Hippocampal-Sparing Alzheimer Disease

Hippocampal-Sparing Alzheimer Disease

Article
Brain & Nerve
Behavioral & Mental Health
+2
Contributed byKrish Tangella MD, MBASep 11, 2023

What are the other Names for this Condition? (Also known as/Synonyms)

  • Alzheimer's Disease, Hippocampal Sparing subtype
  • Hippocampal-Sparing AD
  • Hippocampal-Sparing Alzheimer’s Disease

What is Hippocampal-Sparing Alzheimer Disease? (Definition/Background Information)

  • Hippocampal-Sparing Alzheimer Disease is a distinct subtype of Alzheimer's disease (AD) characterized by the preservation of the hippocampus; a region vital for memory formation. While typical Alzheimer's is associated with significant hippocampal atrophy, this subtype exhibits relatively intact hippocampal volume despite the presence of Alzheimer's pathology
  • This form of Alzheimer's disease often manifests with atypical clinical features, including language difficulties or visual-spatial impairments. The signs and symptoms of Hippocampal-Sparing Alzheimer Disease can include cognitive impairment, memory loss, language difficulties, and changes in behavior. The preservation of the hippocampus might lead to initially milder memory-related symptoms compared to typical AD
  • A diagnosis of Hippocampal-Sparing Alzheimer Disease involves comprehensive clinical evaluation, cognitive testing, brain imaging studies (MRI or PET scans), and sometimes analysis of cerebrospinal fluid (CSF) markers to differentiate it from other forms of dementia
  • The treatment strategies for Hippocampal-Sparing Alzheimer Disease are generally similar to those for other AD subtypes, including cognitive-enhancing medications, behavioral interventions, and supportive care to manage symptoms and improve quality of life
  • The prognosis for Hippocampal-Sparing Alzheimer Disease can vary, but the preserved hippocampus might lead to a somewhat slower progression of memory-related symptoms compared to typical AD. However, cognitive decline and functional impairment still occur over time

Who gets Hippocampal-Sparing Alzheimer Disease? (Age and Sex Distribution)

  • Hippocampal-Sparing Alzheimer Disease typically affects older individuals, with the onset usually occurring after the age of 65
  • Both males and females can develop this subtype, and there does not appear to be a significant difference in its distribution between genders
  • This disorder is observed worldwide, and no particular racial or ethnic preference is observed 

What are the Risk Factors for Hippocampal-Sparing Alzheimer Disease? (Predisposing Factors)

  • While the specific risk factors for Hippocampal-Sparing Alzheimer Disease are still being explored, advancing age remains the primary risk factor for all forms of Alzheimer's disease
  • Other potential factors may include the following:
    • Genetic predisposition
    • Family history of dementia
    • Presence of certain genetic variants associated with Alzheimer's disease

Ongoing research aims to identify additional risk factors unique to this subtype.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hippocampal-Sparing Alzheimer Disease? (Etiology)

The causes of Hippocampal-Sparing Alzheimer Disease align with those of typical Alzheimer's disease (AD).

  • The accumulation of beta-amyloid plaques and tau protein tangles in the brain, leading to neurodegeneration and subsequent cognitive decline, is the hallmark of the disease
  • The exact mechanisms that contribute to the sparing of the hippocampus in this subtype are not yet fully understood

Unlike typical Alzheimer's disease, where the hippocampus is prominently affected, individuals with Hippocampal-Sparing AD experience more pronounced atrophy in other brain parts.

What are the Signs and Symptoms of Hippocampal-Sparing Alzheimer Disease?

The signs and symptoms of Hippocampal-Sparing Alzheimer Disease may vary from one individual to another and may be mild or severe. These include:

  • Language difficulties: Difficulty finding words, expressing thoughts, or understanding speech
  • Visual-spatial impairments: Challenges with perception, depth perception, and navigation
  • Executive function decline: Impaired judgment, decision-making, and planning abilities
  • Memory loss: While memory may be relatively preserved, it may still be affected in later stages
  • Behavioral changes: Mood swings, apathy, and personality alterations

How is Hippocampal-Sparing Alzheimer Disease Diagnosed?

Diagnosing Hippocampal-Sparing Alzheimer Disease involves a comprehensive evaluation, which may include the following:

  • Thorough medical history and physical examination
  • Neuropsychological assessments to evaluate cognitive function and identify atypical features
  • Brain imaging, such as MRI or PET scans, to assess changes in brain structure and detect patterns of atrophy

In general, a neuropathological examination after death may provide definitive confirmation of the diagnosis.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hippocampal-Sparing Alzheimer Disease?

Hippocampal-Sparing Alzheimer Disease can lead to several complications, including:

  • Progressive cognitive decline: Although the hippocampus may be spared, cognitive impairment still worsens over time
  • Behavioral and psychological symptoms: Mood disturbances, agitation, and psychosis may arise
  • Functional decline: Difficulties with self-care, mobility, and daily activities increase as the disease progresses
  • Caregiver burden: Family members or caregivers may experience physical, emotional, and financial strain in providing care and support

How is Hippocampal-Sparing Alzheimer Disease Treated?

Currently, there is no cure for Hippocampal-Sparing Alzheimer Disease. The treatment primarily focuses on managing the signs and symptoms and providing supportive care. The approaches may include:

  • Medications: Cholinesterase inhibitors may help alleviate cognitive symptoms to some extent
  • Symptom management: Behavioral interventions, therapy, and support groups can assist with managing behavioral and psychological changes
  • Caregiver support: Education, counseling, and respite care can help support caregivers in managing the challenges associated with the disease

How can Hippocampal-Sparing Alzheimer Disease be Prevented?

  • As the underlying causes of Hippocampal-Sparing Alzheimer Disease are not fully understood, prevention strategies specific to this subtype are yet to be established
  • However, adopting a brain-healthy lifestyle that includes regular exercise, a balanced diet, mental stimulation, and social engagement may promote overall brain health and potentially reduce the risk of various forms of Alzheimer's disease

What is the Prognosis of Hippocampal-Sparing Alzheimer Disease? (Outcomes/Resolutions)

The prognosis of Hippocampal-Sparing Alzheimer Disease varies, and the rate of disease progression may differ among individuals.

  • As research on this specific subtype is ongoing, it is important to consult with healthcare professionals for the most up-to-date information regarding prognosis, potential treatments, and management strategies
  • Early detection and comprehensive care can help improve the quality of life and provide the necessary support for individuals and their caregivers

Hippocampal-Sparing AD is an area of active research, and more work is needed to better understand its unique features, underlying mechanisms, and optimal treatment approaches.

Additional and Relevant Useful Information for Hippocampal-Sparing Alzheimer Disease:

There are voluntary organizations and support groups for Alzheimer’s disease that provide counsel, help, and understanding to the affected individuals and families.

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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