Publications by authors named "Francesco Bono"

50 Publications

Sudden Onset, Fixed Dystonia and Acute Peripheral Trauma as Diagnostic Clues for Functional Dystonia.

Mov Disord Clin Pract 2021 Oct 10;8(7):1107-1111. Epub 2021 Sep 10.

Neurology Unit, Movement Disorders Division, Department of Neurosciences, Biomedicine and Movement Sciences University of Verona Verona Italy.

Background: The differentiation of functional dystonia from idiopathic dystonia may be clinically challenging.

Objective: To identify clinical features suggestive of functional dystonia to guide physicians to distinguish functional dystonia from idiopathic dystonia.

Methods: Patient data were extracted from the Italian Registry of Functional Motor Disorders and the Italian Registry of Adult Dystonia. Patients with functional and idiopathic dystonia were followed up at the same clinical sites, and they were similar in age and sex.

Results: We identified 113 patients with functional dystonia and 125 with idiopathic dystonia. Sudden onset of dystonia, evidence of fixed dystonia, and acute peripheral trauma before dystonia onset were more frequent in the functional dystonia group. No study variable alone achieved satisfactory sensitivity and specificity, whereas a combination of variables yielded 85% sensitivity and 98% specificity. A diagnostic algorithm was developed to reduce the risk of misclassifying functional dystonia.

Conclusion: Our findings extend the current diagnostic approach to functional dystonia by showing that clinical information about symptom onset, fixed dystonia, and history of peripheral trauma may provide key clues in the diagnosis of functional dystonia.
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http://dx.doi.org/10.1002/mdc3.13322DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8485608PMC
October 2021

Functional gait disorders: Demographic and clinical correlations.

Parkinsonism Relat Disord 2021 Aug 25;91:32-36. Epub 2021 Aug 25.

Clinical Neurology Unit, Department of Medical, Surgical and Health Services, University of Trieste, Trieste, Italy.

Objective: We aimed to describe the prevalence and clinical-demographical features of patients with functional gait disorders (FGDs) and to compare them to patients with functional motor disorders (FMDs) without FGDs (No-FGDs).

Methods: In this multicenter observational study, we enrolled patients with a clinically definite diagnosis of FMDs in 25 tertiary movement disorders centers in Italy. Each subject with FMDs underwent a comprehensive clinical assessment, including screening for different subtypes of functional gait disorders. Multivariate regression models were implemented in order to estimate the adjusted odds ratio (OR; 95% confidence interval) of having FGDs in relation to sociodemographic and clinical characteristics.

Results: Out of 410 FMDs, 26.6% (n = 109) of patients exhibited FGDs. The most frequent FGDs were slow gait (n = 43, 39.4%), astasia-abasia (n = 26, 23.8%), and knee buckling (n = 24, 22%). They exhibited single FGDs in 51.4% (n = 56) or complex FGDs (more than one type of FGDs) in 48.6% (n = 53) of cases. On multivariate regression analysis, the presence of FGDs was more likely associated with older age (OR 1.03, 95% CI 1.01-1.04), functional visual symptoms (OR 2.19, 95% CI 1.08-4.45), and the diagnosis of somatic symptoms disorder (OR 2.97, 95% CI 1.08-8.17). FGDs were also more likely to undergo physiotherapy (OR 1.81, 95% CI 1.08-3.03).

Conclusions: People with FMDs may present with different and overlapping types of FGDs, which may occur in older age. The association of FGDs with functional visual symptoms and somatic symptoms disorder opens up to new avenues to the understanding of the neural mechanisms of these disorders.
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http://dx.doi.org/10.1016/j.parkreldis.2021.08.012DOI Listing
August 2021

Long-term (48 weeks) effectiveness, safety, and tolerability of erenumab in the prevention of high-frequency episodic and chronic migraine in a real world: Results of the EARLY 2 study.

Headache 2021 Oct 26;61(9):1351-1363. Epub 2021 Jul 26.

Headache and Neurosonology Unit, Policlinico Universitario Campus Bio-Medico, Rome, Italy.

Objective: To evaluate the long-term effectiveness, safety, and tolerability of erenumab in a real-world migraine population, looking for putative predictors of responsiveness.

Background: Erenumab proved to be effective, safe, and well tolerated in the prevention of episodic migraine (EM) and chronic migraine (CM) in long-term extension studies of double-blind, placebo-controlled trials in patients with no more than two (EM) or three (CM) prior preventive treatment failures.

Methods: A 48-week, multicenter, longitudinal cohort real-life study was conducted at 15 headache centers across eight Italian regions between December 20, 2018 and July 31, 2020. We considered all consecutive patients with high-frequency episodic migraine (HFEM) or CM aged 18-65 years. Each patient was treated with erenumab 70 mg, administered monthly. The dose was switched to 140 mg in nonresponders and in responders who had become nonresponders for at least 4 weeks. Change in monthly migraine days (MMDs) or monthly headache days (MHDs) at Weeks 45-48 compared with baseline was the primary efficacy endpoint. Secondary endpoints encompassed variation in monthly analgesic intake, achievement of a ≥50%, ≥75%, or 100% reduction in migraine or headache days, and any change in the Visual Analogue Scale (VAS) and Headache Impact Test-6 scores (HIT-6) during the same time interval.

