system (CNS), typically extent. [12] Still, many cases of ADEM can occurring after or vaccinations.[1] The present without any visual evidence of the precise annual population-based incidence of disease, even after multiple scans, and on some ADEM is unknown, given its rarity and unclear occasions, ADEM lesions may appear several diagnostic criteria, but estimates range between weeks following the onset of symptoms. [13,14] In 0.4-0.8 per 100,000.[2] With a mean age of onset the past five years, increasing interest has been between 5 and 8 years old, ADEM is much more devoted to diagnostic testing for myelin common in children, although it can occur at any oligodendrocyte glycoprotein (MOG) auto- age. [1] In the midst of the COVID-19 pandemic, antibodies, which may represent a patho-logical ADEM in adults has been increasingly reported substrate of ADEM and other demye-linating in association with SARS-COV-2 .[3,4,5] disorders. [15] There are no defined and accepted diagnostic We present this case to illustrate the diagnostic criteria for ADEM among adults, although limited and radiologic challenges to diagnosing ADEM information suggests its prognosis might be in adults and how one must have a high index of more severe in adults.[6,7] The time course for suspicion to identify it successfully. ADEM symptoms has also not been well- established among adults, though more detailed Case description records have been reported among children.[8] A 58-year-old, right-handed, White male The onset generally ranges between 2 to 30 presented with cognitive decline and gait days post-vaccination [1], and it can progress changes. His memory was poor, he had difficulty over hours to a peak in 4-5 days.[9] The clinical formulating sentences, and he had the syndrome can initially present with systemic countenance of a patient with Parkinson’s symptoms (e.g., malaise, , nausea, disease. vomiting) and is rapidly followed by focal and The patient’s premorbid medical and psychiatric [9] multifocal neurological deficits. A wide range history was unremarkable with the exception of of neurological features have been associated back surgery in the 1990s. Family medical with ADEM, including altered consciousness, history was remarkable for diabetes mellitus and , visual defects, speech disturbances, limb hypertension. Psychosocial history was also paresis, cerebellar disturbances, and unremarkable; the patient was employed as a [10] . safety technician in a refinery and was Given the lack of distinct clinical signs, it can be reportedly performing well at work. He difficult to reliably diagnose ADEM and completed a high school education and resided distinguish it from other demyelinating diseases with his wife and one adult daughter. (e.g., MS). Thus, diagnosis is often based on a A wild dog bit his left leg in a parking lot at work. combination of clinical features that are For safety, he underwent three rounds of rabies temporally tied to an immunization or infection, vaccinations in March 2015. After the first and the exclusion of other inflammatory vaccination, the patient reported feeling ill and [11] neurological or systemic conditions. faint. He received a second vaccination Laboratory and imaging findings for ADEM in approximately three days later. Within minutes adults can be quite variable. Brain magnetic of receiving the second vaccine, his skin resonance imaging (MRI) findings are among became clammy and pale, and his blood the most widely applied diagnostic tool. [2] No pressure rapidly increased. He was packed in IJCR: https://escipub.com/international-journal-of-case-reports/ 2 Lucia Cavanagh et al., IJCR, 2021; x:xx ice. After the third injection, the patient began gait and motor changes, including muscle experiencing seizures and right arm and leg stiffness and . He had an irregular, tremors. While in the hospital, there were two constant right arm and leg , which varied instances in which he stopped breathing, in frequency, direction and amplitude. The perhaps in association with a , but he tremor was suppressible and mildly entrainable. began breathing spontaneously again without His diagnosis was unclear and included a CPR or intubation. He also experienced psychogenic etiology. numbness and decreased motor abilities in his Neuropsychological assessment legs and loss of vision on one side, which fully Comprehensive neuropsychological testing was recovered within minutes. performed in August 2016 (Table 1). During the Initial laboratory and radiological findings interim, the patient and his family reported mild Lumbar puncture was unrevealing. Tests for improvements in stuttering and gait difficulties. West Nile and Lyme disease were negative. A His seizure activity had decreased in frequency head CT was unremarkable. His awake EEG and intensity. His tremors continued to be was essentially normal but showed a present, reportedly persisting even while predominantly drowsy pattern. No true focal sleeping. There were on-going cognitive paroxysmal features were seen. Brain MRI difficulties, depression and anxiety, and revealed mild cortical atrophy. emotional lability: his emotions were easily Neurological evaluation triggered by environmental influences, such as television commercials. He was easily startled, His team referred him to a neurologist after withdrawn, and minimally responsive at times. hospital discharge. His General mental status during the evaluation fell showed significant declines in speech, including significantly below expectation (MoCA = 7/30). stuttering, short-term memory loss, and increased distractibility. The neurologist noted

