Publications by authors named "Enrico Alfonsi"

65 Publications

Case Report: Laryngospasm as Initial Manifestation of Amyotrophic Lateral Sclerosis in a Long-Survival Patient With Heterozygous p.D90A - Mutation.

Front Neurol 2021 30;12:708885. Epub 2021 Sep 30.

Neuro-Oncology Unit, IRCCS Mondino Foundation, Pavia, Italy.

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. Although its etiology is still unknown, many genes have been found to be implicated in ALS pathogenesis. The Cu/Zn superoxide dismutase () gene was the first to be identified. Currently, more than 230 mutations in the gene have been reported. p.D90A (p. Asp90Ala) is the most common mutation worldwide. It shows both autosomal and recessive inheritance in different populations. To date, five Italian patients with the heterozygous p.D90A mutation have been reported. None of them complained of laryngological symptoms as the initial manifestation of ALS, although they had atypical clinical features. We describe a long-survival patient carrying heterozygous p.D90A mutation who presented with severe laryngospasm due to bilateral vocal cord paralysis. We suggest that genetic analysis may help to diagnose ALS with insidious onset like hoarseness, laryngospasm, and other type of voice disturbances.
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http://dx.doi.org/10.3389/fneur.2021.708885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8514733PMC
September 2021

Consensus on the treatment of dysphagia in Parkinson's disease.

J Neurol Sci 2021 Sep 27;430:120008. Epub 2021 Sep 27.

Department of Clinical and Movement Neurosciences, UCL, Queen Square Institute of Neurology, London, UK.

Background: Dysphagia is common in Parkinson's disease (PD). The effects of antiparkinsonian drugs on dysphagia are controversial. Several treatments for dysphagia are available but there is no consensus on their efficacy in PD.

Objective: To conduct a systematic review of the literature and to define consensus statements on the treatment of dysphagia in PD and related nutritional management.

Methods: A multinational group of experts in the field of neurogenic dysphagia and/or Parkinson's disease conducted a systematic evaluation of the literature and reported the results according to PRISMA guidelines. The evidence from the retrieved studies was analyzed and discussed in a consensus conference organized in Pavia, Italy, and the consensus statements were drafted. The final version of statements was subsequently achieved by e-mail consensus.

Results: The literature review retrieved 64 papers on treatment and nutrition of patients with PD and dysphagia, mainly of Class IV quality. Based on the literature and expert opinion in cases where the evidence was limited or lacking, 26 statements were developed.

Conclusions: The statements developed by the Consensus panel provide a guidance for a multi-disciplinary treatment of dysphagia in patients with PD, involving neurologists, otorhinolaryngologists, gastroenterologists, phoniatricians, speech-language pathologists, dieticians, and clinical nutritionists.
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http://dx.doi.org/10.1016/j.jns.2021.120008DOI Listing
September 2021

Characterization and management of neurological adverse events during immune-checkpoint inhibitors treatment: an Italian multicentric experience.

Neurol Sci 2021 Aug 23. Epub 2021 Aug 23.

"C. Mondino" National Neurological Institute, IRCCS Mondino Foundation, Via Mondino 2, 27100, Pavia, Italy.

Background: Neurological immune-related adverse events (nirAEs) are rare toxicities of immune-checkpoint inhibitors (ICI). With the increase of ICI oncological indications, their incidence is growing. Their recognition and management remain nevertheless challenging.

Methods: A national, web-based database was built to collect cases of neurological symptoms in patients receiving ICI and not attributable to other causes after an adequate workup.

Results: We identified 27 patients who developed nirAEs (20 males, median age 69 years). Patients received anti-PD1/PDL1 (78%), anti-CTLA4 (4%), or both (19%). Most common cancers were melanoma (30%) and non-small cell lung cancer (26%). Peripheral nervous system was mostly affected (78%). Median time to onset was 43.5 days and was shorter for peripheral versus central nervous system toxicities (36 versus 144.5 days, p = 0.045). Common manifestations were myositis (33%), inflammatory polyradiculoneuropathies (33%), and myasthenia gravis (19%), alone or in combination, but the spectrum of diagnoses was broad. Most patients received first-line glucocorticoids (85%) or IVIg (15%). Seven patients (26%) needed second-line treatments. At last follow-up, four (15%) patients were deceased (encephalitis, 1; myositis/myasthenia with concomitant myocarditis, 2; acute polyradiculoneuropathy, 1), while seven (26%) had a complete remission, eight (30%) partial improvement, and six (22%) stable/progressing symptoms. ICI treatment was discontinued in most patients (78%).

Conclusions: Neurological irAEs are rare but potentially fatal. They primarily affect neuromuscular structures but encompass a broad range of presentations. A prompt recognition is mandatory to timely withheld immunotherapy and administrate glucocorticoids. In corticoresistant or severely affected patients, second-line treatments with IVIg or plasmapheresis may result in additional benefit.
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http://dx.doi.org/10.1007/s10072-021-05561-zDOI Listing
August 2021

A multinational consensus on dysphagia in Parkinson's disease: screening, diagnosis and prognostic value.

J Neurol 2021 Aug 21. Epub 2021 Aug 21.

Department of Clinical and Movement Neurosciences, UCL, Queen Square Institute of Neurology, London, UK.

Background: Parkinson's disease (PD) is a neurodegenerative disorder characterized by a combination of motor and non-motor dysfunction. Dysphagia is a common symptom in PD, though it is still too frequently underdiagnosed. Consensus is lacking on screening, diagnosis, and prognosis of dysphagia in PD.

Objective: To systematically review the literature and to define consensus statements on the screening and the diagnosis of dysphagia in PD, as well as on the impact of dysphagia on the prognosis and quality of life (QoL) of PD patients.

