Autoimmune chronic active hepatitis in Down''s syndrome

Hashimoto's thyroiditis, autoimmune adrenalitis, pernicious anaemia, and diabetes mellitus are all recognised associations with Down's syndrome. In addition chronic active hepatitis (CAH) resulting from chronic hepatitis B antigenaemia is known to occur in these patients, but an association of autoimmune CAH and Down's syndrome has not previously been described. We report a case in which Down's syndrome was associated with autoimmune CAH, Hashimoto's thyroiditis, and alopecia areata.     

Rapid diagnosis of rotavirus gastroenteritis by a commercial latex agglutination test.

The Rotalex test, a commercial latex agglutination test for rotavirus, was compared with direct electron microscopy (EM) and the Rotazyme test I, a commercial enzyme immunoassay, for detection of rotavirus in stools of children and neonates. For initial stool specimens from 265 children (less than 3 years old) with diarrhea, the Rotalex test had a sensitivity of 81.7% and specificity of 99.5% compared with EM results. Positive and negative predictive values were 98 and 94.9%, respectively. The Rotalex test was slightly more sensitive and specific than the Rotazyme test. When daily stool specimens from patients with rotavirus gastroenteritis were examined, the sensitivity of the Rotalex test varied depending on the time of stool collection relative to the onset of symptoms. Sensitivity was 100 (20/20), 96 (23/24), and 54% (7/13) during 1 to 4, 5 to 7, and 8 to 18 days, respectively, after the onset of symptoms. The sensitivity of the Rotazyme test varied similarly with days from onset. We also examined 214 EM-negative stool specimens from asymptomatic newborns. False positivity by the Rotalex test was only 3.3% (7/214) compared with 4.2% (9/215) for the Rotazyme test. The Rotalex test was as sensitive and specific as EM for detection of rotavirus during the acute stage of illness and much faster and cheaper than EM or the Rotazyme test. The test appears to be suitable for routine use in small hospitals, emergency wards, or even the physician's office for rapid diagnosis of rotavirus gastroenteritis.     

Prospective validation of consensus criteria for the diagnosis of dementia with Lewy bodies

OBJECTIVE: To determine the validity of a clinical diagnosis of probable or possible dementia with Lewy bodies (DLB) made using International Consensus criteria. BACKGROUND: Validation studies based on retrospective chart reviews of autopsy-confirmed cases have suggested that diagnostic specificity for DLB is acceptable but case detection rates as low as 0.22 have been suggested. METHODS: We evaluated the first 50 cases reaching neuropathologic autopsy in a cohort to which Consensus clinical diagnostic criteria for DLB, National Institute for Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria for AD, and National Institute of Neurological Disorders and Stroke-Association Internationale pour la Recherche et l'Enseignement en Neurosciences criteria for vascular dementia (VaD) had been prospectively applied. RESULTS: Twenty-six clinical diagnoses of DLB, 19 of AD, and 5 of VaD were made. At autopsy, 29 DLB cases, 15 AD, 5 VaD, and 1 progressive supranuclear palsy were identified. The sensitivity and specificity of a clinical diagnosis of probable DLB in this sample were 0.83 and 0.95. Of the five cases receiving a false-negative diagnosis of DLB, significant fluctuation was present in four but visual hallucinations and spontaneous motor features of parkinsonism were generally absent. Thirty-one percent of the DLB cases had additional vascular pathology and in two cases this contributed to a misdiagnosis of VaD. No correlations were found between the distribution of Lewy bodies and clinical features. CONCLUSION: The Consensus criteria for DLB performed as well in this prospective study as those for AD and VaD, with a diagnostic sensitivity substantially higher than that reported by previous retrospective studies. DLB occurs in the absence of extrapyramidal features and in the presence of comorbid cerebrovascular disease. Fluctuation is an important diagnostic indicator, reliable measures of which need to be developed further.     

One year follow-up of parkinsonism in dementia with Lewy bodies

The progression of parkinsonism over 1 year was evaluated in a prospective cohort of patients (n = 338), suffering from dementia with Lewy bodies (DLB), Alzheimer's disease (AD) or vascular dementia (VaD). Parkinsonism was assessed using the modified Unified Parkinson's Disease Rating Scale. Significant parkinsonism was significantly commoner in DLB sufferers (71%) than amongst patients with AD (7%) or VaD (10%). DLB patients with established parkinsonism had an annual increase in severity of 9%, but progression was more rapid (49% in 1 year) in patients with early parkinsonism. Parkinsonism was frequent at all severities in DLB patients, but usually only present in other dementias when MMSE <10.     

Neuropathological substrates of dementia and depression in vascular dementia, with a particular focus on cases with small infarct volumes

The neuropathological substrates of dementia and depression were evaluated in 30 patients with cerebrovascular disease and significant cognitive impairment (VaD), with a particular focus on patients with small infarct volumes (<15 ml). VaD patients with small infarct volumes had a similar degree of cognitive impairment to those with larger infarct volumes (>15 ml) but were significantly more likely to be depressed and to have areas of microinfarction. A review of individual cases with small infarct volumes suggested that the combination of microinfarction, diffuse white matter disease and perivascular changes, or the overlap of neurodegenerative pathologies and microvascular changes were particularly important. Microinfarction was also significantly associated with major depression.     

Comment on “The effect of omalizumab on hematological and inflammatory parameters in patients with chronic spontaneous urticarial”: reply from authors

Abstract

We read Cosansu’s commentary entitled “Effectiveness of the new inflammatory parameters in patients with chronic spontaneous urticarial” to our study with great interest. The author remarked that a limited number of patients had C-reactive protein levels and it was not specified whether there were any other drugs used by the patients and no information was given about the severity of the disease in our study.     

Analysis of persistent geotropic and apogeotropic positional nystagmus of the lateral canal benign paroxysmal positional vertigo

Objective:This study aims to analyze the clinical characteristics of persistent geotropic and apogeotropic positional nystagmus of LC-BPPV in view of light and ...     

Hepatic involvement of disseminated actinomycosis

A 35-year-old woman was admitted for weight loss, fatigue, and epigastric and back pain. She had undergone uterine myomectomy 6 weeks previously, and light microscopic examination revealed actinomycosis. Radiologic examination showed 5×4×4 cm and 2×2×2 cm solid lesions in the left liver lobe. Ultrasound-guided fine-needle aspiration biopsy could not exclude the possibility of malignancy. We decided to perform an explorative laparotomy. Frozen sections indicatedEchinococcus alveolaris infection or granulomatous inflammatory disease, and left hepatic lobectomy was performed. Histologic examination revealed hepatic actinomycosis. After 6 months' penicillin treatment, there was no sign of recurrence. This case of hepatic actinomycosis following uterine infection without gross involvement of any other abdominal organ raises questions on the dissemination pathway. The other interesting feature of this case is the short interval from the presumed initiatory event to the presence of the hepatic deposits.