Table 1: Summary of Neuropsychological Evaluation Cognitive Domain Measure Clinical Classification General mental status Montreal Cognitive Assessment (MoCA) Below expectation Premorbid level of functioning ACS Test of Premorbid Functioning Borderline (TOPF) Attention/concentration Wechsler Adult Intelligence Scale – 4th Deficient Edition (WAIS-IV Digit Span) Verbal Sustained Attention Test (VSAT) Speed: Deficient Accuracy: Deficient Information Trail Making Test A Deficient with 3 errors processing speed Stroop Word Deficient Language Boston Naming Test (BNT) Deficient Animal Fluency Deficient Visual-perceptual Rey Complex Figure Test (RCFT) Copy Deficient Clock Drawing Deficient WAIS-IV, Visual Puzzles Deficient Verbal Memory Wechsler Memory Scale, 4th Edition Immediate recall: Deficient (WMS-IV), Logical Memory Delayed recall: Deficient Recognition: Deficient Hopkins Verbal Learning Test, Revised Immediate recall: Flat and deficient (HVLT-R) Delayed recall: Deficient

IJCR: https://escipub.com/international-journal-of-case-reports/ 3 Lucia Cavanagh et al., IJCR, 2021; x:xx

Recognition: Deficient (due to False Positive errors) Executive functioning Trail Making Test B Discontinued* WAIS-IV, Similarities Deficient Controlled Oral Word Association Test Deficient (COWAT) – Lexical fluency Stroop Color-Word Naming Test Discontinued* Motor functioning Apraxia Examination Deficient bilaterally Note: Clinical descriptors identify performance with the range of Standard Scores (average = 100, standard deviation = 15) indicated in parentheses: Very Superior (> 130), Superior (120-129), High Average (110-119), Average, (90-109), Low Average (80-89), Borderline (70-79), and Deficient (< 69). *Task was discontinued due to motor limitations and/or cognitive difficulties with task demands.

Attention and processing speed: Auditory and lexical fluency. He was unable to complete attention (repetition of digits forward, backward tasks of verbal inhibition and set-shifting/mental and sequenced) was deficient (3 digits forward, flexibility. 2 digits backward, and 2 digits when mentally re- Motor: The patient demonstrated persistent ordering sequentially). Working memory for rote tremors, which impacted his performance during mental exercises was deficient for speed and for testing. Tapping noises triggered involuntary accuracy. Visual attention tracking was deficient movements, though symptoms subsided within due to slow processing speed, with 3 errors. the first 20 minutes of testing. He had bilateral Processing speed for single-word reading was impairments of transitive and intransitive apraxia deficient, and he was unable to complete a to command and imitation. speeded color-naming task. Behavioral and psychiatric symptoms: On a Memory: List learning for a 12-word item list was questionnaire regarding neurobehavioral deficient across three learning trials (5, 6, and 5 symptoms, his family endorsed increased words per trial). Recall following a 25-minute symptoms of anxiety, irritability, and nighttime delay was nil. Recognition memory was also behaviors, which resulted in a moderate level of within the deficient range (11/12 correct emotional distress for his primary caregivers. responses, but 12 false positive errors). Due to cognitive difficulties and tremors, the Immediate recall of short verbal prose was patient was unable to complete self-report deficient with nil delayed recall and deficient questionnaires to better assess mood, though recognition memory (14/30). Recall of culturally he reported symptoms of anxiety and based general knowledge was borderline depression. impaired. Functional status: There were significant Language: Performance was deficient on tasks declines in basic and instrumental activities of of confrontation naming and semantic fluency. daily living (ADLs). The patient stopped working However, his speech during the evaluation was in March 2015 and discontinued driving, per his coherent and goal-oriented. For instance, he doctor’s recommendation. An informant-report would express concerns related to his difficulties questionnaire (Table 2) indicated that he when offered praise for hard work. required complete assistance with finances and Visual-perceptual: His copy of a complex design medication management, as well as daily tasks demonstrated multiple omission errors and such as using the telephone, shopping, meal detail distortions. His ability to mentally arrange preparation, housekeeping, and laundry. He puzzle pieces was deficient. also required assistance completing self-care Executive functioning: Performance was ADLs, including feeding, dressing, grooming, deficient on tasks of abstract verbal reasoning and bathing. IJCR: https://escipub.com/international-journal-of-case-reports/ 4 Lucia Cavanagh et al., IJCR, 2021; 5:206 Table 2: Summary of Informant reports

