Cognition and Personality

Sundowning and Cognitive Decline in Superficial Siderosis


Sundowning in late-stage SS is less common than in other neurodegenerative diseases. However, some patients may still experience symptoms, especially if there’s a coexisting disease or early signs of dementia. Progression to true dementia in superficial siderosis is still considered very rare. Sundowning refers to an increase in confusion, restlessness, agitation, and behavioral problems that typically occur during the late afternoon or evening, ergo the term “sundowning.” The underlying reasons are not fully understood, but disruptions to the brain’s internal clock and a decreased tolerance for stress as the brain’s resources or neurological reserve dwindle toward the end of the day may be contributing factors.

Cognitive Decline

Cognitive decline is a progressive reduction in cognitive function in patients with SS, especially in the late stages of the condition. Cognitive processes include memory, attention, language, problem-solving, and the ability to live a fully independent life. Iron-induced damage to neurons can lead to significant cognitive impairment as SS progresses. The specific cognitive deficits will depend on which brain regions are most affected by iron deposition. For example, damage to the hippocampus, a brain region involved in memory formation, can result in memory deficits.

Management Strategies for Caregivers

Managing the symptoms of sundowning and cognitive decline in late-stage SS can be challenging, but there are strategies that caregivers can use:

1. Maintain a Consistent Schedule: Keeping a consistent routine can help orient the patient and reduce confusion. Try having meals, wake-up times, and bedtimes the same daily.

2. Modify the Environment: Keep their living environment well-lit, especially as the sun begins to set. This can help minimize confusion and anxiety that might be associated with sundowning. Reducing noise and creating a peaceful environment can also be helpful for those not affected by hearing loss.

3. Engage in Calming Activities: Here are some calming activities tailored for individuals with hearing impairment, mobility issues, or those who cannot walk.

  • Sensory Activities: Sensory stimulation can be soothing and engaging. This could be as simple as handling textured materials, using aromatherapy with calming scents such as lavender, or employing weighted blankets or cushions for deep pressure stimulation.
  • Art Therapy: Painting, drawing, or molding clay can be done even when seated. Even if their ability to create detailed work has diminished, the act of creating and the tactile experience can be soothing.
  • Looking at Picture Books or Albums: Photos from their past or picture books about topics they’re interested in can help stimulate memory and provide a calming activity.
  • Pet Therapy: Interacting with animals has been shown to reduce anxiety and agitation in individuals with dementia. A calm, well-behaved pet or therapy animal can provide comfort and companionship.
  • Music Therapy: Even with hearing impairment, some patients can still perceive and appreciate music, especially if it’s a familiar song from their past. Vibration speakers can also be used to allow patients to feel the rhythm.
  • Tactile Games or Puzzles: Tactile games like sorting objects by shape or color, simple puzzles, or fidget toys can provide calming stimulation and engagement.
  • Gentle Touch or Massage: Depending on the comfort level and familiarity of the person, a gentle hand massage using a lightly scented lotion could be calming.
  • Indoor Gardening: Tending to indoor plants can be a soothing activity. Even individuals with mobility issues can water plants or touch their leaves when the plants are brought to them.
  • Watching Aquariums or Virtual Nature Videos: Watching the slow, gentle movements of fish in an aquarium can be very calming. If an aquarium isn’t available, videos of nature scenes or fish tanks can provide a similar benefit.
  • Breathing Exercises and Meditation: Guided breathing exercises can be beneficial. Although they might not be able to hear instructions, a simple demonstration can help them understand the exercise.

4. Promote Good Sleep Hygiene: Encourage activities during the day to help the patient sleep better at night. Later-stage SS patients may sleep all day, so try to discourage daytime napping if possible and make the sleep environment as comfortable as possible.

5. Cognitive Stimulation and Physical Activity: Regular cognitive stimulation, such as puzzles or memory games, may help slow cognitive decline, while physical activities can enhance overall health and well-being.

6. Regular Check-ups: Regular check-ups with healthcare providers are important for managing the progression of SS and addressing any new or worsening symptoms.

7. Medication Management: Some patients may benefit from medications to manage specific symptoms or to slow disease progression. Always consult with a healthcare provider regarding potential benefits and side effects.

8. Support for the Caregiver: Caregivers also need support. Respite care services, caregiver support groups, and counseling can be very helpful. Remember, taking care of oneself is essential for being able to care for another.

The Iron Connection

Iron is an essential nutrient for various biological processes, but in excess, it can cause damage through a process known as oxidative stress. This involves the generation of reactive oxygen species (ROS), which are chemically reactive molecules that contain oxygen. ROS can damage cells, including neurons, in the brain, leading to functional impairment and cell death.

How does this process contribute to cognitive decline?

  1. Neuronal Damage: Iron accumulates in neurons and other cells of the brain in superficial siderosis, where it catalyzes the formation of ROS. These reactive molecules can damage various cellular components, including proteins, lipids, and DNA. This damage can impair the normal functioning of neurons and eventually lead to their death. As neurons die, the cognitive functions they support can start to decline.
  2. Demyelination: Myelin is a fatty substance that insulates neurons and enhances the speed and efficiency of signal transmission. ROS generated by excess iron can damage myelin, disrupting the communication between neurons. This demyelination can lead to various neurological symptoms, including cognitive decline.
  3. Impaired Neurotransmitter Function: Iron synthesizes several neurotransmitters, chemicals neurons use to communicate. Excessive iron can disrupt the balance of these neurotransmitters, impairing the communication between neurons and contributing to cognitive decline.
  4. Neuroinflammation: Oxidative stress can also trigger an inflammatory response in the brain. Chronic neuroinflammation is harmful and has been implicated in the progression of neurodegenerative diseases and cognitive decline.
  5. Alteration in Synaptic Plasticity: Synaptic plasticity, the ability of synapses to strengthen or weaken over time, is crucial for learning and memory. Oxidative stress and inflammation from excess iron can interfere with synaptic plasticity, impairing learning, and memory processes and contributing to cognitive decline.

It’s important to note that the distribution of iron deposition in the brain may also play a role in the specific cognitive deficits observed in superficial siderosis. For example, if iron primarily accumulates in regions of the brain involved in memory (such as the hippocampus), memory loss may be a prominent symptom. Similarly, if iron accumulates in regions involved in executive functions (like the frontal lobes), impairments in planning, problem-solving, and decision-making may be more pronounced. Treatment for superficial siderosis often involves addressing the source of bleeding, if possible, managing symptoms, and, if a patient is able to, using iron chelation therapy to help remove excess iron. However, these strategies have limitations, and further research is needed to develop more effective treatments.

Caring for someone with late-stage SS can be incredibly challenging. It’s crucial to remember that support is available, and reaching out to healthcare professionals and support networks can make a significant difference.

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Rori Daniel

Living With Superficial Siderosis began as a way to keep family and acquaintances updated after my husband Gary was diagnosed with Superficial siderosis in 2014. In 2019, became a partner in the Superficial Siderosis Research Alliance. Together our alliance has expanded into research, advocacy, and patient education. Rori Daniel, Editor,

One Comment

  1. Thank you Rori for your thoughtful and well-researched articles. I hope you know that your contributions have been THE most important support for my husband and family. And for me. 

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