Diagnostic Criterias for Amyotrophic Lateral Sclerosis

         No test can provide a definite diagnosis of ALS, although the presence of interference at UMN and LMN in the body is very suggestive. The diagnosis of ALS is primarily based on signs and symptoms experienced by patients and through a series of investigations to rule out other diseases. Doctors dig deeply patient medical history and usually conduct a thorough neurological examination and to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity worsened progressively. Because the symptoms of ALS can be similar to other illnesses, diseases that can be cured, appropriate tests should be performed to rule out other diseases.

There are two commonly used diagnostic criteria, namely El-Escorial and WFN (World Fedaral Neurology)

A. El-Escorial
Diagnostic criteria according to El-Escorial divided broadly:
1. Found:
- Weakness of lower motor neurons, as evidenced both by clinical examination, electrophysiology and neuropathology examination
- Upper motor neuron degeneration by clinical examination
- Symptoms in one region or several regions progressively based on history and neurological examination.
2. Must no Found:
- Other diseases which give symptoms of LMN or UMN degeneration proven electrophysiological or pathological
- The presence of other disease processes as evidenced by neuroimaging studies based on clinical and electrophysiological.

In the clinical symptoms of UMN and LMN there are 4 regions:
- Central Nervous System and the brain stem (bulbar cranial motor neurons)
- Cervical spinal cord (anterior horn)
- Thoracic spinal cord (anterior horn)
- Lumbosacral spinal cord (anterior horn)

 

Enforcement diagnosis without pathological examination can be made in several levels, among others:
1 . ALS "clinically definite":

Characterized by symptoms of UMN and LMN in three regions

2 . ALS " definitive family" , laboratory -supported ALS
Found by UMN or LMN symptoms , at least one region can be identified and the cause of the SOD1 gene mutation and proband or family positively affected by ALS by identifying the presence of mutations in the SOD1 gene family.

3 . " Possible " in Clinical ALS :
Obtained when the symptoms of UMN and LMN least 2 regions with UMN symptoms rostral above LMN

4 . " Possible " laboratory supported ALS
- support ALS , with symptoms of the disorder UMN and LMN only one region or UMN symptoms only in one region , and LMN symptoms evidenced by EMG abnormalities in at least two limbs.

5 . Clinically  " Possible " ALS ,
if found only one symptom of LMN and UMN dysfunction in one region  simultaneously, or UMN disorder symptoms in two or more regions , or rostral LMN symptoms .

6 . Suspected ALS :
When only found pure LMN syndrome .
• Signs UMN : Clonus, sign Babinsky, no abdominal skin reflex, hypertonia, loss of dexterity
• LMN signs : atrophy, weakness. If only fasciculation: search by EMG to active denervation
• Part of nerves : bulbar, cervical , chest and lumbosacral

 

B.  World Federation of Neurology (WFN)

If using other criteria of the World Federation of Neurology (WFN), where there must be:
- Evidence of UMN lesion
- Evidence of the lesion LMN
- Evidence of progression

In using WFN criteria, there are 4 regions that must be known:
1. bulbar: Muscles of the face, mouth, throat.
2. Cervical: Muscle back of the head, neck, shoulder, shoulders, upper extremities.
3. Thoracic: chest and abdominal muscles, and the central part of the spinal muscles.
4. lumbosacral: The muscle behind the shoulder bottom, thighs, and lower extremity