Autoimmune nodopathy with anti-contactin 1 antibody characterized by cerebellar dysarthria: a case report and literature review.

Background: Autoimmune nodopathy (AN) has emerged as a novel diagnostic category that is pathologically different from classic chronic inflammatory demyelinating polyneuropathy. Clinical manifestations of AN include sensory or motor neuropathies, sensory ataxia, tremor, and cranial nerve involvement. AN with a serum-positive contactin-1 (CNTN1) antibody usually results in peripheral nerve demyelination. In this study, we reported a rare case of AN with CNTN1 antibodies characterized by the presence of CNTN1 antibodies in both serum and cerebrospinal fluid, which is associated with cerebellar dysarthria. Methods: A 25-year-old man was admitted to our hospital due to progressive dysarthria with limb tremors. The patient was initially diagnosed with peripheral neuropathy at a local hospital. Three years after onset, he was admitted to our hospital due to dysarthria, apparent limb tremor, and limb weakness. At that time, he was diagnosed with spinocerebellar ataxia. Eight years post-onset, during his second admission, his condition had notably deteriorated. His dysarthria had evolved to typical distinctive cerebellar characteristics, such as tremor, loud voice, stress, and interrupted articulation. Additionally, he experienced further progression in limb weakness and developed muscle atrophy in the distal limbs. Magnetic resonance imaging (MRI), nerve conduction studies (NCS), and autoimmune antibody tests were performed. Results: The results of the NCS suggested severe demyelination and even axonal damage to the peripheral nerves. MRI scans revealed diffuse thickening of bilateral cervical nerve roots, lumbosacral nerve roots, cauda equina nerve, and multiple intercostal nerve root sheath cysts. Furthermore, anti-CNTN1 antibody titers were 1:10 in the cerebrospinal fluid (CSF) and 1:100 in the serum. After one round of rituximab treatment, the patient showed significant improvement in limb weakness and dysarthria, and the CSF antibodies turned negative. Conclusion: Apart from peripheral neuropathies, cerebellar dysarthria (central nervous system involvement) should not be ignored in AN patients with CNTN1 antibodies.

Recurrent stroke in a reproductive age women with patent foramen ovale.

BACKGROUND: Patent foramen ovale (PFO) is a known cause of ischemic stroke in young adults and combined oral contraceptives (COCs) are widely used by women of reproductive age. If young women with PFO are taking COCs, they may be subjected to a synergistic increase in the occurrence of stroke, though reports of ischemic stroke in this population are rare. We report a woman of reproductive age who was taking COC suffered repetitive ischemic strokes before a patent foramen ovale (PFO) was detected and closed, which may raise concerns in this field. CASE PRESENTATION: A 31-year-old woman presented to the emergency department with sudden-onset right upper- and lower-limb weakness and dysarthria for 1 hour, whose only risk factor of stroke was oral contraceptive use. On admission, she was alert with left gaze deviation, dysarthria, and right-sided hemiplegia. Her symptoms improved after receiving the revascularization therapy. About 24 hours later, her left eye experienced sudden painless vision loss. Then the PFO with a substantial right-to-left shunt was detected and then she received a trans-catheter closure of the defect. Over 3 months of follow-up, there were no signs of stroke, but visual loss persisted. CONCLUSION: This case of disabling stroke raises concerns regarding optimal management in primary and secondary prevention of stroke in young women on COCs with additional risk factors of stroke.

Uncertainty of Vowel Predictions as a Digital Biomarker for Ataxic Dysarthria.

Dysarthria is a common manifestation across cerebellar ataxias leading to impairments in communication, reduced social connections, and decreased quality of life. While dysarthria symptoms may be present in other neurological conditions, ataxic dysarthria is a perceptually distinct motor speech disorder, with the most prominent characteristics being articulation and prosody abnormalities along with distorted vowels. We hypothesized that uncertainty of vowel predictions by an automatic speech recognition system can capture speech changes present in cerebellar ataxia. Speech of participants with ataxia (N=61) and healthy controls (N=25) was recorded during the "picture description" task. Additionally, participants' dysarthric speech and ataxia severity were assessed on a Brief Ataxia Rating Scale (BARS). Eight participants with ataxia had speech and BARS data at two timepoints. A neural network trained for phoneme prediction was applied to speech recordings. Average entropy of vowel tokens predictions (AVE) was computed for each participant's recording, together with mean pitch and intensity standard deviations (MPSD and MISD) in the vowel segments. AVE and MISD demonstrated associations with BARS speech score (Spearman's rho=0.45 and 0.51), and AVE demonstrated associations with BARS total (rho=0.39). In the longitudinal cohort, Wilcoxon pairwise signed rank test demonstrated an increase in BARS total and AVE, while BARS speech and acoustic measures did not significantly increase. Relationship of AVE to both BARS speech and BARS total, as well as the ability to capture disease progression even in absence of measured speech decline, indicates the potential of AVE as a digital biomarker for cerebellar ataxia.

