Major Neurocognitive Disorder DSM-5 294.1x (F02.8x) (Probable) or 331.9 (G31.9) (Possible)

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DSM-5 Category: Neurocognitive Disorders

Introduction

Major neurocognitive disorder is a sub-diagnosis used to indicate the severity of other mental disorders, including those due to cognitive disorders, the multiple forms of dementia, and traumatic brain injuries. While symptoms may be similar, both minor and major neurocognitive disorders are distinct from pervasive developmental disorders and intellectual disabilities (The American Psychiatic Association, 2013).

Symptoms of Major Neurocognitive Disorder

Major neurocognitive disorder typically presents as a significant cognitive decline from previous levels of performance in at least one cognitive domain, such as attention span, executive functioning, memory retention, learning abilities, perceptual-motor skills, social cognition, and language comprehension and production. Declines in functioning are typically noted by either the individual or their caretaker, or are noticed by the clinician themselves (The American Psychiatric Association, 2013).

Major neurocognitive disorder is diagnosed in individuals who demonstrate a substantial impairment in cognitive performance, particularly when this impairment is documented through the use of neurophychological testing or other clinical assessment. These impairments directly interfere with independence in daily functioning, requiring assistance with activities including bill paying, managing medications, or maintaining hygiene and nutrition (The American Psychiatric Association, 2013).

In order to qualify for diagnosis as major neurocognitive disorder, symptoms must not occur exclusively in the context of delirium or be better explained by another cognitive or developmental disorder, however, this diagnosis is often included alongside other diagnoses as a means of indicating the severity of symptoms (The American Psychiatric Association, 2013).

Mood disturbances, including sudden increases in depression, bi-polar like mood swings or disinhibition, agitation, anxiety, or a sudden onset of apathy or dythymia are often early indicators of the cognitive decline characteristic of major neurocognitive disorder. Insomnia, hypersomnia, and circadian rhythm disorders are also common (The American Psychiatric Association, 2013).

Diagnosis of Major Neurocognitive Disorder

Major neurocognitive disorder is diagnosed based on clinical evidence of cognitive decline, including reduction in memory retention and decreased performance on cognitive tasks. The use of the Mini Mental Status Exam (MMSE), the Global Assessment of Functioning (GAF) scale, and other standardized neurophychological testing should also be used (The American Psychiatric Association, 2013).

There is some indication that the MMSE, while the historical assessment of choice for cognitive functioning, may actually fail to detect early stage minor neurocognitive disorder, often a precursor to major neurocognitive disorder in progressive mental illnesses such as the various forms of dementia. There is evidence that the use of the Saint Louis University Mental Status (SLUMS) assessment may lead to earlier detection of cognitive decline (Tariq, Tumosa, Chibnall, Perry, & Morley, 2006).

When evaluating patients in the process of diagnosis with major neurocognitive disorder, any standardized assessment results should be evaluated against the norms for the appropriate age, educational level, and cultural background of the individual. While slight declines consistent with minor neurocognitive disorder may remain within the normal range, declines indicative of a diagnosis of major neurocognitive disorder should be readily apparent (The American Psychiatric Association, 2013).

Overall, females are at significantly higher risk for the development of most forms of dementia, and therefor major neurocognitive disorder as the disease progresses. The earliest stages of decline are typically diagnosed in individuals whose recreational or occupational activities require complex cognitive tasks, while the later stages hallmarking the transition to major neurocognitive disorder will be apparent in most individuals regardless of occupation or cultural background (The American Psychiatric Association, 2013).

Co-morbidity of Major Neurocognitive Disorder

Since both minor and major neurocognitive disorders are used as secondary diagnosis to indicate the severity of cognitive decline in other disorders, comorbidity often occurs with at least one other disorder. It is important to note whether behavioral disturbances accompany cognitive decline, and use the appropriate diagnostic code matching the causitive comorbid condition (The American Psychiatric Association, 2013).

Major neurocognitive disorder is typically due to traumatic brain injury or the advancement of other degenerative neurological diseases. Later stages of Alzheimer's disease, Lewy body disease, Parkinson's disease, Huntington's disease, prion diseases, HIV infection, vascular disease, and frontal lobar degeneration are typically co-diagnosed with minor neurocognitive disorder initially, with diagnosis transitioning to major neurocognitive disorder over time. Traumatic brain injury, either due to physical trauma or substance abuse, and symptoms related to stroke may present as major neurocognitive disorder from onset (The American Psychiatric Association, 2013).

The severity of psychotic symptoms in mood disorders, bipolar disorders, and schizophrenia spectrum disorders show a strong predictive correlation with cognitive functioning, with diagnosis of both minor and major neurocogitive disorder being predictive of future episodes of psychosis (Simonsen, et al., 2011). Similarly, diagnosis of major neurocognitive disorder along with major depressive disorder often shows that severity of depressive symptoms is predictive of the level of neurocognitive decline (McClintock, Husain, Greer, & Cullum, 2010).

When considering diagnoses occuring comorbidly with major neurocognitive disorder, it is important to consider the connection to physical health as well. It has long been established in the medical field that critical illness has an impact on cognition, with life-threatening illnesses often leading to either temporary or permanent impairments diagnosed as either minor or major neurocognitive disorder (Hopkins & Jackson, 2006).