Results: A total of 242 patients with migraine received at least one dose of erenumab 70 mg and were considered for safety analysis, whereas 221 received a monthly erenumab dose for ≥48 weeks and were included in the effectiveness and safety analysis set. All patients had previously been treated unsuccessfully with ≥3 migraine-preventive medication classes. From baseline to Weeks 45-48, erenumab treatment reduced MMD by 4.3 ± 5.3 (mean ± SD) in patients with HFEM, and MHD by 12.8 ± 8.9 (mean ± SD) in subjects with CM. VAS and HIT-6 scores were decreased by 1.8 ± 1.9 (mean ± SD) and 12.3 ± 11 (mean ± SD) in HFEM, and by 3.0 ± 2.2 (mean ± SD) and 13.1 ± 11.2 (mean ± SD) in CM. Median monthly analgesic intake passed from 11.0 (interquartile range [IQR] 10.0-13.0) to 5 (IQR 2.0-8.0) in HFEM and from 20.0 (IQR 15.0-30.0) to 6.0 (IQR 3.8-10.0) in CM. The ≥50% responders were 56.1% (32/57) in HFEM and 75.6% (124/164) in CM; ≥75% responders were 31.6% (18/57) and 44.5% (73/164); and 100% responders were 8.8% (5/57) and 1.2% (2/164), respectively. At Week 48, 83.6% (137/164) of patients with CM had reverted to EM. Erenumab was safe and well tolerated. Responsiveness to erenumab was positively associated with cutaneous allodynia (OR: 5.44, 95% CI: 1.52-19.41; p = 0.009) in HFEM. In patients with CM, ≥50% responsiveness was positively associated with male sex (OR: 2.99, 95% CI: 1.03-8.7; p = 0.044) and baseline migraine frequency (OR: 1.12, 95% CI: 1.05-1.20; p = 0.001) and negatively associated with psychiatric comorbidities (OR: 0.37, 95% CI: 0.15-0.87; p = 0.023) and prior treatment failures (OR: 0.77, 95% CI: 0.64-0.92; p = 0.004).

Conclusions: Long-term (48-week) erenumab treatment provides sustained effectiveness, safety, and tolerability in real-life patients with HFEM or CM with ≥3 prior preventive treatment failures. The dose of 140 mg was required in most patients along the study and should be taken into consideration as the starting dose. Allodynia (in HFEM), male sex, and baseline migraine frequency (in CM) might represent positive responsiveness predictors. Conversely, psychiatric comorbidities and multiple prior preventive treatment failures could be negative predictors in patients with CM.
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http://dx.doi.org/10.1111/head.14194DOI Listing
October 2021

A video of best practice recommendations for making botulinum toxin therapy safe during the COVID-19 pandemic.

Neurol Sci 2021 Sep 19;42(9):3937-3938. Epub 2021 Jul 19.

Neurological Unit 1, Fondazione IRCSS, Istituto Neurologico "Carlo Besta", Milan, Italy.

The physician and patient come into close contact during botulinum toxin treatments, increasing the chances of COVID-19 infection. Therefore, it is essential to use an effective injection method that can prevent infection with SARS-CoV-2 virus. In order to minimize the risk of SARS-CoV-2 transmission during botulinum toxin treatment in the COVID-19 era, the Italian Botulinum Toxin Network study group of the Italian Society of Neurology has prepared a video of best practice recommendations on how to organize the work of a clinic performing botulinum toxin treatments.
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http://dx.doi.org/10.1007/s10072-021-05375-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8287114PMC
September 2021

Clinical features, disease progression, and use of healthcare resources in a large sample of 866 patients from 24 headache centers: A real-life perspective from the Italian chROnic migraiNe (IRON) project.

Headache 2021 06 14;61(6):936-950. Epub 2021 Jun 14.

Unit of Clinical and Molecular Epidemiology, IRCCS San Raffaele Pisana, Roma, Italy.

Objective: To develop a dedicated Italian chronic migraine (CM) database (IRON project) to overcome disease misconceptions, improve clinical administration, reduce patients' burden, and rationalize economic resource allotment.

Background: Proper CM management requires a comprehensive appraisal of its full clinical, social, and economic complexity.

Methods: In this cross-sectional study, CM patients were screened in 24 certified headache centers with face-to-face interviews. Information on sociodemographic factors, medical history, characteristics of CM, and of prior episodic migraine (EM), and healthcare resource use was gathered using a semistructured web-based questionnaire.

Results: A total of 866 CM patients were enrolled. CM started ~20 years after EM onset (age at EM onset 17.4 ± 9.1 vs. age at CM onset 35.3 ± 12.5 [mean ± SD]). CM prophylaxis, used by 430/866 (49.6%) of the patients, was often ineffective, not tolerated, and prematurely discontinued. Medications and diagnostic workup, frequently inappropriate, were mostly subsidized by the Italian national health service. CM patients with ≥25 headache days/month revealed substantial clinical differences and heavier disability and economic burden compared with those with <25 headache days/month.

Conclusions: CM is a heterogeneous headache disorder deserving more in-depth clinical characterization, sharper diagnostic criteria, and tailored treatments. CM registries are expected to improve clinical management, resulting in increased disease awareness, better healthcare resource allocation, and reduced economic burden.
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http://dx.doi.org/10.1111/head.14123DOI Listing
June 2021

Spread of segmental/multifocal idiopathic adult-onset dystonia to a third body site.

Parkinsonism Relat Disord 2021 06 12;87:70-74. Epub 2021 May 12.

IRCCS Mondino Foundation, Pavia, Italy; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

Background: Adult-onset focal dystonia can spread to involve one, or less frequently, two additional body regions. Spread of focal dystonia to a third body site is not fully characterized.

Materials And Methods: We retrospectively analyzed data from the Italian Dystonia Registry, enrolling patients with segmental/multifocal dystonia involving at least two parts of the body or more. Survival analysis estimated the relationship between dystonia features and spread to a third body part.

Results: We identified 340 patients with segmental/multifocal dystonia involving at least two body parts. Spread of dystonia to a third body site occurred in 42/241 patients (17.4%) with focal onset and 10/99 patients (10.1%) with segmental/multifocal dystonia at onset. The former had a greater tendency to spread than patients with segmental/multifocal dystonia at onset. Gender, years of schooling, comorbidity, family history of dystonia/tremor, age at dystonia onset, and disease duration could not predict spread to a third body site. Among patients with focal onset in different body parts (cranial, cervical, and upper limb regions), there was no association between site of focal dystonia onset and risk of spread to a third body site.

