Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Didic, Mira; Ceccaldi, Mathieu; Poncet, Michel

doi:10.1159/000007914

Cited by 33 publications

(24 citation statements)

References 14 publications

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“…Most noteworthy, 11% of patients had AOS without any accompanying aphasia or dysarthria, a finding that supports those of single case or case series reports which suggest that AOS can be the only communication problem in neurodegenerative disease, at least early in its evolution (e.g., Broussolle et al, 1996;Cohen et al, 1993;Didic et al, 1998;Fukui et al, 1996;Sakurai et al, 1998;Tebartz Van Elst et al, 2002). When this is the case, a diagnostic designation of primary progressive AOS is appropriate and useful, both clinically and for research purposes.…”

Section: Presenting Complaints and Temporal Relationshipssupporting

confidence: 57%

Apraxia of speech in degenerative neurologic disease

2006

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Background: Only a limited number of case reports or case series have provided unequivocal evidence that apraxia of speech (AOS) can be the first or predominant sign of neurodegenerative disease. This may reflect a very low frequency of occurrence but may also reflect a failure in many studies to identify AOS or distinguish it from the diagnosis of primary progressive aphasia (PPA). The distinction may be important to clinical neurological localisation and diagnosis, prognosis, management, and histopathology. Aims: To describe the demographic characteristics, presenting complaints, perceptual attributes, associated speech-language and related clinical findings, neuroimaging findings, and clinical neurological diagnoses for a large group of patients with AOS due to degenerative neurologic disease. Methods & Procedures:Retrospective review of patients seen between 1985 and 2004, who had AOS that was never less severe than any aphasia that might have been present and whose neurologic evaluation concluded that the cause was degenerative.Outcomes & Results: A total of 80 patients met inclusion criteria. Of these patients, 61% were men. Average age at initial evaluation was 69 years (range536-86 years). Speechlanguage difficulty was the first symptom to emerge in 80% and the only patient complaint at the time of initial evaluation in 56%. Average duration of symptoms at initial evaluation was 29 months. Aphasia and dysarthria were unequivocally present in 49% and 50% of the sample, respectively. Of the 80 patients with AOS, 11% had neither aphasia nor dysarthria. When abnormal, neuroimaging often identified left or left.right hemisphere abnormalities. Of these patients, 44% received purely descriptive clinical neurologic diagnoses that were largely determined by speech-language findings; an additional 18% received diagnoses related to focal/asymmetric cortical atrophy syndromes that localised to the left hemisphere; 29% received diagnoses of diseases with prominent motor manifestations, most often corticobasal degeneration, progressive supranuclear palsy, or motor neuron disease. Conclusions: AOS can be the first and most prominent manifestation of neurodegenerative disease. It often co-occurs with aphasia and/or dysarthria, but it can be the only speech-language disorder. It does not appear to be associated with any single neurologic disease but specific neurologic diagnoses, when they are possible, tend to reflect conditions with prominent motor manifestations. It is important to distinguish primary progressive AOS from PPA.

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Section: Presenting Complaints and Temporal Relationshipssupporting

confidence: 57%

Apraxia of speech in degenerative neurologic disease

2006

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“…Mao et al [19] advocated that decreased gag reflex is a sign that can distinguish anterior operculum syndrome from other pseudobulbar palsies; however, retaining and decreasing the gag reflex does not always seem a decisive finding of significance for a diagnosis of anterior operculum syndrome, because Davies et al [9] reported that 37% of 140 healthy people lacked a gag reflex. Several investigators have reported progressive loss of speech output in anterior operculum syndrome [5,6,8,10,14,15,22,23,27,30,32]. Although progressive anarthria was common in these cases, the other clinical symptoms and the prognosis seemed to be heterogenous.…”

Section: Discussionmentioning

confidence: 99%

Progressive anterior operculum syndrome due to FTLD-TDP: a clinico-pathological investigation

et al. 2010

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Pathological investigation of progressive anterior operculum syndrome has rarely been reported. We describe clinico-pathological findings in a patient with progressive anterior operculum syndrome. A 74-year-old right-handed man had noticed speech and swallowing difficulties 1 year previously. Neurological examinations showed no abnormality other than a slight limitation of upward gaze and slow tongue movement without fibrillation. We investigated the patient using neuroimaging and neuropsychological examinations and observed him for 2 years until his death, at which point we obtained pathological findings. The patient's facial and masseteric muscles seemed hypotonic with drooling, but he could laugh and yawn normally, showing automatic voluntary dissociation. Palatal and pharyngeal reflexes were normal. Magnetic resonance imaging showed cortical atrophy in the temporal lobes bilaterally. (123)IMP single photon emission computed tomography and positron emission tomography showed decreased blood flow and activity in the frontotemporal lobes, predominantly on the left side. Neuropsychological examinations showed no aphasia, dementia or other neuropsychological abnormality. Intubation fiberscopy, laryngoscopy and video fluorography showed no abnormality. After 6 months his anarthria and dysphagia became aggravated. He died of aspiration pneumonia 2 years after onset. Postmortem examination revealed neuronal degeneration with TDP-43-positive inclusions in the frontal, temporal and insular cortices, consistent with frontotemporal lobar degeneration with TDP inclusions (FTLD-TDP). However, neuronal loss with gliosis was more prominent in the inferior part of the motor cortices, bilaterally. Progressive anterior operculum syndrome could be classified as a variant of FTLD-TDP.

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“…However, these congenitally dysarthric/anarthric patients could have developed alternative strategies, as is the case of callosal agenesia with respect to callosotomy. Agrammatism and orthographic errors (unfortunately a standard written language examination is lacking) are occasionally reported in the case of vascular pure anarthric (mute) patients (Vallar and Cappa, 1987;Nagy et al, 1999;Santens et al, 1999) and are almost invariably present in the case of slowly progressive anarthric patients (Tyrrell et al, 1991;Broussolle et al, 1996;Didic et al 1998;Silveri et al, 2003). In the latter case the writing deficits are similar to those reported in speech production, such as letter omissions, repetitions and transpositions (see, for example, the series reported by Broussolle et al, 1996).…”

Section: Introductionmentioning

confidence: 88%

Is slowly progressive anarthria a “pure” motor-speech disorder? Evidence from writing performance

Lucchelli

Papagno

2005

Neurocase

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It is usually assumed that writing is normal in patients with anarthria, but a careful examination of the literature shows that they produce deletions, transpositions and insertions. Indeed, a matter of debate concerns the distinction between primary progressive aphasia (PPA) and slowly progressive anarthria (SPA). If writing deficits were purely linguistic errors, then there would be no reason to consider slowly progressive anarthria as distinct from non-fluent PPA. We report the case of a patient with SPA in whom writing abilities were specifically assessed. No lexical-semantic deficits were detected, but errors were deletions, substitutions or transpositions, with no frequency, length or lexicality effect; moreover, controls produced the same kind of errors during articulatory suppression. It is suggested that subvocal rehearsal plays a role in writing, allowing the conversion/assembly of the phonological string in a graphemic representation. Therefore, writing deficits do not appear to have a linguistic basis and SPA seems distinguishable from nonfluent forms of aphasia.

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Progressive Loss of Speech: A Neuropsychological Profile of Premotor Dysfunction

Cited by 33 publications

References 14 publications

Apraxia of speech in degenerative neurologic disease

Apraxia of speech in degenerative neurologic disease

Progressive anterior operculum syndrome due to FTLD-TDP: a clinico-pathological investigation

Is slowly progressive anarthria a “pure” motor-speech disorder? Evidence from writing performance

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