Serum autoantibodies do not differentiate PANDAS and Tourette syndrome from controls.
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Animals↗
Biomedical subjects
D Martino
Publications and source records attributed to D Martino.
Pain and motor complications in Parkinson's disease.
AIMS: To study the association of pain with motor complications in 117 patients with Parkinson's disease. METHODS: Patients were asked to refer any pain they experienced at the time of study and lasting since at least 2 months. Basic parkinsonian signs and motor complications (including motor fluctuations and dyskinesia) were assessed and Unified Parkinson's Disease Rating Scale (UPDRS) motor score part III (during on) and part IV were calculated. Information on age, sex, duration of disease, use of dopamine agonists and levodopa, years of levodopa treatment and current levodopa dosage, medical conditions possibly associated with pain, and depression were collected. Single and multiple explanatory variable logistic regression models were used to check the association of pain with the investigated variables. RESULTS: Pain was described by 47 patients (40%) and could be classified into dystonic (n.19) and non dystonic pain (n.16); in 12 patients both types coexisted. Multiple explanatory variable logistic regression models indicated a significant association of pain with motor complications (adjusted OR, 5.7; 95% CI, 2 to 16.5; p = 0.001). No association was found between pain, dystonic or non dystonic, and the other investigated variables including medical conditions known to be associated to pain in the general population. There was a significant correlation (r = 0.31, p<0.05) between severity of pain (measured on a Visual Analogue Scale) and severity of motor complications (UPDRS part IV). CONCLUSIONS: Pain may be a representative feature of Parkinson's disease frequently associated with motor complications. The association is independent of a number of potentially relevant demographic and clinical variables.
Aged↗
A family study on primary blepharospasm.
BACKGROUND: Previous family studies provided evidence that blepharospasm (BSP) can aggregate in families but did not give accurate and reliable information on the characteristics and degree of familial clustering. AIM: To evaluate the proportion of familial and non-familial BSP cases, the clinical expression of dystonia within families, the inheritance pattern, and the extent of penetrance. METHODS: The study was based on the examination of the first degree relatives of 56 probands with primary BSP. RESULTS: The 56 families produced a potential population of 436 first degree relatives of whom 296 were alive and 233 were examined. The proportion of index patients with at least one first degree relative affected by BSP, or adult onset dystonia other than BSP, was 27%. There was a remarkable degree of phenotypic variability of dystonia within families. Similar segregation ratios were calculated for probands' siblings and children. Under the assumption of autosomal dominant transmission of adult onset dystonia, penetrance was about 20%. CONCLUSIONS: The findings of this family study are relevant for accurately counselling the families of patients with BSP and may help identify the most appropriate study design to explore genetic susceptibility in BSP.
Adolescent↗
Soluble adhesion molecules in Gilles de la Tourette's syndrome.
To investigate the immune-mediated response in TS, and its relationship with streptococcal infection, we measured serum levels of soluble intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1) and E-selectin in patients with TS, compared to healthy and diseased controls. Soluble VCAM-1 and sE-selectin were significantly elevated in children and adults with TS, and sVCAM-1 was higher among anti-basal ganglia antibodies (ABGA)-positive adults with TS. No correlation of adhesion molecule levels to clinical severity or anti-streptococcal antibodies was observed. Children with Sydenham's chorea and paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) showed an increased level of sICAM-1, but not sVCAM-1 and sE-selectin. These results provide initial evidence for a role of adhesion molecules and systemic inflammation in TS, and support the hypothesis of an ongoing immune-mediated process in this condition.
Adolescent↗
Tracing Sydenham's chorea: historical documents from a British paediatric hospital.
Sydenham's chorea (SC) became a well defined nosological entity only during the second half of the nineteenth century. Such progress was promoted by the availability of large clinical series provided by newly founded paediatric hospitals. This paper analyses the demographic and clinical features of patients with chorea admitted to the first British paediatric hospital (the Hospital for Sick Children, Great Ormond Street, London) between 1852 and 1936. The seasonal and demographic characteristics of SC during this time appear strikingly similar to those observed today, and witness the introduction of modern "statistically averaging" techniques in the approach to complex paediatric syndromes. Great Ormond Street (GOS) hospital case notes provide detailed descriptions of the "typical cases" of SC, and show that British physicians working in the early age of paediatric hospitals succeeded in recognising the most distinctive clinical features of this fascinating condition.