Methods: A multinational group of experts in the field of neurogenic dysphagia and/or PD conducted a systematic revision of the literature published since January 1990 to February 2021 and reported the results according to PRISMA guidelines. The output of the research was then analyzed and discussed in a consensus conference convened in Pavia, Italy, where the consensus statements were drafted. The final version of statements was subsequently achieved by e-mail consensus.

Results: Eighty-five papers were used to inform the Panel's statements even though most of them were of Class IV quality. The statements tackled four main areas: (1) screening of dysphagia: timing and tools; (2) diagnosis of dysphagia: clinical and instrumental detection, severity assessment; (3) dysphagia and QoL: impact and assessment; (4) prognostic value of dysphagia; impact on the outcome and role of associated conditions.

Conclusions: The statements elaborated by the Consensus Panel provide a framework to guide the neurologist in the timely detection and accurate diagnosis of dysphagia in PD.
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http://dx.doi.org/10.1007/s00415-021-10739-8DOI Listing
August 2021

Electrokinesiographic Study of Oropharyngeal Swallowing in Neurogenic Dysphagia.

Dysphagia 2021 Jul 27. Epub 2021 Jul 27.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Via Mondino 2, 27100, Pavia, Italy.

Electrokinesiographic study of swallowing (EKSS) can be useful for the assessment of patients with suspected or overt neurogenic dysphagia. EKSS consists of multichannel recording of the electromyographic (EMG) activity of the suprahyoid/submental muscle complex (SHEMG), the EMG activity of the cricopharyngeal muscle (CPEMG), and the laryngopharyngeal mechanogram (LPM). The LPM is an expression of the mechanical changes that the laryngopharyngeal structures undergo during the pharyngeal phase of swallowing. This method allows detailed evaluation of the magnitude, duration and temporal relations of the different events that characterize oropharyngeal swallowing, and thus in-depth exploration both of physiological deglutition mechanisms and of pathophysiological features of swallowing in neurogenic dysphagia. Furthermore, EKSS can guide dysphagia treatment strategies, allowing identification of optimal solutions for single patients. For instance, CPEMG recording can identify incomplete or absent relaxation of the upper esophageal sphincter during the pharyngeal phase of swallowing, thus suggesting a therapeutic approach based on botulinum toxin injection into the cricopharyngeal muscle. More recently, the 'shape' of SHEMG and the reproducibility of both SHEMG and LPM over repeated swallowing acts have been implemented as novel electrokinesiographic parameters. These measures could be valuable for straightforward non-invasive investigation of dysphagia severity and response to dysphagia treatment in clinical practice.
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http://dx.doi.org/10.1007/s00455-021-10336-xDOI Listing
July 2021

COVID-19 in patients with myasthenia gravis: Epidemiology and disease course.

Muscle Nerve 2021 08 12;64(2):206-211. Epub 2021 Jun 12.

Neuroncology Unit, IRCCS Mondino Foundation, Pavia, Italy.

Introduction/aims: Coronavirus disease 2019 (COVID-19), a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, has become a global pandemic. Patients with myasthenia gravis (MG), often treated with immunosuppressants, might be at higher risk of developing COVID-19 and of demonstrating a severe disease course. We aimed to study prevalence and describe features of COVID-19 in MG patients.

Methods: In May 2020, we conducted telephonic interviews with MG patients followed at our referral center. We collected structured data regarding MG and COVID-19, which was diagnosed as probable or confirmed according to the European Centre for Disease Prevention and Control case definition. We compared confirmed-COVID-19 prevalence calculated from the beginning of the pandemic in MG patients with that of the overall Pavia district.

Results: We interviewed 162 MG patients (median age, 66 y; interquartile range 41-77; males 59.9%), 88 from the Pavia district. Three patients had SARS-CoV-2-confirmed by polymerase chain reaction and eight had probable-COVID-19. In the Pavia district, the prevalence of confirmed-COVID-19 among MG patients (1/88, 1.14%) and overall population (4777/546 515, 0.87%) did not differ (P = .538). Higher Myasthenia Gravis Foundation of America clinicalclass and the need for recent rescue treatment, but not ongoing immunosuppressive treatments, were associated with COVID-19 risk. Of 11 MG patients with probable/confirmed-COVID-19, 3 required ventilator support, and 2 elderly patients died of COVID-19 respiratory insufficiency. Only 1 of11 patients experienced worsening MG symptoms, which improved after increasing their steroid dose.

Discussion: The risk of COVID-19 in MG patients seems to be no higher than that of the general population, regardless of immunosuppressive therapies. In our cohort, COVID-19 barely affected MG course.
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http://dx.doi.org/10.1002/mus.27324DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8242475PMC
August 2021

Expression pattern of matrix metalloproteinases-2 and -9 and their tissue inhibitors in patients with chronic inflammatory demyelinating polyneuropathy.

Neurol Sci 2021 Oct 14;42(10):4297-4300. Epub 2021 May 14.

Department of Biomedicine, Neuroscience and advanced Diagnostic, University of Palermo (BIND), Palermo, Italy.

Background: Matrix metalloproteinases (MMPs) are a heterogeneous family of endopeptidases that play a role in many physiological functions, including the immune response. An imbalance between the activity of MMPs and their physiological tissue inhibitors (TIMPs) has been proposed in the pathophysiology of different autoimmune disorders. We aimed to assess the plasmatic levels of MMP-2, MMP-9, and their inhibitors TIMP-1 and -2 in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

Subjects And Methods: Twenty patients with CIDP and 20 age- and sex-matched healthy controls were enrolled. Plasma concentrations of MMP-2, MMP-9, TIMP-1, and TIMP-2 were determined by the enzyme-linked immunosorbent assay.

Results: CIDP subjects had higher MMP-9 concentrations along with TIMP-1 downregulation when compared to controls, with the consequent increase in the MMP-9/TIMP-1 ratio (p<0.000002 for all measures). Conversely, the concentration of MMP-2 was lower in the CIDP group (p<0.01) without changes in the TIMP-2 concentration. The MMP-2/TIMP-2 ratio was decreased in the patients' group (p<0.02).

