G31.09 - Other frontotemporal neurocognitive disorder

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Behavioral variant of frontotemporal dementia
• CHMP2B-related frontotemporal dementia
• Dementia of frontal lobe type
• Frontal lobe degeneration
• Frontotemporal dementia
• Frontotemporal dementia with parkinsonism-17
• GRN-related frontotemporal dementia
• Inclusion body myopathy with early-onset Paget disease and frontotemporal dementia
• Myopathy with cytoplasmic inclusions
• Right temporal lobar atrophy
• Semantic dementia

Clinical Information

• Frontotemporal Dementia-. the most common clinical form of frontotemporal lobar degeneration, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.
• Frontotemporal Lobar Degeneration-. heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. patients exhibit progressive changes in social, behavioral, and/or language function. multiple subtypes or forms are recognized based on presence or absence of tau protein inclusions. ftld includes three clinical syndromes: frontotemporal dementia, semantic dementia, and primary progressive nonfluent aphasia.
• Amyotrophic Lateral Sclerosis 10, With or Without Frontotemporal Dementia|ALS10-. an autosomal dominant form of amyotrophic lateral sclerosis caused by mutation(s) in the tardbp gene, encoding tar dna-binding protein 43.
• Amyotrophic Lateral Sclerosis 14, With or Without Frontotemporal Dementia|ALS14-. a form of amyotrophic lateral sclerosis caused by heterozygous mutation(s) in the vcp gene, encoding transitional endoplasmic reticulum atpase.
• Amyotrophic Lateral Sclerosis 6, With or Without Frontotemporal Dementia|ALS6-. a form of amyotrophic lateral sclerosis caused by heterozygous mutation(s) in the fus gene, encoding rna-binding protein fus.
• Frontotemporal Dementia-. a syndrome caused by progressive degeneration of the frontal or temporal lobes of the brain. it is manifested with personality changes and deterioration of the language skills.
• Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis 1|FTDALS1-. an autosomal dominant condition caused by heterozygous hexanucleotide repeat expansion in a noncoding region of the c9orf72 gene , encoding guanine nucleotide exchange c9orf72. it is characterized by amyotrophic lateral sclerosis with frontotemporal dementia.
• Inclusion Body Myopathy with Early-Onset Paget Disease with or without Frontotemporal Dementia 1|IBMPFD1-. a rare autosomal dominant inherited disorder caused by mutations in the vcp gene. it can affect the muscles, bones, and brain. patients may develop myopathy that initially involves the muscles of the hips and shoulders and as the disorder progresses it may affect the cardiac and respiratory muscles, leading to life-threatening cardiac and pulmonary failure. approximately half of the adults develop paget disease of bone, and approximately one-third develop frontotemporal dementia.

Revised 2023 ICD-10 Code

G31.09 was revised for the FY 2023, effective October 1, 2022.

Convert to ICD-9 Code

Patient Education

Dementia

What is dementia?

Dementia is a loss of mental functions that is severe enough to affect your daily life and activities. These functions include:

• Memory
• Language skills
• Visual perception (your ability to make sense of what you see)
• Problem solving
• Trouble with everyday tasks
• The ability to focus and pay attention

It is normal to become a bit more forgetful as you age. But dementia is not a normal part of aging. It is a serious disorder which interferes with your daily life.

What are the types of dementia?

The most common types of dementia are known as neurodegenerative disorders. These are diseases in which the cells of the brain stop working or die. They include:

• Alzheimer's disease, which is the most common form of dementia among older people. People with Alzheimer's have plaques and tangles in their brain. These are abnormal buildups of different proteins. Beta-amyloid protein clumps up and forms plaques in between your brain cells. Tau protein builds up and forms tangles inside the nerve cells of your brain. There is also a loss of connection between nerve cells in the brain.
• Lewy body dementia, which causes movement symptoms along with dementia. Lewy bodies are abnormal deposits of a protein in the brain.
• Frontotemporal disorders, which cause changes to certain parts of the brain:
  • Changes in the frontal lobe lead to behavioral symptoms
  • Changes in the temporal lobe lead to language and emotional disorders
• Vascular dementia, which involves changes to the brain's blood supply. It is often caused by a stroke or atherosclerosis (hardening of the arteries) in the brain.
• Mixed dementia, which is a combination of two or more types of dementia. For example, some people have both Alzheimer's disease and vascular dementia.

Other conditions can cause dementia or dementia-like symptoms, including:

• Creutzfeldt-Jakob disease, a rare brain disorder
• Huntington's disease, an inherited, progressive brain disease
• Chronic traumatic encephalopathy (CTE), caused by repeated traumatic brain injury
• HIV-associated dementia (HAD)

Who is at risk for dementia?

Certain factors can raise your risk for developing dementia, including:

• Aging. This is the biggest risk factor for dementia.
• Smoking
• Uncontrolled diabetes
• High blood pressure
• Drinking too much alcohol
• Having close family members who have dementia

What are the symptoms of dementia?

The symptoms of dementia can vary, depending on which parts of the brain are affected. Often, forgetfulness is the first symptom. Dementia also causes problems with the ability to think, problem solve, and reason. For example, people with dementia may:

• Get lost in a familiar neighborhood
• Use unusual words to refer to familiar objects
• Forget the name of a close family member or friend
• Forget old memories
• Need help doing tasks that they used to do by themselves

Some people with dementia cannot control their emotions and their personalities may change. They may become apathetic, meaning that they are no longer interested in normal daily activities or events. They may lose their inhibitions and stop caring about other peoples' feelings.

Certain types of dementia can also cause problems with balance and movement.

The stages of dementia range from mild to severe. In the mildest stage, it is just beginning to affect a person's functioning. In the most severe stage, the person is completely dependent on others for care.

How is dementia diagnosed?

Your health care provider may use many tools to make a diagnosis:

• A medical history, which includes asking about your symptoms
• A physical exam
• Tests of your thinking, memory, and language abilities
• Other tests, such as blood tests, genetic tests, and brain scans
• A mental health evaluation to see whether a mental disorder is contributing to your symptoms

What are the treatments for dementia?

There is no cure for most types of dementia, including Alzheimer's disease and Lewy body dementia. Treatments may help to maintain mental function longer, manage behavioral symptoms, and slow down the symptoms of disease. They may include:

• Medicines may temporarily improve memory and thinking or slow down their decline. They only work in some people. Other medicines can treat symptoms such as anxiety, depression, sleep problems, and muscle stiffness. Some of these medicines can cause strong side effects in people with dementia. It is important to talk to your health care provider about which medicines will be safe for you.
• Occupational therapy to help find ways to more easily do everyday activities
• Speech therapy to help with swallowing difficulties and trouble speaking loudly and clearly
• Mental health counseling to help people with dementia and their families learn how to manage difficult emotions and behaviors. It can also help them plan for the future.
• Music or art therapy to reduce anxiety and improve well-being

Can dementia be prevented?

Researchers have not found a proven way to prevent dementia. Living a healthy lifestyle might influence some of your risk factors for dementia.

What Are Frontotemporal Disorders?

Code History

• FY 2023 - Code Updated, effective from 10/1/2022 through 9/30/2023
• New Description: Other frontotemporal neurocognitive disorder
• Previous Description:
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)