Symptom Target Measure Clinical Classification

Functional independence Lawton and Brody’s Physical Self- Low function, dependent Maintenance Scale (PSMS)

Lawton and Brody’s Independent Low function, dependent Activities of Daily Living Scale (IADLS)

Neuropsychiatric symptoms Neuropsychiatric Inventory (NPI-Q) Severity: Moderate Distress: Moderate

Overall, his neuropsychological profile revealed Follow-up Neuropsychiatric and Radio- cognitive and functional declines, (compared to logical Examinations age- and education-matched peers) across all Follow up neuroimaging was completed in assessed domains, as well as psychiatric and August 2016. Brain MRI was unremarkable. His behavioral changes, based on informant- DAT SPECT scan was normal, indicating that reports. He was referred for follow-up his Parkinsonism was not due to substantia radiological and neuropsychiatric examinations nigra insufficiency (Figure 1). to consider additional diagnostic opinions.

Figure 1: Normal DAT Scan in this patient. Note that the patient is tilted in the scanner giving the impression of an asymmetric, and hence positive scan.

On neurobehavioral examination, he was again His MoCA score remained impaired (6/30), noted to have parkinsonian features, impaired though with partially intact orientation (4/6). He mental and functional abilities, and psychiatric demonstrated word-finding and comprehension symptoms. Cogwheel rigidity was present difficulties in casual conversation. His family bilaterally. Startle and right-sided reported he frequently got lost and had ongoing intention tremor were observed, accompanied difficulties with staying oriented and sustaining by rest tremors in the right upper extremity. He attention. They reported poor memory, had a stooped, shuffling gait and en-bloc turning, estimating that he could recall approximately with decreased dominant arm swing. 30% of his daily activities. He developed Ambulation was slowed approximately 2.5-fold. changes in sleep patterns and mild symptoms of No sensory abnormalities were noted.