Levodopa may modulate specific speech impairment in Parkinson's disease: an fMRI study.

Hypokinetic dysarthria (HD) is a difficult-to-treat symptom affecting quality of life in patients with Parkinson's disease (PD). Levodopa may partially alleviate some symptoms of HD in PD, but the neural correlates of these effects are not fully understood. The aim of our study was to identify neural mechanisms by which levodopa affects articulation and prosody in patients with PD. Altogether 20 PD patients participated in a task fMRI study (overt sentence reading). Using a single dose of levodopa after an overnight withdrawal of dopaminergic medication, levodopa-induced BOLD signal changes within the articulatory pathway (in regions of interest; ROIs) were studied. We also correlated levodopa-induced BOLD signal changes with the changes in acoustic parameters of speech. We observed no significant changes in acoustic parameters due to acute levodopa administration. After levodopa administration as compared to the OFF dopaminergic condition, patients showed task-induced BOLD signal decreases in the left ventral thalamus (p = 0.0033). The changes in thalamic activation were associated with changes in pitch variation (R = 0.67, p = 0.006), while the changes in caudate nucleus activation were related to changes in the second formant variability which evaluates precise articulation (R = 0.70, p = 0.003). The results are in line with the notion that levodopa does not have a major impact on HD in PD, but it may induce neural changes within the basal ganglia circuitries that are related to changes in speech prosody and articulation.

Topographic Consideration on the Occurrence of Ipsilesional Facial Paresis in Lateral Medullary Infarction.

INTRODUCTION: The purpose of this study was to identify course of the corticobulbar tract and factors associated with the occurrence of facial paresis (FP) in lateral medullary infarction (LMI). METHODS: Patients diagnosed with LMI who were admitted to tertiary hospital were retrospectively investigated and divided into two groups based on the presence of FP. FP was defined as grade 2 or more by the House-Brackmann scale. Differences between the two groups were analyzed with respect to anatomical location of the lesions, demographic data (age, sex), risk factors (diabetes, hypertension, smoking, prior stroke, atrial fibrillation, and other cardiac risk factors for stroke), large vessel involvement on magnetic resonance angiography, other symptoms and signs (sensory symptoms, gait ataxia, limb ataxia, dizziness, Horner syndrome, hoarseness, dysphagia, dysarthria, nystagmus, nausea/vomiting, headache, neck pain, diplopia, and hiccup). RESULTS: Among 44 LMI patients, 15 patients (34%) had FP, and all of them had ipsilesional central-type FP. The FP group tended to involve upper (p < 0.0001) and relative ventral (p = 0.019) part of the lateral medulla. Horizontally large lesion was also related to the presence of FP (p = 0.044). Dysphagia (p = 0.001), dysarthria (p = 0.003), and hiccups (p = 0.034) were more likely to be accompanied by FP. Otherwise, there were no significant differences. CONCLUSION: The results of present study indicate that the corticobulbar fibers innervating the lower face decussate at the upper level of the medulla and ascend through the dorsolateral medulla, where the concentration of the fibers is densest near the nucleus ambiguus.

Sex Differences in the Speech of Persons With and Without Parkinson's Disease.

BACKGROUND: Sex differences are apparent in the prevalence and the clinical presentation of Parkinson's disease (PD), but their effects on speech have been less studied. METHOD: Speech acoustics of persons with (34 females and 34 males) and without (age- and sex-matched) PD were examined, assessing the effects of PD diagnosis and sex on ratings of dysarthria severity and acoustic measures of phonation (fundamental frequency standard deviation, smoothed cepstral peak prominence), speech rate (net syllables per second, percent pause ratio), and articulation (articulatory-acoustic vowel space, release burst precision). RESULTS: Most measures were affected by PD (dysarthria severity, fundamental frequency standard deviation) and sex (smoothed cepstral peak prominence, net syllables per second, percent pause ratio, articulatory-acoustic vowel space), but without interactions between them. Release burst precision was differentially affected by sex in PD. Relative to those without PD, persons with PD produced fewer plosives with a single burst: females more frequently produced multiple bursts, whereas males more frequently produced no burst at all. CONCLUSIONS: Most metrics did not indicate that speech production is differentially affected by sex in PD. Sex was, however, associated with disparate effects on release burst precision in PD, which deserves further study. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24388666.

