Acute Disseminated Encephalomyelitis: Difference between revisions
No edit summary |
No edit summary |
||
| Line 1: | Line 1: | ||
== Introduction == | <div class="editorbox"> '''Original Editor '''- [[User:Hagar Ashraf Abdul-Raziq|Hagar Ashraf Abdul-Raziq]] '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}</div> | ||
==Introduction== | |||
Acute Disseminated Encephalomyelitis (ADEM), a rare disorder of the central nervous system,<ref name=":1">Imam MA, Noushin L. [https://www.researchgate.net/profile/Md-Imam-2/publication/336835285_Efficiency_of_Early_Diagnosis_and_Integrated_Approaches_of_Physical_Therapy_on_Acute_Disseminated_Encephalomyelitis_A_Case_Report/links/5db5311fa6fdccc99da3f6a6/Efficiency-of-Early-Diagnosis-and-Integrated-Approaches-of-Physical-Therapy-on-Acute-Disseminated-Encephalomyelitis-A-Case-Report.pdf Efficiency of Early Diagnosis and Integrated Approaches of Physical Therapy on Acute Disseminated Encephalomyelitis: A Case Report.]</ref> is an inflammatory demyelinating disorder with multifocal and aggressive neurological impairment associated with a viral infection or vaccination.<ref name=":2">Dominguez N. ''[https://core.ac.uk/download/pdf/288116118.pdf The Differential Diagnosis of ADEM vs MS: A Case Report]'' (Doctoral dissertation, University of Iowa).</ref> | Acute Disseminated Encephalomyelitis (ADEM), a rare disorder of the central nervous system,<ref name=":1">Imam MA, Noushin L. [https://www.researchgate.net/profile/Md-Imam-2/publication/336835285_Efficiency_of_Early_Diagnosis_and_Integrated_Approaches_of_Physical_Therapy_on_Acute_Disseminated_Encephalomyelitis_A_Case_Report/links/5db5311fa6fdccc99da3f6a6/Efficiency-of-Early-Diagnosis-and-Integrated-Approaches-of-Physical-Therapy-on-Acute-Disseminated-Encephalomyelitis-A-Case-Report.pdf Efficiency of Early Diagnosis and Integrated Approaches of Physical Therapy on Acute Disseminated Encephalomyelitis: A Case Report.]</ref> is an inflammatory demyelinating disorder with multifocal and aggressive neurological impairment associated with a viral infection or vaccination.<ref name=":2">Dominguez N. ''[https://core.ac.uk/download/pdf/288116118.pdf The Differential Diagnosis of ADEM vs MS: A Case Report]'' (Doctoral dissertation, University of Iowa).</ref> | ||
| Line 6: | Line 9: | ||
It frequently has a monophasic course and a good functional recovery.<ref name=":0">Wang CX. [https://link.springer.com/article/10.1007/s40272-021-00441-7#ref-CR46 Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient.] Pediatric Drugs. 2021 May;23(3):213-21.</ref> Over the course of several weeks, patients progressively improve, with 50–70% achieving full recovery. However, multiphasic and recurrent forms have also been documented. <ref name=":4">Sharma GS, Gupta A, Nashi S, Naveen BP, Khanna M. [https://www.thieme-connect.com/products/ejournals/html/10.1055/s-0041-1726650 Rehabilitation in a Patient with Acute Disseminated Encephalomyelitis Presenting as Tetraplegia with Cortical Blindness.] Journal of Neurosciences in Rural Practice. 2021 Apr 13;12(02):452-4.</ref> | It frequently has a monophasic course and a good functional recovery.<ref name=":0">Wang CX. [https://link.springer.com/article/10.1007/s40272-021-00441-7#ref-CR46 Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient.] Pediatric Drugs. 2021 May;23(3):213-21.</ref> Over the course of several weeks, patients progressively improve, with 50–70% achieving full recovery. However, multiphasic and recurrent forms have also been documented. <ref name=":4">Sharma GS, Gupta A, Nashi S, Naveen BP, Khanna M. [https://www.thieme-connect.com/products/ejournals/html/10.1055/s-0041-1726650 Rehabilitation in a Patient with Acute Disseminated Encephalomyelitis Presenting as Tetraplegia with Cortical Blindness.] Journal of Neurosciences in Rural Practice. 2021 Apr 13;12(02):452-4.</ref> | ||
== Clinical Presentation == | ==Clinical Presentation == | ||
Encephalopathy (irritability, confusion, somnolence, lethargy, and stupor) and multifocal deficits in the nervous system are the primary signs of ADEM.<ref name=":0" /> | Encephalopathy (irritability, confusion, somnolence, lethargy, and stupor) and multifocal deficits in the nervous system are the primary signs of ADEM.<ref name=":0" /> | ||
| Line 13: | Line 16: | ||
Typical: | Typical: | ||
* prodromal symptoms (headache, malaise, irritability, fever, nausea, and vomiting) | *prodromal symptoms (headache, malaise, irritability, fever, nausea, and vomiting) | ||
* Altered mental status | * Altered mental status | ||
* Focal neurological symptoms (based on the part of the brain that is affected) | *Focal neurological symptoms (based on the part of the brain that is affected) | ||
