Lateral cerebellothalamic tract activation underlies DBS therapy for Essential Tremor.

BACKGROUND: While deep brain stimulation (DBS) therapy can be effective at suppressing tremor in individuals with medication-refractory Essential Tremor, patient outcome variability remains a significant challenge across centers. Proximity of active electrodes to the cerebellothalamic tract (CTT) is likely important in suppressing tremor, but how tremor control and side effects relate to targeting parcellations within the CTT and other pathways in and around the ventral intermediate (VIM) nucleus of thalamus remain unclear. METHODS: Using ultra-high field (7T) MRI, we developed high-dimensional, subject-specific pathway activation models for 23 directional DBS leads. Modeled pathway activations were compared with post-hoc analysis of clinician-optimized DBS settings, paresthesia thresholds, and dysarthria thresholds. Mixed-effect models were utilized to determine how the six parcellated regions of the CTT and how six other pathways in and around the VIM contributed to tremor suppression and induction of side effects. RESULTS: The lateral portion of the CTT had the highest activation at clinical settings (p < 0.05) and a significant effect on tremor suppression (p < 0.001). Activation of the medial lemniscus and posterior-medial CTT was significantly associated with severity of paresthesias (p < 0.001). Activation of the anterior-medial CTT had a significant association with dysarthria (p < 0.05). CONCLUSIONS: This study provides a detailed understanding of the fiber pathways responsible for therapy and side effects of DBS for Essential Tremor, and suggests a model-based programming approach will enable more selective activation of lateral fibers within the CTT.

Home-based biofeedback speech treatment improves dysarthria in repeat-expansion SCAs.

CAG repeat-expansion spinocerebellar ataxias (CAG-SCAs) are genetically defined multisystemic degenerative diseases, resulting in motor symptoms including dysarthria with a substantial impact on daily living. Whilst speech therapy is widely recommended in ataxia, very limited evidence exists for its use. We evaluated the efficacy of a home-delivered, ataxia-tailored biofeedback-driven speech therapy in CAG-SCA in 16 individuals with SCA1, 2, 3, or 6. Treatment was delivered intensively over 20 days. Efficacy was evaluated by blinded ratings of intelligibility (primary) and acoustic measures (secondary) leveraging an intra-individual control design. Intelligibility improved post-treatment (Z = -3.18, p = 0.004) whilst remaining stable prior to treatment (Z = 0.53, p = 1.00).

The Effects of Intensive Voice Treatment in Mandarin Speakers With Parkinson's Disease: Acoustic and Perceptual Findings.

PURPOSE: This study investigated the effects of intensive voice treatment on subjective and objective measures of speech production in Mandarin speakers with hypokinetic dysarthria. METHOD: Nine Mandarin speakers with hypokinetic dysarthria due to Parkinson's disease received 4 weeks of intensive voice treatment (4 × 60 min per week). The speakers were recorded reading a passage before treatment (PRE), immediately after treatment (POST), and at 6-month follow-up (FU). Listeners (n = 15) rated relative ease of understanding (EOU) of paired speech samples on a visual analogue scale. Acoustic analyses were performed. Changes in EOU, vocal intensity, global and local fundamental frequency (f o) variation, speech rate, and acoustic vowel space area (VSA) were examined. RESULTS: Increases were found in EOU and vocal intensity from PRE to POST and from PRE to FU, with no change found from POST to FU. Speech rate increased from PRE to POST, with limited evidence of an increase from PRE to FU and no change from POST to FU. No changes in global or local f o variation or in VSA were found. CONCLUSIONS: Intensive voice treatment shows promise for improving speech production in Mandarin speakers with hypokinetic dysarthria. Vocal intensity, speech rate, and, crucially, intelligibility, may improve for up to 6 months posttreatment. In contrast, f o variation and VSA may not increase following the treatment. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.19529017.

Feasibility of a standardized protocol for respiratory training with intermitted positive pressure breathing ventilator application in dysphonia and dysarthria.

