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Background: Sildenafil citrate (Viagra®) is used to treat male erectile dysfunction; however, little is known about the effects of sildenafil overdose and intoxication. We report a patient who presented with cerebral infarction and rhabdomyolysis after intentional sildenafil intoxication., Case Report: A 61-year-old man visited the Emergency Department complaining of dysarthria about 1 h after taking more than 30 sildenafil tablets with the intention to commit suicide. Dysarthria and dizziness were observed, but there were no other neurological symptoms. The creatine kinase level was elevated to 3118 U/L, and the patient was diagnosed with rhabdomyolysis. Brain magnetic resonance imaging revealed multiple scattered acute cerebral infarctions in both midbrain artery branches. At 4 h post-intoxication, the dysarthria had improved and we initiated dual antiplatelet therapy for cerebral infarction. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should be able to anticipate and treat complications like cerebral infarction and rhabdomyolysis after sildenafil intoxication., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Ischemic stroke is relatively rare in children, leading to a low level of suspicion and delayed diagnosis, particularly in cases of posterior circulation occlusion when symptoms are less indicative. Occlusion of the artery of Percheron (AOP) results in nonspecific neurologic symptoms, including drowsiness, aphasia or dysarthria, ophthalmoplegia, ataxia, and dysmetria. Previous reports, mainly in adults, described late diagnosis and severe residual disability., Case Report: We report a case of a 16-year-old male who presented to the pediatric emergency department with altered mental status. There was no history of trauma or intoxication. The main symptoms included confusion, slurred speech, and multiple falls starting 1 h before arrival to the emergency department. No motor deficits or other focal signs were noticed. The patient's consciousness gradually decreased followed by apneic events. Routine laboratory tests, urinary toxic screen, and a computed tomography scan of the head were normal. A magnetic resonance imaging scan of the brain revealed bilateral restrictive changes in the thalamus. A diagnosis of AOP occlusion was made, and the patient was treated with tissue plasminogen activator (6 h after symptom onset). He was extubated on day 4 and discharged on the day 10 of admission without any neuropsychological deficit. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Posterior circulation stroke in the pediatric population is a diagnostic challenge that often results in suboptimal treatment and unfavorable outcomes. Prompt imaging studies in children with nonspecific altered mental status enable timely diagnosis and thrombolytic treatment that may substantially improve the outcome., (Copyright © 2022. Published by Elsevier Inc.)