Optimising verbal fluency analysis in neurological patients with dysarthria: examples from Parkinson's disease and hereditary ataxia.

BACKGROUND: Verbal fluency tests (VFTs) are widely used to assess cognitive-linguistic performance in neurological diseases. However, the influence of dysarthria on performance in tests requiring oral responses is unclear in ataxia and Parkinson's disease. OBJECTIVES: To determine the impact of dysarthria on VFT performance and evaluate the validity and reliability of alternative methods for analyzing VFT data. METHOD: Trained raters evaluated dysarthria using VFT recordings in people with ataxia (N = 61) or Parkinson's disease (PD; N = 69). Total Correct Items scores and qualitative parameters (intrusions, ambiguous verbalizations, perseverations, and interjections) were compared across semantic, phonemic, and alternating fluency tasks. Disease severity was considered as a covariate in the regression model. RESULTS: VFT dysarthria ratings correlated with the benchmark (ground truth) dysarthria scores derived from a monologue. Ambiguous responses resulting from unclear speech impeded the rater's ability to determine if a response was correct. Regression analysis indicated that more severe dysarthria ratings predicted diminished scores in all three tasks (semantic fluency, phonemic fluency and alternating fluency) in the ataxia group. The contribution of disease severity to semantic, phonemic and alternating fluency was reduced substantially in the ataxia group after accounting for dysarthria severity in the model in both groups. CONCLUSIONS: Dysarthria severity can be estimated based on speech samples derived from VFT. Dysarthria can lead to lower total correct items and is associated with more ambiguous verbalizations in VFT. Dysarthria severity should be considered when interpreting VFT performance in common movement disorders.

Dysphagia and pulmonary complications in acute cerebrovascular disease: A retrospective observational study.

INTRODUCTION: Dysphagia is a common post-stroke complication, which may result in serious pulmonary sequelae. Early detection of dysphagia and aspiration risk can reduce morbidity, mortality and length of hospitalization. OBJECTIVES: This study aims to identify association between dysphagia and acute cerebrovascular disease, and evaluate the prevalence and impact of pulmonary complications on readmissions and mortality. MATERIAL AND METHODS: Retrospective observational study based on 250 clinical records of patients with acute cerebrovascular disease: clinical history, neurological examination, imaging and Gugging Swallowing Screen in the first 48h. Patients were followed for 3 months via medical records to estimate 3-month mortality and readmissions. RESULTS: Out of 250 clinical records analyzed, 102 (40.8%) were evaluated for dysphagia. The prevalence of dysphagia was 32.4%. The risk was higher in older patients (p<0.001), in severe stroke (p<0.001) and in the hemorrhagic subtype (p=0.008). An association was found with dysarthria and aphasia (p=0.003; p=0.017). Respiratory tract infections occurred in 14.4% of all patients (GUSS group 11.8% versus no GUSS group 16.2%), and in 75% of those with severe dysphagia (p<0.001). Mortality at 3 months was 24.2% in dysphagic patients, especially high in the severe dysphagia group (75%, p<0.001). CONCLUSIONS: The type of cerebrovascular disease, NIHSS and GCS scores, age, dysarthria, and aphasia were significant associated factors to dysphagia. The prevalence of respiratory tract infections was higher in patients with no GUSS record, and no statistical significance was observed in related readmissions. Mortality at 3 months was superior in the severe dysphagia group.

Compensatory articulatory mechanisms preserve intelligibility in prodromal Parkinson's disease.