Discussion: We provide first preliminary evidence that the plasmatic pattern of MMPs and TIMPs is markedly altered in patients with CIDP. Future studies are needed to assess the potential usefulness of these new biomarkers in the clinical setting.
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http://dx.doi.org/10.1007/s10072-021-05314-yDOI Listing
October 2021

RFC1 expansions are a common cause of idiopathic sensory neuropathy.

Brain 2021 06;144(5):1542-1550

Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.

After extensive evaluation, one-third of patients affected by polyneuropathy remain undiagnosed and are labelled as having chronic idiopathic axonal polyneuropathy, which refers to a sensory or sensory-motor, axonal, slowly progressive neuropathy of unknown origin. Since a sensory neuropathy/neuronopathy is identified in all patients with genetically confirmed RFC1 cerebellar ataxia, neuropathy, vestibular areflexia syndrome, we speculated that RFC1 expansions could underlie a fraction of idiopathic sensory neuropathies also diagnosed as chronic idiopathic axonal polyneuropathy. We retrospectively identified 225 patients diagnosed with chronic idiopathic axonal polyneuropathy (125 sensory neuropathy, 100 sensory-motor neuropathy) from our general neuropathy clinics in Italy and the UK. All patients underwent full neurological evaluation and a blood sample was collected for RFC1 testing. Biallelic RFC1 expansions were identified in 43 patients (34%) with sensory neuropathy and in none with sensory-motor neuropathy. Forty-two per cent of RFC1-positive patients had isolated sensory neuropathy or sensory neuropathy with chronic cough, while vestibular and/or cerebellar involvement, often subclinical, were identified at examination in 58%. Although the sensory ganglia are the primary pathological target of the disease, the sensory impairment was typically worse distally and symmetric, while gait and limb ataxia were absent in two-thirds of the cases. Sensory amplitudes were either globally absent (26%) or reduced in a length-dependent (30%) or non-length dependent pattern (44%). A quarter of RFC1-positive patients had previously received an alternative diagnosis, including Sjögren's syndrome, sensory chronic inflammatory demyelinating polyneuropathy and paraneoplastic neuropathy, while three cases had been treated with immune therapies.
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http://dx.doi.org/10.1093/brain/awab072DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8262986PMC
June 2021

Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment.

Parkinsonism Relat Disord 2021 05 30;86:124-132. Epub 2021 Mar 30.

Department of Neurology, New York University School of Medicine, New York, NY, USA. Electronic address:

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.
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http://dx.doi.org/10.1016/j.parkreldis.2021.03.027DOI Listing
May 2021

Buprenorphine may be effective for treatment of paramyotonia congenita.

Muscle Nerve 2021 07 20;64(1):95-99. Epub 2021 Apr 20.

IRCCS Mondino Foundation, Pavia, Italy.

Introduction/aims: Paramyotonia congenita (PMC) is a skeletal muscle sodium channelopathy characterized by paradoxical myotonia, cold sensitivity, and exercise/cold-induced paralysis. Treatment with sodium-channel-blocking antiarrhythmic agents may expose patients to a risk of arrhythmia or may be poorly tolerated or ineffective. In this study we explored the effectiveness of non-antiarrhythmic sodium-channel blockers in two patients with PMC.

Methods: Earlier treatment with mexiletine was discontinued for gastrointestinal side effects in one of the patients and lack of clinical benefit in the other. One patient received lacosamide, ranolazine, and buprenorphine, and the other was given buprenorphine only. Drug efficacy was assessed by clinical scores, timed tests, and by long and short exercise tests.

Results: In both patients, buprenorphine improved pain scores by at least 50%, stiffness and weakness levels, and handgrip/eyelid-opening times. The fall in compound muscle action potential (CMAP) during short exercise normalized in both patients at baseline, and improved after cooling. During long exercise, one patient showed an earlier recovery of CMAP, and the other patient had a less severe decrease (<60%). With buprenorphine, the fall in CMAP induced by cooling normalized in one patient (from -72% to -4%) and improved (from -49% to -37%) in the other patient.

Discussion: Buprenorphine showed promising results for the treatment of exercise-induced paralysis and cold intolerance in the two patients assessed. The exercise test may be useful for quantitative assessment of treatment response. Further studies on a larger number of patients, under carefully controlled conditions, should be considered to address the effectiveness and long-term tolerability of this therapeutic option.
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http://dx.doi.org/10.1002/mus.27249DOI Listing
July 2021

Camptocormia in idiopathic normal pressure hydrocephalus: a case report.

Acta Neurol Belg 2021 Apr 3. Epub 2021 Apr 3.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Via Mondino 2, 27100, Pavia, Italy.

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http://dx.doi.org/10.1007/s13760-021-01666-6DOI Listing
April 2021

Clinical and Electrophysiological Outcome Measures of Patients With Post-Infectious Neurological Syndromes Related to COVID-19 Treated With Intensive Neurorehabilitation.

Front Neurol 2021 12;12:643713. Epub 2021 Mar 12.

Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

The clinical spectrum of coronavirus disease 2019 (COVID-19), due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, may be quite wide, including neurological symptoms. Among them, para-infectious or post-infectious neurological syndromes (PINS), caused by an inflammatory response against the central and/or peripheral nervous system, have been reported. The aim of this paper is to illustrate the functional and neurophysiological recovery in a series of subjects with COVID-19-related PINS who underwent intensive neurorehabilitation. Five patients with PINS associated with COVID-19 were evaluated at baseline and followed up for 6 months. Three of them had polyradiculoneuropathy and two patients had myelitis. The onset of the neurological syndromes was temporally associated with the SARS-CoV-2 infection. After completing the acute neurological treatments in the intensive care unit, patients underwent a personalized multidisciplinary rehabilitation program. An in-depth clinical, functional, and electrophysiological assessment was carried out at baseline and at 3- and 6-month follow-ups. Among patients with polyradiculoneuropathy, the electrophysiological evaluation at baseline disclosed an acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in two patients and an acute motor and sensory axonal neuropathy (AMSAN) in the third patient. At follow-up, the electrophysiological features improved in one subject with AIDP and were stable in the remaining two cases. The functional assessment after neurorehabilitation showed global recovery and full independence in walking and in activities of daily life in one patient and mild improvement in the other two cases. Of the two subjects with myelitis, the baseline electrophysiological examination showed a prolonged central motor conduction time, which returned to normal in one patient, whereas it improved but remained pathological in the other patient at follow-up. The neurorehabilitation led to a substantial functional improvement in both subjects. This is the first study to describe clinical and electrophysiological aspects along with medium-term outcome in patients with COVID-19-related neurological manifestations who underwent an intensive rehabilitation program. The functional outcome following neurorehabilitation in patients with PINS related to SARS-CoV-2 infection is variable. In our small case series, subjects with polyradiculoneuropathy had a poorer recovery compared to patients with myelitis. The clinical course largely paralleled the follow-up electrophysiological findings.
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http://dx.doi.org/10.3389/fneur.2021.643713DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7994589PMC
March 2021

Electrophysiological features of acute inflammatory demyelinating polyneuropathy associated with SARS-CoV-2 infection.

Neurophysiol Clin 2021 Mar 18;51(2):183-191. Epub 2021 Feb 18.

Statistics Unit, Department of Economics, University "G. d'Annunzio", Pescara, Italy.

Objective: To assess whether patients with acute inflammatory demyelinating polyneuropathy (AIDP) associated with SARS-CoV-2 show characteristic electrophysiological features.

Methods: Clinical and electrophysiological findings of 24 patients with SARS-CoV-2 infection and AIDP (S-AIDP) and of 48 control AIDP (C-AIDP) without SARS-CoV-2 infection were compared.

Results: S-AIDP patients more frequently developed respiratory failure (83.3% vs. 25%, P=0.000) and required intensive care unit (ICU) hospitalization (58.3% vs. 31.3%, P=0.000). In C-AIDP, distal motor latencies (DMLs) were more frequently prolonged (70.9% vs. 26.2%, P=0.000) whereas in S-AIDP distal compound muscle action potential (dCMAP) durations were more frequently increased (49.5% vs. 32.4%, P=0.002) and F waves were more often absent (45.6% vs. 31.8%, P=0.011). Presence of nerves with increased dCMAP duration and normal or slightly prolonged DML was elevenfold higher in S-AIDP (31.1% vs. 2.8%, P=0.000);11 S-AIDP patients showed this pattern in 2 nerves.

Conclusion: Increased dCMAP duration, thought to be a marker of acquired demyelination, can also be oserved in critical illness myopathy. In S-AIDP patients, an increased dCMAP duration dissociated from prolonged DML, suggests additional muscle fiber conduction slowing, possibly due to a COVID-19-related hyperinflammatory state. Absent F waves, at least in some S-AIDP patients, may reflect α-motor neuron hypoexcitability because of immobilization during the ICU stay. These features should be considered in the electrodiagnosis of SARS-CoV-2 patients with weakness, to avoid misdiagnosis.
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http://dx.doi.org/10.1016/j.neucli.2021.02.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7891083PMC
March 2021

Isolated bulbar palsy after SARS-CoV-2 infection.

Lancet Neurol 2021 03;20(3):169-170

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, 27100 Pavia, Italy; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

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http://dx.doi.org/10.1016/S1474-4422(21)00025-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7906749PMC
March 2021

A Rare Syndrome Causing Neurogenic Dysphagia.

Dysphagia 2020 Nov 4. Epub 2020 Nov 4.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Via Mondino 2, 27100, Pavia, Italy.

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http://dx.doi.org/10.1007/s00455-020-10208-wDOI Listing
November 2020

COVID-19 and Guillain-Barré Syndrome: A Case Report and Review of Literature.

Front Neurol 2020 21;11:909. Epub 2020 Aug 21.

Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

During the recent coronavirus disease 2019 (COVID-19) outbreak in Northern Italy, we observed a 57-year-old man developing acute motor-sensory axonal neuropathy, a variant of Guillain-Barré syndrome (GBS), 12 days after severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. Similarly to other bacterial and viral infections, dysregulation of the immune system due to post-infectious mechanisms, such as the molecular mimicry, could lead to an indirect damage of the peripheral nervous system related to SARS-CoV-2. GBS causes motor dysfunctions that are not easily recognizable in non-neurological settings or in patients requiring ventilatory assistance. Several reports also suggested that GBS and Miller Fisher syndrome (MFS) could be neurological complications of COVID-19. Therefore, we performed a review of the 29 articles so far published, describing 33 GBS cases and five MFS cases associated with SARS-CoV-2 infection. We recommend awareness of this rare, but treatable, neurological syndrome, which may also determine a sudden and otherwise unexplained respiratory deterioration in COVID-19 patients.
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http://dx.doi.org/10.3389/fneur.2020.00909DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7471770PMC
August 2020

KCTD17-related myoclonus-dystonia syndrome: clinical and electrophysiological findings of a patient with atypical late onset.

Parkinsonism Relat Disord 2020 09 6;78:129-133. Epub 2020 Aug 6.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Pavia, Italy.

Introduction: Myoclonus-dystonia is a rare syndrome typically occurring during childhood or adolescence, mainly due to SGCE pathogenic variants. Early-onset, atypical presentations of myoclonus-dystonia have recently been associated with KCTD17 variants. In these cases, laryngeal involvement was reported in the advanced stages.

Methods: We evaluated a 52-year-old man with myoclonus-dystonia and positive family history. He underwent an electromyographic investigation of vocal cord and forearm muscles. Whole-exome sequencing was also performed.