IJCR: https://escipub.com/international-journal-of-case-reports/ 5 Lucia Cavanagh et al., IJCR, 2021; x:xx paranoia, marked by concerns about people psychogenic tremor. After following him and taking things from him. evaluating him extensively, his diagnosis was Differential Diagnosis changed to post-vaccination ADEM. This diagnosis was made based on the clinical Reaction rates to immunization have varied features and the temporal correlation of across monitoring systems, in part due to the symptoms with rabies vaccine. This case purity of the inactivated virus. [16] Among highlights the challenges of differential diagnosis reported adverse events, rabies vaccination is and effective symptom management for ADEM, most commonly associated with mild, self- which is an uncommon disorder, particularly limited, and localized reactions. Pain at the site among adults. of injection, redness, and swelling occur in 35- 45% of recipients. 5-15% have reported mild Several factors contributed to the diagnostic systemic reactions such as fever, headache, challenges in this case. First, the base rates for dizziness and gastrointestinal symptoms. More ADEM in adults are relatively low, particularly serious adverse events are rare, but there are following rabies vaccination. While there are reports of allergic reactions, Guillen-Barre historical accounts of a high incidence of ADEM syndrome, and ADEM. [17,18] post-rabies vaccination, these findings were linked to the use of poorly purified and Additionally, ADEM is frequently associated with contaminated vaccines. [22] Currently, the rate of multifocal MRI lesions, but there are several ADEM caused by vaccination is quite low (less reports of ADEM with normal imaging. For than 5% of cases across the lifespan), and prior instance, 5 of 10 patients had normal MRIs in a infection is the more commonly recognized longitudinal study of ADEM in adults [19] and 2 of precipitant (50-75% of cases).[2,23,24] Tselis and 7 patients had normal brain MRI in a study of Lisak[25] summarized available data on the ADEM post-rabies vaccination. [20] It is also not incidence of ADEM post-measles (1:1000), uncommon for the MRI to be normal at initial post-varicella (1:10,000), and post- presentation, and to develop abnormalities at (1:20,000). Given its low incidence, the index of delays between 5 and 14 days. [18] Notably, suspicion for ADEM in this context must be findings based on pathologically-determined relatively high for successful identification. ADEM suggest that the presence of MRI lesions alone is not a reliable criterion for distinguishing Second, the diagnostic criteria for ADEM were ADEM from other demyelinating disorders; not well codified until recently. Höllinger and rather, a depressed level of consciousness may colleagues.[19] initially provided criteria to be a more specific criterion. [20] facilitate ADEM diagnosis, including (1) acute onset; (2) monophasic disease course; (3) Considering the acute onset of cognitive and disseminated diffuse CNS disease with motor symptoms 8-20 days after initial neurologic findings; (4) history of recent infection vaccination, the lack of progression over time or vaccination; and (5) absence of metabolic or and, in fact, some improvement, and in the infectious disorders. Thereafter, consensus absence of alternate etiologies for his criteria for the diagnosis in children were symptoms, the patient was diagnosed with established by the International Pediatric ADEM due to rabies vaccination. He was started Study Group (IPMSG) in on Aricept (5mg) to improve his attention. 2007, with a more recent update in 2013. [26] Discussion While these clinical criteria were developed A 58-year-old, right-handed, White male patient specifically for children, they are often applied in developed the sudden onset of cognitive, motor, adults due to the absence of alternative criteria. behavioral and speech declines following rabies [15] Per these criteria, ADEM requires all of the vaccinations. He was initially diagnosed with a following: (1) a first polyfocal, clinical CNS event

