2024 ICD-10-CM Diagnosis Code I68.0

Approximate Synonyms

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

• Cerebral amyloid angiopathy
• Cerebral amyloid angiopathy
• Cerebral amyloid angiopathy
• Cerebral amyloid angiopathy associated with systemic amyloidosis
• Cerebral non-neuropathic heredofamilial amyloidosis angiopathy
• Cerebral secondary systemic amyloid angiopathy
• Familial non-neuropathic amyloidosis
• Hereditary cerebral hemorrhage with amyloidosis
• Hereditary cerebrovascular amyloidosis
• Localized hereditary amyloidosis
• Secondary systemic amyloidosis

Clinical Information

• Amyloid Neuropathies, Familial

  inherited disorders of the peripheral nervous system associated with the deposition of amyloid in nerve tissue. the different clinical types based on symptoms correspond to the presence of a variety of mutations in several different proteins including transthyretin (prealbumin); apolipoprotein a-i; and gelsolin.

• Cerebral Amyloid Angiopathy

  a heterogeneous group of sporadic or familial disorders characterized by amyloid deposits in the walls of small and medium sized blood vessels of cerebral cortex and meninges. clinical features include multiple, small lobar cerebral hemorrhage; cerebral ischemia (brain ischemia); and cerebral infarction. cerebral amyloid angiopathy is unrelated to generalized amyloidosis. amyloidogenic peptides in this condition are nearly always the same ones found in alzheimer disease. (from kumar: robbins and cotran: pathologic basis of disease, 7th ed., 2005)

• Cerebral Amyloid Angiopathy, Familial

  a familial disorder marked by amyloid deposits in the walls of small and medium sized blood vessels of cerebral cortex and meninges.

• Cerebral Amyloid Angiopathy

  a disorder characterized by the deposition of amyloid in the wall of the vessels in the brain.

• Cerebral Amyloid Angiopathy, APP-Related|HCHWAD|Hereditary Cerebral Hemorrhage with Amyloidosis-Dutch Type

  an autosomal dominant form of cerebral amyloid angiopathy caused by mutation(s) in the app gene, encoding amyloid-beta a4 protein. the deposition of amyloid in cerebral blood vessels wall may lead to degenerative vascular changes that may result in cerebral hemorrhage. mutation(s) in the app gene may also cause autosomal dominant alzheimer disease 1.

Code Edits

The Medicare Code Editor (MCE) detects and reports errors in the coding of claims data. The following ICD-10-CM Code Edits are applicable to this code:

• Manifestation diagnoses - Manifestation codes describe the manifestation of an underlying disease, not the disease itself, and therefore should not be used as a principal diagnosis.

Convert I68.0 to ICD-9-CM

• ICD-9-CM Code: 437.8 - Cerebrovasc disease NEC
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Amyloidosis

Amyloidosis occurs when abnormal proteins called amyloids build up and form deposits. The deposits can collect in organs such as the kidney and heart. This can cause the organs to become stiff and unable to work the way they should.

There are three main types of amyloidosis:

• Primary - with no known cause
• Secondary - caused by another disease, including some types of cancer
• Familial - passed down through genes

Symptoms can vary, depending upon which organs are affected. Treatment depends on the type of amyloidosis you have. The goal is to help with symptoms and limit the production of proteins. If another disease is the cause, it needs to be treated.

[Learn More in MedlinePlus]

Brain Diseases

Your brain is the control center of your body. It controls your thoughts, memory, speech, and movement. It regulates the function of many organs. It's part of your nervous system, which also includes your spinal cord and peripheral nerves. The nervous system sends signals between your brain and the rest of the body. Your nerves take in information from your senses and send it to the brain to be processed. Your brain and nerves also communicate to help you move and to control your body's functions.

When the brain is healthy, it works quickly and automatically. But when you have a brain disease, it may affect how well you can function and do your daily activities. Some common brain diseases include:

• Brain tumors, which can press on nerves and affect brain function.
• Degenerative nerve diseases, which can affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Types include Alzheimer's disease and Parkinson's disease.
• Encephalitis (inflammation in the brain), which can lead to problems such as vision loss, weakness, and paralysis.
• Genetic brain disorders, which are caused by changes in genes (also called variants or mutations). These disorders can affect the development and function of the brain.
• Strokes, which can cause a loss of brain cells and can affect your ability to think clearly.
• Traumatic brain injuries (TBIs), which can affect brain function. They may range from mild to severe. The effects of a TBI may be temporary or permanent.

The symptoms of brain diseases vary widely, depending on the specific problem. In some cases, damage is permanent. In other cases, treatments such as surgery, medicines, or therapies such as physical, occupational, and speech therapies, may cure the disease or improve the symptoms.

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Hereditary cerebral amyloid angiopathy

Hereditary cerebral amyloid angiopathy is a condition characterized by an abnormal buildup of protein clumps called amyloid deposits in the blood vessels in the brain, causing vascular disease (angiopathy). People with hereditary cerebral amyloid angiopathy often have progressive loss of intellectual function (dementia), stroke, and other neurological problems starting in mid-adulthood. Due to neurological decline, this condition is typically fatal in one's sixties, although there is variation depending on the severity of the signs and symptoms. Most affected individuals die within a decade after signs and symptoms first appear, although some people with the disease have survived longer.

There are many different types of hereditary cerebral amyloid angiopathy. The different types are distinguished by their genetic cause, which determines whether areas of the brain other than blood vessels are affected, and the signs and symptoms that occur. The various types of hereditary cerebral amyloid angiopathy are named after the regions where they were first diagnosed.

The Dutch type of hereditary cerebral amyloid angiopathy is the most common form. Stroke is frequently the first sign of the Dutch type and is fatal in about one third of people who have this condition. Survivors often develop dementia and have recurrent strokes. About half of individuals with the Dutch type who have one or more strokes will have recurrent seizures (epilepsy).

People with the Flemish and Italian types of hereditary cerebral amyloid angiopathy are prone to recurrent strokes and dementia. Individuals with the Piedmont type may have one or more strokes and typically experience impaired movements, numbness or tingling (paresthesias), confusion, or dementia.

The first sign of the Icelandic type of hereditary cerebral amyloid angiopathy is typically a stroke followed by dementia. Strokes associated with the Icelandic type usually occur earlier than the other types, with individuals typically experiencing their first stroke in their twenties or thirties.

Strokes are rare in people with the Arctic type of hereditary cerebral amyloid angiopathy, in which the first sign is usually memory loss that then progresses to severe dementia. Strokes are also uncommon in individuals with the Iowa type. This type is characterized by memory loss, problems with vocabulary and the production of speech, personality changes, and involuntary muscle twitches (myoclonus).

Two types of hereditary cerebral amyloid angiopathy, known as familial British dementia and familial Danish dementia, are characterized by dementia and movement problems. Strokes are uncommon in these types. People with the Danish type also have clouding of the lens of the eyes (cataracts) and deafness.

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Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• 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. This was the first year ICD-10-CM was implemented into the HIPAA code set.