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Published on: November 4, 2017
Gait Disturbance as the Presenting Symptom in Young Children With Anti-NMDA Receptor Encephalitis
Anusha K Yeshokumar1, Lisa R Sun2, Jessica L Klein3
1Division of Pediatric Neurology, and ayeshok1@jhmi.edu.
Insights
Gait disturbance is an early sign of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in children under three. This finding aids in earlier diagnosis of this autoimmune encephalitis in toddlers.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Autoimmune Encephalitis
Background:
- Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a significant cause of pediatric encephalitis, often presenting with speech and sleep disturbances.
- While common in children, anti-NMDAR encephalitis is less frequently reported in infants and toddlers.
- Early diagnosis and treatment are crucial but often delayed due to non-specific initial symptoms.
Observation:
- This case series highlights a novel presentation of anti-NMDAR encephalitis in four children under three years old.
- The primary initial symptom in all cases was gait disturbance, including unsteady walking and inability to bear weight.
- Seizures and cerebellar ataxia-like symptoms were also noted at presentation.
Findings:
- Gait disturbance, particularly in the context of other neurological signs, should prompt consideration of anti-NMDAR encephalitis in young children.
- This contrasts with the more typical initial symptoms of disrupted speech and sleep patterns seen in older children.
- The study identifies a specific early clinical phenotype in a rarely reported age group.
Implications:
- Recognizing gait disturbance as an early indicator can lead to faster diagnosis and intervention for anti-NMDAR encephalitis in toddlers.
- This expands the understanding of clinical variability in pediatric anti-NMDAR encephalitis.
- Early diagnosis is critical for improving outcomes in this potentially severe autoimmune neurological condition.
Abstract:
This case series demonstrates a novel clinical phenotype of gait disturbance as an initial symptom in children <3 years old with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Anti-NMDAR encephalitis is one of the most common causes of encephalitis in children, more common than any of the viral encephalitides and the second most common autoimmune cause after acute disseminated encephalomyelitis. Anti-NMDAR encephalitis in children often presents with disrupted speech and sleep patterns followed by progression to motor dysfunction, dyskinesias, and seizures. Because this condition can present initially with vague symptoms, diagnosis and treatment of anti-NMDAR encephalitis are often delayed. Although nearly 40% of all reported patients are <18 years old, few infants and toddlers have been reported with this disease. Four children <3 years old were diagnosed with anti-NMDAR encephalitis at our institution. Interestingly, each child presented initially with the chief concern of gait disturbance. One child presented with unsteady walking and slurred speech, suggestive of cerebellar ataxia, and 3 had inability to bear weight on a unilateral lower extremity, resulting in unsteady gait. Two of these children had seizures at the time of hospital presentation. All developed classic behavioral changes, insomnia, dyskinesias, or decreased speech immediately before or during hospitalization. When seen in the setting of other neurologic abnormalities, gait disturbance should raise the concern for anti-NMDAR encephalitis in young children. The differential diagnosis for gait disturbance in toddlers and key features suggestive of anti-NMDAR encephalitis are reviewed.
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