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Published on: July 4, 2007
Adult acute disseminated encephalomyelitis associated with poststreptococcal infection
M M Ning1, S Smirnakis, K L Furie
1Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
Abstract:
Acute disseminated encephalomyelitis (ADEM) is a monophasic illness that is thought to develop from antigenic mimicry with antibodies having cross-reactivity to host epitopes in the nervous system. The disorder typically follows an exanthematous or recent viral infection. In contrast, complications from bacterial poststreptococcal infections more commonly give rise to disorders in the pediatric population including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders, and ADEM. We present the novel case of documented streptococcal pharyngitis and elevated antideoxyribonuclease B (ADNB) titers in an adult giving rise to ADEM. Furthermore, the absence of basal ganglia abnormalities on MRI and the degree of leukocytosis in the CSF distinguish the adult form of ADEM from childhood ADEM and adult viral demyelinating diseases.
Insights
Acute disseminated encephalomyelitis (ADEM) in adults can be triggered by streptococcal pharyngitis, distinct from childhood cases. This case highlights unique MRI and CSF findings in adult ADEM.
Area of Science:
- Neuroimmunology
- Infectious Diseases
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is typically a monophasic illness following viral infections, involving immune cross-reactivity to neural epitopes.
- Post-streptococcal infections commonly cause pediatric autoimmune neuropsychiatric disorders, including ADEM, Sydenham's chorea, and PANDAS.
- Adult-onset ADEM is less common and often associated with different triggers and clinical presentations compared to pediatric cases.
Observation:
- A novel case of adult-onset ADEM is presented, clearly linked to documented streptococcal pharyngitis.
- The patient exhibited elevated antideoxyribonuclease B (ADNB) titers, confirming a recent streptococcal infection.
- Cerebrospinal fluid (CSF) analysis revealed significant leukocytosis.
Findings:
- The adult ADEM case presented with an absence of basal ganglia abnormalities on magnetic resonance imaging (MRI).
- Elevated ADNB titers in an adult with ADEM suggest a post-streptococcal etiology.
- Distinctive CSF leukocytosis and lack of basal ganglia lesions differentiate this adult ADEM case from typical childhood ADEM and adult viral demyelinating diseases.
Implications:
- This case expands the understanding of ADEM's etiology, demonstrating a link to streptococcal infections in adults.
- The findings suggest that adult ADEM may have distinct diagnostic criteria, particularly regarding neuroimaging and CSF analysis.
- Recognizing streptococcal triggers in adult ADEM is crucial for accurate diagnosis and potentially targeted treatment strategies.
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