Primary Lateral Sclerosis

Stark, Frederick M.; Moersch, Frederick P.

doi:10.1097/00005053-194510000-00002

Cited by 55 publications

(11 citation statements)

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“…Our five cases correspond perfectly with the descriptions provided by these investigators.6 [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] Thus, based on their nine cases and our five cases, a rather consistent pattern of signs can be established for chronic progressive bilateral spinobulbar spasticity. This picture includes initial symptoms that consist of either pseudobulbar mani¬ festations or walking problems, fol¬ lowed by the progressive appearance of spasmodic tetraparesis, severe dysarthria, moderate dysphagia, in¬ appropriate lability of laughter and crying, and sometimes, as in our cases 1 and 5, facial paresis of central ori¬ gin.…”

Section: Chronic Progressive Bilateral Spinobulbar Spasticitysupporting

confidence: 85%

Chronic Progressive Spinobulbar Spasticity

Gastaut

Michel²,

Figarella‐Branger³

et al. 1988

Arch Neurol

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Section: Chronic Progressive Bilateral Spinobulbar Spasticitysupporting

confidence: 85%

Chronic Progressive Spinobulbar Spasticity

Gastaut

Michel²,

Figarella‐Branger³

et al. 1988

Arch Neurol

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“…The onset is insidious, and the course of the illness, in comparison to ALS, tends to be prolonged. Stark and Moersch (13), in a study of 43 cases of PLS, found an average survival of 13 years, and a recent study of eight cases showed a median survival of 19 years (4, 5).…”

Section: Discussionmentioning

confidence: 97%

Diffusion tensor imaging in the diagnosis of primary lateral sclerosis

Uluğ

Grünewald

Lin

et al. 2003

Magnetic Resonance Imaging

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Purpose:To evaluate the utility of MR diffusion tensor imaging in diagnosing primary lateral sclerosis (PLS). Materials and Methods:Five patients who met clinical criteria for a diagnosis of PLS, and two patients with possible PLS of less than three years duration and eight normal volunteers were studied using MR diffusion tensor imaging.Results: All seven patients showed decreased diffusion anisotropy and increased diffusion constant in the PLIC, with complete separation from a normal control group, including patients early in the course of the illness. Conclusion:Quantitative diffusion tensor imaging appears to be a useful test to detect upper motor neuron damage and hence to help to establish the diagnosis of PLS.

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“…In 1945, Stark and Moersch proposed the first clinical diagnostic criteria, which included insidious onset, slow progression, dysfunction limited to the pyramidal tracts, and a course of more than five years. (2). These criteria were later refined by Pringle et al (3).…”

Section: Introductionmentioning

confidence: 99%

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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Primary Lateral Sclerosis

Cited by 55 publications

References 0 publications

Chronic Progressive Spinobulbar Spasticity

Chronic Progressive Spinobulbar Spasticity

Diffusion tensor imaging in the diagnosis of primary lateral sclerosis

Neuropsychological changes in patients with primary lateral sclerosis

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