Discussion And Conclusion: Spread to a third body site occurs in a relative low percentage of patients with idiopathic adult-onset dystonia affecting two body parts. Regardless of the site of dystonia onset and of other demographic/clinical variables, focal onset seems to confer a greater risk of spread to a third body site in comparison to patients with segmental/multifocal dystonia at onset.
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http://dx.doi.org/10.1016/j.parkreldis.2021.04.022DOI Listing
June 2021

Functional motor phenotypes: to lump or to split?

J Neurol 2021 May 7. Epub 2021 May 7.

IRCCS Mondino Foundation, Pavia, Italy.

Introduction: Functional motor disorders (FMDs) are usually categorized according to the predominant phenomenology; however, it is unclear whether this phenotypic classification mirrors the underlying pathophysiologic mechanisms.

Objective: To compare the characteristics of patients with different FMDs phenotypes and without co-morbid neurological disorders, aiming to answer the question of whether they represent different expressions of the same disorder or reflect distinct entities.

Methods: Consecutive outpatients with a clinically definite diagnosis of FMDs were included in the Italian registry of functional motor disorders (IRFMD), a multicenter data collection platform gathering several clinical and demographic variables. To the aim of the current work, data of patients with isolated FMDs were extracted.

Results: A total of 176 patients were included: 58 with weakness, 40 with tremor, 38 with dystonia, 23 with jerks/facial FMDs, and 17 with gait disorders. Patients with tremor and gait disorders were older than the others. Patients with functional weakness had more commonly an acute onset (87.9%) than patients with tremor and gait disorders, a shorter time lag from symptoms onset and FMDs diagnosis (2.9 ± 3.5 years) than patients with dystonia, and had more frequently associated functional sensory symptoms (51.7%) than patients with tremor, dystonia and gait disorders. Patients with dystonia complained more often of associated pain (47.4%) than patients with tremor. No other differences were noted between groups in terms of other variables including associated functional neurological symptoms, psychiatric comorbidities, and predisposing or precipitating factors.

Conclusions: Our data support the evidence of a large overlap between FMD phenotypes.
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http://dx.doi.org/10.1007/s00415-021-10583-wDOI Listing
May 2021

Galcanezumab for the prevention of high frequency episodic and chronic migraine in real life in Italy: a multicenter prospective cohort study (the GARLIT study).

J Headache Pain 2021 May 3;22(1):35. Epub 2021 May 3.

Headache and Pain Unit, IRCCS San Raffaele Pisana, Rome, Italy.

Background: The clinical benefit of galcanezumab, demonstrated in randomized clinical trials (RCTs), remains to be quantified in real life. This study aimed at evaluating the effectiveness, safety and tolerability of galcanezumab in the prevention of high-frequency episodic migraine (HFEM) and chronic migraine (CM) in a real-life setting.

Methods: This multicenter prospective observational cohort study was conducted between November 2019 and January 2021 at 13 Italian headache centers. Consecutive adult HFEM and CM patients clinically eligible were enrolled and treated with galcanezumab subcutaneous injection 120 mg monthly with the first loading dose of 240 mg. The primary endpoint was the change in monthly migraine days (MMDs) in HFEM and monthly headache days (MHDs) in CM patients after 6 months of therapy (V6). Secondary endpoints were the Numerical Rating Scale (NRS), monthly painkiller intake (MPI), HIT-6 and MIDAS scores changes, ≥50% responder rates (RR), the conversion rate from CM to episodic migraine (EM) and Medication Overuse (MO) discontinuation.

Results: One hundred sixty-three patients (80.5% female, 47.1 ± 11.7 years, 79.8% CM) were included. At V6, MMDs reduced by 8 days in HFEM and MHDs by 13 days in CM patients (both p < .001). NRS, MPI, HIT-6 and MIDAS scores significantly decreased (p < .001). Ten patients (6.1%) dropped out for inefficacy and classified as non-responders. Patients with ≥50%RRs, i.e. responders, were 76.5% in the HFEM and 63.5% in the CM group at V6. Among CM patients, the V6 responders presented a lower body mass index (p = .018) and had failed a lower number of preventive treatments (p = .013) than non-responders. At V6, 77.2% of CM patients converted to EM, and 82.0% ceased MO. Adverse events, none serious, were reported in up to 10.3% of patients during evaluation times.

Conclusions: Galcanezumab in real life was safe, well tolerated and seemed more effective than in RCTs. Normal weight and a low number of failed preventives were positively associated with galcanezumab effectiveness in CM patients.

Trial Registration: ClinicalTrials.gov NCT04803513 .
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http://dx.doi.org/10.1186/s10194-021-01247-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8091153PMC
May 2021

Neuroimaging in idiopathic adult-onset focal dystonia.

Neurol Sci 2021 Jul 3;42(7):2947-2950. Epub 2021 Jan 3.

Department of Medical Science and Public Health, Institute of Neurology, University of Cagliari, Cagliari, Italy.

We aimed to study the attitude of Italian neurologists in the use of conventional MRI in patients with idiopathic adult-onset focal dystonia. Patients were included in the Italian Dystonia Registry by experts working in different Italian centers. MRI was available for 1045 of the 1471 (71%) patients included in the analysis. Using logistic regression analysis, we found that MRI was more likely to be performed in patients with cervical dystonia, spasmodic dysphonia, or non-task-specific upper limb dystonia, whereas it was less likely to be performed in patients with blepharospasm or task-specific upper limb dystonia. We did not find differences in the number of MRIs performed between neurological centers in Northern, Central, and Southern Italy. We conclude that although the diagnosis of idiopathic adult-onset dystonia is mainly based on clinical grounds, many movement disorder experts rely on MRI to confirm a diagnosis of idiopathic dystonia. We suggest that neuroimaging should be used in patients with adult-onset focal dystonia to rule out secondary forms.
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http://dx.doi.org/10.1007/s10072-020-05025-wDOI Listing
July 2021

Erenumab in the prevention of high-frequency episodic and chronic migraine: Erenumab in Real Life in Italy (EARLY), the first Italian multicenter, prospective real-life study.

Headache 2021 02 18;61(2):363-372. Epub 2020 Dec 18.

Headache and Neurosonology Unit, Policlinico Universitario Campus Bio-Medico, Rome, Italy.

