What Are the SARA, LEMOCOT & 9-Hole Peg Tests for ARSACS?

When you visit the neurologist for ARSACS (Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay), your care team will likely ask you to perform a series of walking, tapping, and peg-placing exercises. These are standardized clinical assessments—specifically the SARA, LEMOCOT, and 9-Hole Peg Test. They are designed to objectively measure your coordination, balance, and fine motor skills. By turning your symptoms into numerical scores, your doctor can track how your condition is changing over time, make informed adjustments to your physical or occupational therapies, and collect vital data that helps drive ARSACS research and clinical trials ^[1][2]. (Tip: Because these tests involve moving your arms, legs, and walking, it is helpful to wear comfortable clothing and easy-to-remove shoes to your neurology appointments.)

The Scale for the Assessment and Rating of Ataxia (SARA)

The Scale for the Assessment and Rating of Ataxia (SARA) is a tool used to evaluate the overall severity of ataxia, which is the lack of muscle control and coordination common in ARSACS ^[1][3].

During the SARA assessment, your neurologist will ask you to perform eight different tasks. These evaluate your gait (walking), stance (balance while standing), sitting balance, speech clarity, and how well you can coordinate your arms and legs ^[3].

What the score means:
Scores are added up, and a higher total score indicates a greater level of impairment ^[3][4]. SARA is widely recognized as a reliable way to quickly detect changes in ataxia symptoms during busy clinical visits ^[5][6]. Tracking this score helps your doctor understand your overall disease progression ^[4].

The Lower Extremity Motor Coordination Test (LEMOCOT)

Because ARSACS significantly affects the lower body, your doctor may use the Lower Extremity Motor Coordination Test (LEMOCOT) ^[7]. This test specifically measures how well you can control and coordinate the movements of your legs ^[7].

In this test, you are typically asked to tap your big toe between two targets as quickly and accurately as possible within a 20-second timeframe ^[7].

What the score means:
Your score is based on the number of accurate target taps you complete ^[8]. A lower score indicates poorer lower-limb coordination ^[7]. Tracking this over time is crucial for deciding when physical therapy exercises should be adjusted or when it might be time to consider mobility aids (like a walker or wheelchair). While transitioning to a mobility aid can be an emotionally difficult milestone, tracking your scores helps ensure these tools are introduced at the exact right time to keep you safe and maximize your independence ^[9][10].

The 9-Hole Peg Test (9HPT)

The 9-Hole Peg Test (9HPT) measures manual dexterity and upper-extremity (arm and hand) motor function ^[11][12]. You will be asked to take nine small pegs and place them into nine holes on a board as fast as you can, and then remove them ^[11]. To account for varying levels of ability, you might be asked to complete two trials per hand ^[13].

What the score means:
The score is the amount of time it takes to complete the task ^[11]. Longer completion times indicate a decline in hand coordination and fine motor skills ^[12]. If your time increases significantly over several visits, it signals to your care team that you might benefit from occupational therapy or adaptive tools for daily tasks like buttoning shirts or using utensils ^[11][14].

Why These Scores Matter for Your Care and Research

While ARSACS involves a “triad” of symptoms—ataxia, spasticity (muscle stiffness), and neuropathy (nerve damage)—these three tests specifically focus on tracking your ataxia and motor coordination ^[2]. They provide essential, objective data that benefits you in several ways:

• Adjusting Therapies: If your scores show a decline in specific areas, your care team can proactively adjust your physical and occupational therapies, or introduce new strategies like wheelchair-skills training to improve your daily independence ^[9][15].
• Clinical Trials and Registries: These tests provide a standardized way to measure ARSACS progression ^[1]. When your scores are added to disease registries (with your permission), they help researchers understand the natural history of the disease ^[16]. If a new treatment is developed, these tests will be used to prove whether the therapy is working ^[17][18].
• Comprehensive Assessment: Sometimes, doctors also use the DSI-ARSACS (Disease Severity Index for ARSACS), a scale specifically developed for adults with ARSACS. Because it is closely correlated with your SARA, LEMOCOT, and 9-Hole Peg scores, your doctor can often evaluate your overall disease severity without needing to put you through a completely different set of exhausting physical tasks ^[2].

The Emotional Toll of Testing

Repeating these assessments at every visit and watching your times get slower or your scores change can be emotionally exhausting and discouraging. It is completely normal to feel anxious or upset before these tests. Remember that your doctor is primarily comparing your scores to your own past performance—your personal baseline—not to a “perfect” standard ^[4]. The goal is not to pass or fail, but to understand exactly where you are today so your care team can give you the best possible support.