ADEM Presenting with Bilateral Optic Neuritis
Journal name: The Malaysian Journal of Medical Sciences
Original article title: Acute Disseminated Encephalomyelitis (ADEM) Presenting with Bilateral Optic Neuritis
The Malaysian Journal of Medical Sciences (MJMS) is a peer-reviewed, open-access journal published online at least six times a year. It covers all aspects of medical sciences and prioritizes high-quality research.
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Original source:
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Sapiah Sapuan, Hamidon Basri
The Malaysian Journal of Medical Sciences:
(A peer-reviewed, open-access journal)
Full text available for: Acute Disseminated Encephalomyelitis (ADEM) Presenting with Bilateral Optic Neuritis
Year: 2007
Copyright (license): CC BY 4.0
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Summary of article contents:
Introduction
Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disorder of the central nervous system, often triggered by viral infections or vaccinations. It presents with clinical manifestations such as seizures, altered consciousness, and multifocal neurological deficits, which can mimic primary viral encephalitis. Typically occurring days to weeks post-infection or immunization, ADEM usually affects children and young adults, with a monophasic course characterized by neurological disturbances, including potential coma and death. The diagnosis is supported by characteristic MRI findings and cerebrospinal fluid (CSF) analysis, which shows lymphocytosis.
Importance of MRI in Diagnosis
MRI plays a crucial role in diagnosing ADEM, as it can reveal hyperintense lesions in the white matter that are indicative of the disorder. These lesions are predominantly seen in areas such as the posterior limbs of the internal capsules and the centrum semiovale, distinguishing ADEM from other demyelinating diseases like multiple sclerosis. The absence of enhancement with gadolinium and the normal findings on CT scans further corroborate the diagnosis of ADEM, while CSF analysis typically shows increased protein levels and a predominance of lymphocytes. Early diagnosis and treatment are essential to manage ADEM effectively, as the condition can lead to significant morbidity and mortality, highlighted by the case of a 43-year-old woman who succumbed rapidly despite treatment.
Conclusion
In summary, ADEM is a serious but relatively rare inflammatory disorder of the CNS, primarily affecting younger populations, and is characterized by an acute onset of neurological symptoms following an infectious trigger. The diagnosis is established based on clinical presentations, MRI characteristics, and CSF findings. Treatment typically involves high-dose corticosteroids, with other therapies like intravenous immunoglobulin (IVIG) being considered in severe cases. Despite the available treatments, the mortality rate remains significant, underscoring the need for continued research and improved therapeutic strategies.
FAQ section (important questions/answers):
What is acute disseminated encephalomyelitis (ADEM)?
ADEM is a rare, inflammatory demyelinating disorder of the central nervous system. It is often associated with preceding viral infections or immunizations, leading to multifocal neurological disturbances such as coma, drowsiness, and seizures.
How is ADEM diagnosed?
ADEM diagnosis is based on a combination of clinical history, MRI findings of multifocal lesions, abnormal cerebrospinal fluid analysis showing lymphocytosis, and the absence of identifiably causative pathogens in cultures.
What are the treatment options for ADEM?
Treatment typically involves high-dose intravenous corticosteroids. In severe cases, alternatives like plasmapheresis or intravenous immunoglobulins are also considered if there is insufficient recovery with corticosteroids.
What is the prognosis for ADEM patients?
The mortality rate for ADEM can be up to 20%, and survivors may experience significant neurological sequelae. Early initiation of treatment is crucial for better outcomes.
Glossary definitions and references:
Scientific and Ayurvedic Glossary list for “ADEM Presenting with Bilateral Optic Neuritis”. This list explains important keywords that occur in this article and links it to the glossary for a better understanding of that concept in the context of Ayurveda and other topics.
1) Blood:
In the context of the case report, 'blood' often signifies the results of the full blood count, which can indicate systemic infection or other underlying conditions. Normal blood results helped rule out significant metabolical disturbances, allowing clinicians to focus on the neurological examination and central nervous system imaging for diagnosing ADEM.
2) Coma:
The term 'coma' is medically relevant here as it describes a state of unresponsiveness that the patient entered after initially presenting with bilateral vision loss and other neurological symptoms. The rapid deterioration into a coma indicates a severe progression of ADEM, highlighting its potential fatality and emphasizing the critical nature of the condition.
3) Disease:
A 'disease' refers to a pathological condition, particularly in this report, which describes ADEM as an inflammatory demyelinating disease of the central nervous system. Understanding it as a disease allows for the exploration of underlying mechanisms, causes, symptoms, and possible treatments, which are essential for effective management and research.
4) Sign:
The word 'sign' is pertinent as it relates to the clinical manifestations observed during the examination of the patient. Various neurological signs, including bilateral extensor plantar reflexes and meningeal signs, provide critical information to support the diagnosis of ADEM and distinguish it from other conditions like viral meningoencephalitis.
5) Drowsiness:
'Drowsiness' describes a state of altered consciousness observed upon the patient's admission. It is a significant clinical symptom that aids in assessing the severity of the neurological condition, allowing physicians to monitor changes in mental status and gauge progression or response to treatments targeted at ADEM.
