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Neurology Jan 2013To review descriptions of paroxysmal dysarthria and ataxia in multiple sclerosis (MS), with special attention given to Parker and his 1946 case series.
OBJECTIVE
To review descriptions of paroxysmal dysarthria and ataxia in multiple sclerosis (MS), with special attention given to Parker and his 1946 case series.
METHODS
Evaluation of original publications describing paroxysmal dysarthria and ataxia, bibliographic information, writings, and unpublished letters from the Mayo Clinic Historical Unit.
RESULTS
In 1940, Störring described a patient with MS with paroxysmal symptoms that included dizziness and trouble speaking, but also unilateral extremity weakness. In 1946, Parker published a series of 11 patients with paroxysmal dysarthria and ataxia. Six of these patients had MS, and he recognized this phenomenon as a manifestation of the disease. The term "paroxysmal dysarthria and ataxia" was first used in 1959 by Andermann and colleagues. Since that time, paroxysmal dysarthria and ataxia has become a well-recognized phenomenon in MS. More recent reports have suggested that the responsible lesion is located in the midbrain, near or involving the red nucleus.
CONCLUSIONS
Parker was the first to accurately describe paroxysmal dysarthria and ataxia in patients with MS.
Topics: Ataxia; Dysarthria; History, 20th Century; Humans; Multiple Sclerosis; Neurology
PubMed: 23319475
DOI: 10.1212/WNL.0b013e31827dec0f -
BMJ Case Reports Mar 2020Severe hyperhomocysteinemia (>100 µmol/L) is often associated with inborn errors of homocysteine metabolism. It manifests typically in neonatal period with...
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 B deficiency, a markedly elevated serum homocysteine and low methionine. Despite vitamin supplementation homocysteine levels remained elevated. Molecular studies of 5,10-methylenetetrahydrofolate reductase () 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.
Topics: Age of Onset; Betaine; Dysarthria; Folic Acid; Gait Ataxia; Homocystinuria; Humans; Male; Methylenetetrahydrofolate Reductase (NADPH2); Muscle Spasticity; Psychotic Disorders; Tremor; Vitamin B 12; Young Adult
PubMed: 32161077
DOI: 10.1136/bcr-2019-232241 -
Journal of Speech, Language, and... Jan 2023This study examined spontaneous, spoken-to-a-model, and two sung modes in speakers with Parkinson's disease (PD), speakers with cerebellar disease (CD), and healthy...
PURPOSE
This study examined spontaneous, spoken-to-a-model, and two sung modes in speakers with Parkinson's disease (PD), speakers with cerebellar disease (CD), and healthy controls. Vocal performance was measured by intelligibility scores and listeners' perceptual ratings.
METHOD
Participants included speakers with hypokinetic dysarthria secondary to PD, those with ataxic dysarthria secondary to CD, and healthy speakers. Participants produced utterances in four vocal modes: spontaneous speech, spoken-to-a-model, sung-to-a-model, and spontaneous singing. For spoken-to-a-model and sung-to-a-model modes, written material was provided the model. For spontaneous singing, participants sang songs that they endorsed as familiar.
DEPENDENT VARIABLES
In Experiment I, listeners orthographically transcribed the audio samples of the first three vocal modes. In Experiment IIa, raters evaluated the accuracy of the pitch and rhythm of the spontaneous singing of familiar songs. Finally, familiar songs and sung-to-a-model utterances were rated on a competency scale by a second group of raters (Experiment IIb).
RESULTS
Results showed increases in intelligibility during the spoken-to-a-model mode compared with the spontaneous mode in both PD and CD groups. Singing enhanced the vocal output of speakers with PD more than in speakers with CD, as measured by percent intelligibility. PD participants' pitch and rhythm accuracy and competency in singing familiar songs was rated more favorably than those produced by CD participants.
CONCLUSIONS
The findings reveal a vocal task effect for spoken utterances in both groups. Sung exemplars, more impaired in CD, suggest a significant involvement of the cerebellum in singing.
