Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes

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Abstract

We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6–10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias.

Introduction

The syndrome of primary progressive aphasia (PPA) without generalised dementia, first described by Mesulam (Mesulam, 1982), has been related to focal cortical atrophy and hypometabolism of the left perisylvian area (Tyrrell et al., 1990; McDaniel et al., 1991; Snowden et al., 1992). As neuropathological experience in PPA is limited the question remains whether PPA is related to a distinct pathological entity. Most authors report non-specific neuronal loss in the left perisylvian region with spongiform changes within superficial cortical layers (Kirshner et al., 1987; Snowden et al., 1992; Scheltens et al., 1994). Rare cases have been related to a specific disease: Creutzfeldt-Jakob disease (Mandell et al., 1989), Pick's disease (Holland et al., 1985; Graff-Radford et al., 1990; Kertesz et al., 1994), or Alzheimer's disease (Kempler et al., 1990). Most of them however, would not fit with Mesulam's definition of PPA (Mesulam and Weintraub, 1992). Syndromes of non-specific focal cortical degeneration involving the frontal lobes have been also described (Brun, 1987; The Lund and Manchester Groups, 1994), which definition needs also to be improved on both clinical and pathological grounds. Recently, Tyrrell (Tyrrell et al., 1991) described 3 cases of slowly progressive dysarthria with initially intact written language. We could also examine patients sharing similar clinical characteristics, two of which were published as single case studies (Broussolle et al., 1992; Cohen et al., 1993). All presented with progressive anarthria which progressed to muteness and cognitive disturbances of the frontal type. Other signs, as operculum syndrome, pyramidal or extrapyramidal syndromes, were present in the most advanced cases. To delineate more clearly this unusual syndrome, we performed extensive neuropsychological examination, brain imaging and metabolic cerebral studies in a series of 8 cases. This report describes our findings with pathological data in 2 cases. We discuss the possible overlap between this syndrome and other clinical conditions, particularly PPA and fronto-temporal dementias.

Section snippets

Neuropsychological assessment

All patients received the most extensive evaluation possible, given their clinical condition. In some patients, assessment needed adaptation to speech output difficulties, by using written answers instead of the usual oral, or by duplicating, when applicable, the usual tests with nonverbal tests.

Language assessment using the Boston Diagnostic Aphasia Examination, BDAE (French version from Mazaux and Orgogozo, 1983) was performed at least twice in most patients, except Case 4 who was almost mute

Case 1

This housewife had progressive speech difficulties by her 67th year. When first seen three years post-onset, language examination showed an isolated speech disturbance with moderate speech apraxia, mild dysarthria, dysprosody and orofacial apraxia. There was otherwise no aphasia, and no writing abnormalities. Performances with Syntax Comprehension Test were subnormal (score: −1 S.D. of control group mean). There was no semantic disturbance at Binois-Pichot (score: 102; control mean: 100 ± 15)

Results and discussion

We present a series of 8 patients with striking clinical and brain imaging similarities. We will first analyze the symptomatology and the anatomoclinical correlations of this syndrome that we propose to call Slowly Progressive Anarthria (SPA). We will then discuss the nature of SPA and its place among other focal degenerative syndromes of the frontal lobe.

Acknowledgements

We wish to thank P.M. Gonnaud and J.C. Antoine for referring their patient, B. Schott and M. Habib for scientific discussion, and N. Benoit, M. Bourguignon, M. Charensol, N. Foyatier, G. Galy, M.F. Gravejat, E. Gros, A. Jouvet, C. Labbé, P. Landais, F. Lavenne, D. Le Bars, G. Mollaret, J.F. Mosnier, Y. Samson, J.P. Serrat and D. Sosson for their help.

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      Anarthria has been described in association with different lesions: with rapid onset in cases of bulbar onset ALS [54], with opercular syndrome (known as Foix-Chavany-Marie syndrome), a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal and brachial muscles. It may also be a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum [55], after ischemic stroke of the pyramidal decussation causing quadriplegia and anarthria [56], as slowly progressive anarthria with late anterior opercular syndrome [57], and as persistent anarthria following mirror infarction in the anterior choroidal artery territory [58]. In an attempt to depict the roles of central and peripheral structures responsible for speech planning and expression, speech can be likened to a boat: specific areas of the cerebral cortex resemble a boat (= words) builder who chooses the characteristics (type, structure, size, shape) required for a specific task such as floating and transport (specific word message).

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