Background/aim: Levodopa-carbidopa intestinal gel (LCIG) is an effective treatment modality in the management of advanced Parkinson’s disease (PD) despite frequent adverse events and different rates of dropouts. Efficacy and safety data regarding Turkish patients on LCIG are limited. This study aims to report in detail the efficacy and adverse effect profile of LCIG among advanced PD patients from a Turkish center for movement disorders., Materials and Methods: Twenty-two patients (50% male) who started receiving LCIG between December 2014 and March 2020 were recruited. The efficacy of LCIG was assessed with the Unified Parkinson’s Disease Rating Scale (UPDRS III), Clinical Global İmprovement (CGI) scale, and Quality of Life scale (PDQ8). Improvements in gait disorders and nonmotor features were also questioned. Adverse events (AE) were collated into 3 topics: related to percutaneous endoscopic gastrojejunostomy (PEG-J), device-related, and LCIG infusion-related., Results: Mean age and pre-LCIG disease duration were 66.7 (8.8) and 13.3 (8.0) years respectively. UPDRS III scores and H-Y scale assessments significantly improved. Better quality of life scores, clinical global improvements, and improvements in dysarthria, dysphagia, and gait were observed. None of our patients dropped out or died during a mean 17.5-month (12.3) period. Overall 20 (90.9%) patients experienced at least one AE. Twelve patients had PEG-J–related complications; three had acute abdomen. Eight (36.4%) patients had device-associated problems. Half of the patients required at least one additional endoscopic procedure and 7 had a device replaced. Mean body weight decreased from 69.5 to 62.5 kg and seven patients had newly onset PNP at a follow-up electromyography. Dyskinesia related to LCIG infusion was observed in 5 (22.7%) patients. There was no significant increase in hallucination among patients., Conclusion: LCIG is an efficient treatment modality in the management of Turkish patients with advanced Parkinson’s disease. Although most of the patients had at least one AE, none of them dropped out. Patient selection, patient compliance, and collaborative management are important steps affecting the success of modality., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Rationale: Anti-IgLON5 disease was first described as a progressive antibody-associated encephalopathy, with multiple non-specific clinical symptoms including sleep dysfunction, bulbar symptoms, progressive supranuclear palsy-like syndrome, cognitive impairment, and a variety of movement disorders. This newly discovered disease presents with unremarkable or unspecific brain magnetic resonance imagings (MRI), and have poor responsiveness to immunotherapy., Patient Concerns: In this case, a 37-year-old man presented with 4-day history of gait instability, dysarthria, and oculomotor abnormalities. The initial neurologic examination revealed mild unsteady gait, subtle dysarthria, and left abducent paralysis., Diagnosis: The patient was diagnosed with anti-IgLON5 disease, based on clinical features and positive anti-IgLON5 antibodies in serum., Interventions: Initially, the patient was treated with high dosages of methylprednisolone and immunoglobulins.Outcomes: The symptoms of patient rapidly improved after high-dose intravenous methylprednisolone and immunoglobulins., Conclusions: In this paper, we report a new case of anti-IgLON5 disease with major symptoms of gait instability, dysarthria, and oculomotor abnormalities, with distinctive brain MRI findings, and responsive to immunotherapy., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: Investigate the association between levodopa therapy and vocal characteristics in Parkinson's disease patients., Search Strategy: Studies published at MEDLINE, LILACS, and SciELO, from 1960 to December 2016. A systematic review and meta-analysis was performed using the following keywords: Parkinson's disease; levodopa; L-dopa; voice; speech disorders; dysphonia; dysarthria. After analyzing titles and abstracts, two independent reviewers selected all clinical trials that met the eligibility criteria and selected the articles and the data recorded in a previously standardized table., Selection Criteria: Trials published in English between 1960 and December 2016 individuals with clinical diagnosis of Parkinson's disease; use of levodopa therapy in stable doses; acoustic analysis combined or not with auditory-perceptual analysis to evaluate the vocal parameters under investigation., Data Analysis: The following vocal parameters were analyzed: fundamental frequency (F 0), jitter, and vocal intensity. Standardized mean differences (SMD) were calculated using the Comprehensive Meta-analysis V2 software., Results: Nine articles met the eligibility criteria and were selected, with a total of 119 individuals. From these, six articles with 83 individuals were included in the meta-analysis. During the levodopa therapy "on" state, modifications in F 0 (SMD=0.39; 95% CI - 0.21-0.57) and jitter (SMD=0.23; 95% CI - 0.02-0.45) were observed. Vocal intensity was not affected (SMD=0.09; 95% CI - 0.22-0.39) by levodopa ingestion. Data of the included studies were controversial in the auditory-perceptual analysis of voice., Conclusion: Levodopa therapy modifies F0 and jitter. No changes in vocal intensity were observed in either the "on" or "off" states of levodopa therapy.

Background: Sublingual atropine, dosed at 0.4-0.8 mg, is used by dentists as an antisialogogue to facilitate and increase the speed of procedures. Concentrated ophthalmic atropine drops (10 mg/mL) are commonly used off-label for this purpose. These highly concentrated drops may result in medication errors, atropine toxicity, and the antimuscarinic toxidrome. We report a case of a man who suffered acute delirium and dysarthria (from dry mouth) after an iatrogenic overdose from a dental procedure. His symptoms were initially interpreted as a stroke, but they completely resolved with physostigmine., Case Report: A 57-year-old man presented with acute dysarthria and delirium after a dental procedure; 4 hours earlier he was fitted for a temporary replacement of some premolar/molar teeth. He received sublingual atropine to assist in gingival drying for molding of his prosthesis, but a calculation error resulted in the administration of approximately 113 mg. A stroke evaluation was initially planned; however, 2.5 mg of intravenous physostigmine completely reversed his symptoms. His symptoms reoccurred and were successfully treated twice more with physostigmine; the patient was observed overnight with no additional symptoms and safely discharged the next morning. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ophthalmic atropine drops are highly concentrated and may cause an overdose after ingestion of small amounts. This novel case highlights the importance of considering antimuscarinic poisoning in cases of acute delirium or dysarthria after dental procedures and stands as a reminder to inquire about the use of atropine drops in such cases. Timely recognition of the antimuscarinic toxidrome and appropriate use of physostigmine may prevent unnecessary testing while providing an effective therapy. This case also highlights the need for observation after resolution of delirium treated with physostigmine., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Opercular myoclonic-anarthric status epilepticus (OMASE) is a rare form of epilepsia partialis continua presenting as fluctuating dysarthria, or even anarthria. The condition is caused by an epileptogenic lesion involving the opercular cortex of either hemisphere. Speech impairment is secondary to bilateral epileptic activity affecting the glossopharyngeal muscles. This bilateral nature of the condition is due to the fact that innervation of cranial nerves V, VII, IX, X and XII from the opercular area of the primary motor cortex is bilateral. The aetiology of the condition varies, and includes vascular lesions, tumours, and encephalitis, among other causes. A low threshold for clinical suspicion is necessary in order to ensure the timely initiation of antiepileptic treatment, thereby preventing the condition from becoming drug resistant. We present two cases of OMASE which differ in terms of aetiology, clinical course, and treatment response. [Published with video sequences on www.epilepticdisorders.com].