Child↗
Anti-basal ganglia antibodies in patients with atypical dystonia and tics: a prospective study.
Anti-basal ganglia antibodies (ABGA) are associated with movement disorders in children, but have not been assessed in adult onset movement disorders. In a prospective assessment ABGA were positive in 65% of a group of 65 patients with atypical movement disorders, but were very rare in healthy adults and adults with idiopathic dystonia. An autoimmune mechanism may underlie a proportion of cases of atypical movement disorders.
Adolescent↗
Organochlorine compounds and heavy metals in the soft tissue of the mussel Mytilus galloprovincialis collected from Lake Faro (Sicily, Italy).
Three hundred samples of Mytilus galloprovincialis were collected from five stations (north, south, east, west and centre) of Lake Faro to evaluate the concentrations of organochlorine compounds and heavy metals. Quantitative determinations of organochlorine pesticides and PCBs were made by GC-ECD and confirmed with GC-MS. Concentrations of "essential" (Cu, Se and Zn) and "toxic" (As, Cd, Hg and Pb) metals were determined by an atomic absorption spectrophotometer. The results obtained show the low residue levels of p,p'-DDE in six samples of M. galloprovincialis from southern (7.00-11.00 ng/g w.w. and 148.3-275 ng/g l.w.) and western (7.60-15.37 ng/g w.w. and 126.7-256.2 ng/g l.w.) areas of Lake Faro. No appreciable residues of PCBs were found in any of the samples examined. Zn concentrations (range 11.0-18.5 microg/g w.w.) were higher than Cu (range 188.3-396.0 ng/g w.w.) and Se (range 93.5-288.9 ng/g w.w.) in all areas of origin. Cd (range 41.9-63.8 ng/g w.w.), Pb (range 64.8-93.0 ng/g w.w.) and Hg levels (range 5.7-13.1 ng/g w.w.) showed lower concentrations than permitted MRLs. The As levels were below detection limits for the all mussel samples. In conclusion, the absence of PCBs, the low levels of p,p'-DDE, the concentrations of Cd, Hg and Pb below permitted MRLs in M. galloprovincialis, used as a "biological indicator", show that Lake Faro is not at contamination risk from these contaminants and moreover is free from health problems for the consumer of mussel products.
Animals↗
Levels of "toxic" and "essential" metals in samples of bovine milk from various dairy farms in Calabria, Italy.
The aim of this research was to evaluate the possible contamination by heavy metals of milk from cows bred on various farms in Calabria. The concentrations of heavy metals were determined in 40 samples of bovine milk from the various dairy farms. Each sample, homogenized and powdered, was mineralized in a microwave oven. Quantitative analyses of Cd, Cr, Cu, Pb and Se were performed using an atomic absorption spectrophotometer with graphite furnace; As was analyzed by hot vapor generation technique and Zn with the flame method. As regards toxic heavy metals, highest values are those of As (g.m. 37.90 microg/kg w.w.) and Pb (g.m. 1.32 microg/kg w.w.), while lowest concentrations are those of Cd (g.m. 0.02 microg/kg w.w). With regard to essential metals, Zn (g.m. 2016 microg/kg w.w) and Se (g.m. 13.24 microg/kg w.w.) showed the highest concentrations, followed by Cr (g.m. 2.03 microg/kg w.w.) and Cu (g.m. 1.98 microg/kg w.w.). Further investigations of the levels of heavy metals (As) in a greater number of milk samples from various zones of Calabria are necessary, both to examine this problem from the clinical epidemiological point of view and to identify the possible causes of milk contamination.
Animals↗
Primary cervical dystonia and scoliosis: a multicenter case-control study.