Results: Onset of symptoms was at 51 years with dysphonia and vocal tremor. Electromyography disclosed abductor spasmodic dysphonia and laryngeal myoclonus. The patient later developed writer's cramp, upper limb myoclonus, and blepharospasm. Botulinum toxin injection led to improvement of the writer's cramp and to a lesser extent of the spasmodic dysphonia. Genetic analysis identified a heterozygous missense variant in exon 2 of KCTD17: c.229 C > A (p.Leu77Ile), consistently predicted as damaging.

Conclusions: We suggest that the KCTD17-associated phenotypic spectrum may include late onset (even in late adulthood) as well as early and prominent laryngeal involvement.
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http://dx.doi.org/10.1016/j.parkreldis.2020.07.026DOI Listing
September 2020

Reproducibility and reaction time of swallowing as markers of dysphagia in parkinsonian syndromes.

Clin Neurophysiol 2020 09 9;131(9):2200-2208. Epub 2020 Jul 9.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Pavia, Italy.

Objective: To investigate reproducibility and reaction time of oropharyngeal swallowing in patients with Parkinson's disease (PD) and atypical parkinsonisms (APs).

Methods: We enrolled 19 patients with PD, 30 with APs, and 20 healthy subjects. Presence and severity of dysphagia were assessed with clinical and fiberoptic endoscopic evaluations of swallowing. Reproducibility of the oral and pharyngeal phases of swallowing were respectively assessed by calculating the 'similarity index' of the electromyography activity of the submental/suprahyoid muscles and of the laryngeal-pharyngeal mechanogram during consecutive swallows. These were performed both 'on command' and spontaneously. The swallowing reaction time was also recorded.

Results: Reproducibility of the oral phase of swallowing was reduced in patients with dysphagia, mainly when swallowing 'on command'. Swallowing reaction time was prolonged in dysphagic patients. These electrophysiological parameters did not vary among different parkinsonian syndromes and correlated with dysphagia severity.

Conclusions: Increased variability of oral swallowing automatisms and abnormal sensorimotor integration may be of relevance for the pathophysiology of dysphagia in parkinsonian syndromes.

Significance: The electrophysiological assessment represents a valuable tool to investigate swallowing alterations in parkinsonian syndromes. It may also provide useful insights into clinical severity and pathophysiology of dysphagia, giving clues for the choice of the best therapeutic approach.
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http://dx.doi.org/10.1016/j.clinph.2020.06.018DOI Listing
September 2020

Intranasal midazolam for treating acute respiratory crises in a woman with stiff person syndrome.

Neurol Neuroimmunol Neuroinflamm 2020 07 1;7(4). Epub 2020 Apr 1.

From the Department of Brain and Behavioral Sciences (G. Cosentino, C.T.), University of Pavia; Clinical Neurophysiology Unit (G. Cosentino, E.A.), IRCCS Mondino Foundation, Pavia; Neurophysiopathology Department (M.R.), Villa Sofia-Cervello Hospital; Division of Pulmonology (DIBIMIS) (M.A.), Department of Internal Medicine, Villa Sofia-Cervello Hospital, Palermo; Department of Biomedicine Neuroscience and Advanced Diagnostics (F.B.), University of Palermo; Neurorehabilitation Unit (C.T.), IRCCS Mondino Foundation, Pavia; and Institute for Biomedical Research and Innovation (G. Crescimanno), Italian National Research Council, Palermo; Centre for Respiratory Complications of Neuromuscular Rare Disease (G. Crescimanno), Villa Sofia-Cervello Hospital, Palermo, Italy.

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http://dx.doi.org/10.1212/NXI.0000000000000715DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7176251PMC
July 2020

Anodal transcranial direct current stimulation and intermittent theta-burst stimulation improve deglutition and swallowing reproducibility in elderly patients with dysphagia.

Neurogastroenterol Motil 2020 05 23;32(5):e13791. Epub 2020 Jan 23.

Clinical Neurophysiology Unit, IRCCS Mondino Foundation, Pavia, Italy.

Background: Dysphagia in the elderly, known as presbydysphagia, has become a relevant public health problem in several countries. Swallowing disorders may be a consequence of different neurological disorders (secondary presbydysphagia) or the expression of the aging process itself (primary presbydysphagia). We aimed to test the therapeutic potential of two different non-invasive brain stimulation (NIBS) techniques in subjects with primary or secondary presbydysphagia.

Methods: A blinded randomized controlled trial with crossover design was carried out in 42 patients, randomly assigned to anodal transcranial direct current stimulation (tDCS) or intermittent theta-burst stimulation (TBS) group. Both tDCS and TBS were applied for 5 consecutive days over the right swallowing motor cortex. The swallowing function was assessed before and 1 and 3 months after the stimulation using the Dysphagia Outcome and Severity Scale (DOSS), scored based on clinical assessment and fiberoptic endoscopic evaluation of swallowing. An electrophysiological method was also applied to evaluate changes in the reproducibility of the swallowing behavior.

Key Results: Both real tDCS and TBS had beneficial effects on the swallowing function in patients with primary and secondary presbydysphagia. Anodal tDCS resulted in an improvement of 0.5 points in DOSS at 1-month follow-up (P = .014), whereas intermittent TBS induced an increase of 0.7 and 0.6 points at 1- and 3-month follow-up evaluations, respectively (P = .0001 and P = .005, respectively). Reproducibility of both the oral and pharyngeal phases of swallowing significantly increased at 1-month follow-up.

Conclusions And Inferences: Our results suggest that non-invasive cortical stimulation may be useful for dysphagia recovery in elderly patients.
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http://dx.doi.org/10.1111/nmo.13791DOI Listing
May 2020

Vocal cord electromyographic correlates of stridor in multiple system atrophy phenotypes.

