A nationwide survey of excessive daytime sleepiness in Parkinson's disease in France

To determine the prevalence of excessive daytime sleepiness (EDS) and that of dozing and sudden onset of sleep episodes (SOS) while driving in ambulatory patients with Parkinson's disease (PD) in France, a national sample of private and public neurologists was asked to recruit the first 10 consecutive nondemented PD patients. Each patient completed a questionnaire including the Epworth Sleepiness Scale (ESS) and the likelihood of dozing off and experiencing SOS episodes behind the wheel. Clinical and demographic data were collected. One thousand six hundred and twenty‐five patients with PD were included in the survey. Twenty‐nine percent of the patients suffered from EDS (ESS score ≥10) but only 0.8% declared a high chance of dozing while driving and 0.5% reported totally unpredictable SOS episodes while driving. Risk factors for EDS were male gender, reduced activity of daily living, and a high daily levodopa equivalent dosage. Risk factors for SOS episodes while driving were an ESS score ≥10, male gender, and low Hoehn and Yahr staging. EDS is common in ambulatory patients with PD and is a major risk factor for dozing and for SOS episodes behind the wheel in patients who drive. © 2007 Movement Disorder Society     

Hyalinizing trabecular adenoma of the thyroid. Report of two cases, with cytologic, immunohistochemical and ultrastructural studies.

The cytologic, histologic, immunocytochemical and ultrastructural features of 2 cases of hyalinizing trabecular adenoma (HTA) of the thyroid are described. The difficulty of a cytologic diagnosis and the need for an immunohistochemical profile of the lesions for a final histologic diagnosis are emphasized.     

Changes in clinical diagnostic criteria for multiple system atrophy

Multiple system atrophy (MSA) is a clinical and pathological entity characterized by the variable combination of autonomic failure, parkinsonism, cerebellar and pyramidal signs and by cell loss with gliosis and oligodendroglial cytoplasmic inclusions in the nigrostriatal, olivopontocerebellar systems and the spinal cord. Beyond nosology there has been a need for reliable clinical diagnosis criteria for MSA. Such criteria should ideally combine good sensitivity and specificity to diagnose MSA at different stages and should be good predictors (high positive predictive value) of the pathological diagnosis. Difficulties encountered in establishing MSA clinical diagnosis criteria were, among others, the variable expression of the disease, the definition of autonomic failure, of the cerebellar syndrome and of the poor levodopa response of parkinsonism. Quinn in 1989 proposed 3 sets of criteria (revised in 1994) to diagnose "possible", "probable" and "definite" MSA. These criteria are quite simple and partially validated. More recently these criteria evolved towards consensus criteria in which autonomic failure had a more central position. The consensus conference, held in 1998, proposed a precise definition of the various clinical domains and the combination needed for the clinical diagnosis, as well as exclusion criteria. In this article, we review and comment the different criteria for the clinical diagnosis of MSA.     

Video-laparoscopic diagnosis and follow-up of a peritoneal tuberculosis

A 21-year-old woman suffering from abdominal pain and a fever of 39 degrees C was hospitalized. Ultrasonography and computed tomographic scan showed a large amount of ascites and one hepatic node. The serum CA 125 level was elevated. Protein Chain Reaction (PCR) searching tuberculosis antigen in ascitic fluid was normal. A diagnosis of peritoneal tuberculosis was supposed and an exploratory laparoscopic procedure performed. Peroperative observation of the ascites, with multiple sites of adhesion, and pathological examination of the hepatic nodule and peritoneum confirmed initial diagnosis. Antituberculous treatment was given for one year. A second laparoscopic procedure was performed and found no disease remaining.     

Thrombocytopenia in high‐risk patients with antiphospholipid syndrome

Essentials

• The prevalence of thrombocytopenia in patients with antiphospholipid syndrome is not well defined.
• We studied triple positive patients with antiphospholipid syndrome and its catastrophic variant.
• Prevalence of thrombocytopenia was 6% and 100% in patients who developed the catastrophic form.
• In triple positive patients thrombocytopenia is low and platelets drop during the catastrophic form.

Summary

Background

Thrombocytopenia is the most common non‐criteria hematological feature in patients with antiphospholipid syndrome (APS). This condition is more common in patients with catastrophic APS (CAPS).

Objectives

To evaluate the prevalence of thrombocytopenia in a large series of high‐risk patients with APS, and to assess the behavior of the platelet count during CAPS.

Methods/Patients

This was a cross‐sectional study in which we analyzed the platelet counts of a homogeneous group of high‐risk APS patients (triple‐positive). Six of these patients developed a catastrophic phase of the disease, and the platelet count was recorded before the acute phase, during the acute phase, and at recovery.

Results

The mean platelet count in 119 high‐risk triple‐positive patients was 210 × 10⁹ L^?1. With a cut‐off value for thrombocytopenia of 100 × 10⁹ L^?1, the prevalence of thrombocytopenia was 6% (seven patients). No difference between primary APS and secondary APS was found. In patients who suffered from CAPS, a significant decrease from the basal count (212 ± 51 × 10⁹ L^?1) to that at the time of diagnosis (60 ± 33 × 10⁹ L^?1) was observed. The platelet count became normal again at the time of complete remission (220 ± 57 × 10⁹ L^?1). A decrease in platelet count always preceded the full clinical picture.

Conclusions

This study shows that, in high‐risk APS patients, the prevalence of thrombocytopenia is low. A decrease in platelet count was observed in all of the patients who developed the catastrophic form of the disease. A decrease in platelet count in high‐risk APS patients should be considered a warning signal for disease progression to CAPS.

     

Expanding the clinical spectrum of STIP1 homology and U-box containing protein 1-associated ataxia

Journal of Neurology - STUB1 has been first associated with autosomal recessive (SCAR16, MIM# 615768) and later with dominant forms of ataxia (SCA48, MIM# 618093). Pathogenic variations in STUB1...     

Granulo-monocyto apheresis is more effective in mild ulcerative colitis than in moderate to severe disease

AIM:To evaluate whether the effectiveness of Granulomonocyto apheresis(GMA),a technique that consists of the extracorporeal removal of granulocytes and monocytes from the peripheral blood,might vary according to the severity of ulcerative colitis(UC)in patients with mild to moderate-severe disease UC activity.METHODS:We retrospectively reviewed prospectively collected data of patients undergoing GMA at our inflammatory bowel disease centre who had at least a 6 mo of follow-up.The demographics,clinical and laboratory data were extracted from the patients’charts and electronic records.The severity of UC was scored according to the Modified Truelove Witts Severity Index(MTWSI).A clinical response was defined as a decrease from baseline of≥2 points or a value of MTWSI≤2 points.RESULTS:A total of 41(24 males/17 females;meanage 47 years)patients were included in the study.After GMA cycle completion,21/28(75%)of mild UC patients showed a clinical response compared with 7/13(54%)of patients with moderate to severe disease(P=0.27).At 6-mo,14/28(50%)of the mild UC patients maintained a clinical response compared with 2/13(15%)of the patients with moderate to severe disease(P=0.04).After the GMA cycle completion and during the 6-mo follow up period,13/16(81%)and 9/16(56%)of mild UC patients with intolerance,resistance and contraindications to immunosuppressants and/or biologics showed a clinical response compared with 2/6(33%)and 0/6(0%)of patients with moderate to severe disease activity with these characteristics(P=0.05and P=0.04,respectively).CONCLUSION:Patients with mild UC benefit from GMA more than patients with moderate to severe disease in the short-term period.GMA should be considered a valid therapeutic option in cases of contraindications to immunosuppressants,corticosteroids and/or biologics.