Amyotrophic lateral sclerosis

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We were unfortunately unable to download the information for this disease from OMIM.

Prevalence of clinical parameters (%)

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Pubmed id number as a reference Organ system affected
Number of patients in the reference Percent affected patients (Between 0 and 1, eg. 0.1 = 10%)
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List of symptoms

Symptom/sign Organ system Percent affected Pubmed id Added on(yyyy-mm-dd) Edit/add reference
Limb wasting nervous 100 % 17296839 2012-06-10
Hyperactive reflexes nervous 100 % 19487653 2012-08-24
Muscle weakness skeletal 100 % 19487653 2012-08-24
Pyramidal signs nervous 99 % 17296839 2012-06-10
Muscle atrophy skeletal 90 % 19487653 2012-08-24
Bulbar dysfunction nervous 88 % 17296839 2012-06-10
Dysphagia nervous 80 % 19487653 2012-08-24
Dysdiadochokinesia nervous 78 % 19487653 2012-08-24
Weight loss multi 70 % 19487653 2012-08-24
Fasciculations skeletal 70 % 19487653 2012-08-24
Areflexia nervous 70 % 19487653 2012-08-24
Dysarthria nervous 60 % 19487653 2012-08-24
Spasticity nervous 22 % 21702734 2012-06-10
Muscle atrophy skeletal 22 % 21702734 2012-06-10
Dementia nervous 8 % 17296839 2012-06-10

List of references:

Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up.
Maria Carmela Tartaglia, Ann Rowe, Karen Findlater, J B Orange, Gloria Grace, Michael J Strong,

Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum.

Archives of neurology - Feb 2007

Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS.
P H Gordon, B Cheng, I B Katz, H Mitsumoto, L P Rowland,

To determine how clinical features at the first evaluation and in follow-up can be used to suggest a diagnostic outcome for patients with only upper motor neuron (UMN) signs at disease onset.

Neurology - Jun 2009

Uncovering amyotrophic lateral sclerosis phenotypes: clinical features and long-term follow-up of upper motor neuron-dominant ALS.
Mario Sabatelli, Marcella Zollino, Marco Luigetti, Alessandra Del Grande, Serena Lattante, Giuseppe Marangi, Mauro Lo Monaco, Francesca Madia, Emiliana Meleo, Giulia Bisogni, Amelia Conte,

The aim of our study was to analyse the natural history and clinical features of upper motor neuron- dominant (UMN-D) ALS. We studied a large series of sporadic ALS patients admitted in a single referral centre over a 23-year period. UMN-D phenotype was compared with other ALS forms, including classic ALS, flail arm and progressive muscular atrophy. Seven hundred and thirty-four sporadic ALS patients were included of which 163 had UMN-D ALS. The mean age of onset in UMN-D ALS (52 years) was 10 years lower than in classic ALS (61.4 years, p < 0.0001); sex ratio by age groups significantly differed with respect to other phenotypes. The pattern of spread of lower motor neuron signs in UMN-D was characterized by early involvement of upper limb muscles and late impairment of respiratory muscles. Duration of the disease was longer in the UMN-D group (56 months) than in classic ALS (33 months, p < 0.001). The UMN-D phenotype was a strong independent predictor of long survival. In summary, UMN-D ALS showed significant differences in age of onset, sex ratio, pattern of spreading and prognosis with respect to other ALS forms, most probably reflecting biological differences.

Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases - Jul 2011