Objective: To assess the effectiveness, safety, and tolerability of erenumab in a real-life migraine population, while trying to identify responsiveness predictors.

Background: Erenumab is a fully human Ig-2 monoclonal antibody blocking the calcitonin gene-related peptide receptor, indicated for migraine prophylaxis. Phase II and III trials demonstrated that erenumab is effective, safe, and well tolerated in the prevention of episodic and chronic migraine (CM), showing an early onset of action.

Methods: This is a multicenter, prospective, cohort, and real-life study. We considered for enrolment all consecutive patients aged 18-65 affected by high-frequency episodic migraine (HFEM) or CM, with or without medication overuse, visited at nine Italian Headache Centers from December 20, 2018 to September 30, 2019. Each patient was treated with erenumab 70 mg, administered subcutaneously every 4 weeks. Treatment duration was planned to last from 6 to 12 months, depending on the patient's response. The primary endpoint was the change in monthly migraine days (MMDs) at weeks 9-12 compared to baseline. Secondary endpoints included changes in monthly analgesics intake, ≥50%, ≥75%, and 100% responder rates and any variation in the Visual Analog Scale (VAS) and Headache Impact Test scores (HIT).

Results: In total, 372 migraine patients were treated with at least one dose of erenumab 70 mg. At weeks 9-12, erenumab decreased MMDs by 4.5 ± 4.1 days (mean ± SD) in patients with HFEM and by 9.3 ± 9.1 (mean ± SD) days in those with CM compared to baseline. At weeks 9-12 VAS score was reduced by 1.9 ± 1.9 (mean ± SD), HIT score by 10.7 ± 8.8 (mean ± SD), and median monthly analgesics intake passed from 12.0 (interquartile range [IQR] 10.0-14.0) to 5.0 (IQR 3.0-7.0) in HFEM. In CM patients, VAS was reduced by 1.7 ± 2.0 (mean ± SD), HIT by 9.7 ± 10.4 (mean ± SD), and median monthly analgesics intake passed from 20.0 (IQR 15.0-30.0) to 8.0 (IQR 5.0-15.0). At week 12, ≥50% responders were 60/101 (59.4%) for HFEM and 146/263 (55.5%) for CM, ≥75% responders were 17/101 (16.8%) and 59/263 (22.4%) and 100% responders 1/101 (1.0%) and 3/263 (1.1%), respectively. Erenumab responsiveness in HFEM was positively associated with unilateral pain localization (OR: 3.03, 95% CI: 1.24-7.40; p = 0.015), whereas in CM responsiveness was positively associated with and baseline migraine frequency (OR: 1.06, 95% CI:1.02-1.11; p = 0.031), dopaminergic symptoms (OR: 2.01, 95% CI: 1.14-3.52; p = 0.015), and negatively associated with psychiatric comorbidities (OR: 0.43, 95% CI: 0.20-0.93; p = 0.003).

Conclusions: Erenumab 70 mg is effective, safe, and well tolerated in real life. Easily obtainable clinical features might be of help in predicting patient's responsiveness.
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http://dx.doi.org/10.1111/head.14032DOI Listing
February 2021

Functional motor disorders associated with other neurological diseases: Beyond the boundaries of "organic" neurology.

Eur J Neurol 2021 05 2;28(5):1752-1758. Epub 2021 Jan 2.

Movement Disorder Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Background And Purpose: The aims of this study were to describe the clinical manifestations of functional motor disorders (FMDs) coexisting with other neurological diseases ("comorbid FMDs"), and to compare comorbid FMDs with FMDs not overlapping with other neurological diseases ("pure FMDs").

Methods: For this multicenter observational study, we enrolled outpatients with a definite FMD diagnosis attending 25 tertiary movement disorder centers in Italy. Each patient with FMDs underwent a detailed clinical assessment including screening for other associated neurological conditions. Group comparisons (comorbid FMDs vs. pure FMDs) were performed in order to compare demographic and clinical variables. Logistic regression models were created to estimate the adjusted odds ratios (95% confidence intervals) of comorbid FMDs (dependent variable) in relation to sociodemographic and clinical characteristics (independent variables).

Results: Out of 410 FMDs, 21.7% of patients (n = 89) had comorbid FMDs. The most frequent coexisting neurological diseases were migraine, cerebrovascular disease and parkinsonism. In the majority of cases (86.5%), FMDs appeared after the diagnosis of a neurological disease. Patients with comorbid FMDs were older, and more frequently had tremor, non-neurological comorbidities, paroxysmal non-epileptic seizures, major depressive disorders, and benzodiazepine intake. Multivariate regression analysis showed that diagnosis of comorbid FMDs was more likely associated with longer time lag until the final diagnosis of FMD, presence of tremor and non-neurological comorbidities.

Conclusions: Our findings highlight the need for prompt diagnosis of FMDs, given the relatively high frequency of associated neurological and non-neurological diseases.
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http://dx.doi.org/10.1111/ene.14674DOI Listing
May 2021

Clinical Correlates of Functional Motor Disorders: An Italian Multicenter Study.

Mov Disord Clin Pract 2020 Nov 22;7(8):920-929. Epub 2020 Sep 22.

Department of Systems Medicine University of Rome Tor Vergata Rome Italy.

Background: Functional motor disorders (FMDs) are abnormal movements that are significantly altered by distractive maneuvers and are incongruent with movement disorders seen in typical neurological diseases.

Objective: The objectives of this article are to (1) describe the clinical manifestations of FMDs, including nonmotor symptoms and occurrence of other functional neurological disorders (FND); and (2) to report the frequency of isolated and combined FMDs and their relationship with demographic and clinical variables.

Methods: For this multicenter, observational study, we enrolled consecutive outpatients with a definite diagnosis of FMDs attending 25 tertiary movement disorders centers in Italy. Each patient underwent a detailed clinical evaluation with a definition of the phenotype and number of FMDs (isolated, combined) and an assessment of associated neurological and psychiatric symptoms.