IJCR: https://escipub.com/international-journal-of-case-reports/ 6 Lucia Cavanagh et al., IJCR, 2021; x:xx with presumed inflammatory demyelinating sequences are commonly associated with this cause; (2) that cannot be etiology. [6,11] However, as prior studies have explained by fever; (3) no new clinical and MRI noted, there can be normal brain MRI findings in findings emerging 3 months or more after the up to 50% in some settings. [19] It has further onset; (4) brain MRI is abnormal during the been suggested that the prevalence of MRI acute (3-month) phase; and (5) brain MRI findings reported in the ADEM literature may be typically shows diffuse, poorly demarcated, somewhat inflated by a publication bias, as large (>1–2 cm) lesions involving predominantly positive (i.e., abnormal) MRI findings are the cerebral white matter, while T1 hypointense commonly considered as a diagnostic lesions in the white matter are rare. Deep gray prerequisite for inclusion in larger clinical matter lesions (e.g., thalamus or basal ganglia) studies. [23] In this light, it is not surprising then can be present. Unfortunately, stringent that MRI abnormalities in ADEM are highly application of this diagnostic criteria may lower variable, with both white- and gray-matter the apparent rate of ADEM diagnosis, leaving involvement reported across a range of some patients undiagnosed. [15,23] Moreover, locations. For instance, in a large cohort of findings suggest that encephalopathy in ADEM ADEM patients, 38% demonstrated may be subtle, especially early in the course, lesions, 66% infratentorial lesions, 54% and can often be dismissed by clinicians as lesions, 48% cerebellar lesions, 52% “irritability” or “sleepiness” rather than clear periventricular involvement, 77% other cognitive confusion. [27] Thus, the history of supratentorial lesions, and 41% corpus callosum events is perhaps the single most important involvement. [23] Analysis of CSF is another element in the diagnostic criteria. [28] As seen in common diagnostic tool in identifying and this case, the temporal correlation of symptoms differentiating ADEM. Commonly cited CSF with an identified precipitant (i.e., rabies findings include elevated protein and pleocytosis vaccination), coupled with a stable or partially with lymphocytic predominance and a normal improved course over time, can be key IgG index. [30] However, in many cases of ADEM, indicators of an ADEM diagnosis. CSF can be normal or show only minor or [28] In more complex cases, or those with an nonspecific changes. Nevertheless, ambiguous precipitant, ADEM may be an evaluation of CSF remains an essential element exclusionary diagnosis. Therefore, effectively of the workup to rule out other possible ruling out alternative diagnoses is an important conditions, such as Lyme disease or West Nile [10] step in directing suspicion towards ADEM. virus. Ultimately, although abnormal MRI and Unfortunately, the differential diagnoses are CSF findings are commonly elicited in ADEM, extensive, as its symptom presentation can vary neither MRI nor CSF alone are adequate for markedly, depending on location and diagnosing ADEM, as the presence of abnormal degree of brainstem and/or spinal cord findings can vary, and even then, their involvement. [28] Its distinction from other presentation is inconsistent and poorly inflammatory demyelinating diseases (e.g., MS, predictive. neuromyelitis opitca) can be especially To a lesser extent, EEG and CT studies may challenging, as exclusive features for the also be included in the ADEM workup. Often, pathology are not well understood. [29] however, these studies do not demonstrate In the present case, there were unclear significant changes that appreciably distinguish [10] laboratory and neuroimaging findings during the ADEM from other conditions. CT scans of the initial evaluation. Often, MRI findings can cue brain in ADEM are often normal, as in the current [12] providers in to an ADEM diagnosis, as diffuse case. When abnormal, many cases of ADEM white-matter lesions on T2 and FLAIR show non-specific, low attenuation, subcortical

