Huntington’s disease (HD) is a complex, inherited brain disorder that can significantly impact a person's life and the lives of their loved ones. While it's known for noticeable involuntary movements later in its progression, the earliest signs can be subtle and often involve changes in thinking, mood, and behavior, as well as minor physical symptoms. Recognizing these early signs is crucial for seeking timely diagnosis, accessing support, and planning for the future.

This article aims to help families understand what early Huntington's disease can look like, covering the key areas where symptoms typically emerge.

What is Huntington's Disease?

Huntington's disease is a progressive neurodegenerative disorder, meaning it causes nerve cells in the brain to break down over time. It is caused by a genetic mutation – an expanded section of DNA on chromosome 4 in the huntingtin gene. This mutation is passed down from a parent to a child, and anyone who inherits the expanded gene will eventually develop the disease. While the gene is present from birth, symptoms usually appear in adulthood, most often between ages 30 and 50 (McColgan & Tabrizi, 2018; Roos, 2010). A rarer form, called Juvenile Huntington's Disease (JHD), begins before age 20 (Oosterloo et al., 2024).

Why Early Recognition Matters

Identifying the first signs of HD can be challenging because they are often mild and can be mistaken for other conditions or simply personality changes. However, recognizing these early indicators is vital. An early diagnosis allows individuals and families to:

Get access to medical care and symptomatic treatments sooner.
Connect with support groups and resources like therapists, social workers, and financial planners.
Make important decisions about their health, family planning, and future care.
Participate in clinical trials aimed at finding treatments to slow or stop the disease progression.

The Three Main Areas of Early HD Symptoms

Early Huntington's disease symptoms typically fall into three main categories: motor symptoms, cognitive changes, and behavioral/psychiatric changes. These symptoms don't necessarily appear in any specific order, and the combination and severity can vary greatly from person to person.

1. Subtle Motor Symptoms

While the involuntary jerking movements known as chorea are a hallmark of later-stage HD, early motor signs are often much less dramatic. Families might notice:

Minor fidgeting or restlessness: Small, involuntary movements that seem out of character. These subtle motor disturbances are among the early signs (Supporting Info).
Clumsiness or difficulty with coordination: Dropping things more often, tripping, or having trouble with tasks requiring fine motor skills.
Balance problems: Unsteadiness, especially when walking or standing.
Difficulty with speech or swallowing: Though often more noticeable later, subtle changes can occur early.
Muscle stiffness or slowness: In Juvenile HD and some adult cases, motor symptoms may present more as rigidity and slow movements (parkinsonism) rather than chorea. Experts like those in the European Huntington Disease Network's Pediatric Working Group note that rigidity and dystonia (sustained muscle contractions) are common early motor symptoms in JHD, especially in younger children (Oosterloo et al., 2024). Research indicates that a presentation dominated by parkinsonism in early-stage HD might be associated with more severe cognitive and psychiatric issues (Julayanont, Heilman, & McFarland, 2020).

Motor symptoms often begin in the hands, feet, or fingers (distal extremities) before potentially spreading to other parts of the body, including facial muscles (Supporting Info).

2. Cognitive Changes

Changes in thinking and cognitive abilities are a significant part of early HD and can sometimes appear before motor symptoms. These aren't typically problems with just memory recall, but rather with executive functions and processing information. Early cognitive signs may include:

Difficulty with planning, organizing, or prioritizing tasks: Trouble managing daily activities or making decisions.
Problems with flexible thinking: Struggling to switch between tasks or adapt to new situations.
Slowed thinking or processing speed: Taking longer to understand information or respond to questions. Research suggests that changes in brain electrical activity correlate with processing speed issues in early HD (Davis et al., 2023).
Issues with attention and concentration: Easily distracted or finding it hard to focus.
Problems with verbal fluency: Difficulty finding the right words or speaking smoothly. Studies have shown improvements in verbal fluency with certain treatments in early HD patients (Molnar et al., 2022).

These cognitive changes can impact work performance, schoolwork, and the ability to manage personal affairs.

3. Behavioral and Psychiatric Changes

Changes in mood and behavior are very common in the early stages of Huntington's disease and can be particularly challenging for families to navigate. Often, these are the first symptoms to be noticed, sometimes years before motor signs appear (Molnar et al., 2022). Research highlights that neuropsychiatric symptoms significantly affect functional status and daily activities in early HD patients (Gibson et al., 2022; Ruiz-Idiago, Pomarol-Clotet, & Salvador, 2023).

Early behavioral and psychiatric symptoms can include:

Depression: Persistent sadness, loss of interest, fatigue, changes in sleep or appetite. Depression is frequently reported in early HD (Gibson et al., 2022; Ruiz-Idiago, Pomarol-Clotet, & Salvador, 2023).
Irritability and aggression: Increased frustration, short temper, or outbursts. Studies indicate that earlier onset of HD may be related to a higher probability of aggressive behaviors, and irritability can increase with the severity of chorea (Ruiz-Idiago, Pomarol-Clotet, & Salvador, 2023). Unlawful behaviors, including physical violence, have been reported in individuals with HD, particularly young adult males with psychiatric symptoms (McDonell et al., 2021).
Apathy: Lack of motivation, indifference, or loss of interest in previously enjoyed activities. Apathy is strongly associated with reduced functional capacity in early HD (Ruiz-Idiago, Pomarol-Clotet, & Salvador, 2023).
Anxiety: Excessive worry, nervousness, or panic attacks.
Obsessive-compulsive behaviors (OCD): Repetitive thoughts or actions.
Mood swings: Rapid and unpredictable changes in emotional state.
In some cases, psychosis: Hallucinations or delusions, though less common in early stages than other symptoms.

