Rapidly Progressive Gait and Coordination Difficulties

A 59-year-old woman noted sudden onset of slurred speech. Within a few days, she noted double vision, gait unsteadiness, and incoordination of her limbs. She sought care at her local emergency department. Computed tomography and magnetic resonance imaging of the head were negative for stroke. Her symptoms persisted. Neurologic examination indicated a moderate pancerebellar ataxia, without additional abnormalities. Her pursuit eye movements were saccadic. She had binocular diplopia with horizontal, gaze-evoked nystagmus. She had ataxic dysarthria and dysmetria of all limbs. Her steps and walking were irregular and she could not accomplish tandem gait. Additional neural antibody testing was undertaken, beyond the classic paraneoplastic antibodies. Metabotropic glutamate receptor 1-immunoglobulin G was detected in the serum and cerebrospinal fluid. The patient was diagnosed with autoimmune cerebellar ataxia. Because of the reported association of metabotropic glutamate receptor 1-immunoglobulin G with Hodgkin disease and non-Hodgkin lymphoma, positron emission tomography–computed tomography of the trunk (orbits to thighs) was performed, which was negative. After 6 weeks of intravenous methylprednisolone, the patient returned for evaluation. She had a mild ataxic dysarthria and minimal dysmetria of her left upper extremity only. She could tandem walk almost without error, and her gait appeared normal (no longer broad-based). At that point, immunotherapy was discontinued. At last follow-up, her neurologic examination findings remained stable. The subacute onset and rapid progression of ataxic symptoms in this adult patient led to suspicion for an autoimmune cause.

Dysarthria and Speech Intelligibility Following Parkinson’s Disease Globus Pallidus Internus Deep Brain Stimulation

Background: Although earlier studies reported variable speech changes following subthalamic nucleus (STN) deep brain stimulation (DBS) in Parkinson’s disease (PD) patients, the effects of globus pallidus internus (GPi) DBS on speech performance in PD remain largely unknown. Objective: We aimed to characterize speech changes following PD GPi-DBS. Methods: We retrospectively analyzed clinical and speech outcomes of 25 PD patients treated with bilateral GPi-DBS at a single center. Outcome measures included the Unified Parkinson’s Disease Rating Scale (UPDRS), speech subsystem domains (respiratory, laryngeal, resonance, orofacial, rate, prosody, rhythm, and naturalness), and overall speech intelligibility. Scores at baseline were compared with those at 6 months, 1 year, and the longest clinical follow-up available. Results: In the off-medication state, activities of daily living and motor function based on UPDRS II and III significantly improved postoperatively. We observed unique patterns of speech changes in patients with PD following GPi-DBS in the short- (n = 25) and longer-term (n = 8) follow-up periods. Velopharyngeal (resonance), laryngeal components, and prosody worsened after bilateral GPi-DBS (p < 0.015). Speech intelligibility did not worsen after GPi-DBS in the short-term, but there was a trend to deteriorate at long-term follow-up (e.g., one year and beyond). We observed worsening of hypokinetic dysarthria in individual patients. Also, a minority of patients developed stuttering, spastic dysarthria, or ataxic dysarthria. Conclusion: Bilateral GPi-DBS worsened several modalities of parkinsonian speech without compromising overall speech intelligibility. GPi-DBS can potentially worsen or induce hypokinetic dysarthria, stuttering, spastic dysarthria, or ataxic dysarthria. GPi-DBS may have different and variable effects on speech function when compared to STN-DBS.

A study of quantitative and qualitative analysis of standardized speech samples in persons suffering from dysarthria due to various neurological disorder

Background: Dysarthria is manifested as a disorder of movement, it is important to recognize that sensori-motor integration (with tactile, proprioceptive, and auditory feed-back representing the crucial sensory components) is essential to speech motor control, from this standpoint, most or all dysarthria localized to the central nervous system should be thought of as sensori-motor rather than simply motor disturbances.Methods: This non-interventional, cross-sectional comparative, observational study, conducted in 100 study subjects (50 cases and 50 controls) from March 2016 to February 2017 at MGM medical college and MY hospital Indore, MP, India.Results: The mean age of normal population was 53 years and that of dysarthric population was 55 years. Among the dysarthric group, there were 10 cases of ataxic dysarthria, 23 cases of spastic dysarthria, and 9 cases of hypo kinetic dysarthria. There were 20 cases of mild dysarthria 19 cases of moderate dysarthria and 10 cases of severe dysarthria. In ataxic dysarthria, pitch break was found in 6 out of 10 subjects. It was found that there is negative predictive value 93.33%, and positive predictive value, 77.14% in spastic dysarthria and negative predictive value, 83.33% and positive predictive value, 90.90% in ataxic, whereas negative predictive value, 85.71% and positive predictive value, 95.34% in hypo kinetic dysarthria. Conclusions: Different types of dysarthria when analyzed with software tool after extracting pitch and formants showed specific patterns. These patterns correlated with the clinical diagnosis. And Pattern recognition of different dysarthria will help to identify the types of dysarthria in scientific way and prevent inter-subject variability.