IJCR: https://escipub.com/international-journal-of-case-reports/ 7 Lucia Cavanagh et al., IJCR, 2021; x:xx white matter lesions, usually with a gadolinium- deficits more closely than other imaging positive enhancement. [10] Interestingly, among modalities. [35,36] studies that included EEG in the ADEM work-up, With improved purification methods, modern the rate of abnormalities appeared to be quite cell-cultured rabies vaccinations are generally high, with reported prevalence upwards of 80%, safe and well-tolerated; hence, the risk of ADEM and the degree of abnormality in the EEG from vaccines is markedly low compared to viral [19,31] correlates well with clinical symptoms. etiologies. Given its low incidence, the presence These EEG patterns often show nonspecific, of rigorous clinical trials to establish a standard slow waves of high voltage. In the current case, of treatment for ADEM is largely lacking. the EEG did, in fact, show generalized slowing. However, increasing reports of COVID-19 [19] As Hollinger and colleagues previously associated ADEM cases, as well as possible described, such abnormalities on EEG may be exacerbation of pre-existing ADEM following particularly important in bolstering the organic COVID-19 infection, may spark further interest nature of the disease. Given the prevalence and in this area. [3,4,5,37] When it does occur, high- fluctuating nature of neuropsychiatric symptoms dose glucocorticoid therapy is most commonly in ADEM, it is not uncommon to assume a applied as a first-line therapy in acute ADEM. psychogenic etiology, particularly in the context Plasma exchange and intravenous of normal findings on MRI or CSF. immunoglobulin (IVIg) are frequently applied in More recently, MOG antibody testing using cell- ADEM cases that fail to respond to based assays has increasingly been applied as corticosteroids. [18] In cases with long delays a biomarker of several inflammatory between initial onset and final diagnosis, as in demyelinating disorders, including ADEM, optic the current case, treatment becomes largely neuritis, transverse , and neuromyelitis symptomatic. The extent to which delays in optica. While its clinical utility is still developing, receiving care contribute to long-term prognosis MOG antibody assays may prove useful in remains unclear. distinguishing between MS and other non-MS Overall, the medical prognosis for ADEM is som- demyelinating disorders [32] as well as predicting ewhat positive, with full recovery reported in 50- the likelihood of relapse in ADEM and potential 75% of patients. [18] Despite the resolution of benefit from immunotherapy. [33] While neurological ailments, however, long-term promising, MOG seropositivity is not singularly studies of pediatric ADEM suggest that cognitive pathognomonic to ADEM, and thus clinical and psychosocial deficits may linger. [38] The correlation is often required for the differential cognitive and behavioral sequelae of adult-onset diagnosis of ADEM versus other demyelinating ADEM has received markedly less attention, but syndromes and/or autoimmune , suggests more severe deficits. [39] which can have significant crossovers with ADEM. Conclusions As more advanced imaging techniques are ADEM is an inflammatory demyelinating CNS increasingly applied to the study of ADEM, we disease, whose diagnostic picture is challenging hope to better clarify its diagnosis. For instance, because of varied clinical and radiographic positive emission tomography (PET) scanning presentations. Rapid identification of ADEM is has shown decreased global and bilateral necessary to reduce the extent of to the cerebral metabolism and reduced cerebral blood CNS. The current study presents a 58-year-old flow. [34] Alternatively, SPECT abnormalities male who developed ADEM following rabies have captured longer-lasting lesions across vaccine. Unique aspects of the case included an different stages of the illness and may better uncommon precipitant, atypical presentation, reflect the clinical course of neurocognitive and normal radiological and neuroimaging findings. These features complicated diagnosis IJCR: https://escipub.com/international-journal-of-case-reports/ 8 Lucia Cavanagh et al., IJCR, 2021; x:xx and delayed treatment. Ultimately, the temporal [10] Wender, M. (2011). Acute disseminated ence- correlation of presenting symptoms to the rabies phalomyelitis (ADEM). Journal of Neuroimmunology, 231(1), 92–99. vaccine was instrumental in guiding suspicion https://doi.org/10.1016/j.jneuroim.2010.09.019. towards ADEM. Noted abnormalities on EEG [11] Schwarz, S., Mohr, A., Knauth, M., Wildemann, further supported this diagnosis. Future B., & Storch–Hagenlocher, B. (2001). Acute research may evaluate the utility of other disse- minated encephalomyelitis: a follow-up imaging techniques to improve the clinical study of 40 adult patients. Neurology, 56(10), 1313-1318. diagnosis of ADEM. [12] Noorbakhsh, F., Johnson, R. T., Emery, D., & References Power, C. (2008). Acute disseminated [1] Steiner, I., & Kennedy, P. G. E. (2015). Acute encephalomyelitis: clinical and pathogenesis disseminated encephalomyelitis: current features. Neurologic clinics, 26(3), 759-780. knowledge and open questions. Journal of [13] Kawanaka, Y., Ando, K., Ishikura, R., Katsuura, NeuroVirology, 21(5), 473–479. T., Wakata, Y., Kodama, H., ... & Yamakado, K. https://doi.org/10.1007/s13365-015-0353-z. (2019). Delayed appearance of transient hyperin- [2] Menge T., Hemmer B., Nessler, S., Wiendl, H., tensity foci on T1-weighted magnetic resonance Neuhaus, O., Hartung, H.-P., … Stüve, O. (2005). imaging in acute disseminated Acute Disseminated Encephalomyelitis. Archives encephalomyelitis. Japanese journal of of Neurology, 62(11), 1673. radiology, 37(4), 277-282. https://doi.org/10.1001/archneur.62.11.1673. [14] Torisu, H., & Okada, K. (2019). Vaccination- [3] Novi, G., Rossi, T., Pedemonte, E., Saitta, L., associated acute disseminated Rolla, C., Roccatagliata, L., ... & Farinini, D. encephalomyelitis. Vaccine, 37(8), 1126-1129. (2020). Acute disseminated encephalomyelitis [15] Otallah, S. (2020). Acute disseminated after SARS-CoV-2 infection. Neurology- encephalomyelitis in children and adults: A Neuroimmunology Neuroinflammation, 7(5). focused review emphasizing new developments. [4] Parsons, T., Banks, S., Bae, C., Gelber, J., Multiple Sclerosis Journal, 1-8. doi: Alahmadi, H., & Tichauer, M. (2020). COVID-19- 10.1177/1352458520929627. associated acute disseminated encephalomyelitis [16] World Health Organization. (2013). WHO Expert (ADEM). Journal of neurology, 267, 2799-2802. Consultation on Rabies: second report (No. 982). [5] Reichard, R. R., Kashani, K. B., Boire, N. A., World Health Organization. Retrieved from Constantopoulos, E., Guo, Y., & Lucchinetti, C. F. http://www.lca.org.tw/sites/www.lca.org.tw/files/w (2020). Neuropathology of COVID-19: a spectrum ho_report_on_rabies.pdf. of vascular and acute disseminated [17] Siddiqui, A., Usmani, R. I., Anwer, S., & Afsar, S. encephalomyelitis (ADEM)-like pathology. Acta (2005). Guillain-Barre syndrome occurring after neuropathologica, 140(1), 1-6. rabies vaccination. JPMA. The Journal of the [6] Sonneville, R., Klein, I., de Broucker, T., Wolff, M. Pakistan Medical Association, 55(2), 87-88. (2009) Post-infectious encephalitis in adults: [18] Huynh, W., Cordato, D. J., Kehdi, E., Masters, L. diagnosis and management. J Infect, 58, 321– T., & Dedousis, C. (2008). Post-vaccination 328. encephalomyelitis: literature review and illustra- [7] Ketelslegers, I. A., Visser, I. E. R., Neuteboom, R. tive case. Journal of Clinical Neuroscience, F., Boon, M., Catsman-Berrevoets, C. E., & 15(12), 1315-1322. Hintzen, R. Q. (2011). Disease course and [19] Höllinger, P., Sturzenegger, M., Mathis, J., outcome of acute disseminated Schroth, G., & Hess, C. W. (2002). Acute disse- encephalomyelitis is more severe in adults than in minated encephalomyelitis in adults: a reappraisal children. Multiple Sclerosis Journal, 17(4), 441- of clinical, CSF, EEG, and MRI findings. Journal 448. of neurology, 249(3), 320-329. [8] Nishiyama, M., Nagase, H., Tomioka, K., Tanaka, [20] Murthy, J. M. K. (1998). MRI in acute T., Yamaguchi, H., Ishida, Y., ... & Iijima, K. disseminated encephalomyelitis following Semple (2019). Clinical time course of pediatric acute antirabies vaccine. Neuroradiology, 40(7), 420- disseminated encephalomyelitis. Brain and 423. Development, 41(6), 531-537. [21] Young, N. P., Lucchinetti, C. F., & Weinshenker, [9] Tenembaum, S., Chitnis, T., Ness, J., Hahn, J. S., B. G. (2008, February). Acute disseminated & International Pediatric MS Study Group. (2007). encephalomyelitis: current understanding and Acute disseminated encephalomyelitis. Neurolo- controversies. In