INTRODUCTION: Dysarthria is highly prevalent in patients with Parkinson's disease (PD) and speech changes have already been detected in patients with prodromal PD on the acoustic level. However, the present study directly tracks underlying articulatory movements with electromagnetic articulography to investigate early speech alterations on the kinematic level in isolated REM sleep behavior disorder (iRBD) and compares them to PD and control speakers. METHODS: Kinematic data of 23 control speakers, 22 speakers with iRBD, and 23 speakers with PD were collected. Amplitude, duration, and average speed of lower lip, tongue tip, and tongue body movements were analyzed. Naive listeners rated the intelligibility of all speakers. RESULTS: Patients with iRBD produced tongue tip and tongue body movements that were larger in amplitude and longer in duration compared to control speakers, while remaining intelligible. Compared to patients with iRBD, patients with PD had smaller, longer and slower tongue tip and lower lip movements, accompanied by lower intelligibility. Thus, the data indicate that the lingual system is already affected in prodromal PD. Furthermore, lower lip and especially tongue tip movements slow down and speech intelligibility decreases if motor impairment is more pronounced. CONCLUSION: Patients with iRBD adjust articulatory patterns to counteract incipient motor detriment on speech to maintain their intelligibility level.

Effects of Conventional Speech Therapy with Liuzijue Qigong, a Traditional Chinese Method of Breath Training, in 70 Patients with Post-Stroke Spastic Dysarthria.

BACKGROUND Post-stroke spastic dysarthria (PSSD) is a motor speech impairment that impacts patient communication and quality of life. Liuzijue Qigong (LQG), a traditional Chinese method of breath training, could serve as an effective treatment for PSSD. This study compared the effects of conventional speech therapy and conventional speech therapy combined with LQG in patients with PSSD. MATERIAL AND METHODS Seventy patients with PSSD were randomly divided into a control group (conventional speech therapy, n=35, 77.14% cerebral infarction, 22.86% cerebral hemorrhage) and experimental group (LQG combined with conventional speech therapy, n=35, 85.71% cerebral infarction, 14.29% cerebral hemorrhage). Conventional speech therapy included relaxation, breath control, organ articulation, and pronunciation training. LQG involved producing 6 different sounds (Xu, He, Hu, Si, Chui, and Xi) accompanied by breathing and body movements. Patients were treated once a day, 5 times a week, for 4 weeks. The Frenchay Dysarthria Assessment scale (FDA), speech articulation, maximum phonation time (MPT), loudness, and Montreal Cognitive Assessment scale (MoCA) were evaluated. RESULTS At 4 weeks, the experimental group showed significant improvements compared with the control group in the change of FDA (13.26±6.84 vs 18.03±5.32, P=0.028), speech articulation (63.17±22.40 vs 76.51±15.28, P=0.024), MPT (1.34±1.30 vs 3.89±3.98, P<0.001), loudness (3.46±2.74 vs 7.14±2.56, P=0.009), MoCA (19.40±3.72 vs 22.20±5.30, P=0.020), total effective rate (68.57% vs 88.57%, P=0.041). CONCLUSIONS LQG, when combined with conventional speech therapy, enhanced the comprehensive speech ability of patients with PSSD compared with conventional treatment alone.

Educational Information Improves Listener Attitudes Toward People With Dysarthria Secondary to Parkinson's Disease.

PURPOSE: People with dysarthria have been rated as less confident and less likable and are often assumed by listeners to have reduced cognitive abilities relative to neurotypical speakers. This study explores whether educational information about dysarthria can shift these attitudes in a group of speakers with hypokinetic dysarthria secondary to Parkinson's disease. METHOD: One hundred seventeen listeners were recruited via Amazon Mechanical Turk to transcribe sentences and rate the confidence, intelligence, and likability of eight speakers with mild hypokinetic dysarthria. Listeners were assigned to one of four conditions. In one condition, listeners were provided with no educational information prior to exposure to speakers with dysarthria (n = 29). In another condition, listeners were given educational statements from the American Speech-Language-Hearing Association website (n = 29). In a third condition, listeners were given additional information stating that dysarthria does not indicate reduced intelligence or understanding (n = 30). Finally, in a fourth condition, listeners only heard samples from neurotypical, age-matched adults (n = 29). RESULTS: Results revealed statistically significant effects of educational statements on ratings of speakers' confidence, intelligence, and likability. However, educational statements did not affect listeners' transcription accuracy. CONCLUSIONS: This study presents preliminary evidence that educational material can positively influence listener impressions of speakers with hypokinetic dysarthria, especially when it is explicitly stated that the disorder does not affect intelligence or understanding. This initial examination provides preliminary support for educational awareness campaigns and self-disclosure of communicative difficulties in people with mild dysarthria.

Distinct phenotypes of stimulation-induced dysarthria represent different cortical networks in STN-DBS.