SUPPLEMENTAL MATERIAL
https://doi.org/10.23641/asha.21809544.
Topics: Humans; Singing; Speech Intelligibility; Parkinson Disease; Music; Dysarthria; Spinocerebellar Ataxias
PubMed: 36608288
DOI: 10.1044/2022_JSLHR-22-00274 -
American Journal of Speech-language... Jun 2021Purpose This study describes motor speech disorders and associated communication limitations in six variants of progressive supranuclear palsy (PSP). Method The...
Purpose This study describes motor speech disorders and associated communication limitations in six variants of progressive supranuclear palsy (PSP). Method The presence, nature, and severity of dysarthria and apraxia of speech (AOS) were documented, along with scores on the Apraxia of Speech Rating Scale-Version 3 (ASRS-3) for 77 (40 male and 37 female) patients with PSP. Clinician-estimated and patient-estimated communication limitations were rated using the Motor Speech Disorders Severity Rating (MSDSR) Scale and the Communicative Effectiveness Survey (CES), respectively. Descriptive statistics were calculated for each of these dependent variables. One-tailed tests were conducted to test mean differences in ASRS-3 and CES between participants with and without AOS and between participants with and without dysarthria. Spearman rank correlations were calculated between ASRS-3 scores and clinical judgments of AOS and dysarthria severity and between MSDSR and CES ratings. Results Nine participants (12%) had normal speech. Eighty-seven percent exhibited dysarthria; hypokinetic and mixed hypokinetic-spastic dysarthria were observed most frequently. AOS was observed in 19.5% of participants across all variants, but in only 10% exclusive of the PSP speech and language variant. Nearly half presented with AOS in which neither phonetic nor prosodic features clearly predominated. The mean ASRS-3 score for participants with AOS was significantly higher than for those without and correlated strongly with clinician judgment of AOS severity. Mean ASRS-3 was higher for participants with dysarthria than for those without but correlated weakly with dysarthria severity. Mean MSDSR and CES ratings were lower in participants with AOS compared to those without and moderately correlated with each other. Conclusions Motor speech disorders that negatively impact communicative effectiveness are common in PSP and occur in many variants. This is the first description of motor speech disorders across PSP variants, setting the stage for future research characterizing neuroanatomical correlates, progression of motor speech disorders, and benefits of targeted interventions. Supplemental Material https://doi.org/10.23641/asha.14111837.
Topics: Apraxias; Dysarthria; Female; Humans; Language; Male; Speech; Speech Disorders; Supranuclear Palsy, Progressive
PubMed: 33719524
DOI: 10.1044/2020_AJSLP-20-00126 -
American Journal of Speech-language... Aug 2023Few studies have reported on the vowel space area (VSA) in both acoustic and kinematic domains. This study examined acoustic and kinematic VSAs for speakers with and...
PURPOSE
Few studies have reported on the vowel space area (VSA) in both acoustic and kinematic domains. This study examined acoustic and kinematic VSAs for speakers with and without dysarthria and evaluated effects of normalization on acoustic and kinematic VSAs and the relationship between these measures.
METHOD
Vowel data from 12 speakers with and without dysarthria, presenting with a range of speech abilities, were examined. The speakers included four speakers with Parkinson's disease (PD), four speakers with brain injury (BI), and four neurotypical (NT) speakers. Speech acoustic and kinematic data were acquired simultaneously using electromagnetic articulography during a passage reading task. Raw and normalized VSAs calculated from corner vowels /i/, /æ/, /ɑ/, and /u/ were evaluated. Normalization was achieved through score transformations to the acoustic and kinematic data. The effect of normalization on variability within and across groups was evaluated. Regression analysis was used across speakers to assess the association between acoustic and kinematic VSAs for both raw and normalized data.
