Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy.

Pillon, B.; Gouider-Khouja, N.; Deweer, Bernard; Vidailhet, Marie; Malapani, Chara; Dubois, Bruno; Agid, Yves

doi:10.1136/jnnp.58.2.174

Cited by 152 publications

(80 citation statements)

References 30 publications

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“…This test evaluates memory performance both quantitatively and qualitatively, perseverations, intrusions, sensitivity to interference, capacities to initiate semantic and serial clustering, consistency of recall and recognition (Delis et al, 1987). Executive functions were evaluated with the frontal score (Pillon et al, 1995) including the simpli®ed version of the Wisconsin Card Sorting Test (WCST) (Nelson, 1976) and two 1-min verbal¯uency tasks (words beginning with letter`m' and names of animals) (Thuillard and Assal, 1991). Mood was evaluated with the Montgomery and Asberg Depression Rating Scale (MADRS) (Montgomery and Asberg, 1979).…”

Section: Neuropsychological Testsmentioning

confidence: 99%

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies

Ber¹

2003

Brain

213

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SummaryAtaxia with ocular motor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia (ARCA) associated with oculomotor apraxia, hypoalbuminaemia and hypercholesterolaemia. The gene APTX, which encodes aprataxin, has been identi®ed recently. We studied a large series of 158 families with non-Friedreich progressive ARCA. We identi®ed 14 patients (nine families) with ®ve different missense or truncating mutations in the aprataxin gene (W279X, A198V, D267G, W279R, IVS5+1), four of which were new. We determined the relative frequency of AOA1 which is 5%. Mutation carriers underwent detailed neurological, neuropsychological, electrophysiological, oculographic and biological examinations, as well as brain imaging. The mean age at onset was 6.8 T 4.8 years (range 2±18 years). Cerebellar ataxia with cerebellar atrophy on MRI and severe axonal sensorimotor neuropathy were present in all patients. In contrast, oculomotor apraxia (86%), hypoalbuminaemia (83%) and hypercholesterolaemia (75%) were variable. Choreic movements were frequent at onset (79%), but disappeared in the course of the disease in most cases. However, a remarkably severe and persistent choreic phenotype was associated with one of the mutations (A198V). Cognitive impairment was always present. Ocular saccade initiation was normal, but their duration was increased by the succession of multiple hypometric saccades that could clinically be confused with`slow saccades'. We emphasize the phenotypic variability over the course of the disease. Cerebellar ataxia and/or chorea predominate at onset, but later on they are often partially masked by severe neuropathy, which is the most typical symptom in young adults. The presence of chorea, sensorimotor neuropathy, oculomotor anomalies, biological abnormalities, cerebellar atrophy on MRI and absence of the Babinski sign can help to distinguish AOA1 from Friedreich's ataxia on a clinical basis. The frequency of chorea at onset suggests that this diagnosis should also be considered in children with chorea who do not carry the IT15 mutation responsible for Huntington's disease.

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Section: Neuropsychological Testsmentioning

confidence: 99%

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies

Ber¹

2003

Brain

213

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“…TMT-B has been frequently used as an index of cognitive flexibility or set-shifting, an important aspect of executive attention control (Arbuthnott & Frank, 2000;Horton, 1979;Kortte, Horner, & Windham, 2002;Moll, Oliveira-Souza Rd, Moll, Bramati, & Andreiuolo, 2002). The TMT has been demonstrated to be sensitive to cognitive impairment in a range of agerelated clinical conditions, including Parkinson disease (Goldman, Baty, Buckles, Sahrmann, & Morris, 1998;Pillon et al, 1995), Alzheimer's disease (Chen et al, 2000(Chen et al, , 2001, and other dementias (Hestad, Aukrust, Ellertsen, & Klove, 1996;Heun, Papassotiropoulos, & Jennssen, 1998;Lunn et al, 1991;Paul et al, 2001;Selnes et al, 1991).…”

Section: Introductionmentioning

confidence: 99%

Demographic Influences on Baseline and Derived Scores From the Trail Making Test in Healthy Older Australian Adults

Hester

Kinsella

Ong

et al. 2005

The Clinical Neuropsychologist

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“…MSA-P is the more common manifestation of MSA in Western populations [89, 90]; therefore, cognitive dysfunction in MSA-P had attracted considerable attention, particularly in comparison with PD and/or progressive supranuclear palsy [82,83,84,86,87,88]. MSA-P patients showed significant deficits in the tests previously shown to be sensitive to frontal lobe dysfunction [83, 85, 86, 88].…”

Section: Multiple System Atrophymentioning

confidence: 99%

“…Although dementia consistent with the Diagnostic and Statistical Manual of Mental Disorders (DSM IV) is an exclusion criterion for the diagnosis of MSA [81], some studies have reported that MSA patients have a cognitive decline when compared with controls [82,83,84,85,86,87,88]. MSA-P is the more common manifestation of MSA in Western populations [89, 90]; therefore, cognitive dysfunction in MSA-P had attracted considerable attention, particularly in comparison with PD and/or progressive supranuclear palsy [82,83,84,86,87,88].…”

Section: Multiple System Atrophymentioning

confidence: 99%

Cognitive Impairment in Spinocerebellar Degeneration

et al. 2009

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It has been reported that patients with spinocerebellar degenerations (SCDs) have cognitive dysfunction as well as limb and truncal ataxia, dysarthria and dysphagia. We review cognitive dysfunction in common types of SCD, including spinocerebellar ataxia types 1, 2, 3, 6, and 17, dentatorubral-pallidoluysian atrophy, Friedreich’s ataxia, and multiple system atrophy. There are few studies that address cognitive function in SCD. Although there are few comparison studies among the various SCDs, cognitive dysfunction may be more common and severe in spinocerebellar ataxia type 17 and dentatorubral-pallidoluysian atrophy. While cognitive dysfunction in SCD appears to represent frontal dysfunction, the mechanisms of cognitive dysfunction have not been directly clarified. Nevertheless, various lesions, including those in the cerebrocerebellar circuitry, cortico-striatal-thalamocortical circuitry, and the frontal lobe, may influence cognitive function to various degrees for each disease.

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Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson's disease and progressive supranuclear palsy.

Abstract: To study the neuropsychological pattern of striatonigral degeneration (SND) (7 Neurol Neurosurg Psychiatry 1995;58: 174-179)

Cited by 152 publications

References 30 publications

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies

Demographic Influences on Baseline and Derived Scores From the Trail Making Test in Healthy Older Australian Adults

Cognitive Impairment in Spinocerebellar Degeneration

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