Results: Of 410 FMDs (71% females; mean age, 47 ± 16.1 years) the most common phenotypes were weakness and tremor. People with FMDs had higher educational levels than the general population and frequent nonmotor symptoms, especially anxiety, fatigue, and pain. Almost half of the patients with FMDs had other FNDs, such as sensory symptoms, nonepileptic seizures, and visual symptoms. Patients with combined FMDs showed a higher burden of nonmotor symptoms and more frequent FNDs. Multivariate regression analysis showed that a diagnosis of combined FMDs was more likely to be delivered by a movement disorders neurologist. Also, FMD duration, pain, insomnia, diagnosis of somatoform disease, and treatment with antipsychotics were all significantly associated with combined FMDs.

Conclusions: Our findings highlight the need for multidimensional assessments in patients with FMDs given the high frequency of nonmotor symptoms and other FNDs, especially in patients with combined FMDs.
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http://dx.doi.org/10.1002/mdc3.13077DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7604660PMC
November 2020

Motor and Sensory Features of Cervical Dystonia Subtypes: Data From the Italian Dystonia Registry.

Front Neurol 2020 26;11:906. Epub 2020 Aug 26.

Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.

Cervical dystonia (CD) is one of the most common forms of adult-onset isolated dystonia. Recently, CD has been classified according to the site of onset and spread, in different clinical subgroups, that may represent different clinical entities or pathophysiologic subtypes. In order to support this hypothesis, in this study we have evaluated whether different subgroups of CD, that clinically differ for site of onset and spread, also imply different sensorimotor features. Clinical and demographic data from 842 patients with CD from the Italian Dystonia Registry were examined. Motor features (head tremor and tremor elsewhere) and sensory features (sensory trick and neck pain) were investigated. We analyzed possible associations between motor and sensory features in CD subgroups [focal neck onset, no spread (FNO-NS); focal neck onset, segmental spread (FNO-SS); focal onset elsewhere with segmental spread to neck (FOE-SS); segmental neck involvement without spread (SNI)]. In FNO-NS, FOE-SS, and SNI subgroups, head tremor was associated with the presence of tremor elsewhere. Sensory trick was associated with pain in patients with FNO-NS and with head tremor in patients with FNO-SS. The frequent association between head tremor and tremor elsewhere may suggest a common pathophysiological mechanism. Two mechanisms may be hypothesized for sensory trick: a gating mechanism attempting to reduce pain and a sensorimotor mechanism attempting to control tremor.
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http://dx.doi.org/10.3389/fneur.2020.00906DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7493687PMC
August 2020

Demographic and clinical determinants of neck pain in idiopathic cervical dystonia.

J Neural Transm (Vienna) 2020 10 26;127(10):1435-1439. Epub 2020 Aug 26.

IRCCS Neuromed, Pozzilli, Italy.

Cervical dystonia is associated with neck pain in a significant proportion of cases, but the mechanisms underlying pain are largely unknown. In this exploratory study, we compared demographic and clinical variables in cervical dystonia patients with and without neck pain from the Italian Dystonia Registry. Univariable and multivariable logistic regression analysis indicated a higher frequency of sensory trick and a lower educational level among patients with pain.
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http://dx.doi.org/10.1007/s00702-020-02245-4DOI Listing
October 2020

Headache due to intracranial hypertension at the time of first manifestation of multiple sclerosis.

Neurol Sci 2020 12;41(Suppl 2):491-492

Center for Headache and Intracranial Pressure Disorders, A. O. U. "Mater Domini" of Catanzaro, Viale Europa, 88100, Catanzaro, Italy.

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http://dx.doi.org/10.1007/s10072-020-04671-4DOI Listing
December 2020

Idiopathic Non-task-Specific Upper Limb Dystonia, a Neglected Form of Dystonia.

Mov Disord 2020 11 14;35(11):2038-2045. Epub 2020 Jul 14.

Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy.

Objective: The objective of this study was to describe the clinical and demographic features of idiopathic non-task-specific upper limb dystonia compared with the task-specific form.

Methods: In this retrospective study, adult patients with idiopathic upper limb dystonia, either focal or as part of a segmental/multifocal dystonia, from the Italian Dystonia Registry were enrolled. In patients with focal upper limb dystonia, dystonia spread was estimated by survival analysis.

Results: Of the 1522 patients with idiopathic adult-onset dystonia included in the Italian Dystonia Registry, we identified 182 patients with upper limb dystonia. Non-task-specific dystonia was present in 61.5% of enrolled cases. Women predominated among non-task-specific patients, whereas men predominated in the task-specific group. Peak age of upper limb dystonia onset was in the sixth decade in the non-task-specific group and in the fourth decade in the task-specific group. In both groups, upper limb dystonia started as focal dystonia or as part of a segmental dystonia. Segmental onset was more frequent among non-task-specific patients, whereas focal onset predominated among task-specific patients. Dystonic action tremor was more frequent among non-task-specific patients. No significant differences between groups emerged in terms of sensory trick frequency, rest tremor, or family history of dystonia. In patients with focal upper limb dystonia, dystonia spread was greater in the non-task-specific group.

Conclusion: Novel information on upper limb dystonia patients suggests that non-task-specific and task-specific upper limb dystonia have different demographic and clinical features. However, it remains to be determined whether these differences also reflect pathophysiological differences. © 2020 International Parkinson and Movement Disorder Society.
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http://dx.doi.org/10.1002/mds.28199DOI Listing
November 2020

Botulinum toxin for the management of spasticity in multiple sclerosis: the Italian botulinum toxin network study.

Neurol Sci 2020 Oct 12;41(10):2781-2792. Epub 2020 Apr 12.

Multiple Sclerosis Clinical Care and Research Centre, Department of Neuroscience, Reproductive Sciences and Odontostomatology, "Federico II" University of Naples, Via Sergio Pansini 5, edificio 17 piano terra, 80131, Naples, Italy.

Background: Botulinum toxin (BT) is an effective and safe treatment for spasticity, with limited evidence in multiple sclerosis (MS). We aim to describe the use of BT for the management of MS spasticity in the clinical practice, its combination with other anti-spastic treatments in MS and possible MS clinical correlates.

