Post-viral syndromes
Avindra Nath, Joseph R. Berger in Clinical Neurovirology, 2020
Sydenham’s chorea is most frequently seen in children ages 8 to 9 years and is rarely seen in adults. Symptoms develop weeks to months after group-A streptococcal pharyngitis. In a study of 50 patients with rheumatic fever, chorea began unilaterally but spread to both sides in 80% of cases [58]. Diagnosis is by clinical Jones criteria (Table 25.4) and exclusion of other etiologies such as stroke, intoxication or infectious encephalitis. Antistreptolysin O or DNAse antibody titers may support evidence of streptococcal infection. Neuroimaging may be normal or transient T2 hyperintensities in the basal ganglia may be seen [59,60]. In one study of 24 children with recent onset of Sydenham’s chorea, while only two showed T2 hyperintensities on MRI in the basal ganglia, quantitative analysis revealed increased size of the basal ganglia relative to age-matched controls [61]. Other studies have demonstrated increased metabolism in the basal ganglia in two patients by PET [61] and six of ten of patients by SPECT imaging [62].
Unexplained Fever In Infectious Diseases: Section 2: Commonly Encountered Aerobic, Facultative Anaerobic, And Strict Anaerobic Bacteria, Spirochetes, And Parasites
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
The work-up for establishing a correct diagnosis comprises: (a) the isolation and characterization of streptococcus species from appropriate cultures (blood or samples taken from the inflammatory site); and (b) the presence and significant rise of streptococcal antibodies in the serum. The usual test is ASO (antistreptolysin titer) which rises in only 70 to 80% of patients. A rising titer is indicative only of current or recent streptococcal infection. Antibodies appear about 2 weeks after infection. Serum need not be taken more than once every 2 weeks. For completion of the ASO test, a hemagglutination test (streptozyme) may be made, being more sensitive in early detection of antibody responses; but, although rapid and simple to perform, it has some limitations (sensitive only for pharyngeal infection).1
Accident and Emergency
Nagi Giumma Barakat in Get Through, 2006
The major manifestations of rheumatic fever include carditis, polyarthritis, erythema marginatum, Sydenham’s chorea and subcutaneous nodules. Minor manifestations include fever, high ESR, elevated antistreptolysin-O-titers, abdominal pain, previous rheumatic fever, arthralgia, acute phase reaction and a prolonged PR interval. The presence of two major criteria or one major and two minor criteria indicates a high possibility of rheumatic fever. Echocardiography will be helpful in the diagnosis. The mitral valve is the first to be involved, with mitral regurgitation, apical systolic murmur and some short diastolic murmur (Carey-Coombs murmur) of mitral stenosis. Congestive cardiac failure could be the first presentation, with tachycardia, vomiting and hepatomegaly.
A case of neutrophilic dermatosis with MEFV gene variant and abnormal activation of peripheral blood monocytes: a case report
Published in Immunological Medicine, 2019
Mizuna Otsuka, Tomohiro Koga, Remi Sumiyoshi, Yuta Koike, Kaori Furukawa, Momoko Okamoto, Yushiro Endo, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Takashi Igawa, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Niino Daisuke, Atsushi Kawakami
On admission, his body temperature was 37.6 °C, blood pressure was 97/51 mmHg, and heart rate was 68 beats/min. A physical examination showed swollen lymph nodes in the left neck, they were soft, movable, and non-tender. Further, he had erythema in the forehead, neck, and abdomen with infiltration. Laboratory investigations revealed the following results: hemoglobin (Hb) 11.4 g/dL, WBC 12200/μL (neutrophils 87.8%), platelet count 420 × 103/μL, lactate dehydrogenase 198 IU/L, alkaline phosphatase 423 U/L, aspirate aminotransferase 41 IU/L, alanine aminotransferase 118 IU/L, γ GTP 110 IU/L, blood urea nitrogen 9 mg/dL, creatinine 1.89 mg/dL, ferritin 1728 ng/ml, and CRP 11.27 mg/dL. The titers of anti-streptolysin O antibody (ASO) and anti-streptokinase antibody (ASK) were 1024 and 20,480, respectively.
Sydenham’s chorea: from pathophysiology to therapeutics
Published in Expert Review of Neurotherapeutics, 2021
Antonio L. Teixeira, Luiz P. Vasconcelos, Maria do Carmo Pereira Nunes, Harvey Singer
In comparison with other ARF major symptoms, i.e. carditis, arthritis and skin lesions, SC usually manifests later in the course of ARF, developing four to eight weeks after GABHS pharyngitis, affecting mainly children and adolescents [7,8]. Accordingly, patietns presenting only with chorea – usually an uncommon presentation [51,52] – may not notice the temporal association between GABHS infection and motor symptoms emergence. Moreover, the results of throat culture, acute phase proteins such as C reactive proteins (CRP), and anti-streptococcal antibodies, i.e. anti-streptolysin-O (ASO) and anti-DNase B, vary significantly, with the percentage of patients with elevated antistreptococcal titers ranging from about 15–80% [6,53]. As single time-point cultures and single antibody titers are often misleading, sequential samples, preferably measuring both ASO and anti-DNase B, more accurately define infection, allowing correlation of titer changes with GABHS infection [54].
Direct Immunofluorescence Results of the Skin Biopsy and Frequency of Systemic Involvement in Children with Henoch-Schonlein Purpura
Published in Fetal and Pediatric Pathology, 2019
Mehdi Ataeepour, Maryam Monajemzadeh, Peyman Sadeghi, Vahid Ziaee
HSP usually tends to have a good prognosis without significant sequelae. However, in a subset of patients, renal involvement results in an adverse outcome. HSP nephritis is generally a self-limited disease, presenting with hematuria and/or mild proteinuria, however in some patients, it may progress to a more severe renal impairment [1]. It is reported in up to 50% of pediatric patients, 1%–7% of which eventually develop renal failure [1]. Although HSP nephritis usually occurs within 4–6 weeks of the initial presentation, it might also be a delayed complication, so a 6-month follow-up with urinary evaluations is recommended after the onset of HSP [1,2]. Renal biopsy is a definitive but invasive way to diagnose renal involvement, yet it is not routinely obtained. It would be advantageous to determine a noninvasive measure to predict renal involvement in HSP patients. In a systematic review by Chan et al, the following risk factors were associated with higher renal involvement in pediatric patients: male gender, age more than 10 years, severe GI symptoms (abdominal pain, GI bleeding, and severe bowel angina), arthritis/arthralgia, persistent purpura or relapse, white blood cells >15,000/µL, platelets >500,000/µL, elevated antistreptolysin O, and low C3 [13].