BACKGROUND: Brain damage can affect several functions related to speech production leading to dysphonia and dysarthria. Most rehabilitation treatments focus on articulation training rather than on pneumophonic coordination and respiratory muscle strength. Respiratory training using an intermitted positive pressure breathing (IPPB) ventilator can be used for this last purpose; no agreement on a standard protocol has been reached to date. AIM: To evaluate the feasibility and the effectiveness of a standardized incremental protocol of respiratory training using IPPB to treat dysphonia and dysarthria. DESIGN: Case series study. SETTING: Neuropsychological Rehabilitation Unit in an Italian Neurorehabilitation Division. POPULATION: Thirty-two subjects with dysphonia and dysarthria resulting from neurological lesion. METHODS: Participants were assessed using clinical evaluation scales (GIRBAS scale of dysphonia, Robertson dysarthria profile), respiratory function test, and arterial blood gas analysis in air. The evaluations were performed at baseline and after 20 sessions of respiratory training with IPPB. The protocol provided a default increment of ventilator parameters. All subjects also underwent a standard speech and language therapy treatment. A satisfaction survey to assess acceptability and the Goal Attainment Scale were applied. RESULTS: All participants fulfilled the protocol. No complications or discomfort were reported. Subjects' satisfaction at survey was 97.7%. After respiratory training, all respiratory function parameters increased, but only maximal voluntary ventilation (MVV), maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP) were statistically significant (P<0.05). Clinical evaluation scales significantly improved (P<0.05). Correlation between respiratory function parameters and clinical evaluation scales showed a moderate correlation between MVV, MEP, MIP, and Robertson dysarthria profile (P<0.01). A weak correlation was found between MIP, MVV, and GIRBAS scale (P<0.05). CONCLUSIONS: Our protocol showed to be practical and well-tolerated. After respiratory training, MVV, MIP and MEP improved in significantly. Clinical scale scores improved in all participants. CLINICAL REHABILITATION IMPACT: Respiratory training using IPPB ventilator can be useful in implementing speech and language treatments in subjects with dysphonia and dysarthria linked to brain injury.

Isolated acute pseudobulbar palsy with infarction of artery of percheron: case report and literature review.

INTRODUCTION: Pseudobulbar palsy (PBP) is characterized by supranuclear lesions in the corticobulbar pathway. Neoplasia, inflammatory, demyelinating, and stroke are possible etiologies of this disorder. CASE REPORT: We report an elderly female who presented with dysarthria. She was dysarthric with a hypernasal voice, no apraxia or aphasia was observed. Tongue movements were slow with limited amplitude. Her soft palate dropped bilaterally; gag reflex was present. Also, she reported swallowing difficulty and choking with her saliva. Bilateral vertical and horizontal gaze were intact to either voluntary or oculocephalic movements. A cranial CT scan was suggestive of artery of Percheron (AOP) infarction. Brain magnetic resonance imaging showed hypersignal on diffusion-weighted and T2-weighted images and hyposignal on apparent diffusion coefficient in both thalami. CT angiography scan revealed an AOP originating from the left posterior cerebral artery. The swallowing study with a videofluoroscopic demonstrated oral and pharyngeal phases with severe dysfunction. CONCLUSION: To the authors' knowledge, there are two cases of individuals with artery of Percheron infarction who developed PBP associated with other clinical syndromes. Still, isolated PBP following infarction of Percheron's artery was not reported. We hypothesized that the PBP may have occurred because of the existence of vascular territory variations in the perforating arteries that arise from the AOP.

Liuzijue qigong versus traditional breathing training for patients with post-stroke dysarthria complicated by abnormal respiratory control: Results of a single-center randomized controlled trial.

