Brain SPECT imaging in Sydenham's chorea

Abstract

The objective of the present study was to determine whether brain single-photon emission computed tomography (SPECT) imaging is capable of detecting perfusional abnormalities. Ten Sydenhams chorea (SC) patients, eight females and two males, 8 to 25 years of age (mean 13.4), with a clinical diagnosis of SC were submitted to brain SPECT imaging. We used HMPAO labeled with technetium-99m at a dose of 740 MBq. Six examinations revealed hyperperfusion of the basal ganglia, while the remaining four were normal. The six patients with abnormal results were females and their data were not correlated with severity of symptoms. Patients with abnormal brain SPECT had a more recent onset of symptoms (mean of 49 days) compared to those with normal SPECT (mean of 85 days) but this difference did not reach statistical significance. Brain SPECT can be a helpful method to determine abnormalities of the basal ganglia in SC patients but further studies on a larger number of patients are needed in order to detect the phase of the disease during which the examination is more sensitive.

Functional neuroimaging and chorea: a systematic review

“…Of note, imaging was performed a mean of 49 days from symptom onset in the patients who exhibited hyperperfusion, while scans were performed after 85 days in the patients with normal SPECT findings, although this difference was not statistically significant [57]. In a case report of an 18-year-old man with bilateral Sydenham chorea, a 99m Tc HMPAO-SPECT during the first week of symptom onset showed hypoperfusion in the left basal ganglia [58].…”

Subtle cognitive deficits in adults with a previous history of Sydenham's chorea during childhood

“…Some studies suggest that its etiopathogenesis is related to immunologic dysfunction located at the basal ganglia, mediated by antibodies that cross‐react against antigens in these structures after a group A streptococcus pharyngitis infection (9, 10). Autopsy findings (11) and studies of anatomic (12, 13) and functional (14) neuroimaging show involvement of the basal ganglia and their different frontostriatal circuits.…”

Behavioral and neural effects of intra-striatal infusion of anti-streptococcal antibodies in rats

“…Moreover, the abnormal behaviors in GAS-exposed rats were attenuated by pharmacological treatments used to treat the corresponding symptoms in human patients (i.e., a selective serotonin reuptake inhibitor (SSRI) and a D2 blocker, respectively). Immunologically, IgG was found in the striatum, prefrontal cortex (PFC) and thalamus of GAS-exposed rats (Brimberg et al, 2012), corresponding to the brain regions implicated in GAS-related neuropsychiatric disorders (Barsottini et al, 2002; Citak et al, 2004; Dilenge et al, 1999; Giedd et al, 2000; Huyser et al, 2009). IgG in sera obtained from GAS-exposed rats demonstrated strong immunoreactivity with D1 and D2 dopamine receptors and activated calcium/calmodulin-dependent protein kinase II signaling, as has previously been found for IgG in sera obtained from SC and PANDAS patients (Kirvan et al, 2003; Kirvan et al, 2006).…”