**11. Diagnostic criteria for motor neuron disease (amyotrophic lateral sclerosis, ALS) by\*\* Leigh and Ray – Chaudhuri 1994**

#### **The diagnosis of ALS requires the presence of:**


#### **11.1 Diagnostic categories**


Definite ALS Upper and lower motor neuron signs in at least three body

**Clinically probable ALS** Upper and lower motor neuron signs in at least two

other causes. **Clinically possible ALS** Clinical signs of upper and lower motor neuron

**11. Diagnostic criteria for motor neuron disease (amyotrophic lateral** 

**Probable ALS:** UMN plus LMN signs in two regions with UMN signs rostral to LMN

 **Possible ALS**: UMN plus LMN signs in one region, or UMN signs in two or three regions, such as in monomelic ALS, progressive bulbar palsy, and primary lateral

**Suspected ALS**: LMN signs in two or three regions, such as in progressive muscular

**Clinically probable ALS: Laboratory-supported ALS** 

\* (Brooks, 1994 and Brooks, et al. 2000) \*\* Als: amyotrophic lateral sclerosis

Progression of the disorder

**11.1 Diagnostic categories** 

UMN signs

signs

sclerosis

Table 4. Revised El Escorial criteria for the diagnosis of ALS.\*

**sclerosis, ALS) by\*\* Leigh and Ray – Chaudhuri 1994** 

**Definite ALS**: UMN plus LMN signs in three regions\*\*

LMN signs (including EMG features in clinically normal muscles)

**The diagnosis of ALS requires the presence of:** 

atrophy, and other motor syndromes.

regions (upper limb, lower limb, bulbar, thoracic).

Clinical signs of upper and lower motor neuron dysfunction in only one region, or when upper motor neuron signs alone are present in one region and lower motor neuron signs defined by electromyographic criteria are present in at least two limbs, with proper application of neuroimaging and clinical laboratory protocols to exclude

rostoral to the lower motor neuron signs

regions, with some upper motor neuron signs necessarily

dysfunction are together in only one region, or upper motor neuron signs are found alone in two or more regions, or lower motor neuron signs are found rostral to upper motor neuron signs and the diagnosis of clinically probable: laboratory-supported ALS cannot be proven by evidence on clinical grounds in conjunction with electrodiagnostic, neurophysiological, neuroimaging or clinical laboratory studies. Other diagnoses must have been excluded

### **11.2 The diagnosis of ALS requires the absence of**


### **11.3 The diagnosis of ALS is supported by**


Regions are defined as follows: brainstem, brachial, thorax and trunk, crural. UMN= Upper motor neuron; LMN= lower motor neuron.