* Meningism | *Meningism | ||
Atypical: | Atypical: | ||
* Progressive onset, recurrent seizures (most common in pediatric cases), persistent headaches, stroke-like episodes, dystonia or parkinsonism, and neuropsychiatric symptoms. | *Progressive onset, recurrent seizures (most common in pediatric cases), persistent headaches, stroke-like episodes, dystonia or parkinsonism, and neuropsychiatric symptoms. | ||
Common exam findings also include ataxia, extremity weakness, urinary retention<ref name=":0" /> | Common exam findings also include ataxia, extremity weakness, urinary retention<ref name=":0" /> | ||
| Line 26: | Line 29: | ||
Children with ADEM also reported feeling more exhausted, having less energy to exercise, and having impaired motor function.<ref name=":3" /> | Children with ADEM also reported feeling more exhausted, having less energy to exercise, and having impaired motor function.<ref name=":3" /> | ||
== Causes == | ==Causes == | ||
ADEM is an autoimmune CNS disorder that affects genetically susceptible people and is brought on by a trigger from the environment.<ref name=":5" /> | ADEM is an autoimmune CNS disorder that affects genetically susceptible people and is brought on by a trigger from the environment.<ref name=":5" /> | ||
| Line 38: | Line 41: | ||
Clinical finding that characterizes ADEM are: | Clinical finding that characterizes ADEM are: | ||
* A first polyfocal, clinical CNS event with a suspected inflammatory demyelinating cause | *A first polyfocal, clinical CNS event with a suspected inflammatory demyelinating cause | ||
* encephalopathy that is not attributable to postictal symptoms, fever, or a systemic illness. | *encephalopathy that is not attributable to postictal symptoms, fever, or a systemic illness. | ||
* Three months after the onset, there are no new findings on magnetic resonance imaging (MRI) or clinical examination. | *Three months after the onset, there are no new findings on magnetic resonance imaging (MRI) or clinical examination. | ||
* Brain MRI abnormalities occur during the acute phase (three months). | *Brain MRI abnormalities occur during the acute phase (three months). | ||
Diagnostic tools includes:<ref name=":0" /><ref name=":5" /> | Diagnostic tools includes:<ref name=":0" /><ref name=":5" /> | ||
* Neuroimaging | *Neuroimaging | ||
** MRI brain | **MRI brain | ||
** MRI spine | **MRI spine | ||
* CSF studies | *CSF studies | ||
* Serum studies | *Serum studies | ||
* Adjunctive tests for alternative diagnoses, such as vascular, infectious, neoplastic, or disorders related to metabolism, genetics, or mitochondrial abnormalities. | *Adjunctive tests for alternative diagnoses, such as vascular, infectious, neoplastic, or disorders related to metabolism, genetics, or mitochondrial abnormalities. | ||
Inpatient therapists and rehabilitation doctors can evaluate a child receiving immunotherapies for deficiencies in gross motor, fine motor, and speech/swallowing function. | Inpatient therapists and rehabilitation doctors can evaluate a child receiving immunotherapies for deficiencies in gross motor, fine motor, and speech/swallowing function. | ||
== Intervention== | ==Intervention== | ||
It is crucial to maintain breathing, heart, and airway function. For patients who are bedridden, preventive measures against venous thromboembolism are also necessary.<ref name=":5" /> In order to shorten the illness's duration and severity, it is imperative that therapies aimed at lowering inflammation and immune activation be initiated. these interventions consist of high-dose corticosteroids (first-line therapy), intravenous immunoglobulin (IVIG), and therapeutic plasma exchange (PLEX) <ref name=":0" /> | It is crucial to maintain breathing, heart, and airway function. For patients who are bedridden, preventive measures against venous thromboembolism are also necessary.<ref name=":5" /> In order to shorten the illness's duration and severity, it is imperative that therapies aimed at lowering inflammation and immune activation be initiated. these interventions consist of high-dose corticosteroids (first-line therapy), intravenous immunoglobulin (IVIG), and therapeutic plasma exchange (PLEX) <ref name=":0" /> | ||
When appropriate, starting physical, occupational, and speech therapy early can promote a quicker and more thorough recovery. <ref name=":0" /> Physical therapy modalities can include ADL training, PROM, NDT, PRET, and biofeedback therapy.<ref name=":1" /> | When appropriate, starting physical, occupational, and speech therapy early can promote a quicker and more thorough recovery. <ref name=":0" /> Physical therapy modalities can include ADL training, PROM, NDT, PRET, and biofeedback therapy.<ref name=":1" /> | ||
