lvPPA is a specific type of primary progressive aphasia that leads to difficulties in finding and using words while speaking.

Primary progressive aphasia (PPA) refers to a group of neurodegenerative diseases primarily affecting language. It includes three variants: semantic, nonfluent/agrammatic, and logopenic. Each variant affects distinct aspects of language processing and comprehension.

Logopenic PPA (lvPPA), in particular, causes difficulties in single-word retrieval, sentence repetition, and phonological errors, and is frequently associated with Alzheimer’s disease (AD).

lvPPA is a neurodegenerative disease that affects language abilities, particularly in terms of word finding and sentence formation.

It’s one of three recognized variants of PPA, the other two being semantic variant PPA (svPPA) and nonfluent/agrammatic variant PPA (nfvPPA).

Unlike svPPA, where word comprehension is impaired, and nfvPPA, which involves difficulties with grammar, lvPPA primarily manifests as a challenge in word retrieval and fluency.

In lvPPA, individuals often have challenges retrieving and producing the right words while talking, leading to hesitations and frequent use of filler words. They might also have difficulties retaining short-term information and may complain of memory difficulties.

As your disease progresses, speech may be lost entirely, and symptoms may become more typical of AD, such as memory loss and difficulty recognizing familiar faces or finding your way.

It’s estimated that there are approximately 3.6–8.1 people with PPA per 100,000 individuals, with an even smaller proportion representing the lvPPA variant.

Symptoms of lvPPA:

  • word-finding difficulties
  • trouble holding short-term information and following instructions
  • complaints of memory difficulties
  • difficulty in noisy environments
  • difficulty with phonological language processing
  • slow and hesitant speech with pauses
  • trouble repeating sentences
  • challenges with calculations and memory tasks
  • potential progression to loss of speech
  • development of AD-like symptoms over time
  • motor skill and swallowing issues in advanced stages

Common early symptoms of lvPPA may include struggling to retrieve words, frequent hesitations in speech, difficulties with short-term memory tasks, or challenges in understanding speech in noisy environments.

What are examples of logopenic primary progressive aphasia?

Here are a few examples of how a person might experience lvPPA.

Jane, middle-aged, has difficulties with word retrieval in conversations. While chatting about a recent vacation, she hesitates, resorting to vague descriptions like “that place we went to” instead of specific names. Frustration mounts as she increasingly relies on filler words to convey her thoughts.

Mark, a retiree, enjoys socializing with friends at his local coffee shop. But he starts avoiding these gatherings because he finds it increasingly hard to follow conversations in the bustling environment. The background noise makes it difficult for him to distinguish between individual voices, and he often misses important details.

Unlike other neurodegenerative diseases like AD or Parkinson’s disease, PPA doesn’t have a standardized progression of stages. But symptoms do appear to get worse over time.

In an unpublished study (pending peer review), researchers aimed to develop a clear framework for understanding the general stages of PPA. Based on information they’d collected from caregivers of people with PPA, the researchers created a six-stage chart ranging from “very mild” at level 1 to “profound” at level 6.

  • Stage 1, very mild: Symptoms might go unnoticed or may be blamed on stress or aging. Their irregular nature makes them difficult to detect and may only be evident in retrospect.
  • Stage 2, mild: Communication difficulties and subtle daily challenges become noticeable to both the individual and those around them.
  • Stage 3, moderate: Daily tasks may require help, potentially leading to leaving work. Difficulty in communication might affect personal goals and social interactions, causing frustration.
  • Stage 4, severe: Communication difficulties get worse, potentially compromising independent living.
  • Stage 5, very severe: Cognitive and behavioral changes are significant and obvious, and meaningful communication becomes increasingly rare. Assistance is needed with personal care, and there are pronounced physical symptoms like limited coordination and walking difficulties.
  • Stage 6, profound: Communication becomes nearly impossible, and awareness of surroundings decreases. Mobility significantly declines.

The exact cause of lvPPA isn’t fully understood, but it’s believed to result from underlying neurodegenerative processes affecting the regions of your brain responsible for language. This disease is often linked to abnormal protein buildup, such as tau or TDP-43, disrupting nerve cell function and leading to brain tissue degeneration.

It’s estimated that 86% of people with lvPPA have pathology related to AD pathology (presence of plaques and tangles).

Genetic and environmental factors may also contribute to its development, but more research is needed for a comprehensive understanding.

Risk factors for lvPPA

While the exact risk factors for lvPPA aren’t definitively established, here are some factors that might contribute:

  • Age: Like many neurodegenerative diseases, your risk of lvPPA increases with your age. On average, individuals with the lvPPA experienced symptom onset around the age of 69 years (typically within a 10-year range).
  • Genetics: Research suggests that mutations in genes like C9ORF72 and progranulin (GRN) have been associated with various forms of frontotemporal dementia, including lvPPA. A family history of similar diseases could indicate a genetic tendency.
  • Underlying protein abnormalities: Evidence suggests that lvPPA is associated with the accumulation of specific proteins, such as tau and TDP-43, in your brain cells. These abnormalities might be influenced by genetic factors or other processes.
  • Neurological conditions: Certain neurological diseases or brain diseases may heighten lvPPA risk. It can be associated with AD pathology in some cases.
  • Environmental factors: Environmental factors, especially exposure to toxins like lead, aluminum, mercury, and pesticides, are linked to neurodegenerative disorders, leading to characteristic features like plaques and tangles. But specific research on lvPPA is lacking.

Research also shows that PPA affects more men than women, in contrast to other neurodegenerative diseases like AD.

Language matters

You’ll notice that the language used to share statistics and other data points is pretty binary, fluctuating between “female/male” and “women/men.”

Although we typically avoid language like this, specificity is key when reporting on research participants and clinical findings.

The studies and surveys referenced in this article didn’t report data on, or include, participants who were transgender, nonbinary, gender nonconforming, genderqueer, agender, or genderless.

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Currently, there’s no cure for lvPPA. Since the predominant underlying pathology is often AD, healthcare professionals may prescribe cholinesterase inhibitors like donepezil (Aricept) or rivastigmine (Exolon), similar to their use in classical AD treatment.

In cases where individuals with lvPPA experience symptoms of depression or anxiety, selective serotonin reuptake inhibitors (SSRIs) might be considered as a treatment option.

Treatments like speech therapy, cognitive training, and supportive strategies may help with communication skills.

What is the life expectancy of someone with lvPPA?

The average life expectancy of someone who’s received a diagnosis of lvPPA is about 7.6 years after onset. But it’s important to note that this is an average, and individual outcomes can vary.

The disease lvPPA is a distinct variant of PPA, a neurodegenerative disease that primarily affects language abilities.

lvPPA is characterized by challenges in word retrieval and fluency, leading to hesitations, filler words, and difficulties in repeating sentences.

As with any medical condition, early diagnosis and appropriate medical care are crucial. If you or a loved one is experiencing symptoms consistent with lvPPA, it’s important to reach out to a medical professional who specializes in neurology or speech and language diseases.