Methods: This is a multicentre cross-sectional observational study including 386 MS patients, receiving BT for spasticity in 19 Italian centres (age 53.6 ± 10.9 years; female 228 (59.1%); disease duration 18.7 ± 9.2 years; baseline Expanded Disability Status Scale (EDSS) 6.5 (2.0-9.0)).

Results: BT was used for improving mobility (n = 170), functioning in activities of daily living (n = 56), pain (n = 56), posturing-hygiene (n = 63) and daily assistance (n = 41). BT formulations were AbobotulinumtoxinA (n = 138), OnabotulinumtoxinA (n = 133) and IncobotulinumtoxinA (n = 115). After conversion to unified dose units, higher BT dose was associated with higher EDSS (Coeff = 0.591; p < 0.001), higher modified Ashworth scale (Coeff = 0.796; p < 0.001) and non-ambulatory patients (Coeff = 209.382; p = 0.006). Lower BT dose was used in younger patients (Coeff = - 1.746; p = 0.009), with relapsing-remitting MS (Coeff = - 60.371; p = 0.012). BT dose was higher in patients with previous BT injections (Coeff = 5.167; p = 0.001), and with concomitant treatments (Coeff = 43.576; p = 0.022). Three patients (0.7%) reported on post-injection temporary asthenia/weakness (n = 2) and hypophonia (n = 1).

Conclusion: BT was used for spasticity and its consequences from the early stages of MS, without significant adverse effects. MS-specific goals and injection characteristics can be used to refer MS patients to BT treatment, to decide for the strategy of BT injections and to guide the design of future clinical trials and observational studies.
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http://dx.doi.org/10.1007/s10072-020-04392-8DOI Listing
October 2020

Does acute peripheral trauma contribute to idiopathic adult-onset dystonia?

Parkinsonism Relat Disord 2020 02 13;71:40-43. Epub 2020 Jan 13.

Neurology Department, Asti Hospital, Asti, Italy.

Background: Acute peripheral trauma is a controversial risk factor for idiopathic dystonia.

Materials And Methods: We retrospectively analyzed data from the Italian Dystonia Registry regarding the occurrence of acute peripheral trauma severe enough to require medical attention in 1382 patients with adult-onset idiopathic dystonia and 200 patients with acquired adult-onset dystonia.

Results: Patients with idiopathic and acquired dystonia showed a similar burden of peripheral trauma in terms of the number of patients who experienced trauma (115/1382 vs. 12/200, p = 0.3) and the overall number of injuries (145 for the 1382 idiopathic patients and 14 for the 200 patients with secondary dystonia, p = 0.2). Most traumas occurred before the onset of idiopathic or secondary dystonia but only a minority of such injuries (14 in the idiopathic group, 2 in the acquired group, p = 0.6) affected the same body part as that affected by dystonia. In the idiopathic group, the elapsed time between trauma and dystonia onset was 8.1 ± 9.2 years; only six of the 145 traumas (4.1%) experienced by 5/1382 idiopathic patients (0.36%) occurred one year or less before dystonia onset; in the acquired dystonia group, the two patients experienced prior trauma to the dystonic body part 5 and 6 years before dystonia development.

Discussion And Conclusion: Our data suggest that the contribution of peripheral acute trauma to idiopathic dystonia is negligible, if anything, and likely involves only a small subset of patients.
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http://dx.doi.org/10.1016/j.parkreldis.2020.01.002DOI Listing
February 2020

Isolated cerebrospinal fluid hypertension in chronic headache: diagnostic innovations and clinical implications.

Authors:
Francesco Bono

Neurol Sci 2019 May;40(Suppl 1):71-74

Center for Headache and Intracranial Pressure Disorders, University Hospital "Mater Domini", Catanzaro, Italy.

Isolated cerebrospinal fluid hypertension (ICH) is a condition of increased cerebrospinal fluid (CSF) pressure in the cranial-spinal compartment without an identifiable cause. Isolated headache is the most common symptom of ICH, while missing may be signs such as papilledema or sixth nerve palsy. This fact makes difficult the clinical diagnosis of headache attributable to ICH in headache sufferers. Another source of confusion stems from the CSF pressure measurement. It has been observed that a single-spot CSF opening pressure measurement may be insufficient to identify elevated CSF pressure in headache sufferers. A new method of CSF pressure measurement has been able to identify pressure-related features of isolated CSF hypertension (ICH). In fact, nocturnal or postural headache and abnormal pressure pulsations are the more common pressure-related features of ICH in patients with chronic headache. The compressive action of these abnormal pressure pulsations causes the periventricular white matter microstructure alterations leading to the focal diffusion tensor imaging findings in patients with ICH. Abnormal pressure pulsations are a marker of ICH in chronic headache. The identification of the CSF pressure-related features may be useful for differentiating headache sufferers with ICH from those with primary headache disorder in clinical practice. The therapeutic strategy in these headache sufferers with ICH includes the CSF removal and a medical treatment.
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http://dx.doi.org/10.1007/s10072-019-03836-0DOI Listing
May 2019

Periventricular white matter changes in idiopathic intracranial hypertension.

Ann Clin Transl Neurol 2019 02 18;6(2):233-242. Epub 2019 Jan 18.

Department of Medical and Surgical Sciences Center for Headache and Intracranial Pressure Disorders Institute of Neurology Magna Græcia University of Catanzaro Catanzaro Italy.

Objective: To evaluate whether increased cerebrospinal fluid (CSF) pressure causes alteration of periventricular white matter (WM) microstructure in patients with idiopathic intracranial hypertension (IIH).

Methods: In a prospective study, patients with refractory chronic headache with and without IIH performed a neuroimaging study including 3T MRI, 3D Phase Contrast MR venography, and diffusion tensor imaging (DTI) of the brain. Whole-brain voxel-wise comparisons of DTI abnormalities of WM were performed using tract-based spatial statistics. A correlation analysis between DTI indices and CSF opening pressure, highest peak, and mean pressure was also performed in patients with IIH.