==Differential Diagnosis == | ==Differential Diagnosis== | ||
Classified as demyelinating inflammatory diseases affecting the central nervous system, ADEM and MS can be difficult to distinguish at first clinical presentation.<ref name=":2" /> | Classified as demyelinating inflammatory diseases affecting the central nervous system, ADEM and MS can be difficult to distinguish at first clinical presentation.<ref name=":2" /> | ||
Systemic symptoms, which are generally uncommon in pediatric MS, such as headache, fever, nausea, vomiting, and altered mental status, are present in the majority of patients with pediatric ADEM. <ref name=":5" /> | Systemic symptoms, which are generally uncommon in pediatric MS, such as headache, fever, nausea, vomiting, and altered mental status, are present in the majority of patients with pediatric ADEM. <ref name=":5" /> | ||
== Case Reports== | ==Case Reports== | ||
A 30-year-old woman with loss of vision and tetraplegia. The main goals of the physiotherapy program were bed mobility, stretching exercises for all joints, and passive range of motion. The main goals of occupational therapy were environment and home modifications, as well as training in functional abilities through audio-visuo-tactile stimulation. Her motor, cognitive, and functional recovery was notable following a month-long stay in an inpatient rehabilitation facility. She required only minimal assistance to stand and move around.<ref name=":4" /> | A 30-year-old woman with loss of vision and tetraplegia. The main goals of the physiotherapy program were bed mobility, stretching exercises for all joints, and passive range of motion. The main goals of occupational therapy were environment and home modifications, as well as training in functional abilities through audio-visuo-tactile stimulation. Her motor, cognitive, and functional recovery was notable following a month-long stay in an inpatient rehabilitation facility. She required only minimal assistance to stand and move around.<ref name=":4" /> | ||
Revision as of 21:51, 27 November 2023
Introduction[edit | edit source]
Acute Disseminated Encephalomyelitis (ADEM), a rare disorder of the central nervous system,[1] is an inflammatory demyelinating disorder with multifocal and aggressive neurological impairment associated with a viral infection or vaccination.[2]
In general, young adults in their twenties and thirties are thought to be affected by this illness. [3]
It frequently has a monophasic course and a good functional recovery.[4] Over the course of several weeks, patients progressively improve, with 50–70% achieving full recovery. However, multiphasic and recurrent forms have also been documented. [5]
Clinical Presentation[edit | edit source]
Encephalopathy (irritability, confusion, somnolence, lethargy, and stupor) and multifocal deficits in the nervous system are the primary signs of ADEM.[4]
Symptoms are classified to typical and atypical[6]
Typical:
- prodromal symptoms (headache, malaise, irritability, fever, nausea, and vomiting)
- Altered mental status
- Focal neurological symptoms (based on the part of the brain that is affected)
- Meningism
Atypical:
- Progressive onset, recurrent seizures (most common in pediatric cases), persistent headaches, stroke-like episodes, dystonia or parkinsonism, and neuropsychiatric symptoms.
Common exam findings also include ataxia, extremity weakness, urinary retention[4]
Children with ADEM also reported feeling more exhausted, having less energy to exercise, and having impaired motor function.[3]
Causes[edit | edit source]
ADEM is an autoimmune CNS disorder that affects genetically susceptible people and is brought on by a trigger from the environment.[6]
There are numerous pathogens that have been linked to ADEM, including measles, rubella, varicella zoster, influenza, Epstein–Barr virus, herpes simplex virus (HSV), and beta-hemolytic Streptococcus [4]
Multiple cases of ADEM were reported in 2020 as a result of the global pandemic linked to SARS-CoV-2 infection, which caused severe acute respiratory syndrome.[7]
Diagnostic Procedure[edit | edit source]
Due to the lack of specific biomarkers and the variability of clinical manifestations, ADEM diagnosis is difficult. The initial step of evaluation is a thorough clinical history and neurologic examination, which frequently reveals multifocal symptoms. [6]
Clinical finding that characterizes ADEM are:
- A first polyfocal, clinical CNS event with a suspected inflammatory demyelinating cause
- encephalopathy that is not attributable to postictal symptoms, fever, or a systemic illness.