OBJECTIVE: The aim of the study was to investigate whether liuzijue qigong could improve the ability of respiratory control and comprehensive speech in patients with stroke dysarthria. DESIGN: A randomized controlled trial. SETTING: The research was carried out in the department of rehabilitation. PARTICIPANTS: Altogether, a total of 98 stroke patients with dysarthria participated in the study. INTERVENTIONS: Patients were randomly divided into two groups (the experimental group: basic articulation + liuzijue qigong, 48 patients or the control group: basic articulation + traditional breathing training, 50 patients). All therapies were conducted once a day, five times a week for three weeks. MAIN MEASURES: Primary outcome measure: Speech breathing level of the modified Frenchay Dysarthria Assessment. Secondary outcome measures: the modified Frenchay Dysarthria Assessment, maximum phonation time, maximal counting ability, /s/, /z/, s/z ratio, and the loudness level. All outcome measures were assessed twice (at baseline and after three weeks). RESULTS: At three weeks, There were significant difference between the two groups in the change of speech breathing level (81% vs 66%, P = 0.011), the modified Frenchay Dysarthria Assessment (5.54 (4.68-6.40) vs 3.66 (2.92-4.40), P = 0.001), maximum phonation time (5.55 (4.92-6.18) vs 3.01(2.31-3.71), P < 0.01), maximal counting ability (3.08(2.45-3.71) vs 2.10 (1.53-2.67), P = 0.018), and /s/ (3.08 (2.39-3.78) vs 1.87 (1.23-2.51), P = 0.004), while no significant differences were found in the change of /z/ (3.08 (2.31-3.86) vs 2.10 (1.5-2.64), P = 0.08), s/z ratio (1.26 (0.96-1.55) vs 1.03 (0.97-1.09), P = 0.714), and the change of loudness level (69% vs 60%, P = 0.562). CONCLUSIONS: Liuzijue qigong, combined with basic articulation training, could improve the respiratory control ability, as well as the comprehensive speech ability of stroke patients with dysarthria. TRIAL REGISTRATION: ChiCTR-INR-16010215.

Adult-onset methylenetetrahydrofolate reductase deficiency.

Severe hyperhomocysteinemia (>100 µmol/L) is often associated with inborn errors of homocysteine metabolism. It manifests typically in neonatal period with developmental delay, hypotonia, feeding problems or failure to thrive. Adult-onset forms are rare and include less severe manifestations. Early diagnosis is crucial because effective treatment is available. A 23-year-old man presented with a 3-week history of speech and gait impairment, and numbness in lower limbs. Neurological examination revealed dysarthria, decreased vibratory sensation in both legs and appendicular and gait ataxia. Brain MRI revealed T2-hyperintense symmetric white matter lesions and cortical atrophy. He had folate and vitamin B12 deficiency, a markedly elevated serum homocysteine and low methionine. Despite vitamin supplementation homocysteine levels remained elevated. Molecular studies of 5,10-methylenetetrahydrofolate reductase (MTHFR) gene revealed a new pathogenic mutation (c.1003C>T (p.Arg335Cys)) and a polymorphism (C677T (p.Ala222Val)) associated with hyperhomocysteinemia, both in homozygosity. The patient started betaine with clinical and biochemical improvement.

Respiratory muscle training in stroke patients with respiratory muscle weakness, dysphagia, and dysarthria - a prospective randomized trial.

OBJECTIVE: To examine the efficacy of combined inspiratory and expiratory respiratory muscle training (RMT) with respect to the swallowing function, pulmonary function, functional performance, and dysarthria in patients with stroke. DESIGN: Prospective, randomized controlled trial. SETTING: Tertiary hospital. PARTICIPANTS: The trial included 21 subjects (12 men, 9 women) aged 35 to 80 years presenting with 6 months history of unilateral stroke, respiratory muscle weakness (≥70% predicted maximal inspiratory pressure (MIP) and/or ≤70% maximal expiratory pressure (MEP)), dysphagia, or dysarthria. These subjects were randomly assigned to the control (n = 10, rehabilitation) and experimental (n = 11, rehabilitation with RMT) groups. INTERVENTION: Inspiratory RMT starting from 30% to 60% of MIP and expiratory RMT starting from 15% to 75% of MEP for 5 days/week for 6 weeks. MAIN OUTCOME MEASURES: MIP, MEP, pulmonary function, peak cough flow, perception of dyspnea, Fatigue Assessment Scale, Modified Rankin Scale, Brunnstrom stage, Barthel index, Functional Oral Intake Scale (FOIS), and parameters of voice analysis. RESULTS: Significant differences were observed between both groups in terms of MIP, forced vital capacity (FVC), and forced expiratory volume per second (FEV1) of the percentage predicted. Significant difference was found with respect to the change in fatigue, shimmer percent, amplitude perturbation quotient, and voice turbulence index (VTI) according to the acoustic analysis in the RMT group. The FEV1/FVC ratio was negatively correlated with jitter percent, relative average perturbation, pitch perturbation quotient, and VTI; the maximum mid-expiratory flow (MMEF) and MMEF% were also negatively correlated with VTI. Significant differences among participants of the same group were observed while comparing the Brunnstrom stage before and after training of the affected limbs and the Barthel scale and FOIS scores in both the groups. CONCLUSIONS: Altogether, 6-week combined inspiratory and expiratory RMT is feasible as adjuvant therapy for stroke patients to improve fatigue level, respiratory muscle strength, lung volume, respiratory flow, and dysarthria.Clinical trial registration number (Clinical Trial Identifier): NCT03491111.