BACKGROUND: Deep brain stimulation of the subthalamic nucleus is an effective treatment of Parkinson's disease, yet it is often associated with a general deterioration of speech intelligibility. Clustering the phenotypes of dysarthria has been proposed as a strategy to tackle these stimulation-induced speech problems. METHODS: In this study, we examine a cohort of 24 patients to test the real-life application of the proposed clustering and attempt to attribute the clusters to specific brain networks with two different approaches of connectivity analysis. RESULTS: Both our data-driven and hypothesis-driven approaches revealed strong connections of variants of stimulation-induced dysarthria to brain regions that are known actors of motor speech control. We showed a strong connection between the spastic dysarthria type and the precentral gyrus and supplementary motor area, prompting a possible disruption of corticobulbar fibers. The connection between the strained voice dysarthria and more frontal areas hints toward a deeper disruption of the motor programming of speech production. CONCLUSIONS: These results provide insights into the mechanism of stimulation-induced dysarthria in deep brain stimulation of the subthalamic nucleus and may guide reprogramming attempts for individual Parkinson's patients based on pathophysiological understanding of the affected networks.

The Effects of Deep Brain Stimulation on Speech Motor Control in People With Parkinson's Disease.

PURPOSE: Despite the overall benefits of deep brain stimulation (DBS) in Parkinson's disease (PD), its effects on speech production have been mixed when examined using auditory-perceptual and acoustic measures. This study investigated the effects of DBS on the lip and jaw kinematics during sentence production in individuals with dysarthria secondary to PD. METHOD: Twenty-seven participants from three groups were included in the study: (a) individuals with PD and without DBS (PD group), (b) individuals with PD and with DBS (PD-DBS group), and (c) neurologically healthy control speakers (HC group). Lip and jaw movements during speech were recorded using optical motion capture and analyzed for path distance, speed, duration, articulatory stability, and interarticulator coordination. RESULTS: The PD-DBS group showed (a) increased path distance compared with the PD and HC groups and (b) increased speed compared with the PD group but not the HC group. Both PD and PD-DBS groups exhibited lengthened sentence duration compared with the HC group. Articulatory stability was greater for the two PD groups, PD and PD-DBS, compared with the HC group. Spatial, but not temporal, coordination was lower for the PD group than for the other two groups. The only kinematic changes between the DBS on and off conditions within the PD-DBS group were increases in spatial coordination. CONCLUSIONS: These data suggest that DBS primarily affects the amplitude scaling of articulatory movements, but not the temporal scaling, in individuals with PD. The findings are discussed with respect to the DBS-induced neural changes and their effects on speech motor control in PD.

Motor Speech Phenotypes in Children With Epilepsy: Preliminary Findings.

PURPOSE: This exploratory study aimed to characterize motor speech impairments in a small sample of children with epilepsy, both with and without a known seizure etiology. A secondary aim was to evaluate the validity of the Profile for Childhood Apraxia of speech and Dysarthria (ProCAD), a newly developed tool for differential diagnosis of childhood apraxia of speech and dysarthria. METHOD: Thirteen children with seizure disorders completed a comprehensive speech and language assessment. Three expert speech-language pathologists rated the presence of auditory-perceptual features of motor speech impairment using the ProCAD. Motor speech features, diagnoses, and standardized test scores were compared between children with a known seizure etiology and children with idiopathic epilepsy. RESULTS: Nine of the 13 children exhibited motor speech impairment; dysarthria was the most common diagnosis. Most children (11/13) exhibited language impairment. Group comparisons showed that children with a known seizure etiology had more atypical motor speech features and lower language scores than children with idiopathic seizures. CONCLUSION: These preliminary findings suggest a high rate of motor speech impairment among children with epilepsy.

The Effects of Speech Task on Lexical Stress in Parkinson's Disease.