Purpose: This research characterised perioral muscle reciprocity and amplitude ratio in lower lip during bilabial syllable production [pa] at three rates to understand the neuromotor dynamics and scaling of motor speech patterns in individuals with Parkinson's disease (PD)., Method: Electromyographic (EMG) signals of the orbicularis oris superior [OOS], orbicularis oris inferior [OOI] and depressor labii inferioris [DLI] were recorded during syllable production and expressed as polar-phase notations., Result: PD participants exhibited the general features of reciprocity between OOS, OOI and DLI muscles as reflected in the EMG during syllable production. The control group showed significantly higher integrated EMG amplitude ratio in the DLI:OOS muscle pairs than PD participants. No speech rate effects were found in EMG muscle reciprocity and amplitude magnitude across all muscle pairs., Conclusion: Similar patterns of muscle reciprocity in PD and controls suggest that corticomotoneuronal output to the facial nucleus and respective perioral muscles is relatively well-preserved in our cohort of mild idiopathic PD participants. Reduction of EMG amplitude ratio among PD participants is consistent with the putative reduction in the thalamocortical activation characteristic of this disease which limits motor cortex drive from generating appropriate commands which contributes to bradykinesia and hypokinesia of the orofacial mechanism.

Purpose: In this preliminary study, we examined self-rated communication-related quality of life (CR-QoL) of 10 control participants and 10 individuals with oromandibular dystonia (OMD) and dysarthria receiving therapeutic botulinum toxin (BoNT-A) injections., Method: Participants with OMD and associated dysarthria self-rated CR-QoL pre- and post- BoNT-A injection using the American Speech-Language-Hearing Association's Quality of Communication Life Scale (ASHA QCL; Paul et al., 2004). Control participants self-rated CR-QoL during a single experimental visit., Results: Significant differences were found between control participants and participants with OMD on ratings of CR-QoL across all 5 domains and subdomains of the ASHA QCL. No significant differences in CR-QoL were found over the course of the BoNT-A treatment cycle., Conclusions: CR-QoL was rated lower by participants with OMD as compared with control participants across all ASHA QCL domains/subdomains with "socialization/activities" and "confidence/self-concept" having the largest effect sizes. No differences in CR-QoL were found over the course of the treatment cycle. We advocate for outcome measures that include patient report. The use of patient-reported outcome measures in conjunction with objective or impairment-based outcome measures can help inform meaningful clinical indicators of treatment success. This study adds novel information that may aid our understanding of the experience of living with OMD in this underserviced clinical population.

In a 51-year-old patient of consanguineous parents with a severe neuromuscular phenotype of early-onset ataxia, myoclonia, dysarthria, muscle weakness and exercise intolerance, exome sequencing revealed a novel homozygous variant (c.-264_31delinsCTCACAAATGCTCA) in the mitochondrial FAD-transporter gene SLC25A32. Flavin adenine dinucleotide (FAD) is an essential co-factor for many mitochondrial enzymes and impaired mitochondrial FAD-transport was supported by a reduced oxidative phosphorylation complex II activity in the patient's muscle, decreased ATP production in fibroblasts, and a deficiency of mitochondrial FAD-dependent enzymes. Clinically, the patient showed improvement upon riboflavin treatment, which is a precursor of FAD. Our results confirm the recently reported case of SLC25A32 as a cause of riboflavin-responsive disease. Our patient showed a more severe clinical phenotype compared with the reported patient, corresponding with the (most likely) complete absence of the SLC25A32-encoding MFT (Mitochondrial Folate Transporter) protein.

Botulinum toxin A (Btx-A) injections are used to treat limb spasticity in children with cerebral palsy (CP) resulting in improved gross and fine motor control. This treatment has also been reported to have additional functional effects, but the effect of treatment on speech has not been reported. This report presents results of longitudinal speech evaluation of two children with CP given injections of Btx-A for treatment of limb spasticity. Speech evaluations were accomplished at baseline (date of injections) and 4- and 10-weeks post-injections. Improvements in production of consonants, loudness control, and syllables produced per breath were found. Parental survey also suggested improvements in subjects' speech production and willingness to speak outside the testing situation. Future larger studies are warranted to assess the nature of the changes observed related to Btx-A.