Automated Assessment of Oral Diadochokinesis in Multiple Sclerosis Using a Neural Network Approach: Effect of Different Syllable Repetition Paradigms.

Slow and irregular oral diadochokinesis represents an important manifestation of spastic and ataxic dysarthria in multiple sclerosis (MS). We aimed to develop a robust algorithm based on convolutional neural networks for the accurate detection of syllables from different types of alternating motion rate (AMR) and sequential motion rate (SMR) paradigms. Subsequently, we explored the sensitivity of AMR and SMR paradigms based on voiceless and voiced consonants in the detection of speech impairment. The four types of syllable repetition paradigms including /ta/, /da/, /pa/-/ta/-/ka/, and /ba/-/da/-/ga/ were collected from 120 MS patients and 60 matched healthy control speakers. Our neural network algorithm was able to correctly identify the position of individual syllables with a very high average accuracy of 97.8%, with the correct temporal detection of syllable position of 87.8% for 10 ms and 95.5% for 20 ms tolerance value. We found significantly altered diadochokinetic rate and regularity in MS compared to controls across all types of investigated tasks ( ). MS patients showed slower speech for SMR compared to AMR tasks, whereas voiced paradigms were more irregular. Objective evaluation of oral diadochokinesis using different AMR and SMR paradigms may provide important information regarding speech severity and pathophysiology of the underlying disease.

Psychosocial Impact of Dysarthria: The Patient-Reported Outcome as Part of the Clinical Management.

BACKGROUND: Dysarthria in neurological disorders can have psychosocial consequences. The dysarthric speaker's perspective towards the disorder's psychosocial impact is essential in its global assessment and management. For such purposes, assessment tools such as the Dysarthria Impact Profile (DIP) are indispensable. OBJECTIVE: We aimed to confirm the relevance of using the DIP to quantify the psychosocial consequences of dysarthria in neurological diseases. METHODS: We studied 120 participants, 15 healthy controls and 105 patients with different kinds of dysarthria induced by several neurological disorders (Parkinson's disease [PD], Huntington's disease, dystonia, cerebellar ataxia, progressive supranuclear palsy [PSP], multiple system atrophy, lateral amyotrophic sclerosis). All participants underwent a cognitive evaluation and a speech intelligibility assessment and completed three self-reported questionnaires: the 36-Item Short Form Health Survey, the Voice Handicap Index (VHI), and the DIP. RESULTS: The psychometric properties of the DIP were confirmed, including internal consistency (α = 0.93), concurrent validity (correlation with the VHI: r = -0.77), and discriminant validity (accuracy = 0.93). Psychosocial impact of dysarthria was revealed by the DIP for all patients. Intelligibility loss was found strongly correlated with the psychosocial impact of dysarthria: for a similar level of intelligibility impairment, the DIP total score was similar regardless of the pathological group. However, our findings suggest that the psychosocial impact measured by the DIP could be partially independent from the severity of dysarthria (indirectly addressed here via speech intelligibility): the DIP was able to detect patients without any intelligibility impairment, but with a psychosocial impact. CONCLUSIONS: All patients reported a communication complaint, attested by the DIP scores, despite the fact that not all patients, notably PD, ataxic, and PSP patients, had an intelligibility deficit. The DIP should be used in clinical practice to contribute to a holistic evaluation and management of functional communication in patients with dysarthria.

Integration of a neurodynamic approach into the treatment of dysarthria for patients with idiopathic Parkinson's disease: A pilot study.