Parkinsonism Relat Disord 2020 01 27;70:31-35. Epub 2019 Nov 27.

Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy.

Introduction: Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by dysautonomia in combination with parkinsonian and cerebellar signs. Stridor may also occur and it is associated with life-threatening events and poor prognosis. The pathophysiology of stridor in MSA is still debated.

Objective: To define correlations between diurnal electromyographic (EMG) abnormalities of vocal cord muscles and stridor in MSA phenotypes.

Methods: We recruited 60 patients with "probable" MSA (45 with parkinsonian [MSA-P] and 15 with cerebellar phenotype [MSA-C]). Nocturnal stridor was detected with video-polysomnography, whereas diurnal stridor was clinically noted when present. A diurnal kinesiologic EMG study of the adductor thyroarytenoid and the abductor posterior cricoarytenoid muscles was also performed.

Results: Among subjects with nocturnal stridor, MSA-P patients predominantly showed a paradoxical burst-like activation of the adductor thyroarytenoid muscle during inspiration. This dystonic pattern was associated with nocturnal stridor in MSA-P (odds ratio [OR] = 23.64, 95% confidence interval [CI] 3.42-70.77, p < 0.001). Conversely, MSA-C patients with nocturnal stridor mainly had additional neurogenic findings of vocal cord muscles. This dystonic-plus pattern correlated with nocturnal stridor in MSA-C (OR = 17.21, 95% CI 4.17-74.92, p < 0.01). The findings of diurnal stridor paralleled the observations for nocturnal stridor.

Conclusions: The pathophysiology of stridor may differ between MSA phenotypes, possibly related to dysfunctional supranuclear mechanisms in MSA-P (dystonic pattern) and to additional nuclear damage in MSA-C (dystonic-plus pattern).
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http://dx.doi.org/10.1016/j.parkreldis.2019.11.025DOI Listing
January 2020

Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype.

Neurol Neuroimmunol Neuroinflamm 2020 01 21;7(1). Epub 2019 Nov 21.

From the Department of Brain and Behavioral Sciences (A.C., I.C., G.C., R.C., E.V.), University of Pavia, Pavia, Italy; Department of Neuromuscular Disease (A.C.), UCL Queen Square Institute of Neurology, London, United Kingdom; Neuroalgology Unit (R.L., P.D., L.P., G.L.), IRCCS Fondazione Istituto Neurologico "Carlo Besta," Milan, Italy; Department of Neurosciences (C.B., M.R., A.S.), University of Padova, Padova, Italy; IRCCS Mondino Foundation (I.C., G.C., E.Z., R.C., M.G., E.V., E.A., A.B., D.F.), Pavia, Italy; Department of Neuroscience (L.B., C.D.M., A.S.), Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DiNOGMI), University of Genova, Genova, Italy; IRCCS Ospedale Policlinico San Martino (L.B., C.D.M., A.S.), Genova, Italy; Department of Neurosciences (F.M., E.S., A.T.), Odontostomatological and Reproductive Sciences, University of Naples "Federico II," Naples, Italy; Fondazione Policlinico Universitario Agostino Gemelli-IRCCS. UOC Neurologia (M.L., A.R., M.S.), Rome, Italy; Università Cattolica del Sacro Cuore (M.L., A.R., M.S.), Rome, Italy; Section of Neurology (S.F., S.M.), Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy; Department of Neurology and Stroke Unit (A.M.C.), Ospedale di Circolo/Fondazione Macchi, Varese, Italy; Department of Systems Medicine (G.A.M., G.M.), University of Rome Tor Vergata, Rome, Italy; Neurological Department (A.R.), ASST Lecco; Ospedale Treviglio ASST Bergamo Ovest (M.C.), Italy; Department of Neurology (R.F., F.C.), San Raffaele Scientific Institute, Milan, Italy; Department of Neurorehabilitation Sciences (M.C.), Casa Cura Policlinico (CCP), Milan, Italy; Department of Clinical and Experimental Medicine (A.M.), University of Messina, Messina, Italy; Department of Medicine, Surgery and Neurosciences (F.G.), University of Siena, Italy; Referral Center for Neuromuscular Diseases and ALS (L.K., E.M.), AP-HM, Timone University Hospital, Marseille, France; Université de Bordeaux (C.M.), Interdisciplinary Institute for Neuroscience, Bordeaux, France; Humanitas Clinical and Research Center (C.G., P.D., E.N.-O.), Milan University, Milan, Italy; IRCCS Centro Neurolesi "Bonino Pulejo" (C.S.), Messina, Italy; Department of Biomedical and Clinical Sciences "Luigi Sacco" (G.B., G.L.), University of Milan, Milan, Italy; and Institute for Neurosciences of Montpellier (J.D.), INSERM U1051, Montpellier University, Hopital Saint Eloi, Montpellier, France.

Objective: To assess the prevalence and isotypes of anti-nodal/paranodal antibodies to nodal/paranodal proteins in a large chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) cohort, compare clinical features in seronegative vs seropositive patients, and gather evidence of their isotype-specific pathogenic role.

Methods: Antibodies to neurofascin-155 (Nfasc155), neurofascin-140/186 (Nfasc140/186), contactin-1 (CNTN1), and contactin-associated protein 1 (Caspr1) were detected with ELISA and/or cell-based assay. Antibody pathogenicity was tested by immunohistochemistry on skin biopsy, intraneural injection, and cell aggregation assay.

