Post-streptococcal autoimmune dystonia with isolated bilateral striatal necrosis

Abstract

Infantile bilateral striatal necrosis (IBSN) is characterized by a dystonic movement disorder and basal ganglia imaging abnormalities. Acute IBSN often occurs after upper respiratory tract infections although no specific micro-organism which may cause IBSN has been identified. We present 2 children (1 year 2 months and 4 years) with acute IBSN after clinical pharyngitis. Both IBSN patients had serological evidence of recent beta-haemolytic streptococcal infection. Due to the association of post-streptococcal disorders with anti-basal ganglia antibodies (ABGA), we examined both patients for anti-neuronal antibodies. For comparison, 20 children with dystonia (9 females, 11 males; mean age 4 years 1 month), and 20 children with uncomplicated streptococcal infection (12 females, 8 males; mean age 5 years 9 months) were examined. Both IBSN patients had antibodies reactive against basal ganglia constituents of molecular weight 40 kDa. Immunohistochemistry showed antibody reactivity against large striatal neurons only. Other anti-neuronal antibodies were negative, supporting striatal specificity. All controls were negative for ABGA. Acute IBSN is part of the poststreptococcal autoimmune neuropsychiatric spectrum. An autoimmune aetiology should be considered in this phenotype, as immunomodulatory therapies may reduce morbidity and mortality.

Progressive striatal necrosis associated with anti-NMDA receptor antibodies

“…Immune mediated BSN is a rare clinical entity [1] which has been associated with a variety of infectious agents including streptococci [2] and mycoplasma [3]. An infectious or parainfectious pathomechanism has been proposed, but the pathogenesis of these disorders remains unclear.…”

Post‐streptococcal autoimmune disorders of the central nervous system

“…9,24,25 In addition, Parkinsonism has been described, either associated with SC, 26 or as an isolated post-streptococcal phenomenon. 24 It is not known why some patients develop particular movement disorders. The clinical neurological phenotype is relatively specific to movement disorders, although encephalopathy (and disseminated encephalomyelitis) are sometimes described.…”

“…[51][52][53][54] In post-streptococcal dystonia and Parkinsonism, inflammatory changes involving the basal ganglia appear to be more common. 24,30 More sophisticated techniques using volumetric studies have shown specific enlargement of the caudate and putamen during the acute phase of SC and PANDAS. 49,55 This volumetric enlargement resolves during remission.…”

“…Patients with post-streptococcal dystonia and Parkinsonism also have antineuronal antibodies (in serum and cerebrospinal fluid [CSF]) to the 40, 45, and 60 kDa autoantigens, similar to those seen in SC. 24,29,30,37 The 40, 45, and 60 kDa antigens are predominantly brain specific or brain enriched. 30 The identity of these brain autoantigens is the subject of current investigation, and will help define the possible role that antineuronal antibodies have in post-streptococcal disorders of the CNS.…”

Cytokine Production by Mononuclear Cells from Patients with Familial Infantile Bilateral Striatal Necrosis

“…Current data, although very limited because of the rarity of the disease, suggest that the onset of familial IBSN is often preceded by a mild infection such as pharyngitis, an upper respiratory tract infection [10, 11], measles [12], human herpes virus-6 infection [13], Mycoplasma infection [14], and pneumonitis [15]. Together, these findings raise the question of an immune system imbalance in patients with IBSN.…”