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Published on: November 21, 2013
Pediatric neuropsychiatric disorders with motor and nonmotor phenomena
1Department of Clinical Medicine and Neuroscience, CUNY School of Medicine, New York, NY, United States; Department of Medicine, Section of Internal Medicine and Neurology, White Plains Hospital, White Plains, NY, United States.
Insights
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections (PANDAS) and related conditions are increasingly recognized. These disorders form a spectrum, including PANS, with shared neuroimaging findings.
Area of Science:
- Neuroscience
- Pediatrics
- Immunology
Background:
- Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections (PANDAS) is a well-established concept.
- The spectrum of these disorders has expanded to include other triggers and conditions like Pediatric Acute-onset Neuropsychiatric Syndrome (PANS).
Approach:
- Investigating the spectrum of post-infectious autoimmune neuropsychiatric disorders.
- Utilizing advanced neuroimaging techniques like FDG PET-MRI to identify common pathophysiological features.
Key Points:
- PANDAS and PANS share clinical features such as tics and obsessive-compulsive disorder.
- Brain imaging reveals hypometabolism in the medial temporal lobe and hippocampus in affected children.
- These disorders exist on a spectrum, with Tourette syndrome and PANDAS at one end and PANS at the other.
Conclusions:
- The concept of PANDAS has evolved into a broader spectrum of pediatric neuropsychiatric disorders.
- PANS, often an exclusionary diagnosis, highlights current challenges in nosology and classification.
- Further research is needed to refine diagnostic criteria and understand the full spectrum of these conditions.
Abstract:
The concept of pediatric autoimmune neuropsychiatric disorders associated with group A beta-hemolytic streptococcus (PANDAS) has become seminal since first introduced more than two decades ago. At the time of this writing, most neurologists, pediatricians, psychiatrists, and general pediatricians will probably have heard of this association or treated an affected child with PANDAS. The concept of an acute-onset, and typically self-limited, postinfectious autoimmune neuropsychiatric disorder resembling PANDAS manifesting vocal and motor tics and obsessive-compulsive disorder has broadened to other putative microbes and related endogenous and exogenous disease triggers. These disorders with common features of hypometabolism in the medial temporal lobe and hippocampus in brain 18fluorodeoxyglucose positron emission tomography fused to magnetic resonance imaging (FDG PET-MRI), form a spectrum: with the neuropsychiatric disorder Tourette syndrome and PANDAS with its well-defined etiopathogenesis at one end, and pediatric abrupt-onset neuropsychiatric syndrome (PANS), alone or associated with specific bacterial and viral pathogens, at the other end. The designation of PANS in the absence of a specific trigger, as an exclusionary diagnosis, reflects the current problem in nosology.
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