Primary lateral sclerosis: a case report with SPECT study

Arruda, Walter Oleschko; Neto, Maurício Coelho

doi:10.1590/s0004-282x1998000300018

Cited by 5 publications

(2 citation statements)

References 12 publications

(4 reference statements)

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“…Behavioural changes with bilateral atrophy of the frontal and temporal lobes have been reported in association with PLS in one patient (16). Frontal lobe dysfunction has been evidenced in PLS by magnetic resonance imaging (MRI) studies (17,18), and positron emission tomography (PET) and single photon emission computed tomography (SPECT) studies (4,12,19). The cognitive impairment reported by Caselli et al was observed in neuropsychological tests sensitive to frontal lobe functions and memory (13).…”

Section: Introductionmentioning

confidence: 98%

Neuropsychological changes in patients with primary lateral sclerosis

Piquard

Forestier

Baudoin-Madec

et al. 2006

Amyotrophic Lateral Sclerosis

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Primary lateral sclerosis (PLS) has been defined as rare, and the neuropsychological changes remain poorly defined. We studied 20 patients with a diagnosis of PLS. We carried out an extensive psychometric testing including a general assessment (memory, language, attention, visual-constructional ability and praxis) and a more specific assessment of prefrontal and premotor cortex functions in order to characterize the neuropsychological profile of the patients compared to matched controls, and explore executive functions and premotor cortex functions. None of the PLS patients was demented but they all presented memory deficits reflecting an executive dysfunction. All patients but three had signs of premotor and/or prefrontal cortex deficits. The cognitive impairment in PLS, specifically related to a frontal lobe dysfunction, seems qualitatively similar to ALS. Our results suggest a patchy distribution of cortical involvement in PLS but it remains difficult to draw any definite conclusion as to the spatio-temporal progression of the disease into the different regions of the frontal lobe.

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Section: Introductionmentioning

confidence: 98%

Neuropsychological changes in patients with primary lateral sclerosis

Piquard

Forestier

Baudoin-Madec

et al. 2006

Amyotrophic Lateral Sclerosis

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“…We will not discuss single-photon emission computed tomography (SPECT) studies except to mention a single case report in a patient with PLS of reduced signal in both frontal lobe pericentral areas (87). PET findings in the one with reportedly mild signs could not be distinguished from normal.…”

Section: Introductionmentioning

confidence: 99%

Neuroimaging in primary lateral sclerosis

Pioro

Turner

Bede

2020

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Increased interest in the underlying pathogenesis of primary lateral sclerosis (PLS) and its relationship to amyotrophic lateral sclerosis (ALS) has corresponded to a growing number of CNS imaging studies, especially in the past decade. Both its rarity and uncertainty of definite diagnosis prior to 4 years from symptom onset have resulted in PLS being less studied than ALS. In this review, we highlight most relevant papers applying magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and positron emission tomography (PET) to analyzing CNS changes in PLS, often in relation to ALS. In patients with PLS, mostly brain, but also spinal cord has been evaluated since significant neurodegeneration is essentially restricted to upper motor neuron (UMN) structures and related pathways. Abnormalities of cortex and subcortical white matter tracts have been identified by structural and functional MRI and MRS studies, while metabolic and cell-specific changes in PLS brain have been revealed using various PET radiotracers. Future neuroimaging studies will continue to explore the interface between the PLS-ALS continuum, identify more changes unique to PLS, apply novel MRI and MRS sequences showing greater structural and neurochemical detail, as well as expand the repertoire of PET radiotracers that reveal various cellular pathologies. Neuroimaging has the potential to play an important role in the evaluation of novel therapies for patients with PLS.

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