Saturday, 28 May 2022

ICD-11 Criteria for Dementia due to Diseases Classified Elsewhere (6D85 )

ICD-11 Criteria for Dementia due to Diseases Classified Elsewhere (6D85 )

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.0        Dementia due to Parkinson disease

Dementia due to Parkinson disease develops among individuals with idiopathic Parkinson disease and is characterized by impairment in attention, memory, executive and visuo-spatial functions. Mental and behavioral symptoms such as changes in affect, apathy and hallucinations may also be present. Onset is insidious and the course is one of gradual worsening of symptoms.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.1          Dementia due to Huntington disease

Dementia due to Huntington disease occurs as part of a widespread degeneration of the brain due to a trinucleotide repeat expansion in the HTT gene, which is transmitted through autosomal dominance. Onset of symptoms is insidious typically in the third and fourth decade of life with gradual and slow progression. Initial symptoms typically include impairments in executive functions with relative sparing of memory, prior to the onset of motor deficits (bradykinesia and chorea) characteristic of Huntington disease.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

Inclusions:    

Dementia in Huntington chorea

6D85.2           Dementia due to exposure to heavy metals and other toxins

Dementia due to exposure to heavy metals and other toxins caused by toxic exposure to specific heavy metals such as aluminium from dialysis water, lead, mercury or manganese. The characteristic cognitive impairments in Dementia due to exposure to heavy metals and other toxins depend on the specific heavy metal or toxin that the individual has been exposed to but can affect any cognitive domain. Onset of symptoms is related to exposure and progression can be rapid especially with acute exposure. In many cases, symptoms are reversible when exposure is identified and ceases. Investigations such as brain imaging or neurophysiological testing may be abnormal. Lead poisoning is associated with abnormalities on brain imaging including widespread calcification and increased signal on MRI T2-weighted images of periventricular white matter, basal ganglia hypothalamus and pons. Dementia due to aluminium toxicity may demonstrate characteristic paroxysmal high-voltage delta EEG changes. Examination may make evident other features such as peripheral neuropathy in the case of lead, arsenic, or mercury.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

Exclusions: 

Dementia due to psychoactive substances including medications (6D84)

6D85.3          Dementia due to human immunodeficiency virus

Dementia due to human immunodeficiency virus develops during the course of confirmed HIV disease, in the absence of a concurrent illness or condition other than HIV infection that could explain the clinical features. Although a variety of patterns of cognitive deficit are possible depending on where the HIV pathogenic processes have occurred, typically deficits follow a subcortical pattern with impairments in executive function, processing speed, attention, and learning new information. The course of Dementia due to human immunodeficiency virus varies including resolution of symptoms, gradual decline in functioning, improvement, or fluctuation in symptoms. Rapid decline in cognitive functioning is rare with the advent of antiretroviral medications.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.4           Dementia due to multiple sclerosis

Dementia due to multiple sclerosis is a neurodegenerative disease due to the cerebral effects of multiple sclerosis, a demyelinating disease. Onset of symptoms is insidious and not secondary to the functional impairment attributable to the primary disease (i.e., multiple sclerosis). Cognitive impairments vary according to the location of demyelination but typically include deficits in processing speed, memory, attention, and aspects of executive functioning.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.5           Dementia due to prion disease

Dementia due to prion disease is a primary neurodegenerative disease caused by a group of spongiform encephalopathies resulting from abnormal prion protein accumulation in the brain. These can be sporadic, genetic (caused by mutations in the prion-protein gene), or transmissible (acquired from an infected individual). Onset is insidious and there is a rapid progression of symptoms and impairment characterised by cognitive deficits, ataxia, and motor symptoms (myoclonus, chorea, or dystonia). Diagnosis is typically made on the basis of brain imaging studies, presence of characteristic proteins in spinal fluid, EEG, or genetic testing.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.6        Dementia due to normal pressure hydrocephalus

Dementia due to normal pressure hydrocephalus results from excess accumulation of cerebrospinal fluid in the brain as a result of idiopathic, non-obstructive causes but can also be secondary to haemorrhage, infection or inflammation. Progression is gradual but intervention (e.g., shunt) may result in improvement of symptoms, especially if administered earlier in the course of the condition. Typically, cognitive impairments include reduced processing speed and deficits in executive functioning and attention. These symptoms are also typically accompanied by gait abnormalities and urinary incontinence. Brain imaging to reveal ventricular volume and characterize brain displacement is often necessary to confirm the diagnosis.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.7          Dementia due to injury to the head

Dementia due to injury to the head is caused by damage inflicted on the tissues of the brain as the direct or indirect result of an external force. Trauma to the brain is known to have resulted in loss of consciousness, amnesia, disorientation and confusion, or neurological signs. The symptoms characteristic of Dementia due to injury to the head must arise immediately following the trauma or after the individual gains consciousness and must persist beyond the acute post-injury period. Cognitive deficits vary depending on the specific brain areas affected and the severity of the injury but can include impairments in attention, memory, executive functioning, personality, processing speed, social cognition, and language abilities.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.8           Dementia due to pellagra

Dementia due to pellagra is caused by persistent lack of vitamin B3 (niacin) or tryptophan either in the diet or due to poor absorption in the gastrointestinal tract due to disease (e.g., Crohn disease) or due to the effects of some medications (e.g., isoniazid). Core signs of pellagra include dermatological changes (sensitivity to sunlight, lesions, alopecia, and oedema) and diarrhoea. With prolonged nutritional deficiency cognitive symptoms that include aggressivity, motor disturbances (ataxia and restlessness), confusion, and weakness are observed. Treatment with nutritional supplementation (e.g., niacin) typically results in reversal of symptoms.

Coding Note:     Code aslo the causing condition

6D85.9          Dementia due to Down syndrome

Dementia due to Down syndrome is a neurodegenerative disorder related to the impact of abnormal increased production and accumulation of amyloid precursor protein (APP) leading to formation of beta-amyloid plaques and tau tangles. APP gene expression is increased due to its location on chromosome 21, which is abnormally triplicated in Down syndrome. Cognitive deficits and neuropathological features are similar to those observed in Alzheimer disease. Onset is typically after the fourth decade of life with a gradual decline in functioning, and may impact 50% or more of individuals with Down syndrome.

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.

6D85.Y         Dementia due to other specified diseases classified elsewhere

Coding Note:     This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere.

When dementia is due to multiple aetiologies, code all that apply.


REFERENCE:

International Classification of Diseases Eleventh Revision (ICD-11). Geneva: World Health Organization; 2022. License: CC BY-ND 3.0 IGO.

https://creativecommons.org/licenses/by-nc-nd/3.0/igo/


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