Similarly to the connections between major neurocognitive disorder and critical illness, there is also a well-established link between major neurocognitive disorder and both Human Immunodeficiency Virus (HIV) and Acquired Immunodeficiency Syndrome (AIDS), which may also be referred to as HIV-associated neurocognitive disorder, or HAND, in non-psychiatric medical literature (Singh, 2009). There is significant evidence that activated monocytes and macrophages within the cerebrospinal fluid may play a roll in HAND, despite the use of combination antiretroviral therapy. When monitoring patients presenting with HIV/AIDs and major neurocognitive disorder, it is important to consider monitoring the levels of these critical immune cells through cerebrospinal fluid testing, as this may allow the progression of the associated major neurocognitive disorder to be monitored (Kamat, et al., 2012).

Prevalence of Major Neurocognitive Disorder

The prevalence of major neurocognitive disorder is difficult to assess, though it is safe to assume that it will closely follow the prevalence rates of the associated neurodegenerative diseases. Rates of major neurocognitive disorder increase sharply with age, with the most significant rise occurring in patients over the age of 60. It is estimated that approximately 1-2% of those aged 65 and older, and as much as 30% of those aged 85 and older, have been diagnosed with dementia, and therefore more than likely also meet the criteria for either minor or major neurocognitive disorder (The American Psychiatric Association, 2013).

Treatment of Major Neurocognitive Disorder

While there are no direct treatments for major neurocognitive disorder or the dementia it is typically associated with, many of the comorbid diseases associated with major neurocognitive disorder have treatment options available. There is evidence that group-based cognitive remediation treatment in patients with major neurocognitive disorder and bipolar disorder may improve verbal memory, attention, executive function, and psychosocial function, indicating that this treatment option may be viable for patients whose diagnosis of major neurocognitive disorder is caused by other etiologies (Demant, Almer, Vinberg, Kessing, & Miskowiak, 2013).

In addition, there is significant evidence that the use of ATP-enhancing metabolic agents may improve cognitive function, while possibly slowing the decline of the associated neurodegenerative disorder. This class of medications may hold new promise for those suffering from major neurocognitive disorder or other cognitive declines (Owen & Sunram, 2011). However, it is important to continue the use of medications historically used to treat the underlying causative disorders associated with major neurocognitive disorder, including viral load control in HIV-associated cognitive decline and those used to improve memory and other impairments in cognitive disorders (The American Psychiatric Association, 2013).

Outcomes for Major Neurocognitive Disorder

Unfortunately, due to the nature of major neurocognitive disorder and its association with aging and dementia, it is unlikely that symptoms will improve or that decline will stabilize. It is important that patients remain in a life-long relationship with healthcare workers, including psychologists, psychiatrists, neurologists, and any other specialist related to underlying disease process. If patients diagnosed with major neurocognitive disease are carefully monitored and treated using a multidisciplinary approach, it may be possible to extend or improve quality of life for these individuals (The American Psychiatric Association, 2013).


References

American Psychiatric Association (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Arlington, VA: American Psychiatric Publishing.

Demant, K.M., Almer, G.M., Vinberg, M., Kessing, L.V., & Miskowiak, K.W. (2013). Effects of cognitive remediation on cognitive dysfunction in partially or fully remitted patients with bipolar disorder: Study protocol for a randomized controlled trial. Trials, 14, 338.

 

Hopkins, R.O., & Jackson, J.C. (2006). Long-term neurocognitive function after critical illness. CHEST, 130(3), 869-878.

Kamat, A., Lyons, J.L., Misra, V., Uno, H., Morgello, S., Singer, E.J., & Gabuzda, D. (2012). Monocyte activation markers in cerebrospinal fluid associated with impaired neurocognitive testing in advanced HIV infection. JAIDS-Journal of Acquired Immune Deficiency Syndromes, 60(3), 234-243.

McClintock, S.A., Husain, M.M., Greer, T.L., & Cullum, C.M. (2010). Association between depression severity and neurocognitive function in major depressive disorder: A review and synthesis. Neuropsychology, 24(1), 9-34.

Owen, L. & Subran-Lea, S.I. (2011). Metabolic agents that enhance ATP can improve cognitive functioning: A review of the evidence for glucose, oxygen, pyruvate, creatine, and l-carnitine. Nutrients, 3(8), 735-755.

Simonsen, C., Sundet, K., Vaskinn, A., Birkenaes, A.B., Engh, J.A., Faerden, A., Jonsdottir, H., Ringen, P.A., Opjordsmoen, S., Melle, I., Friis, S., & Andreassen, O.A. (2011). Neurocognitive disfunction in bipolar and schizophrenia spectrum disorders depends on history of psychosis rather than diagnostic group. Schizophrenia Bulletin, 37(1), 73-83.

Singh, D. (2009). Neurocognitive impairment in PLWHA: Clinical features and assessment. Southern African Journal of HIV Medicine, 35, 30-34.

Tariq, S.H., Tumosa, N., Chibnall, J.T., Perry, M.H., & Morley, J.E. (2006). Comparison of the Saint Louis University Mental Status examination and the Mini-Mental State Examination for detecting dementia and Major neurocognitive disorder – A pilot study. American Journal of Geriatric Psychiatry, 14(11), 900-910.


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