Introduction: Individuals with Parkinson's disease (PD) have to deal with several aspects of voice and speech decline and thus alteration of communication ability during the course of the disease. Among these communication impairments, 3 major challenges include: (1) dysarthria, consisting of orofacial motor dysfunction and dysprosody, which is linked to the neurodegenerative processes; (2) effects of the pharmacological treatment, which vary according to the disease stage; and (3) particular speech modifications that may be language-specific, that is, dependent on the language spoken by the patients. The main objective of the FraLusoPark project is to provide a thorough evaluation of changes in PD speech as a result of pharmacological treatment and disease duration in 2 different languages (French vs European Portuguese)., Methods and Analysis: Individuals with PD are enrolled in the study in France (N=60) and Portugal (N=60). Their global motor disability and orofacial motor functions is assessed with specific clinical rating scales, without (OFF) and with (ON) pharmacological treatment. 2 groups of 60 healthy age-matched volunteers provide the reference for between-group comparisons. Along with the clinical examinations, several speech tasks are recorded to obtain acoustic and perceptual measures. Patient-reported outcome measures are used to assess the psychosocial impact of dysarthria on quality of life., Ethics and Dissemination: The study has been approved by the local responsible committees on human experimentation and is conducted in accordance with the ethical standards. A valuable large-scale database of speech recordings and metadata from patients with PD in France and Portugal will be constructed. Results will be disseminated in several articles in peer-reviewed journals and in conference presentations. Recommendations on how to assess speech and voice disorders in individuals with PD to monitor the progression and management of symptoms will be provided., Trial Registration Number: NCT02753192, Pre-results., Competing Interests: Conflicts of Interest: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Background: Paroxysmal ataxia and dysarthria (PAD) is a relatively rare symptom in Multiple Sclerosis patients. PAD involves transient dysfunction in control, coordination and initiation of speech and/or limb movements., Objective: To describe the successful use of levetiracetam for the treatment of PAD., Methods: Case report., Results: A 37-year-old woman with MS developed PAD approximately 3 months after a multifocal MS relapse. Brain MRI showed a lesion in the posterior aspect of the midbrain as well as in the right posterior internal capsule, both of which were adjacent to the red nucleus. Attack frequency was reduced after starting levetiracetam at a dose of 500mg twice daily, and attacks stopped completely once the dose was increased to 750mg twice daily., Conclusions: Given its advantages (in terms of side effects, safety profile and ease of use compared to other anticonvulsants), we suggest that levetiracetam be considered for management of PAD, and perhaps for other paroxysmal MS symptoms as well., (Published by Elsevier B.V.)

Intravenous (IV) thrombolysis is approved and proven treatment for acute ischemic stroke in the window period of 4.5 hours. The therapeutic benefit is not extended to many patients with prior stroke and recurrent stroke as they are excluded in the protocol for thrombolysis. We report a case of successful IV thrombolysis in a young patient with recent prior stroke and recurrent stroke. A 35-year-old male presented in our emergency with recurrent stroke had a history of acute onset vertigo, headache, and vomiting. He was diagnosed to have posterior circulation stroke before 5 days on the basis of clinical history and neuroimaging. On the day of presentation to our hospital, he had developed new symptom of acute onset right hemiparesis with dysarthria. IV tissue plasminogen activator administered within 2 hours of onset of new symptoms resulted in significant improvement in spite of the recent prior stroke., (Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Paroxysmal dysarthria-ataxia is a rare neurological condition due to ephaptic transmission, generally appearing in multiple sclerosis patients characterized by stereotyped attacks of slurred speech usually accompanied by ataxia, appearing many times a day. Here we describe a patient with an unusual remitting-relapsing form of Bickerstaff's-like brainstem encephalitis who manifested PDA after a relapse with the involvement of a peculiar region below the red nuclei and benefited from lamotrigine., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Paroxysmal ataxia and dysarthria are part of the spectrum of transient neurological disturbances that can be frequently encountered in multiple sclerosis (MS). Prompt recognition of these symptoms is important because they can be the only manifestation of a MS relapse and symptomatic therapy is often beneficial. We report a patient who developed paroxysmal ataxia and dysarthria, documented by video imaging, while he was recovering from a MS relapse. Treatment with carbamazepine resulted in the complete reversal of the paroxysmal ataxia and dysarthria., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Paroxysmal dysarthria is an unusual condition characterised by brief episodes of dysarthria with the sudden onset and frequent recurrence. It has been mainly reported in multiple sclerosis and an association with midbrain lesions has been claimed; however, most of the reported patients had multiple brain alterations so it was difficult to associate this symptom with a specific lesion site. We illustrate the cases of two patients with an isolated demyelinating midbrain lesion presenting paroxysmal dysarthria as the only symptom; both participants had oligoclonal bands in the cerebrospinal fluid and an unremarkable follow-up. Both patients had benefit from carbamazepine treatment, similarly to previously reported cases. Our report confirms that a demyelinating midbrain lesion is sufficient to provoke paroxysmal dysarthria. It is noteworthy that an erroneous diagnosis of psychogenic disorders was initially made in both cases, highlighting the importance not to underestimate isolated paroxysmal symptoms in clinical practice.