PSP-like syndrome after aortic surgery in adults (Mokri syndrome)

BackgroundA rare progressive supranuclear palsy–like syndrome seemingly triggered by aortic surgery was first described in 2004. This largest case series to date describes the features of this syndrome.MethodsWe searched the Mayo Clinic electronic medical records using the advanced cohort explorer search engine for patients evaluated for neurologic symptoms after cardiac-aortic surgery in the past 30 years. Data were extracted to Microsoft Excel from the identified patients and included clinical and neuroimaging features and outcomes.ResultsTwenty-five patients met the inclusion criteria. All surgeries were performed under thoracic aortic bypass and deep hypothermia. Surgery included aortic aneurysm, aortic valve repair, and/or aortic dissection repair. Surgical records were unavailable, although surgery was documented in the Mayo record as uncomplicated in 60% of cases. In the remaining cases, no particular intraoperative or postoperative complications were documented at a high frequency. A typical triad was documented: supranuclear gaze palsy (SNGP; 100%), gait imbalance (80%), and dysarthria (96%). Part or all of the triad was observed before hospital discharge and stabilized over the course of days-weeks. A second phase of symptom worsening plus new symptoms developed up to a year later; this decline continued for up to several years before stabilization. Delayed epileptic seizures occurred in 32% of patients. Brain MRI revealed only nonspecific findings.ConclusionThis syndrome following adult thoracic aortic bypass surgery with deep hypothermia remains unexplained. It follows a biphasic course and is characterized by the triad of SNGP, unsteady gait, and a predominantly ataxic dysarthria.

Dysarthria Profiles in Adults With Hereditary Ataxia

Purpose This preliminary study examined whether speech profiles exist for adults with hereditary ataxia based on 2 competing frameworks: a pattern of instability/inflexibility or a pattern of differential subsystem involvement. Method Four dysarthria experts rated the speech samples of 8 adults with dysarthria from hereditary ataxia using visual analog scales and presence/severity rating scales of speech characteristics. Speaking tasks included diadochokinetics, sustained phonation, and a monologue. Results Speech profiles aligned with the instability/inflexibility framework, with the pattern of instability being the most common. Speech profiles did not emerge for the majority of speakers using the differential subsystem framework. Conclusions The findings extend previous research on pure ataxic dysarthria and suggest a possible framework for understanding the speech heterogeneity associated with the ataxias. The predominance of the instability profile is consistent with the notion of impaired feedforward control in speakers with cerebellar disruption.

Generalized Adaptation to Dysarthric Speech

Purpose Generalization of perceptual learning has received limited attention in listener adaptation studies with dysarthric speech. This study investigated whether adaptation to a talker with dysarthria could be predicted by the nature of the listener's prior familiarization experience, specifically similarity of perceptual features, and level of intelligibility. Method Following an intelligibility pretest involving a talker with ataxic dysarthria, 160 listeners were familiarized with 1 of 7 talkers with dysarthria—who differed from the test talker in terms of perceptual similarity (same, similar, dissimilar) and level of intelligibility (low, mid, high)—or a talker with no neurological impairment (control). Listeners then completed an intelligibility posttest on the test talker. Results All listeners benefited from familiarization with a talker with dysarthria; however, adaptation to the test talker was superior when the familiarization talker had similar perceptual features and reduced when the familiarization talker had low intelligibility. Conclusion Evidence for both generalization and specificity of learning highlights the differential value of listeners' prior experiences for adaptation to, and improved understanding of, a talker with dysarthria. These findings broaden our theoretical knowledge of adaptation to degraded speech, as well as the clinical application of training paradigms that exploit perceptual processes for therapeutic gain.