6) Study (Studying):
In the clinical context, 'study' refers to research findings that enrich understanding of ADEM. The article references studies that document imaging findings, clinical features, and responses to therapy, aimed at validating the diagnosis and informing treatment strategies for conditions like ADEM, facilitating improved patient outcomes.
7) Antibiotic (Antibacterial):
The mention of 'antibiotic' is relevant as the initial management of the patient included empirical antibiotic therapy due to suspicion of meningoencephalitis. This reflects a common practice in managing central nervous system infections while pending definitive diagnoses, thereby ensuring broad-spectrum coverage against potential infectious agents.
8) Pradhan:
The name 'Pradhan' appears in the context of studies that suggest effective treatment options for ADEM. It highlights collaborative research efforts by various clinicians, sharing experiences and findings that contribute to understanding how alternative therapies like intravenous immunoglobulin can aid in managing severe cases of ADEM.
9) Family:
'Family' is significant as it refers to the patient's social history, revealing no familial history of neurological diseases. This information helps in evaluating genetic predisposition, supporting the understanding of the patient's acute condition's isolated nature and determining potential environmental or infectious triggers for ADEM.
10) Inflammation:
The term 'inflammation' relates directly to the pathophysiology of ADEM, which includes acute inflammatory responses leading to demyelination in the central nervous system. Understanding the role of inflammation aids clinicians in identifying the disease process, guiding appropriate therapeutic interventions to mitigate damage and improve patient prognosis.
11) Blindness:
'Blindness' is a critical symptom that the patient experienced, indicating significant neurological impairment. This feature aids in diagnosing ADEM, as it is often associated with optic neuritis. Highlighting such acute visual disturbances accentuates the serious nature of the condition and its impact on the patient's quality of life.
12) Edema (Oedema):
The term 'oedema' refers to swelling often observed in various pathological conditions, including ADEM. It can occur due to inflammation in the brain and is typically seen on MRI scans. Understanding oedema's presence contributes to diagnosing ADEM and assessing the severity of neurological compromise in affected patients.
13) Gupta (Guptā):
'Gupta' pertains to authors referenced in the literature discussing ADEM treatment options. These mentions signify the contributions of researchers to the body of knowledge regarding clinical management, including the efficacy of therapies like intravenous immunoglobulin, establishing important precedents for patient care in severe cases.
14) India:
In the case report, 'India' serves as geographical context, indicating the location of this clinical case. It also reflects the research landscape where studies on ADEM and its treatments are conducted, encompassing demographic and epidemiological aspects that can influence disease presentation and healthcare approaches within different populations.
15) Pulse:
'Pulse' is clinically relevant for monitoring vital signs, indicating the patient's cardiovascular status upon admission. Stability of pulse rate is crucial, suggesting lack of systemic shock or severe distress, thereby guiding clinical assessment and management decisions in the context of neurological illness such as ADEM.
16) Death:
The term 'death' highlights the severe outcome of this case, illustrating the mortality risk associated with ADEM, especially when rapid progression occurs. It emphasizes the importance of prompt diagnosis and management to potentially improve outcomes and reduce fatality rates linked to this condition.
17) Bird:
Mention of 'bird' relates to the patient's travel and exposure history, which could be relevant in investigating potential environmental or infectious triggers for ADEM. Understanding such factors can help in broader epidemiological studies and identifying potential links between viral infections and subsequent neurological diseases.
18) Post:
'Post' in medical literature typically relates to events following a significant occurrence, in this case referring to the patient's viral infection that preceded the onset of ADEM symptoms. This highlights the temporal relationship often seen in ADEM cases, emphasizing the need for awareness of infectious triggers.
19) Drug:
The term 'drug' encompasses medications administered to manage the patient's condition, including antihypertensive drugs and corticosteroids. Understanding drug response and interactions is critical in treating complex neurological disorders like ADEM, as the choice of medication can significantly impact patient outcomes and recovery trajectories.
20) Pur (Pūr):
'Poor' relates to the prognosis discussed in the context of ADEM, indicating the potential for significant neurological sequelae in survivors. This highlights the need for early intervention and effective treatment strategies to mitigate long-term outcomes, emphasizing the importance of clinical awareness and timely management in severe cases.
Other Health Sciences Concepts:
Discover the significance of concepts within the article: ‘ADEM Presenting with Bilateral Optic Neuritis’. Further sources in the context of Health Sciences might help you critically compare this page with similair documents:
Central nervous system, Loss of vision, Optic nerve, Multiple sclerosis, Clinical manifestation, Viral Infection, Acute phase, Magnetic resonance imaging, Photophobia, Cerebrospinal Fluid, Acute disseminated encephalomyelitis, Upper motor neuron lesion, Cerebrospinal Fluid (CSF), Intravenous immunoglobulin therapy, Acute disseminated encephalomyelitis (ADEM), High-dose corticosteroids, Lymphocytosis, Cerebrospinal fluid examination, Neurological sequelae, Cerebral Function, Viral encephalitis, Brain CT, Hyperintense lesion, IVIG treatment, Focal neurological deficit, Ventilatory support, Multifocal white matter involvement, Oral prednisolone, Neurological sign, Bilateral optic neuritis, Electrophysiological study, Magnetic resonance imaging study, White matter, White matter lesions, Mental state changes.