Introduction: CLIPPERS syndrome (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory process of the central nervous system whose distinguishing features are the enhancing punctiform lesions in the brainstem that appear in the magnetic resonance images. Clinically, it is accompanied by dysarthria, ataxia and diplopia, and usually responds to treatment with corticoids. Pathologically, T lymphocytes appear infiltrated in the perivascular spaces of the brainstem., Case Report: We report the case of a 40-year-old woman with an initial subacute clinical picture of binocular diplopia, ataxia and dysarthria. The magnetic resonance brain scan revealed T2 hyperintense punctiform lesions in the stem, cerebellum, diencephalons and cortico-subcortical areas of both hemispheres, which were enhanced with contrast. An aetiological study was performed to rule out any underlying infectious, neoplastic or inflammatory origin, the results being negative. The patient was treated on two occasions with methylprednisolone, with a gradual lowering of the dosage, the response being favourable., Conclusions: Diplopia and ataxia, as in our case, are practically always present. The MR findings consist of punctiform enhancing lesions located in the pons extending towards the cerebellum, basal ganglia and corpus callosum, the enhancement gradient becoming lower as the distance increases rostrally away from the cortex, and caudally towards the spinal cord. In the case of our patient, this gradient is not respected, and the density found was similar to that of lesions at the supratentorial level. The differential diagnosis is wide-ranging and justifies an extensive diagnostic study with, in certain cases, a biopsy study of brain tissue. The disease courses in a relapsing-remitting pattern and the earlier steroid therapy is established and the more prolonged it is, the better the prognosis will be.

We report a five-year-old girl presenting with dysphagia, dysarthria, drooling, and generalized tonic convulsions in whom the final diagnosis was acquired epileptiform opercular syndrome. Levetiracetam monotherapy at a dosage of 40 mg/kg/day improved the clinical findings, and seizures were controlled at the end of the first month of treatment. Six months after the initial diagnosis, she presented with speech deterioration and dysarthria. At this time, although sleep and awake electroencephalography (EEG) were normal, FDG-PET showed hypometabolic and hypermetabolic regions in the anterior/inferior and anterior regions of the right frontal lobe, respectively. By increasing before levetiracetam dosage to 50 mg/kg/day, the clinical findings resolved and the patient is still seizure free. Acquired epileptiform opercular syndrome is a rare epileptic disorder in which the seizures are resistant to conventional antiepileptic drugs. Levetiracetam may be an effective antiepileptic drug in controlling seizures and other clinical findings in acquired opercular epileptiform syndrome. Hypometabolic and hypermetabolic regions in FDG-PET study may be due to ongoing seizure activity or impaired glucose metabolism in this disorder., (Copyright © 2012 Elsevier Inc. All rights reserved.)

The word dysphagy was suggested by Nicolatopoulos (1907) and derives from the ancient Greek "duz", which means "difficulty" and "katapinein", which means "to swallow". Generally, the dysphagia is defined on the basis of its origin: oral, pharynx and oesophagus, otherwise by its mechanical or neurological aetiology. The symptoms are dependent on the nature of the lesions in the affected organs. The swallow is a complex motor sequence dependent on the coordinate contraction of the muscle of mouth, of larynx and of the oesophagus. The mechanical action of the swallow helps the liquid or solid food progression from mouth to stomach thanks to cooperation of 31 muscles and 5 cranial nerves and allows swallowing about 580 times approximately. The dysphagy in neurological diseases is mainly due to the following reasons. Increase of vascular cerebral disease, increase of population age and increase of road and work traumas. The difficulties in swallowing causes heavy social problems like meager diet, social isolation and worsening of quality of life. The speech rehabilitation requires the involvement of care givers through a re-educational program that takes place in two periods: the first of relaxation, and the second of restoration of phonodeglutition praxis.