PURPOSE: Hypokinetic dysarthria associated with Parkinson's disease (PD) is characterized by dysprosody, yet the literature is mixed with respect to how dysprosody affects the ability to mark lexical stress, possibly due to differences in speech tasks used to assess lexical stress. The purpose of this study was to compare how people with and without PD modulate acoustic dimensions of lexical stress-fundamental frequency, intensity, and duration-to mark lexical stress across three different speech tasks. METHOD: Twelve individuals with mild-to-moderate idiopathic PD and 12 age- and sex-matched older adult controls completed three speech tasks: picture description, word production in isolation, and word production in lists. Outcome measures were the fundamental frequency, intensity, and duration of the vocalic segments of two trochees (initial stress) and two iambs (final stress) spoken in all three tasks. RESULTS: There were very few group differences. Both groups marked trochees by modulating intensity and fundamental frequency and iambs by modulating duration. Task had a significant impact on the stress patterns used by both groups. Stress patterns were most differentiated in words produced in isolation and least differentiated in lists of words. CONCLUSIONS: People with PD did not demonstrate impairments in the production of lexical stress, suggesting that dysprosody associated with PD does not impact all types of prosody in the same way. However, there were reduced distinctions in stress marking that were more apparent in trochees than iambs. In addition, the task used to assess prosody has a significant effect on all acoustic measures. Future research should focus on the use of connected speech tasks to obtain more generalizable measures of prosody in PD.

Acoustic Predictors of Ease of Understanding in Spanish Speakers With Dysarthria Associated With Parkinson's Disease.

PURPOSE: The purpose of this study was to examine selected baseline acoustic features of hypokinetic dysarthria in Spanish speakers with Parkinson's disease (PD) and identify potential acoustic predictors of ease of understanding in Spanish. METHOD: Seventeen Spanish-speaking individuals with mild-to-moderate hypokinetic dysarthria secondary to PD and eight healthy controls were recorded reading a translation of the Rainbow Passage. Acoustic measures of vowel space area, as indicated by the formant centralization ratio (FCR), envelope modulation spectra (EMS), and articulation rate were derived from the speech samples. Additionally, 15 healthy adults rated ease of understanding of the recordings on a visual analogue scale. A multiple linear regression model was implemented to investigate the predictive value of the selected acoustic parameters on ease of understanding. RESULTS: Listeners' ease of understanding was significantly lower for speakers with dysarthria than for healthy controls. The FCR, EMS from the first 10 s of the reading passage, and the difference in EMS between the end and the beginning sections of the passage differed significantly between the two groups of speakers. Findings indicated that 67.7% of the variability in ease of understanding was explained by the predictive model, suggesting a moderately strong relationship between the acoustic and perceptual domains. CONCLUSIONS: Measures of envelope modulation spectra were found to be highly significant model predictors of ease of understanding of Spanish-speaking individuals with hypokinetic dysarthria associated with PD. Articulation rate was also found to be important (albeit to a lesser degree) in the predictive model. The formant centralization ratio should be further examined with a larger sample size and more severe dysarthria to determine its efficacy in predicting ease of understanding.

Spinocerebellar ataxia type 31: A clinical and radiological literature review.

Spinocerebellar ataxia type 31 (SCA31) is an autosomal dominant disease, classified amongst pure cerebellar ataxias (ADCA type 3). While SCA31 is the third most prevalent autosomal dominant ataxia in Japan, it is extremely rare in other countries. A literature review was conducted on PubMed, where we included all case reports and studies describing the clinical presentation of original SCA31 cases. The clinical and radiological features of 374 patients issued from 25 studies were collected. This review revealed that the average age of onset was 59.1 ± 3.3 years, with symptoms of slowly progressing ataxia and dysarthria. Other common clinical features were oculomotor dysfunction (38.8%), dysphagia (22.1%), hypoacousia (23.3%), vibratory hypoesthesia (24.3%), and dysreflexia (41.6%). Unfrequently, abnormal movements (7.4%), extrapyramidal symptoms (4.5%) and cognitive impairment (6.9%) may be observed. Upon radiological examination, clinicians can expect a high prevalence of cerebellar atrophy (78.7%), occasionally accompanied by brainstem (9.1%) and cortical (9.1%) atrophy. Although SCA31 is described as a slowly progressive pure cerebellar syndrome characterized by cerebellar signs such as ataxia, dysarthria and oculomotor dysfunction, this study evaluated a high prevalence of extracerebellar manifestations. Extracerebellar signs were observed in 52.5% of patients, primarily consisting of dysreflexia, vibratory hypoesthesia and hypoacousia. Nonetheless, we must consider the old age and longstanding disease course of patients as a confounding factor for extracerebellar sign development, as some may not be directly attributable to SCA31. Clinicians should consider SCA31 in patients with a hereditary, pure cerebellar syndrome and in patients with extracerebellar signs.

Sex differences in the symptom presentation of stroke: A systematic review and meta-analysis.