OBJECTIVE: To study the distribution of prior scoliosis among patients with primary adult-onset cervical dystonia (CD) and matched control subjects. METHODS: Case and control subjects were selected among consecutive outpatients attending four Italian centers. Control outpatients were matched for age (+/-5 years), sex, and referral center. Information on prior scoliosis, other spine diseases, and family history of dystonia was obtained by a standardized questionnaire and supported by medical records. Conditional logistic regression models were used to adjust simultaneously for age, disease duration, and education level and to determine the independent association of exposure variables with the outcome. RESULTS: Prior scoliosis developing in middle or late childhood or at around the puberty occurred more frequently among 72 case patients than among 144 neurologic control subjects. No subject reported conditions considered to be responsible for secondary scoliosis. The association of scoliosis and CD was not confounded by age, duration of disease, education level, other spine diseases, or family history of dystonia (adjusted odds ratio [OR] 6.8; 95% CI 1.5 to 29.5; p = 0.011). The OR of family history of dystonia (18.7; 95% CI 2.4 to 147.5; p = 0.005) fell to 11.7 (95% CI 1.3 to 103; p = 0.03) after controlling for scoliosis. CONCLUSIONS: Prior scoliosis may increase the risk of developing CD. The observed decrease in the magnitude of the association between family history of dystonia and CD after controlling for scoliosis suggests a link between the two conditions.
Adolescent↗
Frequency of familial aggregation in primary adult-onset cranial cervical dystonia.
Family history of definite/probable dystonia was studied in 36 probands with primary adult-onset cranial/cervical dystonia. Of the 157 relatives who were examined, 8 from 7 families and 11 from 10 families were diagnosed as having definite or probable dystonia, respectively. The frequency of familial occurrence of definite dystonia was 19.4%, 33% when considering both definite and probable dystonia. There was a tendency for relatives affected by either definite or probable dystonia to have the same type of dystonia as the index patient. Similar segregation ratios were found for parents, siblings, and children with either definite or probable dystonia. These observations raise the possibility that probable dystonia represents formes frustes/mild phenotypes of dystonia rather than another movement disorder.
Adult↗
Does sex influence age at onset in cranial-cervical and upper limb dystonia?
The relation between age at dystonia onset and sex was investigated in 264 patients with cranial-cervical dystonia and 56 patients with upper limb dystonia. In cranial-cervical dystonia, women had a significantly greater age at the onset of dystonia than men. The association was independent of duration of disease and distance of referral, but it was no longer detectable after adjustment for educational level. In upper limb dystonia, men and women did not differ for age at dystonia onset, duration of disease, education level, or distance of referral. A significant inverse association between age at the onset of dystonia and education was observed in both cranial-cervical dystonia and upper limb dystonia series.
Adult↗
Agreement among neurologists on the clinical diagnosis of dystonia at different body sites.
OBJECTIVE: To study the reliability of the diagnosis of blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp among neurologists. METHODS: 12 patients with adult onset focal segmental dystonia were videotaped in a standardised way. The tape was sent to six neurologists who are involved in clinical practice without a specific interest in movement disorders (general neurologists), and to four neurologists expert in movement disorders. The observers had to recognise whether the patients were affected by dystonia and to distinguish among blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp. Interobserver reliability was assessed by kappa statistics, and the degree of agreement was classified according to the Landis classification. RESULTS: The 10 neurologists reached slight to moderate agreement on the diagnosis of these four disorders. When the observers were subdivided according with their professional experience in the field, a moderate to perfect agreement on the diagnosis was achieved by specialists in movement disorders, and a fair to moderate agreement by the general neurologists. CONCLUSIONS: Neurologists may have different ability to recognise adult onset focal dystonia, depending on their experience and on the type of dystonia.
Adult↗
Influence of age on the association between primary hemifacial spasm and arterial hypertension.