Results: Of 342 patients with CIDP, 19 (5.5%) had antibodies against Nfasc155 (n = 9), Nfasc140/186 and Nfasc155 (n = 1), CNTN1 (n = 3), and Caspr1 (n = 6). Antibodies were absent from healthy and disease controls, including neuropathies of different causes, and were mostly detected in patients with European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) definite CIDP (n = 18). Predominant antibody isotypes were immunoglobulin G (IgG)4 (n = 13), IgG3 (n = 2), IgG1 (n = 2), or undetectable (n = 2). IgG4 antibody-associated phenotypes included onset before 30 years, severe neuropathy, subacute onset, tremor, sensory ataxia, and poor response to intravenous immunoglobulin (IVIG). Immunosuppressive treatments, including rituximab, cyclophosphamide, and methotrexate, proved effective if started early in IVIG-resistant IgG4-seropositive cases. Five patients with an IgG1, IgG3, or undetectable isotype showed clinical features indistinguishable from seronegative patients, including good response to IVIG. IgG4 autoantibodies were associated with morphological changes at paranodes in patients' skin biopsies. We also provided preliminary evidence from a single patient about the pathogenicity of anti-Caspr1 IgG4, showing their ability to penetrate paranodal regions and disrupt the integrity of the Nfasc155/CNTN1/Caspr1 complex.

Conclusions: Our findings confirm previous data on the tight clinico-serological correlation between antibodies to nodal/paranodal proteins and CIDP. Despite the low prevalence, testing for their presence and isotype could ultimately be part of the diagnostic workup in suspected inflammatory demyelinating neuropathy to improve diagnostic accuracy and guide treatment.

Classification Of Evidence: This study provides Class III evidence that antibodies to nodal/paranodal proteins identify patients with CIDP (sensitivity 6%, specificity 100%).
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http://dx.doi.org/10.1212/NXI.0000000000000639DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935837PMC
January 2020

Stridor in multiple system atrophy: Consensus statement on diagnosis, prognosis, and treatment.

Neurology 2019 10;93(14):630-639

From the IRCCS (P.C., G.C.-B., G.G., F.P., L.V.), Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie (P.C., G.C.-B., G.G., P.M., F.P.), Università di Bologna, Bologna, Italy; Department of Neurology (E.E.B., P.A.L.), Mayo Clinic, Rochester, MN; Multidisciplinary Sleep Unit (A.I.), Neurology Service, Hospital Clinic de Barcelona, IDIBAPS CIBERNED, Barcelona, Spain; UCL Queen Square Institute of Neurology (N.Q.), Queen Square, London; Parkinson's Disease and Movement Disorders Unit (E.T.), Neurology Service, Hospital Clinic de Barcelona; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (E.T.), University of Barcelona (UB), and Centre for Networked Biomedical Research on Neurodegenerative Diseases (CIBERNED), Barcelona, Spain; Department of Neurology (G.K.W.), Innsbruck Medical University, Innsbruck, Austria; Department of Neuroscience (G.A.), Rehabilitation, Ophthalmology, Genetics and Maternal Child Health, University of Genoa, Genoa, Italy; The Multiple System Atrophy Coalition, Inc. (P.B.), Charlotte, NC; Neurophysiopathology Unit (E.A.), IRCCS "C. Mondino" Foundation, Pavia, Italy; Department of Clinical Neurophysiology (I.G.), CHU de Bordeaux, Bordeaux, France; Université de Bordeaux (I.G.), Institut de Neurosciences Cognitives et Intégratives d'Aquitaine, Bordeaux, France; CNRS (I.G.), Institut de Neurosciences Cognitives et Intégratives d'Aquitaine, Bordeaux, France; Department of Neurology (T.O.), Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Minami Uonuma, Niigata, Japan; Parkinson's Disease and Movement Disorders Unit (C.P., N.G.P.), IRCCS "C. Mondino" Foundation, Pavia; Dipartimento di Medicina Specialistica (C.V.), Diagnostica e Sperimentale (DIMES), University of Bologna, Bologna, Italy; Dipartimento di Scienze biomediche e chirurgico specialistiche (C.V.), University of Ferrara, Ferrara, Italy; Department of Neurosciences (A.A.), University of Padua, Padua, Italy; Department of Clinical and Motor Neuroscience (A.P.B.), UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London; Spinal Unit (J.B.), Montecatone Rehabilitation Institute, Imola, Italy; Department of Neurology (H.K.), New York University School of Medicine, New York, NY; Department of Medicine (M.T.P.), Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy; "Luigi Sacco" Department of Biomedical and Clinical Sciences (N.P., A.S.), University of Milan, Milan, Italy; Service de Neurologie (F.T., W.G.M.), CRMR Atrophie Multisystématisée, CHU Bordeaux, Bordeaux, France; Univ. de Bordeaux (F.T., W.G.M.), Institut des Maladies Neurodégénératives, Bordeaux, France; and Department Medicine (W.G.M.), University of Otago, Christchurch, and New Zealand Brain Research Institute, Christchurch, New Zealand.

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure, cerebellar ataxia, and parkinsonism. Laryngeal stridor is an additional feature for MSA diagnosis, showing a high diagnostic positive predictive value, and its early occurrence might contribute to shorten survival. A consensus definition of stridor in MSA is lacking, and disagreement persists about its diagnosis, prognosis, and treatment. An International Consensus Conference among experts with methodological support was convened in Bologna in 2017 to define stridor in MSA and to reach consensus statements for the diagnosis, prognosis, and treatment. Stridor was defined as a strained, high-pitched, harsh respiratory sound, mainly inspiratory, occurring only during sleep or during both sleep and wakefulness, and caused by laryngeal dysfunction leading to narrowing of the rima glottidis. According to the consensus, stridor may be recognized clinically by the physician if present at the time of examination, with the help of a witness, or by listening to an audio recording. Laryngoscopy is suggested to exclude mechanical lesions or functional vocal cord abnormalities related to different neurologic conditions. If the suspicion of stridor needs confirmation, drug-induced sleep endoscopy or video polysomnography may be useful. The impact of stridor on survival and quality of life remains uncertain. Continuous positive airway pressure and tracheostomy are both suggested as symptomatic treatment of stridor, but whether they improve survival is uncertain. Several research gaps emerged involving diagnosis, prognosis, and treatment. Unmet needs for research were identified.
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http://dx.doi.org/10.1212/WNL.0000000000008208DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6814413PMC
October 2019

Pisa syndrome in Idiopathic Normal Pressure Hydrocephalus.

