Amyotrophic Lateral Sclerosis and Related Conditions

Prognosis of Motor Neuron Disease in Japan

“…Observation of long-term survival cases, however, revealed a rela tionship between sex and mode of disease progression. Swank and Putnam [15] postulated that there are two modes of progression occurring in longsurvival MND cases: one shows a relatively rapid progression after onset to a severe state and then progresses slowly (corresponding to group I in this study); the second shows relatively slow progression after onset (corre sponding to group II in this study). These two modes of progression were also observed in a study of MND cases on Guam in which it was observed that group I was female-dominant and group II male-dominant [8].…”

Prognosis of Motor Neuron Disease in Japan

“…Observation of long-term survival cases, however, revealed a rela tionship between sex and mode of disease progression. Swank and Putnam [15] postulated that there are two modes of progression occurring in longsurvival MND cases: one shows a relatively rapid progression after onset to a severe state and then progresses slowly (corresponding to group I in this study); the second shows relatively slow progression after onset (corre sponding to group II in this study). These two modes of progression were also observed in a study of MND cases on Guam in which it was observed that group I was female-dominant and group II male-dominant [8].…”

Myelin imaging in amyotrophic and primary lateral sclerosis

“…Clinical, genetic and neuropathological features of PLS support it being a distinct disorder, rather than an extreme of the spectrum of ALS ( Table III ). The nosology of PLS emerged at the end of the 19th century (reviewed in ( 4 )), followed in 1943 by a series of 21 cases contrasted clinically with ALS ( 21 ). The observation that humans have direct corticomotoneuronal connections to anterior horns has been postulated as the reason for UMN involvement more generally in ALS compared to LMN-only degenerations seen in lower-order animals ( 22 ).…”

Nerve Fibre Degeneration in the Brain in Amyotrophic Lateral Sclerosis

“…In looking for degeneration in these brains the need for using stains which demonstrate fibres in a state of active degeneration cannot be stressed too strongly. It is relevant that Swank and Putnam (1943) found, from doing fibre counts, that in the anterior nerve roots of patients with amyotrophic lateral sclerosis, up to 50% of the larger fibres may be lost before ordinary microscopic methods reveal any defect, and they thought that this is true for cortico-spinal fibres also. Their work shows the difficulty in recognizing the loss of even considerable numbers of fibres from tracts, by means of staining the healthy fibres.…”

Prognosis of Motor Neuron Disease in Japan

“…Observation of long-term survival cases, however, revealed a rela tionship between sex and mode of disease progression. Swank and Putnam [15] postulated that there are two modes of progression occurring in longsurvival MND cases: one shows a relatively rapid progression after onset to a severe state and then progresses slowly (corresponding to group I in this study); the second shows relatively slow progression after onset (corre sponding to group II in this study). These two modes of progression were also observed in a study of MND cases on Guam in which it was observed that group I was female-dominant and group II male-dominant [8].…”

Myelin imaging in amyotrophic and primary lateral sclerosis

“…Clinical, genetic and neuropathological features of PLS support it being a distinct disorder, rather than an extreme of the spectrum of ALS ( Table III ). The nosology of PLS emerged at the end of the 19th century (reviewed in ( 4 )), followed in 1943 by a series of 21 cases contrasted clinically with ALS ( 21 ). The observation that humans have direct corticomotoneuronal connections to anterior horns has been postulated as the reason for UMN involvement more generally in ALS compared to LMN-only degenerations seen in lower-order animals ( 22 ).…”

Nerve Fibre Degeneration in the Brain in Amyotrophic Lateral Sclerosis

“…In looking for degeneration in these brains the need for using stains which demonstrate fibres in a state of active degeneration cannot be stressed too strongly. It is relevant that Swank and Putnam (1943) found, from doing fibre counts, that in the anterior nerve roots of patients with amyotrophic lateral sclerosis, up to 50% of the larger fibres may be lost before ordinary microscopic methods reveal any defect, and they thought that this is true for cortico-spinal fibres also. Their work shows the difficulty in recognizing the loss of even considerable numbers of fibres from tracts, by means of staining the healthy fibres.…”

Prognosis of Motor Neuron Disease in Japan

“…Observation of long-term survival cases, however, revealed a rela tionship between sex and mode of disease progression. Swank and Putnam [15] postulated that there are two modes of progression occurring in longsurvival MND cases: one shows a relatively rapid progression after onset to a severe state and then progresses slowly (corresponding to group I in this study); the second shows relatively slow progression after onset (corre sponding to group II in this study). These two modes of progression were also observed in a study of MND cases on Guam in which it was observed that group I was female-dominant and group II male-dominant [8].…”

Myelin imaging in amyotrophic and primary lateral sclerosis

“…Clinical, genetic and neuropathological features of PLS support it being a distinct disorder, rather than an extreme of the spectrum of ALS ( Table III ). The nosology of PLS emerged at the end of the 19th century (reviewed in ( 4 )), followed in 1943 by a series of 21 cases contrasted clinically with ALS ( 21 ). The observation that humans have direct corticomotoneuronal connections to anterior horns has been postulated as the reason for UMN involvement more generally in ALS compared to LMN-only degenerations seen in lower-order animals ( 22 ).…”

Nerve Fibre Degeneration in the Brain in Amyotrophic Lateral Sclerosis

“…In looking for degeneration in these brains the need for using stains which demonstrate fibres in a state of active degeneration cannot be stressed too strongly. It is relevant that Swank and Putnam (1943) found, from doing fibre counts, that in the anterior nerve roots of patients with amyotrophic lateral sclerosis, up to 50% of the larger fibres may be lost before ordinary microscopic methods reveal any defect, and they thought that this is true for cortico-spinal fibres also. Their work shows the difficulty in recognizing the loss of even considerable numbers of fibres from tracts, by means of staining the healthy fibres.…”