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Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset of the symptoms was 11.7 +/- 2.6 years (range, 6-17 years). Of the patients, 63% were female and 37% were male (male/female: 1.7/1). Chorea was generalized in 78.5% of the patients, right hemichorea in 12.3%, and left hemichorea 9.2%. There was a history of rheumatic fever in 30.8% of the patients. Echocardiographic study showed cardiac valve involvement in 70.5% of 61 patients. Brain magnetic resonance imaging, which was performed on only 18 patients, was evaluated as normal in all. Electroencephalography was also performed on only 18 patients and showed abnormal waves in 50% of them. Pimozide was mostly the first choice of drug therapy. Nevertheless, drug therapy was not needed in 18.5% of the patients. The recovery period of the first attack of the chorea was 1 to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenham's chorea is still an important health problem in Turkey with respect to its morbidity.

In a retrospective study, we investigated and compared the angulation and the length of the styloid process between patients operated for Eagle's syndrome and a control group by means of lateral skull and Towne's radiographs. Thirty patients with 51 symptomatic elongated styloid processes underwent surgery. As a control group, patients with chronic otitis media and trauma were included in the study and none of them had symptoms characteristic of an elongated styloid process. The length, medial and anterior angulation of the styloid processes of the patient and the control groups were measured on lateral skull and Towne's radiographs. The mean length of the styloid process was 5 cm on the right and 5.2 cm on the left in the patient group, whereas they were 2.8 and 2.6 cm, respectively, in the control group. The mean degree of anterior angulation in the patient group was 33.6 degrees on the right, 36.7 degrees on the left, whereas these were 21.4 degrees and 18.5 degrees , respectively, in the control group. There was a significant difference between the two groups for length and anterior angulation (P = 0.001). The mean medial angulation was 14 degrees on the right and 18.1 degrees on the left in the patient group, whereas these were 15 degrees and 16.3 degrees in the control group, respectively, and there were no significant differences between the two groups. The anterior angulation and the length of the styloid process are responsible for the symptoms of Eagle's syndrome.

To study the effect of continuous ambulatory peritoneal dialysis on nucleoside levels and clinical course in a patient with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE).Patient: We studied a patient with genetically verified MNGIE, who prior to treatment had lost weight progressively, developed amenorrhea, vomited multiple times daily, and had abdominal pain. Intervention:The patient was treated with peritoneal dialysis for 3 years, and the effect on symptoms and plasma concentrations of thymidine and deoxyuridine were monitored.Results: Dialysis stopped vomiting and reduced abdominal pain, and the patient gained 5 kg in weight and started to menstruate again. Symptoms returned if dialysis was paused. Dialysis did not affect plasma nucleoside levels. Conclusions:This study shows an unambiguous clinical benefit of peritoneal dialysis on gastrointestinal symptoms in MNGIE. Dialysis did not affect nucleoside levels, indicating elevated thymidine and deoxyuridine levels are not solely responsible for the pathogenesis of MNGIE.

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BackgroundMaxillary sinus mucoceles are relatively rare among all paranasal sinus mucoceles. With the introduction of endoscopic sinus surgical techniques, rhinologic surgeons prefer transnasal endoscopic management of sinus mucoceles. The aim of this study is to describe the clinical presentation of maxillary sinus mucoceles and to establish the efficacy of endoscopic management of sinus mucoceles.MethodsBetween 2003 and 2005, 14 patients underwent endoscopic sinus surgery for maxillary sinus mucocele. The presenting sign and symptoms, radiological findings, surgical management and need for revision surgery were reviewed.ResultsThere were eight males and six females with an age range of 14 to 65. Ten patients complained of nasal obstruction, five of nasal drainage, five of cheek pressure or pain and one of proptosis of the eye and cheek swelling. The maxillary sinus and ipsilateral ethmoid sinus involvement on computed tomographic studies was seen in 4 patients. Four patients had history of endoscopic ethmoidectomy surgery for ethmoid sinusitis and one had Caldwell-Luc operation in the past. Ethmoidectomy with middle meatal antrostomy and marsupialization of the mucocele was performed in all patients. Postoperative follow-up ranged between 8 to 48 months. All patients had a patent middle meatal antrostomy and healthy maxillary sinus mucosa. No patients need revision surgery.ConclusionThe most common causes of mucoceles are chronic infection, allergic sinonasal disease, trauma and previous surgery. In 64% of the patients of our study cause remains uncertain. Endoscopic sinus surgery is an effective treatment for maxillary sinus mucoceles with a favorable long-term outcome.

SummaryAutosomal recessive, autosomal dominant and the sporadic forms of hyperimmunoglobulin E syndrome (HIES) are multi-system disorders. Although HIES patients may present with cold abscesses, the vascular features of HIES are not well recognized. The objective of this review is to characterize the nature and spectrum of vascular abnormalities in HIES patients. Vascular abnormalities in HIES patients were reviewed with Medline and Google Scholar-based searches. In brief, the searches combined terms related to HIES with the terms related to vasculature. Furthermore, reference lists from the original studies and review papers identified were screened. There were vascular abnormalities in 25 patients with HIES. These abnormalities were identified as aneurysms (coronary, aortic, carotid and cerebral), pseudoaneurysms, congenital patent ductus venosus, superior vena cava syndrome, vasculitides, vascular ectasia, thrombosis and others. They may be congenital or acquired, in the veins and arteries, affecting both sexes. These abnormalities can be seen in all subtypes of HIES. They could be also fatal in children and adults. Limited pathological investigations revealed the presence of vasculitis. Three of the patients were found to have overlap diseases. In this review, the spectrum of vascular abnormalities in HIES are documented and discussed in detail for the first time. They highlight a previously under-recognized and potentially devastating complication of these disorders. These vascular abnormalities constitute one of the major clinical characteristics in HIES. The presence of hypereosinophilia, vasculitis and defective angiogenesis in HIES may contribute to the formation of vascular abnormalities in HIES.

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