PURPOSE: Differences between standard dysarthria treatment and the same treatment with the integration of neurodynamic techniques tailored to the severity of dysarthria in patients with Parkinson's disease were examined. METHOD: In total, 10 subjects with idiopathic Parkinson's disease and rigid-hypokinetic dysarthria were enrolled in this quasi-randomized, controlled, single-blind, pre-post study. In each of 12 therapy sessions the control group (n = 5) received standard dysarthria treatment (usual care), while the intervention group (n = 5) received the same treatment with the addition of integrated neurodynamic treatment (special care). RESULTS: There was no significant difference between the two groups for either the pre-test (p = 0.739) or the post-test (p = 0.156) results. However, significant differences between the pre-test and post-test results within each group (intervention group p = 0.001; control group p = 0.003) were found. CONCLUSIONS: The significant differences in the pre-post comparison within the groups may indicate a high probability of a positive effect of standard dysarthria treatment on the severity of dysarthria. In between-group comparisons, the study results indicated no evidence of a significant difference between standard dysarthria treatment with or without neurodynamics. Due to the small sample size, the effectiveness of the integration of neurodynamics into speech therapy cannot be definitively concluded for now. In order to be able to have generalized applicability, future studies with larger numbers of participants are required.

Liuzijue Qigong vs traditional breathing training for patients with post-stroke dysarthria complicated with abnormal respiratory control: study protocol of a single center randomized controlled trial.

BACKGROUND: Stroke-induced dysarthria is caused by muscle weakness, sacral or muscular dystonia, and incoordination of the articulatory organ formed by organic lesions caused by cerebral vascular obstruction or sudden bursting of blood vessels in the brain, which may cause abnormal breathing patterns, pronunciation, resonance, rhythm, and unclear articulation. The Six Character Formula, or Liuzijue qigong (LQG), is an essential part of Chinese traditional exercises and focuses on breathing-speech synchronization. The purpose of the present study was to compare the effects of LQG with traditional breathing training (combined with basic articulation training in both groups) in patients with post-stroke dysarthria. METHODS/DESIGN: The proposed study will be a single-center randomized controlled trial. A total of 100 patients, with a modified Frenchay Dysarthria Assessment (FDA) dysarthria assessment score < 27 and with a FDA speech breathing level ≥ b will be randomly divided into study (LQG, n = 50) and control (conventional breathing training, n = 50) groups. Basic articulation training will be conducted once a day, five times a week for 3 weeks. Data collection will be conducted at baseline, 1 week, and 2 weeks post-treatment initiation and after completion of the treatment (3 weeks). Comprehensive analyses will be conducted to measure and compare any differences in speech breathing dysfunction levels, comprehensive evaluation of dysarthria, maximum phonation time (MPT), maximal counting ability, signal-noise (S/Z) ratio, and loudness scales between the study and control groups. DISCUSSION: This trial will provide evidence about the effectiveness of LQG for improvement of speech breathing function and speech ability in patients with post-stroke dysarthria complicated with abnormal breathing. TRIAL REGISTRATION: Chinese Clinical Trial Registry, ChiCTR-INR-16010215. Registered 21 December 2016.

The challenges of dysphagia in treating motor neurone disease.

Motor neurone disease (MND) is a relatively rare degenerative disorder. Its impacts are manifested in progressive loss of motor function and often accompanied by wider non-motor changes. Swallowing and speech abilities are frequently severely impaired. Effective management of dysphagia (swallowing difficulty) symptoms and nutritional care requires a holistic multidisciplinary approach. Care must be patient focused, facilitate patient decision making, and support planning towards end of life care. This article discusses the challenges of providing effective nutritional care to people living with motor neurone disease who have dysphagia.

[Cryptogenic stroke in a young patient with heart disease and kidney failure]. / Ictus criptogenico en un paciente joven con cardiopatia y fallo renal.

