Chapter 16

Delirium is recognized by the DSM-V as a neurocognitive disorder in which the patient is having fluctuations in confusion and disorientation over a short period of time. There will be various degrees of consciousness, perception, cognitive disturbance, and attention deficits. The two main features under the DSM-V are changes in attention and cognition. Illusions and hallucinations are also main features. The patient is easily distractible and often cannot comprehend what is going on. The onset is fast, within hours to days. There will be sleep disturbances and behavior that is harmful to themselves or others. The symptoms of inattention and cognitive impairments can be seen in other disorders, such as autism spectrum disorder, ADHD, and schizophrenia. In delirium, these features are more serious; common sufferers are the elderly, those with dementia, and those suffering from drug intoxication or withdrawal. The DSM lists these criteria: A. Attention disturbance (lack of focus) and decreased awareness B. Quick onset of symptoms that tend to fluctuate C. Cognitive deficit D. Absence of coma or evolving dementia 248 E. Evidence of physiological insult, substance intoxication, substance withdrawal, or toxic exposure F. Specifier that involves hyperactive or hypoactive level of activity or mixed level of activity There is a strong association with delirium and either mental disorders, neurological disorders, medical conditions, or substance/medication use. People with alcohol withdrawal or other drug withdrawal are treated with benzodiazepines, which can actually trigger delirium in some patients. There is a higher incidence of delirium with the elderly or those with underlying Alzheimer’s disease, Parkinson’s disease, or Huntington’s disease. The incidence of delirium is just 1-2 percent in the general population; 14-24 percent in hospitalized patients; and 70-80 percent in intensive care patients. About 83 percent of patients near the end of their life will have delirium. Of people with delirium secondary to a medical condition (versus a substance-related delirium), about 40 percent die within the next year (according to the DSM-V). Poor health and low functioning are positively associated with the presence of delirium. The hallucinations and delusions of delirium must be differentiated from that seen in bipolar disorder, depression, and psychotic disorders. Anxiety and dissociation seen in delirium also can be seen in acute stress disorder. The treatment of delirium is mostly medical and antipsychotics are most commonly prescribed. The diagnostic code for delirium according to the DSM-V must include the probable underlying cause for the delirium. Untreated, it can increase the risk of infections, respiratory failure, and death. There is always a risk of over-sedation in the treatment of delirium but this needs to be weighed against the aggressiveness of the symptoms. Risperidone along with light therapy will improve sleep time and will decrease delirium scores. Exercise and cognitive stimulation will also improve attention; these can be used along with drug therapy for the management of delirium symptoms. 249 MAJOR NEUROCOGNITIVE DISORDER (294.X) This is a broad category involving several different diseases all associated with a severe cognitive impairment. Traumatic brain injuries and several types of dementia will have cognitive deficits that meet the criteria for major neurocognitive disorder; these are different from having an intellectual disability or pervasive developmental disorder. To make the diagnosis, there has to be significant cognitive decline from previous levels of performance in at least one area of cognition. These include language comprehension, language production, perceptual-motor skills, learning abilities, memory retention, executive functioning, and attention span. The patient can detect this themselves but it is often noticed by clinicians or caretakers. The patient cannot manage medications, pay bills, maintain hygiene, or maintain nutrition. Delirium needs to be excluded. Early indicators of a major neurocognitive disorder include sudden depressive symptoms, bipolar-like mood swings, agitation, disinhibition, anxiety, or apathy. Other things commonly seen are hypersomnia, insomnia, and circadian rhythm disorders. The diagnosis can be made using the MMSE (mini mental status examination), the Global Assessment of Functioning (GAF) scale, or other neuropsychological testing. The St. Louis University Mental Status assessment or SLUMS may also detect early cognitive decline, and may be more sensitive than any of the other tests. Women are at a higher risk for developing dementia and individuals who have jobs that require complex cognitive tasks will be detected earlier than most other people. Major neurocognitive disorder can be secondary to many different neurological diseases as well as traumatic brain injury. Patients with degenerative neurological diseases (such as Alzheimer’s disease, Lewy boy disease, Parkinson’s disease, prion disease, Huntington’s disease, etc.) are often diagnosed with mild or “minor” neurocognitive disorder first and then progress to major neurocognitive disorder. Traumatic brain injury and stroke can immediately lead to major neurocognitive disorder. 