Diagnosing ALS

Consider ALS in the differential diagnosis of focal, atrophic weakness without sensory disturbance in an adult of any age¹

• Death of both the upper motor neurons (UMNs) and lower motor neurons (LMNs) is the unique pathologic characteristic of ALS.¹
• Limbs are the initial site of involvement in nearly 80% of patients with ALS; weakness presents in an asymmetric pattern in close to 60% of these patients.¹
• The remaining 20% present with speech or swallowing difficulty (bulbar onset).
• The patient’s history is of prime importance because it establishes the site of onset and allows the pattern of progression to be determined.¹
• A review of symptoms and systems can be used to exclude features that may bring the diagnosis into question, such as: Cognitive, sensory, and bowel/bladder impairment.¹

A prompt diagnosis will provide the most benefit to the patient¹

An early, efficient diagnosis can offer clear advantages¹

• Reduces uncertainty for the patient.
• Prevents long wait for exclusivity tests to be performed.
• Speeds up referral to ALS clinics for specialized care.
• Can result in early treatment with Rilutek.

The full evaluation of ALS can be approached with the aid of an algorithm or decision tree¹

• Optimizes clinical and economic efficiency.¹
• Nodal points in the algorithm should achieve clear changes in direction along the tree.¹

Algorithm for Diagnosing ALS World Federation of Neurology (WFN) criteria have been shown to be efficient and accurate and can be used to accelerate ALS diagnosis 1 Criteria for Early ALS Diagnosis2,3 Weakness/Atrophy/Hyper-reflexia/Spasticity Progression over time Electromyogram (EMG)/Nerve Conduction Velocity (NCV)/Neuroimaging/Biopsy Neuropathology LMN signs only ≥ 1 region UMN signs only ≥ 1 region LMN + UMN 1 region LMN + UMN 1 region or UMN ≥ 1 region LMN + UMN 2 regions LMN + UMN 3 regions Suspected ALS* Possible ALS EMG acute denervation ≥ 2 limbs Probable ALS Definite ALS          Identified DNA gene Probable ALS Laboratory Supported               Definite Familial ALS Laboratory Supported         *Not recognized as an official diagnosis for entry into ALS clinical trials at this time.