Background: The LARGO study demonstrated that rasagiline 1 mg/day as adjunct to levodopa significantly reduces OFF time to the same magnitude as adjunct entacapone. This substudy of LARGO aimed to assess the effect of rasagiline and entacapone on the motor symptoms of PD during the practically defined OFF state., Methods: LARGO was a randomized, double-blind, multicenter trial that assessed the efficacy and safety of rasagiline (1 mg/day), entacapone (200 mg with each levodopa dose), and placebo in 687 levodopa-treated PD patients with motor fluctuations. A substudy of LARGO measured UPDRS motor scores in the practically defined OFF state in 32 rasagiline, 36 entacapone, and 37 placebo patients., Results: Treatment with rasagiline produced a significant improvement over placebo of 5.64 units in UPDRS motor OFF score (P = 0.013 vs. placebo). By contrast, the effect of adjunct entacapone was not significant (P = 0.14 vs. placebo). Whereas rasagiline also showed a trend in reducing the UPDRS-ADL OFF score (P = 0.058 vs. placebo), no such trend was noted for entacapone (P = 0.26 vs. placebo). Retrospective analysis, using the Bonferroni correction, of UPDRS motor subdomains further revealed that rasagiline, but not entacapone, significantly improved bradykinesia (P < 0.001) and showed trends for improvements in facial expression, speech, and axial impairment during OFF time., Conclusions: This study provides the first objectively measured evidence that adjunct rasagiline 1 mg/day is effective in reducing the severity of motor symptoms in the OFF state. This suggests a continuous effect of rasagiline 1 mg/day throughout the day and night and is consistent with its extended duration of therapeutic action., (© 2011 The Author(s). European Journal of Neurology © 2011 EFNS.)

The authors describe herein the magnetoencephalographic findings and long-term outcome of a girl with acquired opercular epilepsy with oromotor dysfunction. She presented with brief episodes of unconsciousness, tremulous movements of the upper limbs, and negative myoclonus, in addition to convulsive seizures. She also had prolonged episodes of dysarthria and oral motor dysfunction, a gradual decrease in speech output, impairment of finger movements, and deterioration in cognitive performance over several years. Her electroencephalography (EEG) recordings showed notable continuous sharp or sharp-slow discharges during sleep. Brain magnetic resonance images revealed no structural anomalies. Magnetoencephalographic analysis showed broadly distributed epileptic foci around the sylvian fissure, including a secondary source, explaining the specific prolonged neurological dysfunction. Antiepileptic drugs could control her seizures; however, they did not improve the other neurological symptoms or epileptiform discharge on EEG. Administration of low-dose prednisolone over a long period was effective for improving the neurological impairments of this patient.

Objective: The aim of our study was the analysis of fundamental frequency (F(0)) variability (fundamental frequency standard deviation [F(0)SD]) and net speech rate (NSR) in the course of reading in Parkinsonian patients' speech, with special emphasis on the changes of F(0)SD and NSR from the first to the last sentence of the task., Patients and Methods: We examined 138 patients with Parkinson's disease (PD) and 50 age-matched control persons using a standardized reading task with subsequent acoustical analysis of F(0)SD and NSR. A subgroup of 20 PD patients underwent a standardized levodopa challenge., Results: F(0)SD in PD patients was significantly reduced compared with the control group when based on the measurement of the entire reading task. Furthermore, in the PD group, NSR and F(0)SD showed significant changes from the first to the last sentence of the reading task, but no correlation was seen between NSR and F(0)SD. Standardized levodopa administration had no effect on NSR and F(0)SD when related to the entire reading passage, but the aforementioned decline of F(0) variability in the course of reading seemed to be counterbalanced by levodopa administration., Conclusions: In this large series of PD patients, previous findings of reduced F(0)SD in PD were confirmed. Additionally, this is the first analysis to show an increasing reduction of F(0) variability in the course of reading mirroring abnormalities in the dynamical aspects of speech in PD. According to the results of the levodopa challenge, dopaminergic stimulation seems to ameliorate dynamic intonation changes over time, whereas overall intonation variability might be a PD symptom independent of dopaminergic control., (Copyright © 2011 The Voice Foundation. Published by Mosby, Inc. All rights reserved.)