INTRODUCTION: Fabry's disease is an infrequent metabolic pathology linked to the X chromosome which causes a wide variety of signs and symptoms. CASE REPORT: A 39-year-old male who was admitted to our stroke unit with right-side hemiparesis (1 + 0) and dysarthria (1). The score on the National Institute of Health Stroke Scale was 2. The patient presented angiokeratomas in both thighs. A computerised axial tomography scan of the head showed left thalamic acute infarction. The duplex scan of the supra-aortic trunks was normal, and the transcranial Doppler reflected a generalised increase in the pulsatility indices. Transthoracic echocardiography showed left ventricular hypertrophy and left atrial dilatation. He was discharged five days later, with antiaggregating medication but asymptomatic. The prolonged Holter-electrocardiogram recording showed paroxysmal atrial fibrillation. One notable value in the urine analysis was microalbuminuria of 281 mg/L. In view of the multi-organic involvement and the family history, a study for Fabry's disease was performed. Activity of the enzyme alpha-galactosidase A was diminished, and the presence of a mutation in the GLA gene was found. The patient's brother, who suffered from kidney failure and atrial fibrillation, was positive for this mutation. The patient is on treatment with agalsidase beta. CONCLUSIONS: Fabry's disease must be suspected in young males with heart disease, stroke or peripheral neuropathy, skin lesions, kidney failure and a history of cases in the family. Hormone replacement therapy must be established at an early stage, as it can improve the prognosis.

TITLE: Ictus criptogenico en un paciente joven con cardiopatia y fallo renal.Introduccion. La enfermedad de Fabry es una patologia metabolica infrecuente ligada al cromosoma X, que provoca una amplia variedad de signos y sintomas. Caso clinico. Varon de 39 antilde;os que ingreso en nuestra unidad de ictus con hemiparesia derecha (1 + 0) y disartria (1). La puntuacion en la National Institute of Health Stroke Scale era de 2. Presentaba angioqueratomas en ambos muslos. La tomografia axial computarizada craneal mostraba un infarto agudo talamico izquierdo. El duplex de los troncos supraaorticos era normal, y el Doppler transcraneal reflejaba un aumento generalizado de los indices de pulsatilidad. El ecocardiograma transtoracico mostraba hipertrofia ventricular izquierda y dilatacion de la auricula izquierda. Recibio el alta cinco dias despues, asintomatico, con antiagregacion. El registro Holter-electrocardiografico prolongado mostraba fibrilacion auricular paroxistica. En la analitica de orina destacaba microalbuminuria de 281 mg/L. En vista de la afectacion multiorganica y la historia familiar, se curso estudio de enfermedad de Fabry. La actividad de la enzima alfa-galactosidasa-A se encontro disminuida, y se demostro la presencia de una mutacion en el gen GLA. Su hermano, que padecia insuficiencia renal y fibrilacion auricular, fue positivo para dicha mutacion. El paciente se encuentra en tratamiento con agalsidasa beta. Conclusiones. La enfermedad de Fabry debe sospecharse en varones jovenes con cardiopatia, ictus o neuropatia periferica, lesiones cutaneas, fallo renal e historia de familiares afectos. El tratamiento hormonal sustitutivo debe comenzarse precozmente, ya que puede mejorar el pronostico.

Speech Prosody Across Stimulus Types for Individuals with Parkinson's Disease.

BACKGROUND: Up to 89% of the individuals with Parkinson's disease (PD) experience speech problem over the course of the disease. Speech prosody and intelligibility are two of the most affected areas in hypokinetic dysarthria. However, assessment of these areas could potentially be problematic as speech prosody and intelligibility could be affected by the type of speech materials employed. OBJECTIVE: To comparatively explore the effects of different types of speech stimulus on speech prosody and intelligibility in PD speakers. METHODS: Speech prosody and intelligibility of two groups of individuals with varying degree of dysarthria resulting from PD was compared to that of a group of control speakers using sentence reading, passage reading and monologue. Acoustic analysis including measures on fundamental frequency (F0), intensity and speech rate was used to form a prosodic profile for each individual. Speech intelligibility was measured for the speakers with dysarthria using direct magnitude estimation. RESULTS: Difference in F0 variability between the speakers with dysarthria and control speakers was only observed in sentence reading task. Difference in the average intensity level was observed for speakers with mild dysarthria to that of the control speakers. Additionally, there were stimulus effect on both intelligibility and prosodic profile. CONCLUSIONS: The prosodic profile of PD speakers was different from that of the control speakers in the more structured task, and lower intelligibility was found in less structured task. This highlighted the value of both structured and natural stimulus to evaluate speech production in PD speakers.

Individualized current-shaping reduces DBS-induced dysarthria in patients with essential tremor.

