Post‐streptococcal autoimmune disorders of the central nervous system

Abstract

Group A Streptococcus can induce autoimmune disease in humans with particular involvement of the heart, joints, and brain. The spectrum of post‐streptococcal disease of the central nervous system (CNS) has been widened recently and includes movement disorders (chorea, tics, dystonia, and Parkinsonism), psychiatric disorders (particularly emotional disorders), and associated sleep disorders. Neuroimaging and pathological studies indicate that the most vulnerable brain region is the basal ganglia. The immunopathogenesis of the disease is incompletely defined, and although there is some support for autoantibody‐mediated disease, several conflicting studies cast doubt on the autoantibody hypothesis. It has been speculated that post‐streptococcal autoimmunity has a role in common neuropsychiatric disease but the evidence is conflicting and routine screening of patients with Tourette syndrome and obsessive‐compulsive disorder for post‐streptococcal autoimmune abnormalities is not be recommended at present. However, post‐streptococcal disorders of the CNS remain a useful model of neuropsychiatric disease, which may improve our understanding of abnormal movements and behaviours in children.

Antibasal Ganglia Antibodies and Antistreptolysin O in Noncomorbid ADHD

“…Our data therefore provide evidence that streptococcal infections and autoimmune reactions against the basal ganglia may play an etiologic role or to modulate the phenotype in some cases of ADHD (Dale, 2005;Loiselle, Wendlandt, Rohde, & Singer, 2003). The pathophysiology of poststreptococcal neuropsychiatric disorders is still undefined.…”

“…Despite the hypothesis of an immunemediated pathogenesis of poststreptococcal neuropsychiatric disorders, imaging studies do not show any abnormality of the blood-brain barrier (BBB) integrity in these patients. Recent observations support the possibility that activated lymphocytes might have access to the CNS even through an intact BBB (Dale, 2005). It is thus theoretically possible that activated autoreactive B cells, which synthesize and release autoantibodies in the serum, might pass an integer BBB and start a local synthesis of the same autoantibodies in the CNS.…”

Bilateral symmetrical basal ganglia and thalamic lesions in children: an update (2015)

“…The classic post-streptococcal neurologic clinical presentation is that of Sydenham's chorea, but recently, other poststreptococcal basal ganglia-associated neurological symptoms have been described in patients who do not meet the criteria for choreiform movements, including myoclonus, dystonia, and parkinsonism [39] (Fig. 7).…”

“…Many autoimmune disorders in the pediatric population can affect the basal ganglia, including post-streptococcal autoimmunity [39], N-methyl-D-aspartate receptor (NMDAR) antibodies [40], neuromyelitis optica (NMO)/neuromyelitis optica spectrum disorders (NMOSD) [41], and ADEM ( Fig. 6) [42].…”

Letters to the Editor

“…There are a number of post‐infective complications recorded following Group A streptococcal pharyngitis which are considered to have an immunopathogenic basis. In addition to rheumatic fever and glomerulonephritis, these include post‐streptococcal reactive arthritis, 4 paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, 5 uveitis, erythema nodosum, tenosynovitis of the superior oblique tendon sheath and polymyalgia. Post‐streptococcal polymyalgia consists of muscle pain, fever, raised antistreptolysin O titres, neutrophilia, raised inflammatory markers and normal muscle enzymes 6 .…”