Recent developments in temporary vocal fold (VF) augmentation materials and office-based laryngeal procedures have expanded the role of vocal fold injection (VFI). Although already performed in the laryngology community, trial VFI is yet to be defined in the literature. We sought to define trial VFI and to emphasize its concept and clinical utility through a review of our institution's experience. The study design was a retrospective review of our surgical database. All patients who received a temporary, deep VFI were identified. Patients with VF paralysis, resolved paresis, isolated scar, or laryngeal trauma were excluded. Twenty-five patients had the diagnosis of VF atrophy and/or paresis, and were confirmed to have had their injection for trial purposes by chart review. Trial VFI was performed using one of three temporary injectibles. Ten patients underwent further permanent augmentation. Subjective outcome and Voice Handicap Index 10 (VHI-10) scores were used as outcome measures. Nineteen of 25 patients had a good or partial response to the trial VFI. Ten patients went on to have permanent medialization. Seventy-five percent of the patients with a good response to trial VFI went on to have permanent VF augmentation. All of these patients had a good result from their permanent procedure. Trial VFI is defined as the use of a temporary injectible to augment the VF when the benefit of permanent augmentation is uncertain. In a review of our patients who underwent trial VFI, those with a good response to trial VFI went on to have permanent augmentation more often than those with a lesser response., (Copyright (c) 2010 The Voice Foundation. Published by Mosby, Inc. All rights reserved.)

While the beneficial effect of levodopa on motor impairment in Parkinson's disease (PD) has been well documented, its effect on speech has rarely been examined and the respective literature is inconclusive. The aim of our study was to analyze the effect of short-term levodopa admission and long-term dopaminergic treatment on speech in PD patients in early stages of the disease. Motor examination according to UPDRS III and speech testing were performed in 23 PD patients (9 males; median age 68, 42-78 years) in the early morning after having abstained from dopaminergic medication overnight ("off" state, t0) after administration of 200 mg of soluble levodopa (t1), and at follow-up after 12-14 weeks under stable dopaminergic medication (t2). Speech examination comprised the perceptual rating of global speech performance and an acoustical analysis based upon a standardized reading task. While UPDRS III showed a significant amelioration after L: -dopa application, none of the parameters of phonation, intonation, articulation and speech velocity improved significantly in the "on" state, neither under short-term levodopa administration (t1) nor on stable dopaminergic treatment (t2). However, there was a positive effect of dopaminergic stimulation on vowel articulation in individual patients. Results indicated significant beneficial effect of short-term levodopa administration or long-term dopaminergic medication on different dimensions of speech in PD patients. As some improvement of vowel articulation was seen in individual patients, the pre-existing pattern of speech impairment might be responsible for the different response to pharmacological treatment.

Hemichorea-hemiballisum in patients with hyperglycemia and striatal hyperintensity on T1-weighted magnetic resonance imaging is now an accepted clinical entity. Usually, both the clinical syndrome and neuroimaging abnormalities are reversible. A transient, reversible metabolic impairment within the basal ganglion has been considered a possible cause of this disorder. However, the pathophysiology remains to be unclear. We report a 56-year-old man with a prolonged, uncontrolled hyperglycemia (HbA1C: 13.8%) and striatal hyperintensity on T1-weighted MR imaging presenting as reversible focal neurological deficit and irreversible neuroimaging abnormalities on the fourth month when blood sugar was under control (HbA1C 6.0 mg/dl). We hypothesize that neuroimaging abnormalities in our case may be a sequence of an "ischemic insult" caused by prolonged, uncontrolled hyperglycemia. Whether the signal abnormalities on neuroimaging studies or the clinical syndrome are reversible (patients with HCHB) or irreversible (such as in our case) are based on the degree of ischemic damage.

Unlabelled: Research has found that speaking rate has an effect on voice onset time (VOT). Given that Parkinson disease (PD) affects speaking rate, the purpose of this study was to examine VOT with the effect of rate removed (VOT ratio), along with the traditional VOT measure, in individuals with PD. VOT and VOT ratio were examined in 9 individuals with PD (before and after taking medication), along with 9 matched controls. Place of articulation and vowel height had significant effects on VOT and VOT ratio for all groups, and there were no PD versus control differences. PD medication had a greater effect on VOT than VOT ratio, reflecting a rate-related VOT change rather than a pure VOT change. These data support the usefulness of examining both VOT and VOT ratio with individuals with PD, as this allows for dissociation between rate-related VOT changes and true VOT changes., Learning Outcomes: The reader will be able to understand and describe: (1) voice onset time (VOT) and VOT ratio; (2) how VOT and VOT ratio are affected in individuals with Parkinson disease; and (3) the effect of medication on VOT and VOT ratio., ((c) 2009 Elsevier Inc. All rights reserved.)