OBJECTIVE: To investigate in patients with essential tremor (ET) treated with thalamic/subthalamic deep brain stimulation (DBS) whether stimulation-induced dysarthria (SID) can be diminished by individualized current-shaping with interleaving stimulation (cs-ILS) while maintaining tremor suppression (TS). METHODS: Of 26 patients screened, 10 reported SID and were invited for testing. TS was assessed by the Tremor Rating Scale and kinematic analysis of postural and action tremor. SID was assessed by phonetic and logopedic means. Additionally, patients rated their dysarthria on a visual analog scale. RESULTS: In 6 of the 10 patients with ET, DBS-ON (relative to DBS-OFF) led to SID while tremor was successfully reduced. When comparing individualized cs-ILS with a non-current-shaped interleaving stimulation (ILS) in these patients, there was no difference in TS while 4 of the 6 patients showed subjective improvement of speech during cs-ILS. Phonetic analysis (ILS vs cs-ILS) revealed that during cs-ILS there was a reduction of voicing during the production of voiceless stop consonants and also a trend toward an improvement in oral diadochokinetic rate, reflecting less dysarthria. Logopedic rating showed a trend toward deterioration in the diadochokinesis task when comparing ON with OFF but no difference between ILS and cs-ILS. CONCLUSION: This is a proof-of-principle evaluation of current-shaping in patients with ET treated with thalamic/subthalamic DBS and experiencing SID. Data suggest a benefit on SID from individual shaping of current spread while TS is preserved. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that in patients with ET treated with DBS with SID, individualized cs-ILS reduces dysarthria while maintaining tremor control.

The safety and efficacy of thalamic deep brain stimulation in essential tremor: 10 years and beyond.

BACKGROUND: Deep brain stimulation (DBS) has proven to be a safe and effective therapy for refractory essential tremor, but information regarding long-term outcomes is lacking. OBJECTIVES: We aimed to assess the long-term safety and efficacy of DBS in patients with essential tremor. METHODS: Patients treated with DBS for essential tremor for at least 8 years were evaluated in the 'on' and 'off' state using the Fahn-Tolosa-Marin tremor rating scale, and their medical records were reviewed to assess complications related to this therapy. RESULTS: We studied 13 patients (7 men): median age at evaluation 79 years (range 47-88), median age at electrode implantation 68 years (range 37-78) and mean time since electrode implantation 132.54±15.3 months (range 114-164). The difference between the 'off' and 'on' state on the motor items of the tremor rating scale was 41.9% (58.62 vs. 34.08, p<0.001) in the non-blinded and 37.2% (56.07 vs. 35.23, p<0.001) in the blinded rating. DBS provided a functional improvement of 31.7% in the 'on' state (15.07 vs. 22.07, p<0.001). A total non-blinded improvement in the tremor rating scale of 39% was observed in the 'on' state (49.15 vs. 80.69, p<0.001). Dysarthria and disequilibrium were common in patients with bilateral stimulation. A DBS-related surgery (electrode revision or internal pulse generator exchange) was necessary on average every 47.9 months to continue with the DBS therapy. CONCLUSIONS: Thalamic DBS is a safe and effective therapy in patients with essential tremor followed for up to 13 years.

Unusual neurological syndrome induced by atmospheric pressure change.

BACKGROUND: We describe a case of a 46-yr-old female who developed hypertension, tachycardia, dysarthria, and leg weakness provoked by pressure changes associated with flying. Typically during the landing phase of flight, she would feel dizzy and note that she had difficulty with speech and leg weakness. After the flight the leg weakness persisted for several days. The symptoms were mitigated when she took a combined alpha-beta blocker (labetalol) prior to the flight. CASE STUDY: To determine if these symptoms were related to atmospheric pressure change, she was referred for testing in a hyperbaric chamber. She was exposed to elevated atmospheric pressure (maximum 1.2 ATA) while her heart rate and blood pressure were monitored. Within 1 min she developed tachycardia and hypertension. She also quickly developed slurred speech, left arm and leg weakness, and sensory changes in her left leg. She was returned to sea level pressure and her symptoms gradually improved. A full neurological workup has revealed no explanation for these findings. She has no air collections, cysts, or other anatomic findings that could be sensitive to atmospheric pressure change. DISCUSSION: The pattern is most consistent with a vascular event stimulated by altitude exposure. This case suggests that atmospheric pressure change can produce neurological symptoms, although the mechanism is unknown.