Validation of the classification criteria for cryoglobulinaemic vasculitis.

OBJECTIVE: The aim of this study was to validate the classification criteria for cryoglobulinaemic vasculitis (CV). METHODS: Twenty-three centres were involved. New patients with CV (group A) and controls, i.e. subjects with serum cryoglobulins but lacking CV based on the gold standard of clinical judgment (group B) and subjects without cryoglobulins but with clinical features that can be observed in the course of CV (group C), were studied. Positivity of serum cryoglobulins was necessary for CV classification. Sensitivity and specificity of the criteria were calculated by comparing group A vs group B. The group A vs group C comparison was done to demonstrate the possible diagnostic utility of the criteria. RESULTS: The study included 268 patients in group A, 182 controls in group B and 193 controls in group C (small vessel vasculitis, 51.8%). The questionnaire (at least 2/3 positive answers) showed 89.0% sensitivity and 93.4% specificity; the clinical item (at least 3/4 clinical involvement) showed 75.7% sensitivity and 89.0% specificity and the laboratory item (at least 2/3 laboratory data) showed 80.2% sensitivity and 62.4% specificity. The sensitivity and specificity of the classification criteria (at least 2/3 positive items) were 89.9% and 93.5%, respectively. The comparison of group A with group C demonstrated the clinical utility of the criteria in differentiating CV from CV mimickers. CONCLUSION: Classification criteria for CV were validated in a second, large, international study confirming good sensitivity and specificity in a complex systemic disease.

Dietary and lifestyle variables in relation to incidence of Parkinson's disease in Greece.

Identification of dietary and lifestyle variables associated with the development of Parkinson's disease (PD) may offer pathogenetic clues and prevention opportunities. In a population-based prospective cohort study, 26,173 participants in the EPIC-Greece cohort had sociodemographic, anthropometric, medical, dietary and lifestyle variables ascertained at enrolment and periodically reassessed with follow-up contacts. Based on these data, subjects were screened as possible PD cases if they (1) reported either a medical diagnosis of PD or use of anti-PD drugs and (2) did not report preceding causes of secondary parkinsonism. For diagnostic validation, possible incident PD cases were assessed by a focused 3-item telephone questionnaire. Cox proportional hazards regression was used to evaluate associations between potential predictors and incident PD. The main multivariate model included gender, age, marital status, schooling years, farming occupation, smoking status, caffeinated coffee, body mass index, physical activity and energy intake. Additional models included all above variables plus one dietary item at a time. Incidence rate adjusted to the European population was 16.9 per 100,000 person-years. In multivariate models, incident PD exhibited strong positive association with consumption of milk, but not cheese or yoghurt. This finding may help narrow down the search for potential dairy product components with a facilitatory role in PD. Concerning other dietary components, inverse association was found between polyunsaturated fat intake and incident PD. Also, inverse association was found with tobacco smoking, in line with previous studies, but not with caffeine.

Differences in EEG delta frequency characteristics and patterns in slow-wave sleep between dementia patients and controls: a pilot study.

PURPOSE: To evaluate the modifications of EEG activity during slow-wave sleep in patients with dementia compared with healthy elderly subjects, using spectral analysis and period-amplitude analysis. METHODS: Five patients with dementia and 5 elderly control subjects underwent night polysomnographic recordings. For each of the first three nonrapid eye movement-rapid eye movement sleep cycles, a well-defined slow-wave sleep portion was chosen. The delta frequency band (0.4-3.6 Hz) in these portions was analyzed with both spectral analysis and period-amplitude analysis. RESULTS: Spectral analysis showed an increase in the delta band power in the dementia group, with a decrease across the night observed only in the control group. For the dementia group, period-amplitude analysis showed a decrease in well-defined delta waves of frequency lower than 1.6 Hz and an increase in such waves of frequency higher than 2 Hz, in incidence and amplitude. CONCLUSIONS: Our study showed (1) a loss of the dynamics of delta band power across the night sleep, in dementia, and (2) a different distribution of delta waves during slow-wave sleep in dementia compared with control subjects. This kind of computer-based analysis can highlight the presence of a pathologic delta activity during slow-wave sleep in dementia and may support the hypothesis of a dynamic interaction between sleep alteration and cognitive decline.

Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

INTRODUCTION: Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. REPORT OF THE CASES: We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. CONCLUSIONS: The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

Sensorineural hearing loss complicating severe rickettsial diseases: report of two cases.

Sub-acute focal neurological manifestations are reported rarely in systemic rickettsial diseases and are considered to be secondary to immune-mediated mechanisms. We present two cases of transient sensorineural hearing loss complicating the course of severe rickettsial diseases caused by Rickettsia typhi and Rickettsia conorii, respectively. The diagnosis was based on the presence of high IgM antibodies and the prompt response to doxycycline treatment. In both cases, hearing loss presented during convalescence and resolved automatically without administration of specific treatment.

Psychosis as presenting symptom in adult-onset Hallervorden-Spatz syndrome.

BACKGROUND: Hallervorden-Spatz syndrome is characterized by pyramidal and extrapyramidal signs, and dysarthria and dementia. Psychiatric symptomatology can emerge in the course of the disorder. Mutations in the pantothenate kinase 2 gene have been found in many cases. We report a case with psychosis as sole presenting symptom. CASE: A 41-year-old man presented with change in behavior and paranoid delusional ideation. Six months later, spasticity, extrapyramidal rigidity and dysarthria were added to the picture. Eventually, the patient became mute and wheel-chair bound. The brain magnetic resonance imaging (MRI) was consistent with iron depositions in the globus pallidus and substantia nigra. CONCLUSIONS: In this case, the combination of clinical and MRI findings was consistent with Hallervorden-Spatz syndrome. The combination of psychiatric and MRI findings should lead to further neurological investigation.