OBJECTIVE: To investigate the association between primary hemifacial spasm and arterial hypertension. SUBJECTS: 114 patients with primary hemifacial spasm and 228 neurological controls, matched for age, sex, and referral centre, were recruited during an 18 month period from consecutive outpatients attending two neurological institutions. DESIGN: The association between exposure variables and case/control status was examined in conditional logistic regression models, adjusting simultaneously for disease duration and education level. RESULTS: Hypertension was more common among the patients with primary hemifacial spasm than among the controls. The association was independent of age, disease duration, years of schooling, and other diseases (adjusted odds ratio (OR) 2.76 (95% confidence interval (CI), 1.43 to 5.33); p = 0.002). Hypertension was associated with hemifacial spasm in both the left sided group (adjusted OR, 2.76 (1.18 to 6.44); p = 0.02) and the right sided group (adjusted OR, 3.02 (1.13 to 8.1); p = 0.03). The association of hypertension with hemifacial spasm was apparently greater in the age group < 60 years (adjusted OR, 4.2 (1.4 to 12); p = 0.008) than in the age group >/= 60 years (adjusted OR, 2.5 (CI 1.3 to 4.6); p = 0.005), but the difference in the OR estimates between the two age groups was not significant. Among hypertensive patients, mean age at the diagnosis of hypertension was significantly lower than mean age at the onset of hemifacial spasm in the age group >/= 60 years, but not significantly different in the age group < 60 years. CONCLUSIONS: The findings support the hypothesised association of primary hemifacial spasm with hypertension and raise the possibility that a different mechanism underlies the association in different age groups.
Adolescent↗
Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy.
The authors ascertained the prevalence of primary blepharospasm (BSP) in a community located in Puglia, a region in Southern Italy, by focusing on neurologists and ophthalmologists. The crude prevalence rates were 133 per million (95% CI, 61--153) for both focal and segmental BSP, 74 per million (95% CI, 24--173) for focal BSP, and 59 per million (95% CI, 16--151) for segmental BSP. Prevalence rate increased with age. Apraxia of eyelid opening and BSP coexisted in one third of cases.
Adult↗
Interferon beta-1a downregulates TNFalpha-induced intercellular adhesion molecule 1 expression on brain microvascular endothelial cells through a tyrosine kinase-dependent pathway.
TNFalpha (100 U/ml, 24 h) upregulated intercellular adhesion molecule 1 (ICAM1) expression on brain microvascular endothelial cell (BMEC) culture. The tyrosine kinase (TK) inhibitor genestein (100 microgram/ml), the protein kinase C (PKC) inhibitor staurosporin (1 nM), and interferon (IF) beta-1a (1000 U/ml) antagonized TNFalpha effect. When an ineffective dose of IFbeta-1a (100 U/ml) was challenged with ineffective doses of either genestein (10 microgram/ml) or staurosporin (0.1 nM), the combination IFbeta-1a-genestein significantly reduced TNFalpha-induced ICAM1 expression whereas IFbeta-1a-staurosporin did not. These findings indicate that a TK- rather than a PKC-dependent mechanism is involved in the modulation of TNFalpha response by IFbeta-1a on BMECs.
Adjuvants, Immunologic↗
Inhibition of protein kinase C counteracts TNFalpha-induced intercellular adhesion molecule 1 expression and fluid phase endocytosis on brain microvascular endothelial cells.
TNFalpha (100 U/ml, 24 h) upregulated intercellular adhesion molecule 1 (ICAM1) expression and fluid phase endocytosis (FPE) of horseradish peroxidase on brain microvascular endothelial cell (BMEC) culture. The protein kinase C (PKC) inhibitor staurosporin (0. 5-10 nM) antagonized ICAM1 expression and FPE due to TNFalpha, whereas the protein kinase A inhibitor H89 (0.5-10 nM) did not. These findings indicate that a PKC-dependent mechanism may affect TNFalpha signalling on different barrier properties of BMECs.
Animals↗
Sulfur Cycling in the Terrestrial Subsurface: Commensal Interactions, Spatial Scales, and Microbial Heterogeneity
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Journal Article↗
[Echo-guided percutaneous treatment of renal cysts: aspiration vs continuous 24-hour drainage].
The results of 2 treatment options, percutaneous aspiration vs percutaneous aspiration and continuous drainage over 24 hours, in the management of simple renal cyst were compared. Thirteen patients were managed with aspiration alone (group 1) while 19 with aspiration and continuous drainage (group 2). Recurrence rate was 100% in group 1 and 73% in group 2 (p: n.s.). Therefore, we believe that the higher cost of continuous drainage are not justified.
Adult↗