Disease modeling in functional movement disorders

Introduction: The mechanisms underlying functional movement disorders are poorly known. We examined whether experience of a movement disorder model in the family and/or the friendships contributes to functional movement disorders. Methods: The hypothesis was tested in a caseecontrol study including 33 patients with functional movement disorders and 66 age- and sex-matched patients with organic movement disorders and using a conditional logistic multivariable analysis (adjusted by age, education, disease duration, chronic medical illnesses and clinical phenotype). Results: Case-control comparison yielded a significant association between functional movement dis- orders and exposure to phenotypically congruent movement disorder models (Odds ratio, 3.9, p 1⁄4 0.01), mainly when disease model came from friendships (Odds ratio, 5.9, p 1⁄4 0.04). By contrast no association was found between functional movement disorders and phenotypically different neurological or non neurological disease models. A significant inverse relationship between exposure to a phenotypically concordant movement disorder model and age of disease onset was also observed. Conclusions: These findings support disease modeling as a factor contributing to the phenomenology of functional movement disorders

Quality of life in patients with craniocervical dystonia: Italian validation of the "Cervical Dystonia Impact Profile (CDIP-58)" and the "Craniocervical Dystonia Questionnaire (CDQ-24)"

Dystonia is a disabling and disfiguring disorder that can often affect many aspects of patients' daily lives, and lower their self-esteem. To date, quality of life (QoL) has been assessed in dystonic patients using generic measures that do not address the specific problems of this diagnostic group. Recently, two disease-specific scales "The Cervical Dystonia Impact Profile (CDIP-58)" and the "Craniocervical Dystonia Questionnaire (CDQ-24)" were validated for measuring QoL in craniocervical dystonia patients. No disease-specific scales for QoL for dystonic patients are currently available in Italian. The aim of our study was to produce and validate the Italian version of the CDIP-58 and CDQ-24. We obtained the Italian version of CDQ-24 and CDIP-58 with a back-translation design. Both scales were applied to a population of 94 craniocervical dystonia patients along with the Short Form 36 health-survey questionnaire (SF-36), both before and 4 weeks after botulinum toxin therapy. A group of 65 controls matched for sex, age and comorbidity underwent the SF-36. Internal consistency was satisfactory for all subscales. Both the CDIP-58 and CDQ-24 showed moderate to high correlations with similar items of the SF-36. Sensitivity to change was confirmed by highly significant improvements in all CDQ-24 subscales and by moderate improvements in three out of eight CDIP-58 subscales and total score. This is the first Italian study on QoL in dystonia patients. We validated the Italian version of two disease-specific questionnaires to evaluate QoL in craniocervical dystonia patients. These scales could be useful for both clinical practice and clinical trials

Ability of goat milk to modulate healthy human peripheral blood lymphomonocyte and polymorphonuclear cell function: in vitro effects and clinical implications

The in vitro effects of goat’s milk from different sources (Jonica, Saanen, and Priska breeds plus a commercial preparation) on healthy human peripheral blood mononuclear cells (PBMCs) were evaluated in terms of nitric oxide (NO) and cytokine release. According to the incubation time (24 h or 48 h) used all milks could induce release of NO from monocytes. In this context, however, in the presence of a commercial milk preparation inhibition of lypopolysaccharide (LPS)-induce NO generation was evident. Also polymorphonuclear cells stimulated with the various milks released detectable amounts of NO. In the case of Priska milk inhibition of LPS-mediated NO generation was observed. Despite a broad array of cytokines tested [Interleukin (IL)-4, IL-5, IL-6, IL-10, IL-12, IL-13, IL-17, Tumor Necrosis Factor (TNF)-, Transforming Growth Factor– and Granulocyte Colony Stimulating Factor] only IL-10, TNF-, and IL-6 were released by PBMCs upon stimulation with various milks. Taken together, these data indicate that goat’s milk for its capacity to produce NO may exert a cardioprotective and anti-atherogenic effect in consumers. Moreover, induction of proinflammatory (TNF- and IL-6) and anti-inflammatory (IL-10) cytokines suggests the ability of this milk to maintain immune homeostasis in the immunocompromised host (e.g., aged people)

Lemierre's syndrome complicated by cerebral venous sinus thrombosis: A life threatening and rare disease successfully treated with empiric antimicrobial therapy and conservative approach

Lemierre's syndrome (LS) is a "forgotten" condition characterized by septic thrombophlebitis of the jugular vein that follows an otolaryngological infection. Fusobacterium necrophorum is the aetiological agent responsible for the syndrome in adolescents and young adults whereas in older people even common bacteria are involved. Complications arise from spreading of septic emboli distally, i.e. to the brain, lungs, bones and internal organs everywhere in the body. We report a middle-aged woman who presented with headache and bilateral sixth cranial nerve palsy following a sphenoidal sinusitis and left mastoiditis. Imaging revealed thrombotic involvement of the left internal jugular vein as well as of several cerebral venous sinuses thrombosis (CVT). Currently, precise management protocols of LS with CVT complication do not exist although a combination of macrolides and second or third-generation cephalosporins, as well as anti-coagulants represent the mainstream of therapeutics. Surgical drainage is associated to remove septic foci but is burdened by severe complications and side effects. Complete recovery was achieved following pharmacological treatment in our patient. This report adds further evidence that LS complicated by CVT may be effectively treated adopting a conservative approach thus avoiding surgical drainage and severe complications

Quality of life in patients with craniocervical dystonia: Italian validation of the “Cervical Dystonia Impact Profile (CDIP-58)” and the “Craniocervical Dystonia Questionnaire (CDQ-24)”

Dystonia is a disabling and disfiguring disorder that can often affect many aspects of patients’ daily lives, and lower their self-esteem. To date, quality of life (QoL) has been assessed in dystonic patients using generic measures that do not address the specific problems of this diagnostic group. Recently, two disease-specific scales ‘‘The Cervical Dystonia Impact Profile (CDIP-58)’’ and the ‘‘Craniocervical Dystonia Questionnaire (CDQ-24)’’ were validated for measuring QoL in craniocervical dystonia patients. No disease- specific scales for QoL for dystonic patients are currently available in Italian. The aim of our study was to produce and validate the Italian version of the CDIP-58 and CDQ-24. We obtained the Italian version of CDQ-24 and CDIP-58 with a back-translation design. Both scales were applied to a population of 94 craniocervical dystonia patients along with the Short Form 36 health-survey questionnaire (SF-36), both before and 4 weeks after botulinum toxin therapy. A group of 65 controls matched for sex, age and comorbidity underwent the SF-36. Internal consistency was satisfactory for all subscales. Both the CDIP-58 and CDQ-24 showed moderate to high correlations with similar items of the SF-36. Sensitivity to change was confirmed by highly significant improvements in all CDQ-24 subscales and by moderate improvements in three out of eight CDIP-58 subscales and total score. This is the first Italian study on QoL in dystonia patients. We validated the Italian version of two disease-specific questionnaires to evaluate QoL in craniocervical dystonia patients. These scales could be useful for both clinical practice and clinical trials