Clinical Study of the Responsible Lesion for Dysarthria in the Cerebellum

Ogawa, Kōichi; Yoshihashi, Hidetaka; Suzuki, Yutaka; Kamei, Satoshi; Mizutani, Tetsuya

doi:10.2169/internalmedicine.49.2913

Cited by 11 publications

(5 citation statements)

References 10 publications

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“…Similarly, using fMRI, Urban (2013) described cerebellar activation during speech production. Ogawa, Yoshihashi, Suzuki, Kamei, and Mizutani (2010) proposed that cerebellar dysarthria is characterized by ‘… ataxic speech, and is mainly caused by impairment of the upper cerebellar hemisphere in the distribution of the superior cerebellar artery (SCA)’ (p. 861). The link between the known cerebellar differences in persons with DS paired with the motor speech disorder results and ataxic dysarthria findings further support the role of the cerebellum in speech production and the potential causative role of cerebellar dysfunction in motor speech disorder and intelligibility deficits in DS.…”

Section: Discussionmentioning

confidence: 99%

Estimates of the prevalence of speech and motor speech disorders in adolescents with Down syndrome

Wilson

Abbeduto

Camarata

et al. 2019

Clinical Linguistics & Phonetics

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Although there is substantial rationale for a motor component in the speech of persons with Down syndrome (DS), there presently are no published estimates of the prevalence of subtypes of motor speech disorders in DS. The goal of this research is to provide initial estimates of the prevalence of types of speech disorders and motor speech disorders in adolescents with DS. Conversational speech samples from a convenience sample of 45 adolescents with DS, ages 10 to 20 years old, were analysed using perceptual and acoustic methods and measures in the Speech Disorders Classification System (SDCS). The SDCS cross-classified participants into five mutually exclusive speech classifications and five mutually exclusive motor speech classifications. For participants meeting criteria for Childhood Dysarthria or for Childhood Dysarthria concurrent with Childhood Apraxia of Speech, the SDCS provided information on participants’ percentile status on five subtypes of dysarthria. A total of 97.8% of participants met SDCS criteria for Speech Disorders and 97.8% met criteria for Motor Speech Disorders, including Childhood Dysarthria (37.8%), Speech Motor Delay (26.7%), Childhood Dysarthria and Childhood Apraxia of Speech (22.2%), and Childhood Apraxia of Speech (11.1%). Ataxia was the most prevalent dysarthria subtype. Nearly all participants with DS in the present sample had some type of speech and motor speech disorder, with implications for theory, assessment, prediction, and treatment. Specific to treatment, the present findings are interpreted as support for motor speech disorders as a primary explanatory construct to guide the selection and sequencing of treatment targets for persons with DS.

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Section: Discussionmentioning

confidence: 99%

Estimates of the prevalence of speech and motor speech disorders in adolescents with Down syndrome

Wilson

Abbeduto

Camarata

et al. 2019

Clinical Linguistics & Phonetics

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“…Dysarthria results in slurred and irregular speech with impaired pronunciation, due to a lack of coordination of the motor-speech system [42]. It results from a combination of ataxia and dystonia and involves pathologies in the cerebellum and basal ganglia.…”

Section: Introductionmentioning

confidence: 99%

Niemann-Pick disease type C symptomatology: an expert-based clinical description

Mengel

Klünemann

Lourenço

et al. 2013

Orphanet J Rare Dis

125

114

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Niemann-Pick disease type C (NP-C) is a rare, progressive, irreversible disease leading to disabling neurological manifestations and premature death. The estimated disease incidence is 1:120,000 live births, but this likely represents an underestimate, as the disease may be under-diagnosed due to its highly heterogeneous presentation. NP-C is characterised by visceral, neurological and psychiatric manifestations that are not specific to the disease and that can be found in other conditions. The aim of this review is to provide non-specialists with an expert-based, detailed description of NP-C signs and symptoms, including how they present in patients and how they can be assessed. Early disease detection should rely on seeking a combination of signs and symptoms, rather than isolated findings. Examples of combinations which are strongly suggestive of NP-C include: splenomegaly and vertical supranuclear gaze palsy (VSGP); splenomegaly and clumsiness; splenomegaly and schizophrenia-like psychosis; psychotic symptoms and cognitive decline; and ataxia with dystonia, dysarthria/dysphagia and cognitive decline. VSGP is a hallmark of NP-C and becomes highly specific of the disease when it occurs in combination with other manifestations (e.g. splenomegaly, ataxia). In young infants (<2 years), abnormal saccades may first manifest as slowing and shortening of upward saccades, long before gaze palsy onset. While visceral manifestations tend to predominate during the perinatal and infantile period (2 months–6 years of age), neurological and psychiatric involvement is more prominent during the juvenile/adult period (>6 years of age). Psychosis in NP-C is atypical and variably responsive to treatment. Progressive cognitive decline, which always occurs in patients with NP-C, manifests as memory and executive impairment in juvenile/adult patients. Disease prognosis mainly correlates with the age at onset of the neurological signs, with early-onset forms progressing faster. Therefore, a detailed and descriptive picture of NP-C signs and symptoms may help improve disease detection and early diagnosis, so that therapy with miglustat (Zavesca®), the only available treatment approved to date, can be started as soon as neurological symptoms appear, in order to slow disease progression.

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“…Functional MRI (fMRI) studies in healthy volunteers show that the cerebellar representation of the tongue and orofacial muscles corresponds to that of the area involved in patients with cerebellar dysarthria due to focal vascular lesions (Urban et al 2003). Impairment of the upper cerebellar hemisphere in the territory of the superior cerebellar artery results in dysarthria (Ogawa et al 2010). Combined parametric data (measurements at the acoustic speech signal, tracking of articulatory movements and fMRI studies) have contributed to redefine the concept of ataxic dysarthria and indicated the cerebellum to support several aspects of speech processing.…”

Section: Dysarthria and Other Speech Deficitsmentioning

confidence: 99%

Cerebellar Motor Disorders

Grimaldi

2021

Handbook of the Cerebellum and Cerebellar Disorders

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Many of the clinical manifestations of cerebellar damage were described at the turn of the nineteenth and twentieth centuries by the pioneers of the cerebellar physiopathology, including Luciani, Babinski, and Holmes. Cerebellar diseases result in lack of coordination and disturbances of accuracy of movements, causing a constellation of symptoms and motor signs which can be grouped into four categories: oculomotor disturbances, speech deficits, deficits of limb movements, and abnormalities of gait and posture. Instability of gaze and nystagmus, hypermetria/hypometria of saccades, saccadic pursuit, skew deviation (ocular misalignment), and disorders of vestibulo-ocular reflex/optokinetic responses are the main oculomotor alterations observed in cerebellar diseases. Gaze-evoked nystagmus is the most common form of nystagmus encountered in disorders of the cerebellum. Ataxic dysarthria has a typical scanning quality; it is often explosive, with a staccato rhythm and a nasal character. Ataxic speech tends to become slow with slurring, and words may be unintelligible. Cerebellar damage typically results in impairment of performance of limb movements. Ataxia of G. Grimaldi (*) Unité d'Etude du Mouvement (UEM), Neurologie -ULB Erasme, Bruxelles, Belgium

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Clinical Study of the Responsible Lesion for Dysarthria in the Cerebellum

Cited by 11 publications

References 10 publications

Estimates of the prevalence of speech and motor speech disorders in adolescents with Down syndrome

Estimates of the prevalence of speech and motor speech disorders in adolescents with Down syndrome

Niemann-Pick disease type C symptomatology: an expert-based clinical description

Cerebellar Motor Disorders

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