These behavioral changes can be difficult to understand and manage, often leading to social withdrawal and strain on relationships (Gibson et al., 2022).

Early Signs in Juvenile Huntington's Disease (JHD)

Juvenile HD is rare, accounting for about 5-10% of HD cases, and presents before age 20. The early signs can differ from adult-onset HD. Instead of chorea, motor symptoms often start as rigidity, slowness, and dystonia. Early signs frequently involve cognitive and behavioral issues that manifest at school, such as:

Learning difficulties: Declining school performance is a common early indicator (Roos, 2010; Oosterloo et al., 2024).
Behavioral problems at school: Changes in conduct, focus, or social interaction (Supporting Info). These can sometimes be confused with conditions like ADHD or autism spectrum disorder, potentially leading to delayed diagnosis (Oosterloo et al., 2024).
Speech difficulties: Problems with articulation or fluency (Oosterloo et al., 2024).
Other possible early signs in JHD: Seizures, ataxia (lack of coordination), and spasticity (muscle tightness) can also occur (Oosterloo et al., 2024).

JHD typically progresses faster than adult-onset HD and is often associated with a larger CAG repeat expansion in the gene (Roos, 2010; Oosterloo et al., 2024).

What to Do If You Notice Potential Early Signs

If you notice a combination of these subtle changes in yourself or a family member, especially if there is a known history of Huntington's disease in the family, it's important to consult a doctor. Describe the specific changes you've observed in motor control, thinking, and behavior.

A doctor can perform a physical and neurological examination and may refer to a neurologist or a specialist HD clinic. Genetic testing can definitively confirm whether a person has the Huntington's disease gene expansion. Genetic counseling is highly recommended before and after testing to discuss the implications.

Conclusion

Spotting Huntington's disease in its earliest stages requires paying attention to subtle changes across motor, cognitive, and behavioral domains. While these signs can be varied and sometimes overlap with other conditions, increased awareness is the first step. If you have concerns, particularly with a family history of HD, seeking medical advice from a healthcare professional is the most important action you can take. Early diagnosis opens the door to necessary support, planning, and management strategies, helping families navigate the complexities of this disease.

References

Davis, M.-C., Hill, A. T., Fitzgerald, P. B., Bailey, N. W., Stout, J. C., & Hoy, K. E. (2023). Neurophysiological correlates of non-motor symptoms in late premanifest and early-stage manifest huntington's disease. Clinical Neurophysiology, 145, 139–148.
Gibson, J. S., Rhoten, B. A., Ridner, S. H., & Claassen, D. O. (2022). Perceived Effects of Neuropsychiatric Symptoms on Functional Status in Early-stage Huntington Disease. Journal of Neuroscience Nursing, 54(5), 203–209.
Julayanont, P., Heilman, K. M., & McFarland, N. R. (2020). Early-Motor Phenotype Relates to Neuropsychiatric and Cognitive Disorders in Huntington's Disease. Movement Disorders, 35(12), 2283–2290.
McColgan, P., & Tabrizi, S. J. (2018). Huntington's disease: a clinical review. European Journal of Neurology, 25(1), 24–34.
McDonell, K. E., Brown, B. K., Hale, L., Darby, R. R., Stovall, J., Compas, B. E., & Claassen, D. O. (2021). Medicolegal Aspects of Huntington Disease. Journal of the American Academy of Psychiatry and the Law Online, 49(3), 321–328.
Molnar, M. J., Molnar, V., Fedor, M., Csehi, R., Acsai, K., Borsos, B., & Grosz, Z. (2022). Improving Mood and Cognitive Symptoms in Huntington's Disease With Cariprazine Treatment. Frontiers in Pharmacology, 13.
Oosterloo, M., Touze, A., Byrne, L. M., Achenbach, J., Aksoy, H., Coleman, A., ... & Quarrell, O. (2024). Clinical Review of Juvenile Huntington's Disease. Movement Disorders Clinical Practice, 11(1), 13–21.
Roos, R. A. C. (2010). Huntington's disease: a clinical review. Orphanet Journal of Rare Diseases, 5(1), 40.
Ruiz-Idiago, J., Pomarol-Clotet, E., & Salvador, R. (2023). Longitudinal analysis of neuropsychiatric symptoms in a large cohort of early-moderate manifest Huntington's disease patients. European Neuropsychopharmacology, 71, 49–59.
Snowden, J. S. (2017). The Neuropsychology of Huntington's Disease. In S. J. Tabrizi & M. Orth (Eds.), Huntington's Disease (pp. 1–10). Springer International Publishing.

Spotting Huntington’s Disease Early: Symptoms Families Should Know