Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

Infectious complications of intrauterine insemination: a case report and literature review.

Intrauterine insemination is commonly performed in the treatment of infertility. Infectious complications associated with intrauterine insemination are frequently cited, though rarely reported. A review of the literature yields only five reported cases of pelvic infections subsequent to intrauterine insemination (IUI), and only two of these show firm evidence of infection, with none presenting bacteriologic confirmation. We report a case of Escherichia coli septicemia subsequent to an IUI performed on a patient with a large adenomyoma. Of 147 patients treated with IUI at Georgetown University from November 1987 through March 1990, this was the only infectious complication. The incidence of infectious complications in our series is thus 0.0068 (6.8 per 1,000 women). A review of infectious complications in 38 reported series on intrauterine inseminations reveals five infections in 3,129 patients. With the addition of our series to the literature, the prevalence of infectious complications is 1.83 per 1,000 women undergoing IUI. The rates were not significantly altered by semen washing with antibiotics, or the administration of prophylactic antibiotics to the woman (P = .81). We conclude that (1) the infection rate following IUI is small, (2) many of the infections subsequent to insemination were not associated with intrauterine insemination, (3) most reported cases of infection fail to show evidence for the actual presence of infection, and (4) the prevalence is unaltered by the administration of prophylactic antibiotics or washing the semen sample with antibiotics.     

Extrapulmonary cryptococcosis in children with acquired immunodeficiency syndrome

There is a paucity of published information available on extrapulmonary cryptococcosis (EC) in children infected with human immunodeficiency virus, the etiologic agent of the acquired immunodeficiency syndrome. We surveyed investigators in pediatric acquired immunodeficiency syndrome around the country regarding their experience with EC. Investigators from 33 (87%) of 38 institutions responded and information on 13 patients from 11 institutions was analyzed. EC was the acquired immunodeficiency syndrome indicator disease in 9 (69%) of 13 patients. Median age was 8 years with a range of 2 to 17 years. Human immunodeficiency virus risk factors were transfusion (5 patients), hemophilia (4 patients) and perinatal exposure (4 patients). Meningitis, seen in 62% of patients, was the most common clinical manifestation. Although 2 patients with fulminant disease died before therapy was started, 10 (91%) of 11 had a clinical response to amphotericin B with or without flucytosine. Our study indicates a spectrum of EC in pediatric human immunodeficiency virus infection ranging from fulminant, fatal fungemia to chronic meningitis and fever of unknown origin. Cryptococcosis was generally not the cause of death in patients who initially responded to amphotericin B therapy. Optimal antifungal therapy, including the role of fluconazole, warrants further study.     

Inter-rater reliability of the International Cooperative Ataxia Rating Scale (ICARS).

We assessed the inter-rater reliability of the 100-point International Cooperative Ataxia Rating Scale (ICARS). Three neurologists independently rated videotaped ICARS examinations of 22 subjects with genetically determined ataxias (spinocerebellar ataxia [SCA] Type 1 in 11; SCA Type 2 in 1; Friedreich's ataxia in 10) and 4 controls. Scores on live ICARS assessment had ranged from 0 to 7 for controls and 11 to 74 for ataxic subjects (clinically very mildly affected to wheelchair-bound). Inter-rater correlation was very high for the total score (Kendall's omega 0.994, 95% confidence interval, 0.988-0.997), and high to very high for each component subscore (0.791 for speech to 0.994 for posture/gait). All correlations were significant at P < 0.00001. The ICARS exhibits very high inter-rater reliability even without prior observer standardisation and is sensitive to a range of ataxia severities from very mild to severe.     

Continuous venous infusion of doxorubicin in advanced sarcomas

Seventeen patients with advanced sarcoma were treated with continuous venous infusion of doxorubicin for a mean of 118 days, achieving total doses up to 1097 mg/m2. Three partial responses and one minor response were obtained. Major toxic effects were stomatitis and hand-foot syndrome. There was a low incidence of leukopenia (18%) and clinical cardiotoxicity (11%). Continuous venous infusion is a safe means of administering doxorubicin, with a response rate similar to that observed with bolus doxorubicin in metastatic sarcoma.     

Gastroduodenal tolerability of indomethacin and acetaminophen. A comparative endoscopic study in healthy subjects

The effect of a 5- and 10-day treatment with indometacin and acemetacin (Rantudil) on the gastroduodenal mucosa was endoscopically evaluated in 16 healthy volunteers. In a randomised double-blind cross-over fashion the volunteers received 50 mg t.i.d. indometacin as well as 60 mg t.i.d. acemetacin. Acemetacin evoked after 5 and 10 days significantly less gastroduodenal lesions than indometacin. Possible reasons for this apparently better tolerability of acemetacin in man are discussed.