Results: We enrolled 62 consecutive patients with refractory chronic headaches. Thirty-five patients with IIH, and 27 patients without increased intracranial pressure. DTI analysis revealed no fractional anisotropy changes, but decreased mean, axial, and radial diffusivity in body (IIH = 0.80 ± 0.04, non-IIH = 0.84 ± 0.4, IIH = 1.67 ± 0.07, non-IIH = 1.74 ± 0.05, IIH = 0.38 ± 0.04, non-IIH = 0.42 ± 0.05 [mm/sec × 10]) of corpus callosum, and in right superior corona radiata (IIH = 0.75 ± 0.04, non-IIH = 0.79 ± 0.05, IIH = 1.19 ± 0.07, non-IIH = 1.28 ± 0.09, IIH = 0.59 ± 0.03, non-IIH = 0.53 ± 0.03 [mm/sec × 10]) of 35 patients with IIH compared with 27 patients without increased intracranial pressure. DTI indices were negatively correlated with high CSF pressures (<0.05). After medical treatment, eight patients showed incremented MD in anterior corona radiata left and right and superior corona radiata right.

Conclusions: There is significant DTI alteration in periventricular WM microstructure of patients with IIH suggesting tissue compaction correlated with high CSF pressure. This periventricular WM change may be partially reversible after medical treatment.
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http://dx.doi.org/10.1002/acn3.685DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389746PMC
February 2019

Cerebrospinal Fluid Pressure-Related Features in Chronic Headache: A Prospective Study and Potential Diagnostic Implications.

Front Neurol 2018 18;9:1090. Epub 2018 Dec 18.

Neurosciences Research Center, Magna Græcia University, Catanzaro, Italy.

To identify the pressure-related features of isolated cerebrospinal fluid hypertension (ICH) in order to differentiate headache sufferers with ICH from those with primary headache disorder. In this prospective study, patients with refractory chronic headaches and suspected of having cerebrospinal fluid-pressure elevation without papilledema or sixth nerve palsy, together with controls, underwent 1-h lumbar cerebrospinal fluid pressure monitoring via a spinal puncture needle. We recruited 148 consecutive headache patients and 16 controls. Lumbar cerebrospinal fluid pressure monitoring showed high pressure and abnormal pressure pulsations in 93 (63 %) patients with headache: 37 of these patients with the most abnormal pressure parameters (opening pressure above 250 mm HO, mean pressure 301 mm HO, mean peak pressure 398 mm HO, and severe abnormal pressure pulsations) had the most severe headaches and associated symptoms (nocturnal headache, postural headache, transient visual obscuration); 56 patients with the less abnormal pressure parameters (opening pressure between 200 and 250 mm HO, mean pressure 228 mm HO, mean peak pressure 316 mm HO, and abnormal pressure pulsations) had less severe headaches and associated symptoms. Nocturnal and postural headache, and abnormal pressure pulsations are the more common pressure-related features of ICH in patients with chronic headache. Abnormal pressure pulsations may be considered a marker of ICH in chronic headache.
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http://dx.doi.org/10.3389/fneur.2018.01090DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305580PMC
December 2018

Expert recommendations for diagnosing cervical, oromandibular, and limb dystonia.

Neurol Sci 2019 Jan 29;40(1):89-95. Epub 2018 Sep 29.

Department of Neurology and Psychiatry, Sapienza University of Rome, and Neuromed Institute IRCCS, Pozzilli, Italy.

Background: Diagnosis of focal dystonia is based on clinical grounds and is therefore open to bias. To date, diagnostic guidelines have been only proposed for blepharospasm and laryngeal dystonia. To provide practical guidance for clinicians with less expertise in dystonia, a group of Italian Movement Disorder experts formulated clinical diagnostic recommendations for cervical, oromandibular, and limb dystonia.

Methods: A panel of four neurologists generated a list of clinical items related to the motor phenomenology of the examined focal dystonias and a list of clinical features characterizing neurological/non-neurological conditions mimicking dystonia. Thereafter, ten additional expert neurologists assessed the diagnostic relevance of the selected features and the content validity ratio was calculated. The clinical features reaching a content validity ratio > 0.5 contributed to the final recommendations.

Results: The recommendations retained patterned and repetitive movements/postures as the core feature of dystonia in different body parts. If present, a sensory trick confirmed diagnosis of dystonia. In the patients who did not manifest sensory trick, active exclusion of clinical features related to conditions mimicking dystonia (features that would be expected to be absent in dystonia) would be necessary for dystonia to be diagnosed.

Discussion: Although reliability, sensitivity, and specificity of the recommendations are yet to be demonstrated, information from the present study would hopefully facilitate diagnostic approach to focal dystonias in the clinical practice and would be the basis for future validated diagnostic guidelines.
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http://dx.doi.org/10.1007/s10072-018-3586-9DOI Listing
January 2019

Validation of a self-reported instrument to assess work-related difficulties in patients with migraine: the HEADWORK questionnaire.

J Headache Pain 2018 Sep 10;19(1):85. Epub 2018 Sep 10.

Headache and Neuroalgology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Background: The degree to which work-related difficulties are recognized in headache research is poor and often carried out with inadequate information such as "reduced ability to work as usual", which do not capture at all the variety of difficulties and the factors that impact over them. The aim of this paper is to present the validation of the HEADWORK questionnaire, which addresses the amount and severity of difficulties in work-related tasks and the factors that impact over them.

Methods: We developed a set of items based on a previous literature review and patients' focus groups and tested it on a wide set of patients with episodic and chronic migraine attending eight different Italian headache centers. HEADWORK factor structure was assessed with exploratory and confirmatory factor analysis; internal consistency and construct validity were addressed as well.

Results: The validation sample (N = 373) was mostly composed of patients with episodic migraine without aura (64.3%) and of females (81%). Factor analysis retrieved two different scales: "Work-related difficulties", composed of eleven items which explain 67.1% of the total variance, and "Factors contributing to work difficulties", composed of six items which explain 52.1% of the total variance. Both HEADWORK subscales have good measurement properties, with higher scores being associated to higher disability, lower quality of life, lower productivity, higher headache frequency and pain intensity.