- Three months after the onset, there are no new findings on magnetic resonance imaging (MRI) or clinical examination.
- Brain MRI abnormalities occur during the acute phase (three months).
Diagnostic tools includes:[4][6]
- Neuroimaging
- MRI brain
- MRI spine
- CSF studies
- Serum studies
- Adjunctive tests for alternative diagnoses, such as vascular, infectious, neoplastic, or disorders related to metabolism, genetics, or mitochondrial abnormalities.
Inpatient therapists and rehabilitation doctors can evaluate a child receiving immunotherapies for deficiencies in gross motor, fine motor, and speech/swallowing function.
Intervention[edit | edit source]
It is crucial to maintain breathing, heart, and airway function. For patients who are bedridden, preventive measures against venous thromboembolism are also necessary.[6] In order to shorten the illness's duration and severity, it is imperative that therapies aimed at lowering inflammation and immune activation be initiated. these interventions consist of high-dose corticosteroids (first-line therapy), intravenous immunoglobulin (IVIG), and therapeutic plasma exchange (PLEX) [4]
When appropriate, starting physical, occupational, and speech therapy early can promote a quicker and more thorough recovery. [4] Physical therapy modalities can include ADL training, PROM, NDT, PRET, and biofeedback therapy.[1]
Differential Diagnosis[edit | edit source]
Classified as demyelinating inflammatory diseases affecting the central nervous system, ADEM and MS can be difficult to distinguish at first clinical presentation.[2]
Systemic symptoms, which are generally uncommon in pediatric MS, such as headache, fever, nausea, vomiting, and altered mental status, are present in the majority of patients with pediatric ADEM. [6]
Case Reports[edit | edit source]
A 30-year-old woman with loss of vision and tetraplegia. The main goals of the physiotherapy program were bed mobility, stretching exercises for all joints, and passive range of motion. The main goals of occupational therapy were environment and home modifications, as well as training in functional abilities through audio-visuo-tactile stimulation. Her motor, cognitive, and functional recovery was notable following a month-long stay in an inpatient rehabilitation facility. She required only minimal assistance to stand and move around.[5]
The 15-year-old boy in this case report underwent physical therapy that included progressive resistance exercise therapy (PRET), bladder training, neurodevelopmental therapy (NDT), striking, balance training, and ADL training. After four months, he was released in a stable state after making steady progress.[1]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 Imam MA, Noushin L. Efficiency of Early Diagnosis and Integrated Approaches of Physical Therapy on Acute Disseminated Encephalomyelitis: A Case Report.
- ↑ 2.0 2.1 Dominguez N. The Differential Diagnosis of ADEM vs MS: A Case Report (Doctoral dissertation, University of Iowa).
- ↑ 3.0 3.1 Toussaint-Duyster LC, Wong YY, Van der Cammen-van Zijp MH, Van Pelt-Gravesteijn D, Catsman-Berrevoets CE, Hintzen RQ, Neuteboom RF. Fatigue and physical functioning in children with multiple sclerosis and acute disseminated encephalomyelitis. Multiple Sclerosis Journal. 2018 Jun;24(7):982-90.
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Wang CX. Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient. Pediatric Drugs. 2021 May;23(3):213-21.
- ↑ 5.0 5.1 Sharma GS, Gupta A, Nashi S, Naveen BP, Khanna M. Rehabilitation in a Patient with Acute Disseminated Encephalomyelitis Presenting as Tetraplegia with Cortical Blindness. Journal of Neurosciences in Rural Practice. 2021 Apr 13;12(02):452-4.
- ↑ 6.0 6.1 6.2 6.3 6.4 6.5 Filippi M, Rocca MA, Filippi M, Rocca MA. Acute disseminated encephalomyelitis. White Matter Diseases: An Update for Neurologists. 2020:109-25.
- ↑ Fueta P, Igbinoba O, Tsou PY, Valdez M, Mathew S. COVID‐19‐Associated Acute Disseminated Encephalomyelitis (ADEM) in a Child.