Seminars in neurology (Vol. 28, gy, 68(16 suppl 2), S23-S36. IJCR: https://escipub.com/international-journal-of-case-reports/ 9 Lucia Cavanagh et al., IJCR, 2021; x:xx No. 01, pp. 084-094). © Thieme Medical Clinical and neuroradiologic features of acute Publishers. disseminated encephalomyelitis in children. [22] Swaddiwudhipong, W., Prayoonwiwat, N., Kuna- Neurology, 56(10), 1308-1312. sol, P., & Choomkasien, P. (1987). A high [32] Waters, P., Woodhall, M., O'Connor, K. C., incidence of neurological complications following Reindl, M., Lang, B., Sato, D. K., ... & Vincent, A. Semple anti-rabies vaccine. The Southeast Asian (2015). MOG cell-based assay detects non-MS journal of tropical medicine and public heal- patients with inflammatory neurologic th, 18(4), 526-531. disease. Neurology-Neuroimmunology [23] Koelman, D. L., Chahin, S., Mar, S. S., Neuroinflammation, 2(3). Venkatesan, A., Hoganson, G. M., Yeshokumar, [33] López-Chiriboga, A. S., Majed, M., Fryer, J., A. K.,... & Benkeser, D. C. (2016). Acute Dubey, D., McKeon, A., Flanagan, E. P., ... & disseminated encephalomyelitis in 228 patients: A Pittock, S. J. (2018). Association of MOG-IgG retrospective, multicenter US study. Neurology, serostatus with relapse after acute disseminated 86(22), 2085-2093. encephalomyelitis and proposed diagnostic [24] Baxter, R., Lewis, E., Goddard, K., Fireman, B., criteria for MOG-IgG–associated disorders. JAMA Bakshi, N., DeStefano, F., ... & Klein, N. P. (2016). neurology, 75(11), 1355-1363. Acute demyelinating events following vaccines: a [34] Tabata, K., Shishido, F., Uemura, K., Inugami, A., case-centered analysis. Clinical Infectious Tomura, N., Higano, S., ... & Nagata, K. (1990). Diseases, 63(11), 1456-1462. Positron emission tomography in acute dissemi- [25] Tselis, A. C., & Lisak, R. P. (1995). Acute nated encephalomyelitis: a case report. Kaku disseminated encephalomyelitis and isolated igaku. The Japanese journal of nuclear medicine, central nervous system demyelinative syndrome- 27(3), 261-265. es. Current opinion in neurology, 8(3), 227-229. [35] Ohya, T., Nagamitsu, S., Yamashita, Y., & [26] Krupp, L. B., Tardieu, M., Amato, M. P., Banwell, Matsuishi, T. (2008). Serial magnetic resonance B., Chitnis, T., Dale, R. C., ... & International imaging and single photon emission computed Pediatric Multiple Sclerosis Study Group. (2013). tomography study of acute disseminated ence- International Pediatric Multiple Sclerosis Study phalomyelitis patient after Japanese encephalitis Group criteria for pediatric multiple sclerosis and vaccination. The Kurume medical journal, 54(3+ immune-mediated central nervous system 4), 95-99. demyelinating disorders: revisions to the 2007 [36] Okamoto, M., Ashida, K. I., & Imaizumi, M. (2001). definitions. Multiple Sclerosis Journal, 19(10), Hypoperfusion following encephalitis: SPECT 1261-1267. with acetazolamide. European journal of neurolo- [27] Fridinger, S. E., & Alper, G. (2014). Defining gy, 8(5), 471-474. encephalopathy in acute disseminated [37] Hussein, O., Abd Elazim, A., & Torbey, M. T. encephalomyelitis. Journal of child neurology, (2020). Covid-19 systemic infection exacerbates 29(6), 751-755. pre-existing acute disseminated encephalomye- [28] Harris, C., & Lee, K. (2007). Acute disseminated litis (ADEM). Journal of Neuroimmunology, 349, encephalomyelitis. Journal of Neuroscience Nur- 577405. sing, 39(4), 208-212. [38] Burton, K. L., Williams, T. A., Catchpoole, S. E., & [29] Palace, J. (2011). Acute disseminated Brunsdon, R. K. (2017). Long-term encephalomyelitis and its place amongst other neuropsychological outcomes of childhood onset acute inflammatory demyelinating CNS acute disseminated encephalomyelitis (ADEM): a disorders. Journal of the neurological meta-analysis. Neuropsychology review, 27(2), sciences, 306(1-2), 188-191. 124-133. [30] Kennedy, P. G. E. (2004). : [39] Adamec, I., Klepac, N., Kolenc, D., Ozretic, D., & causes, differential diagnosis, and management. Habek, M. (2013). Isolated and persistent Journal of Neurology, Neurosurgery & Psychiatry, cognitive dysfunction in a patient with acute 75(suppl 1), i10-i15. disseminated encephalomyelitis. Cognitive and [31] Hynson, J. L., Kornberg, A. J., Coleman, L. T., Behavioral Neurology, 26(1), 30-35. Shield, L., Harvey, A. S., & Kean, M. J. (2001).

IJCR: https://escipub.com/international-journal-of-case-reports/ 10