Objective: To report the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients Յ 18 years old. Methods: Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1. Results: Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were Յ18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women Ͼ18 years old, 31% in girls Յ18 years old ( p ϭ 0.05), and 9% in girls Յ14 years old ( p ϭ 0.008). None of the male patients had tumors. Of 32 patients Յ18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p ϭ 0.03). Interpretation: Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children.Ann Neurol 2009;66:11-18 Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently described disorder with a well defined set of clinical features.1 The associated syndrome has been characterized in adults, frequently young women with teratomas of the ovary who develop changes of mood, behavior, and personality, resembling acute psychosis. The clinical picture usually progresses to include seizures, decreased level of consciousness, dyskinesias, autonomic instability, and hypoventilation.2-5 Despite the severity of the disorder, patients often improve with immunotherapy and removal of the teratoma. 1,3,6 These findings and the discovery that all patients have serum and cerebrospinal fluid (CSF) antibodies that react with the cell surface of neurons suggested an immune-mediated pathogenesis. 1,7,8 Further studies demonstrated that the target antigen of patients' antibodies was the NR1 subunit of the NMDAR. Additionally, application of antibodies into cultures of hippocampal neurons resulted in a significant decrease of postsynaptic NMDAR clusters that was reversed after antibody removal. 9 A recent series of 100 patients showed that the disorder also occurs in patients without teratoma, and that men and children c...
Internists clearly stated the need for better training in congenital and childhood-onset conditions, training of more adult subspecialists, and continued family involvement. They also identified concerns about patients' psychosocial issues and maturity, as well as financial support to care for patients with complex conditions.
ITZ-HUGH-CURTIS SYNDROME-perihepatitis associated with pelvic inflammatory disease-can pose a diagnostic challenge to the clinician, especially when right upper quadrant pain predominates, mimicking acute gall bladder disease. This article reviews the etiology, pathogenesis, diagnosis, and treatment of this syndrome. s HISTORICAL PERSPECTIVE Association with gonorrhea Fitz-Hugh-Curtis syndrome was first described in 1920 by Carlos Stajano, 1 who noted adhesions between the liver capsule and anterior abdominal wall in patients with gonococcal infection and right upper quadrant pain. In the 1930s Thomas Fitz-Hugh and Arthur Curtis also described the syndrome and made the connection between the acute clinical syndrome of right upper quadrant pain following a pelvic infection and the "violinstring" adhesions found in women with evidence of prior salpingitis. 2,3 Curtis described several cases of these very typical adhesions between the liver and the abdominal wall in patients with gonococcal disease and noted that similar adhesions were not found in other causes of peritonitis, suggesting the combination was a unique syndrome. 4 Fitz-Hugh suggested that Neisseria gonorrhoeae was the cause when he found gram-negative diplococci on smears from the liver capsule in patients with the syndrome. 2 Since then, the diagnosis of Fitz-Hugh-Curtis syndrome has largely been a clinical
Human papillomavirus (HPV) is the most common sexually transmitted infection in the United States, accounting for the large majority of cervical cancer and anogenital warts cases. Two HPV vaccines are currently licensed and recommended for women and girls. However, vaccination rates have been suboptimal, with evidence of disparities influencing both uptake and series completion among African American and Hispanic adolescents. There has been a dearth of theory-based, behavioral interventions targeted to prevent HPV infection and increase HPV vaccine uptake among urban adolescents. This article describes the development of two skills-based intervention curricula aimed to increase HPV prevention and vaccination among low-income urban adolescent females 9 to 18 years old. Guided by the theory of planned behavior, elicitation research was conducted to elucidate the social psychological factors that underlie HPV vaccination intentions (N = 141). The findings were subsequently used to identify theoretical mediators of behavioral change to drive the intervention. Culturally relevant strategies to promote HPV vaccination were translated into the curricula content. Both curricula were designed to motivate and empower participants to reduce risk of being infected with HPV. Targeting theoretical mediators of behavioral change, derived from the voices of the community, may prove to be successful in increasing HPV vaccination and preventing HPV.
The Institute of Medicine defines health disparities as "racial or ethnic differences in the quality of health care that are not due to access-related factors or clinical needs, preferences, and appropriateness of intervention" (Smedley, Stith, & Nelson, 2003). In this case, discrimination at the individual patient-provider level is thought to manifest as differences in care resulting from biases, prejudices, and stereotyping in clinical communication and decision making. There is evidence that patients who perceive racial discrimination are more likely to report adverse health outcomes. Previous work has found that minority adults who report ever experiencing discrimination, being prevented from doing something, being hassled, or made to feel inferior in various situations have greater physical and psychiatric symptoms than their counterparts who did not report this experience (Borrell, Kiefe, Williams, Diez-Roux, & Gordon-Larsen, 2006). In addition, individuals who report experiencing discrimination at any time during their lifetime have been found to have poor adherence to treatment, worse health outcomes, and delays in seeking medical care (Casagrande, Gary, LaVeist, Gaskin, & Cooper, 2007). The work of Dr. David Williams (2012) and others has continued to demonstrate a clear relationship between minority race and poorer health outcomes. Racial inequities and disparities are evident in the timing of disease onset (earlier for minorities), severity and progression of disease (more severe for minorities), the effects of some risk factors (less exposure to the risk factor has a greater impact on minorities), and the persistence of health disparities over time (resulting in a lower life expectancy for minorities vs. non-minorities). Racial discrimination is reported frequently by African American adolescents, similar to their adult counterparts (Fisher,
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