RESULTS
When evaluating the speakers as three different groups (i.e., PD, BI, and NT), normalization reduced the standard deviations within each group and changed the relative differences in average magnitude between groups. Regression analysis revealed a significant relationship between normalized, but not raw, acoustic and kinematic VSAs, after the exclusion of an outlier speaker.
CONCLUSIONS
Normalization reduces the variability across speakers, within groups, and changes average magnitudes affecting speaker group comparisons. Normalization also influences the correlation between acoustic and kinematic measures. Further investigation of the impact of normalization techniques upon acoustic and kinematic measures is warranted.
SUPPLEMENTAL MATERIAL
https://doi.org/10.23641/asha.22669747.
Topics: Humans; Speech Intelligibility; Speech Production Measurement; Speech Acoustics; Dysarthria; Biomechanical Phenomena; Acoustics; Parkinson Disease; Phonetics
PubMed: 37105919
DOI: 10.1044/2023_AJSLP-22-00158 -
Journal of the Neurological Sciences Jan 2023Spinocerebellar ataxia type 31 (SCA31) is an autosomal dominant disease, classified amongst pure cerebellar ataxias (ADCA type 3). While SCA31 is the third most... (Review)
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.
Topics: Humans; Middle Aged; Dysarthria; Hypesthesia; Spinocerebellar Ataxias; Cerebellar Ataxia; Atrophy
PubMed: 36563608
DOI: 10.1016/j.jns.2022.120527 -
Folia Phoniatrica Et Logopaedica :... 2021Communication is affected in most people with amyotrophic lateral sclerosis (ALS); up to 80-95% will reach a point where they are no longer able to meet their...
BACKGROUND
Communication is affected in most people with amyotrophic lateral sclerosis (ALS); up to 80-95% will reach a point where they are no longer able to meet their communicative needs with natural speech. The deterioration of speech and communicative abilities presumably has an impact on communicative participation. However, little is known about how these factors relate to each other in this population of patients.
OBJECTIVE
This study aimed to investigate the association between communicative participation, functional deficits, and severity of dysarthria in individuals with ALS.
METHOD
Thirty people with ALS were rated for (1) communicative participation, using the Communicative Participation Item Bank (CPIB, Swedish); and (2) disability related to the disease, using the Revised ALS Functional Rating Scale (Swedish). An expert listening panel assessed intelligibility and severity of dysarthria based on recorded text readings and sentences from the Swedish Test of Intelligibility.
RESULTS
CPIB scores were significantly lower for participants with moderate/severe dysarthria than for those with no/mild dysarthria and correlated with bulbar function and intelligibility.
CONCLUSION
The study found that the CPIB provides a means to rate and discuss communicative participation with persons with ALS, which could assist in the planning of further efforts/services.
Topics: Amyotrophic Lateral Sclerosis; Communication; Dysarthria; Humans; Speech; Speech Disorders; Speech Intelligibility
PubMed: 31918429
DOI: 10.1159/000505022 -
BMJ Case Reports Feb 2021A 73-year-old man who presented with fever and abdominal discomfort was diagnosed to have a liver abscess. He was treated with antimicrobials which included...
A 73-year-old man who presented with fever and abdominal discomfort was diagnosed to have a liver abscess. He was treated with antimicrobials which included metronidazole. One month into treatment, he developed neurological symptoms and signs that were suggestive of cerebellar pathology. MRI of the brain showed T2/fluid attenuated inversion recovery hyperintensities involving bilateral dentate, fastigial and interpositus nuclei. After excluding common aetiologies, the possibility of metronidazole-induced neurotoxicity was considered. After stopping metronidazole, his symptoms and signs resolved. A subsequent MRI scan of the brain showed reversal of changes. Neurotoxicity caused by metronidazole is an uncommon adverse effect of a commonly used antimicrobial drug and should be considered in the appropriate clinical scenario.
