Abstract
A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p
| Original language | English |
|---|---|
| Pages (from-to) | 424-429 |
| Number of pages | 6 |
| Journal | Amyotrophic Lateral Sclerosis |
| Volume | 11 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Oct 2010 |
Fingerprint
Keywords
- ALS
- ALSFRS-R
- Prognosis
ASJC Scopus subject areas
- Clinical Neurology
- Neurology
Cite this
Natural history of upper motor neuron-dominant ALS. / Sorarù, Gianni; Ermani, Mario; Logroscino, Giancarlo; Palmieri, Arianna; D'Ascenzo, Carla; Orsetti, Valeria; Volpe, Marco; Cima, Valentina; Zara, Gabriella; Pegoraro, Elena; Angelini, Corrado.
In: Amyotrophic Lateral Sclerosis, Vol. 11, No. 5, 10.2010, p. 424-429.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Natural history of upper motor neuron-dominant ALS
AU - Sorarù, Gianni
AU - Ermani, Mario
AU - Logroscino, Giancarlo
AU - Palmieri, Arianna
AU - D'Ascenzo, Carla
AU - Orsetti, Valeria
AU - Volpe, Marco
AU - Cima, Valentina
AU - Zara, Gabriella
AU - Pegoraro, Elena
AU - Angelini, Corrado
PY - 2010/10
Y1 - 2010/10
N2 - A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p
AB - A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p
KW - ALS
KW - ALSFRS-R
KW - Prognosis
UR - http://www.scopus.com/inward/record.url?scp=77956620471&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77956620471&partnerID=8YFLogxK
U2 - 10.3109/17482960903300867
DO - 10.3109/17482960903300867
M3 - Article
VL - 11
SP - 424
EP - 429
JO - Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders
JF - Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders
SN - 1466-0822
IS - 5
ER -