Conclusions: HEADWORK is a 17-item, two-scale questionnaire addressing the impact of migraine on work-related difficulties in terms of difficulties in general or specific skills, and the factors contributing to these difficulties, defined as negative impact on work tasks. It can be used to address disability weights for the purpose of calculating the burden of migraine, and to assess the balance between therapeutic and side effects of medication on productivity.
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http://dx.doi.org/10.1186/s10194-018-0914-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131677PMC
September 2018

Relationship between severity of migraine and vitamin D deficiency: a case-control study.

Neurol Sci 2018 Jun;39(Suppl 1):167-168

Neuroscience Research Center, Department of Medical and Surgical Sciences, Magna Graecia University, Catanzaro, Italy.

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http://dx.doi.org/10.1007/s10072-018-3384-4DOI Listing
June 2018

Cardiac parasympathetic index identifies subjects with adult obstructive sleep apnea: A simultaneous polysomnographic-heart rate variability study.

PLoS One 2018 8;13(3):e0193879. Epub 2018 Mar 8.

Institute of Bioimaging and Molecular Physiology, National Research Council, Germaneto, Catanzaro, Italy.

Objective: To evaluate circadian fluctuations and night/day ratio of Heart Rate Variability (HRV) spectral components in patients with obstructive sleep apnea (OSA) in comparison with controls.

Participants And Methods: This is a simultaneous HRV-polysomnographic (PSG) study including 29 patients with OSA and 18 age-sex-matched controls. Four patients with OSA dropped out. All participants underwent PSG and HRV analysis. We measured the 24-hour fluctuations and the night/day ratio of low frequency (LF) and high frequency (HF) spectral components of HRV in all subjects and controls. The LF night/day ratio was termed the cardiac sympathetic index while the HF night/day ratio was termed the cardiac parasympathetic index.

Results: All twenty-five OSA patients were PSG positive (presence of OSA) while 18 controls were PSG negative (absence of OSA). There was no significant difference in LF and HF 24-hour fluctuation values between OSA patients and controls. In OSA patients, LF and HF values were significantly higher during night-time than day time recordings (p<0.001). HF night/day ratio (cardiac parasympathetic index) accurately (100%) differentiated OSA patients from controls without an overlap of individual values. The LF night/day ratio (cardiac sympathetic index) had sensitivity of 84%, specificity of 72.2% and accuracy of 79.1% in distinguishing between groups.

Conclusions: The cardiac parasympathetic index accurately differentiated patients with OSA from controls, on an individual basis.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0193879PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5843243PMC
July 2018

Cough tremor as presenting symptom of intraventricular tumor.

Neurology 2016 09;87(11):1185-6

From the Institute of Neurology (Drs. Mazza, Morelli, Ferrigno, Quattrone, and Bono), Magna Græcia University of Catanzaro; Federico II University of Naples (Drs. Gangemi and Cavallo); and the Institute of Molecular Bioimaging and Physiology (Drs. Vescio, Quattrone, and Bono), National Research Council, Catanzaro, Italy.

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http://dx.doi.org/10.1212/WNL.0000000000003100DOI Listing
September 2016

Reversible deep brain swelling causing REM behavior disorder.

Neurology 2016 Apr;86(14):1360

From the Headache Group, Institutes of Neurology (D.S., M.R.M., L.M., A.Q., F.B.) and Neuroradiology (C.B.), Department of Medical and Surgical Sciences, Magna Græcia University of Catanzaro; and the Neuroimaging Research Unit (M.S., A.Q., F.B.), Institute of Bioimaging and Molecular Physiology, National Research Council, Catanzaro, Italy.

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http://dx.doi.org/10.1212/WNL.0000000000002548DOI Listing
April 2016

Electrophysiological and structural MRI correlates of dystonic head rotation in drug-naïve patients with torticollis.

Parkinsonism Relat Disord 2015 Dec 9;21(12):1415-20. Epub 2015 Oct 9.

Institute of Neurology, Department of Medical and Surgical Sciences, University Magna Græcia, Catanzaro, Italy; Neuroimaging Research Unit, Institute of Bioimaging and Molecular Physiology, National Research Council, Catanzaro, Italy.

Introduction: We tested whether a change in head/neck position initiates head deviation in drug-naïve patients with cervical dystonia and to identify the electrophysiological and neuroanatomical correlates of dystonic head rotation.

Methods: Twenty-five consecutive drug-naïve patients with cervical dystonia and 25 healthy controls underwent the simultaneous surface electromyographic (EMG) recording of sternocleidomastoid (SCM) muscle contractions during head/neck position changes, blink reflex recovery cycle (BRrc), DAT-SPECT, and advanced structural neuroimaging analysis using voxel-based morphometry (VBM).

Results: Surface EMG recordings of SCM muscle activity during changes in head/neck position demonstrated an insignificant asymmetric low amplitude of the SCM muscle contractions in the horizontal position in both patients and controls, but an asymmetric high amplitude in SCM muscle contractions leading to abnormal head movements in vertical positions in patients with cervical dystonia. All controls had a symmetric low increase in amplitude of SCM muscle contractions in response to changes in head/neck position. VBM analysis in 19 patients showed abnormal decreases of gray matter (GM) volume in the bilateral motor (localized in the homunculus of the head) and premotor cortices when compared to controls. In addition, the side of these neuroanatomical changes was asymmetrically related to abnormal head deviations in these patients. All subjects had normal results during BRrc and DAT-SPECT.

Conclusions: The passage from inactive horizontal position to active vertical head/neck posture initiates head deviation in drug-naïve patients with cervical dystonia, and the anatomical correlates of this dystonic head rotation is a restricted abnormal pattern of GM changes in the motor cortices.
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http://dx.doi.org/10.1016/j.parkreldis.2015.09.050DOI Listing
December 2015
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