Topics: Aged; Anti-Bacterial Agents; Ataxia; Cerebellar Diseases; Cerebellar Nuclei; Duration of Therapy; Dysarthria; Humans; Liver Abscess; Magnetic Resonance Imaging; Male; Metronidazole; Neurotoxicity Syndromes
PubMed: 33526534
DOI: 10.1136/bcr-2020-239227 -
Journal of Speech, Language, and... Aug 2023The purpose of this study was to describe, compare, and understand speech modulation capabilities of patients with varying motor speech disorders (MSDs) in a paradigm in...
PURPOSE
The purpose of this study was to describe, compare, and understand speech modulation capabilities of patients with varying motor speech disorders (MSDs) in a paradigm in which patients made highly cued attempts to speak faster or slower.
METHOD
Twenty-nine patients, 12 with apraxia of speech (AOS; four phonetic and eight prosodic subtype), eight with dysarthria (six hypokinetic and two spastic subtype), and nine patients without any neurogenic MSD completed a standard motor speech evaluation where they were asked to repeat words and sentences, which served as their "natural" speaking rate. They were then asked to repeat lower complexity (counting 1-5; repeating "cat" and "catnip" 3 times each) and higher complexity stimuli (repeating "catastrophe" and "stethoscope" 3 times each and "My physician wrote out a prescription" once) as fast/slow as possible. Word durations and interword intervals were measured. Linear mixed-effects models were used to assess differences related to MSD subtype and stimuli complexity on bidirectional rate modulation capacity as indexed by word duration and interword interval. Articulatory accuracy was also judged and compared.
RESULTS
Patients with prosodic AOS demonstrated a reduced ability to go faster; while they performed similarly to patients with spastic dysarthria when counting, patients with spastic dysarthria were able to increase rate similar to controls during sentence repetition; patients with prosodic AOS could not and made increased articulatory errors attempting to increase rate. AOS patients made more articulatory errors relative to other groups, regardless of condition; however, their percentage of errors reduced with an intentionally slowed speaking rate.
CONCLUSIONS
The findings suggest comparative rate modulation abilities in conjunction with their impact on articulatory accuracy may support differential diagnosis between healthy and abnormal speech and among subtypes of MSDs (i.e., type of dysarthria or AOS). Findings need to be validated in a larger, more representative cohort encompassing several types of MSDs.
SUPPLEMENTAL MATERIAL
https://doi.org/10.23641/asha.22044632.
Topics: Humans; Dysarthria; Speech; Apraxias; Phonetics; Speech Production Measurement; Speech Disorders
PubMed: 36780318
DOI: 10.1044/2022_JSLHR-22-00286 -
Muscle & Nerve Sep 2018Herein we present an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) vs. healthy controls.
INTRODUCTION
Herein we present an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) vs. healthy controls.
METHODS
We evaluated 41 children with CDM and 29 healthy controls for speech and swallow function and for lingual and labial strength.
RESULTS
The Iowa Oral Performance Instrument (IOPI), measuring tongue strength, and a lip force meter (LFM), measuring lip strength, had excellent interrater reliability with intraclass correlation coefficients (ICCs) of 0.75 (n = 19, P < 0.001) and 0.96 (n = 20, P < 0.001), respectively. Mean overall lingual strength was 3.5-fold less and labial strength was about 7-fold less in CDM patients than in healthy controls. Eighteen of 24 children with CDM demonstrated dysarthria and an additional 11 participants were nonverbal. Dysarthria correlated moderately with lingual strength, age, and dysphagia. Strength measures correlated moderately with dysphagia.
DISCUSSION
Children with CDM have impaired orofacial functioning that affects communication and swallowing. Reliability of strength measures may be useful for future therapeutic trials. Muscle Nerve 58: 413-417, 2018.
Topics: Adolescent; Child; Child, Preschool; Deglutition Disorders; Dysarthria; Facial Muscles; Female; Humans; Infant; Lip; Male; Muscle Strength; Myotonic Dystrophy; Tongue
PubMed: 29901230
DOI: 10.1002/mus.26176