BACKGROUND: Early diagnosis through symptom recognition is vital in the management of acute stroke. However, women who experience stroke are more likely than men to be initially given a nonstroke diagnosis and it is unclear if potential sex differences in presenting symptoms increase the risk of delayed or missed stroke diagnosis. AIMS: To quantify sex differences in the symptom presentation of stroke and assess whether these differences are associated with a delayed or missed diagnosis. METHODS: PubMed, EMBASE, and the Cochrane Library were systematically searched up to January 2021. Studies were included if they reported presenting symptoms of adult women and men with diagnosed stroke (ischemic or hemorrhagic) or transient ischemic attack (TIA) and were published in English. Mean percentages with 95% confidence intervals (CIs) of each symptom were calculated for women and men. The crude relative risks (RRs) with 95% CI of symptoms being present in women, relative to men, were also calculated and pooled. Any data on the delayed or missed diagnosis of stroke for women compared to men based on symptom presentation were also extracted. RESULTS: Pooled results from 21 eligible articles showed that women and men presented with a similar mean percentage of motor deficit (56% in women vs 56% in men) and speech deficit (41% in women vs 40% in men). Despite this, women more commonly presented with nonfocal symptoms than men: generalized nonspecific weakness (49% vs 36%), mental status change (31% vs 21%), and confusion (37% vs 28%), whereas men more commonly presented with ataxia (44% vs 30%) and dysarthria (32% vs 27%). Women also had a higher risk of presenting with some nonfocal symptoms: generalized weakness (RR 1.49, 95% CI 1.09-2.03), mental status change (RR 1.44, 95% CI 1.22-1.71), fatigue (RR 1.42, 95% CI 1.05-1.92), and loss of consciousness (RR 1.30, 95% CI 1.12-1.51). In contrast, women had a lower risk of presenting with dysarthria (RR 0.89, 95% CI 0.82-0.95), dizziness (RR 0.87, 95% CI 0.80-0.95), gait disturbance (RR 0.79, 95% CI 0.65-0.97), and imbalance (RR 0.68, 95% CI 0.57-0.81). Only one study linking symptoms to definite stroke/TIA diagnosis found that pain and unilateral sensory loss are associated with lower odds of a definite diagnosis in women compared to men. CONCLUSION: Although women showed a higher prevalence of some nonfocal symptoms, the prevalence of focal neurological symptoms, such as motor weakness and speech deficit, was similar for both sexes. Awareness of sex differences in symptoms in acute stroke evaluation, careful consideration of the full constellation of presenting symptoms, and further studies linking symptoms to diagnostic outcomes can be helpful in improving early diagnosis and management in both sexes.

Clear Speech Variants: An Investigation of Intelligibility and Speaker Effort in Speakers With Parkinson's Disease.

PURPOSE: This study investigated the effects of three clear speech variants on sentence intelligibility and speaking effort for speakers with Parkinson's disease (PD) and age- and sex-matched neurologically healthy controls. METHOD: Fourteen speakers with PD and 14 neurologically healthy speakers participated. Each speaker was recorded reading 18 sentences from the Speech Intelligibility Test in their habitual speaking style and for three clear speech variants: clear (SC; given instructions to speak clearly), hearing impaired (HI; given instructions to speak with someone with a hearing impairment), and overenunciate (OE; given instructions to overenunciate each word). Speakers rated the amount of physical and mental effort exerted during each speaking condition using visual analog scales (averaged to yield a metric of overall speaking effort). Sentence productions were orthographically transcribed by 50 naive listeners. Linear mixed-effects models were used to compare intelligibility and speaking effort across the clear speech variants. RESULTS: Intelligibility was reduced for the PD group in comparison to the control group only in the habitual condition. All clear speech variants significantly improved intelligibility above habitual levels for the PD group, with OE maximizing intelligibility, followed by the SC and HI conditions. Both groups rated speaking effort to be significantly higher for both the OE and HI conditions versus the SC and habitual conditions. DISCUSSION: For speakers with PD, all clear speech variants increased intelligibility to a level comparable to that of healthy controls. All clear speech variants were also associated with higher levels of speaking effort than habitual speech for the speakers with PD. Clinically, findings suggest that clear speech training programs consider using the instruction "overenunciate" for maximizing intelligibility. Future research is needed to identify if high levels of speaking effort elicited by the clear speech variants affect long-term sustainability of the intelligibility benefit.