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.

Differences in psychopathology and behavioral characteristics of patients affected by conversion motor disorder and organic dystonia.

PURPOSE: Typically, the diagnosis of conversion motor disorder (CMD) is achieved by the exclusion of a wide range of organic illnesses rather than by applying positive criteria. New diagnostic criteria are highly needed in this scenario. The main aim of this study was to explore the use of behavioral features as an inclusion criterion for CMD, taking into account the relationship of the patients with physicians, and comparing the results with those from patients affected by organic dystonia (OD). PATIENTS AND METHODS: Patients from the outpatient Movement Disorder Service were assigned to either the CMD or the OD group based on Fahn and Williams criteria. Differences in sociodemographics, disease history, psychopathology, and degree of satisfaction about care received were assessed. Patient-neurologist agreement about the etiological nature of the disorder was also assessed using the k-statistic. A logistic regression analysis estimated the discordance status as a predictor to case/control status. RESULTS: In this study, 31 CMD and 31 OD patients were included. CMD patients showed a longer illness life span, involvement of more body regions, higher comorbidity with anxiety, depression, and borderline personality disorder, as well as higher negative opinions about physicians' delivering of proper care. Contrary to our expectations, CMD disagreement with neurologists about the etiological nature of the disorder was not statistically significant. Additional analysis showed that having at least one personality disorder was statistically associated with the discordance status. CONCLUSION: This study suggests that CMD patients show higher conflicting behavior toward physicians. Contrary to our expectations, they show awareness of their psychological needs, suggesting a possible lack of recognition of psychological distress in the neurological setting.

Personality disorders and somatization in functional and organic movement disorders.

Psychiatric disturbances and somatizations are both criteria which support the diagnosis of functional movement disorders. It is unclear, however, whether these factors are helpful in differentiating functional and organic movement disorders. To address this issue, the Structured Clinical Interview for DSM-IV Axis I and II psychiatric disorders, the State-Trait Anxiety Inventory, the Beck Depression Inventory and the "somatization section" of the Dissociative Disorders Interview Schedule were administered to 31 functional movement disorder patients diagnosed, according to Fahn and Williams criteria and 31 sex- and age-matched control outpatients, with adult-onset dystonia. Axis I psychiatric diagnoses were similarly frequent in patients with functional and organic movement disorders. There was a trend to a greater frequency of personality disorders overall; when looking at individual personality disorders, there was no significant between-group difference. Depression and anxiety scores and mean number of somatizations per patient were also greater in the functional group. The number of somatic complaints significantly correlated with depression and anxiety scores. However, the presence of these disturbances in a proportion of patients with organic dystonia indicates that personality disorders and somatizations do not aid in distinguishing functional and organic movement disorders.

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 case-control 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 disorders and exposure to phenotypically congruent movement disorder models (Odds ratio, 3.9, p = 0.01), mainly when disease model came from friendships (Odds ratio, 5.9, p = 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.