We describe three patients with relapsing-remitting multiple sclerosis who presented with paroxysmal dysarthria. In one patient, the symptoms were the only manifestation of an acute relapse. In the other two patients, the attacks appeared during the recovery of a brainstem relapse. All three patients had an acute lower midbrain lesion. The location was central in two patients and left paramedian in the other, and probably involving cerebellothalamocortical pathways. Treatment with carbamazepine was effective.

Two young patients are described who made complete recovery from locked-in syndrome (LIS) after snakebites. LIS was a presenting feature in a patient of presumed snake bite who showed complete response to polyvalent Anti-snake venom (ASV). This case suggests that elapid snake bite should be suspected in unresponsive patient found in early morning in endemic areas of snake bite in monsoon season. The second case was admitted in complete LIS state with history of rapidly progressive ptosis, diplopia, ophthalmoplegia, bulbar symptoms and quadriparesis, 6 hours after snake bite. Complete improvement by ASV in second patient highlights the need of differentiation of complete LIS from coma and brain death in patient of snake bite, as former have good prognosis.

We report a 71 years old man suffering from transient ischemic attack associated with a calcinosis cerebri (Fahr's disease). Arterial hypertension was the unique vascular risk factor disclosed.

The prosodic aspects of hypokinetic dysarthria in Parkinson's disease (PD) have been the focus of numerous reports. Few data on the effects of levodopa on prosody, more specifically on the effects on the variability of prosodic characteristics such as pitch, loudness and speech rate, are available in advanced PD. The relation between these characteristics and comprehensibility is currently unknown. These topics are the focus of the present report. Ten patients with advanced PD were evaluated, with and without medication, by four speech-language pathologists during a reading task. A significant improvement of variability in pitch and loudness was demonstrated after medication intake. Comprehensibility improved following medication administration. Results are compared with previous studies.

Objectives: Hypokinetic dysarthria is commonly encountered in Parkinson's disease (PD). Although the etiology of this dysarthria is multifactorial, disorders of respiration may strongly contribute to it. The aim of this study was to evaluate the effects of levodopa on measures of respiration and intelligibility., Patients and Methods: Vital capacity (VC), sustained vowel phonation (SVP) and phonation quotient (PQ) were determined with and without medication in 25 patients suffering from idiopathic PD. Intelligibility was evaluated by three independent speech pathologists using the word subtest protocol of the Yorkston and Beukelman "Assessment of intelligibility of dysarthric speech" (AIDS)., Results: VC was abnormal in 18/25 patients in the off-condition and 15/25 in the on-condition. SVP was normal in 22/25 patients in the off-state and in all patients in the on-state. PQ was normal in all patients in the off- and on-condition. All three respiratory parameters, as well as intelligibility, improved significantly following administration of levodopa. There were significant differences between men and women for VC and PQ, with men having the highest values for both parameters in both conditions. In none of the two conditions a correlation between respiratory parameters and intelligibility could be demonstrated., Conclusion: This study suggests that thoracic mobility is decreased in PD, and that pharmacological treatment results in improvement, but not in normalization. Moreover, it remains unclear to what extent dyskinesias negatively influence respiratory control. Our results also demonstrate improvement of intelligibility after pharmacological treatment of PD. However, this improvement is not solely the consequence of respiratory changes. Further research on the effects of different characteristics on intelligibility is necessary.

Purpose of Review: We review recent important papers pertaining to acquired aphasia, apraxia of speech and dysarthria with special attention to clinically significant work published in the last 12 months., Recent Findings: The role of the contralateral inferior frontal gyrus in language recovery after stroke is controversial, but is an area of active research, particularly in functional imaging studies. Recent treatment studies in poststroke aphasia have shown that intensity of language therapy may be more important than the method of therapy. Some studies have indicated that amphetamines, piracetam and repetitive transcortical magnetic stimulation may be effective adjuncts to speech and language therapy. Treatment studies for poststroke dysarthria indicate that speech supplementation strategies may be effective and deserve further study., Summary: Recent studies of aphasia provide clues regarding language recovery poststroke, but further studies of the role of the ipsi and contralateral inferior frontal gyrus are necessary, and should be longitudinal. There are relatively few recent studies on the treatment of acquired disorders of speech and language, other than poststroke aphasia.

The Foix-Chavany-Marie syndrome (FCMS) is characterized by apraxia of speech associated to bilateral central facio-linguo-velo-pharyngeal paralysis, with automatic-voluntary dissociation. In Worster-Drought Syndrome (WDS), dysarthria is remarkable. We report an 18-year-old female, with clinical and radiological findings of intermediary form of FCMS/WDS, and showing involuntary movements, an unusual fact.