Parkinsonism Relat Disord 2019 09 28;66:40-44. Epub 2019 Jun 28.

Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic address:

Introduction: Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex syndrome of ventriculomegaly that can include parkinsonian-like features besides the classical triad of cognitive decline, urinary incontinence, and gait/balance disturbances. Pisa syndrome (PS) is a postural abnormality often associated with parkinsonism and defined as lateral trunk flexion greater than 10° while standing that resolves in the supine position. We reported a case series of classical "fixed" PS and one case of "Metronome" recurrent side-alternating PS in iNPH, displaying opposite electromyographic patterns of paraspinal muscles.

Methods: Eighty-five iNPH patients were followed longitudinally for at least one year through scheduled clinical and neuropsychological visits.

Results: Five (5.9%) subjects revealed PS. None of them had nigrostriatal dopaminergic involvement detected by [123I]FP-CIT SPECT. Among these patients, four had "fixed" PS, whereas one showed a recurrent side-alternating PS which repeatedly improved after ventriculo-peritoneal shunt and following adjustments of the valve-opening pressure of the shunt system.

Discussion: This is the first case series of PS in iNPH and the first report of "Metronome" PS in iNPH. The prompt response of the abnormal trunk postures through cerebrospinal fluid (CSF) shunt surgery suggests a causative role of an altered CSF dynamics. PS and gait disorders in iNPH could be explained by a direct involvement of cortico-subcortical pathways and subsequent secondary brainstem involvement, with also a possible direct functional damage of the basal ganglia at the postsynaptic level, due to enlargement of the ventricular system and impaired CSF dynamics. The early detection of these cases supports a proper surgical management.
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http://dx.doi.org/10.1016/j.parkreldis.2019.06.024DOI Listing
September 2019

OnabotulinumtoxinA Reduces Temporal Pain Processing at Spinal Level in Patients with Lower Limb Spasticity.

Toxins (Basel) 2019 06 20;11(6). Epub 2019 Jun 20.

Neurorehabilitation Unit, IRCCS Mondino Foundation, 27100 Pavia, Italy.

Spasticity is a muscle tone disorder associated with different neurological conditions. Spasticity could be associated with pain, high disability, poor functional recovery, and reduced quality of life. Botulinum neurotoxin type A (BoNT-A) is considered a first-line treatment for spasticity and, more recently, it also represents a therapeutic option for various chronic pain conditions. In this open label study, we aim to evaluate the effect of the BoNT-A on the spinal nociception in patients affected by spasticity of the lower limbs with associated pain with predominantly neuropathic features. Ten patients with stroke, 10 with multiple sclerosis and 5 with spinal cord injury were enrolled in the study. They were tested with clinical scales (neuropathic pain scale inventory (NPSI), numerical rating scale (NRS), modified Ashworth scale (MAS) and with the nociceptive withdrawal reflex at lower limbs to explore the spinal temporal summation threshold at baseline and 30 day after BoNT-A injection. OnabotulinumtoxinA (50 to 200 units per site) was injected in the lower limb muscles according to the distribution of spasticity. No significant differences were found at baseline for neurophysiological features across groups. After the BoNT-A injection, we recorded a significant reduction in MAS and NRS scores. Regarding the neurophysiological parameters, we described a significant increase in the temporal summation threshold after the BoNT-A injection. Our data supports the hypothesis that peripherally injected OnabotulinumtoxinA modulates the excitability of spinal cord nociceptive pathways. This activity may take place irrespective of the effect of the drug on spasticity.
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http://dx.doi.org/10.3390/toxins11060359DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6628414PMC
June 2019

Subclinical dysphagia in task-specific mouth tremor triggered by drinking.

Clin Neurophysiol 2019 08 18;130(8):1289-1291. Epub 2019 May 18.

Unit of Neurology & Neurorehabilitation, IRCCS Neuromed, Via Atinense 18, 86077 Pozzilli, IS, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.clinph.2019.05.009DOI Listing
August 2019

A pilot study on the efficacy of transcranial direct current stimulation applied to the pharyngeal motor cortex for dysphagia associated with brainstem involvement in multiple sclerosis.

Clin Neurophysiol 2019 06 11;130(6):1017-1024. Epub 2019 Apr 11.

Unit of Neurology and Neurorehabilitation, IRCCS Neuromed, Via Atinense 18, 86077 Pozzilli, IS, Italy.

Objective: we investigated the effect of anodal transcranial direct current stimulation (tDCS) applied over the pharyngeal motor area in dysphagia associated with multiple sclerosis (MS).

Methods: Eighteen MS patients with dysphagia associated with brainstem involvement were randomized to receive either "real" or "sham" tDCS.

Primary Outcome: The Penetration/Aspiration Scale (PAS).

Secondary Outcomes: changes in electromyographic (EMG) parameters and pharyngeal cortical motor evoked potentials (MEPs). Patients were evaluated at baseline (T), at the end of 5-session cycle of tDCS stimulations (T), after two (T), and four (T) weeks.

Results: the PAS values were significantly lower in the active group than in "sham" group at T, and at T. Over the post-stimulation periods, PAS significantly improved only in the "real" group. As regards the secondary outcomes, we observed a statistically significant difference between the 2 groups only in the MEPs amplitude at T. The comparison between baseline and each of the post-stimulation times showed significant differences only of the "real" group across all the secondary parameters.

Conclusions: Our findings support a beneficial effect of anodal tDCS applied to the pharyngeal motor cortex in MS-associated dysphagia.

Significance: Considering its safety and efficacy, tDCS may represent an important resource in MS-associated dysphagia.
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http://dx.doi.org/10.1016/j.clinph.2019.04.003DOI Listing
June 2019
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