250 Individuals with mood disorders, bipolar disorder, or schizophrenia spectrum disorders with severe psychosis have a higher likelihood of developing a neurocognitive disorder. The severity of depressive symptoms is also predictive of neurocognitive decline and major neurocognitive disorder. Having a life-threating illness will also predict the future development of a major neurocognitive disorder. HIV disease is also predictive of major neurocognitive disorder so that there is a separate diagnosis for neurocognitive disorder secondary to HIV. The prevalence of major cognitive disorder is difficult to know as there are many different etiologies. The prevalence is about 1-2 percent in those over 65 years but it is 30 percent in those 85 years or older. There are no actual treatments for major neurocognitive disorder; however, comorbid diseases can be treated. The type of neurocognitive disorder determines which treatment strategy is employed. Most people do not recover from this type of disorder and tend to gradually decline in function over time. MILD NEUROCOGNITIVE DISORDER (331.83) Patients with mild neurocognitive disorder will present with a decline in cognitive functioning to include memory impairment, decreased ability to perform ADLs, and difficulty with language, social skills, and/or perceptual-motor skills. Mood disturbances are often seen as early findings, some of which may rise to the level of another psychiatric disorder. Like major neurocognitive disorders, the GAF scale, the MMSE, and the SLUMS assessment are used to diagnose the presence of early cognitive decline in mild neurocognitive disorder. It is important to remember that both major and minor neurocognitive disorders are secondary diagnoses so that there will always be a primary diagnosis to explain the neurocognitive deficit, except in cases of unspecified neurocognitive disorder and neurocognitive disorder due to multiple etiologies. It is a good idea to make the diagnosis when the patient has mild neurocognitive disorder so that, if any interventions can take place, they can take place before the disease can worsen. 251 Treatment is based on the primary underlying cause. There are treatments for mild Alzheimer’s disease, for Parkinson’s disease, and to reduce the viral load in patients who have HIV disease. Each of these has the potential to manage mild neurocognitive disorder and to prevent it from becoming severe. Metabolic agents that enhance ATP, the primary molecule used for cellular energy, have the potential to slow or prevent further cognitive decline in patients with mild deficits. More research is necessary to determine if these agents will be helpful. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO ALZHEIMER’S DISEASE As mentioned, major or minor neurocognitive disorder is generally secondary to another disorder. The most common disorder underlying neurocognitive disorders is Alzheimer’s disease or AD. Patients will have the incursion of neurofibrillary tangles and beta amyloid plaques in the brain, leading to progressive cognitive decline. Acetylcholine production of the nearby neurons decreases, which affects memory and behavior. There are three criteria for this disorder, including the following: A. The patient must meet the criteria for major or minor neurocognitive disorder B. The decline must be slow and insidious with ongoing cognitive deficits that worsen over years rather than months C. The patient must have “probable” or “possible” Alzheimer’s disease because of the genetic mutation being detected, the presence of steady cognitive decline, prominence of memory impairment, and a lack of other probable cause found. There are specifiers that indicate probable or possible disease, depending on which criteria are met (or not met). The onset occurs as “early onset” disease in the 50s and 60s, although it can occur as late as the 90s with late onset disease. The prevalence increases with age so that 25 percent of people aged 80 or older have this disorder. Risk factors for Alzheimer’s disease include having a traumatic brain injury and old age. Disrupted sleep is an early marker for Alzheimer’s disease. The strongest predictor for Alzheimer’s disease is daytime sleepiness. A common comorbidity is Down syndrome with 75 252 percent comorbidity in Down syndrome patients over 65 years of age. The differential diagnoses include thyroid disease (hypothyroidism), major depression, and any of the other causes of degenerative brain diseases and dementias. Polypharmacy can also cause cognitive impairment that will mimic Alzheimer’s disease. MAJOR OR MILD FRONTOTEMPORAL NEUROCOGNITIVE DISORDER WITH FRONTOTEMPORAL DISEASE There are actually six frontotemporal dementias (FTDs) involving either the frontal or temporal lobes. These include behavioral variant of FTD, semantic variant primary progressive aphasia, non-fluent agrammatic variant primary progressive aphasia, corticobasal syndrome, progressive supranuclear palsy, and FTD associated with motor neuron disease. The disease was previously called Pick’s disease and only one type is now referred to as Pick disease. It is the second most common type of dementia in the US and accounts for 20 percent of young-onset dementia cases, causing symptoms between 45 and 65 years of age in both men and women. The major feature is social and personal behavioral changes, emotional apathy, and language deficits. There is no cure but there are treatments that may alleviate some of the symptoms. It is a heterogeneous disease with three major symptom classifications. These include the following: A. Behavioral variant FTD with social behavioral and conduct changes B. Semantic dementia, which is the loss of word comprehension with fluent speech C. Progressive nonfluent aphasia, which involves changes in speech production Things that tend to be preserved in FTD patients are memory, motor task abilities, spatial skills, and perception. Binge eating and compulsive behaviors are a major feature of FTD with prominence of abnormal eating preferences and behaviors. There is difficulty with executive functioning and working memory. 253 MAJOR OR MILD NEUROCOGNITIVE DISORDER WITH LEWY BODIES Lewy body dementia or LBD includes both Parkinson’s disease and dementia with Lewy bodies (DLB), two dementias characterized by the development of abnormal deposits of alphasynuclein (a protein) in the brain. These are collectively known as synucleinopathies. REM sleep behavior disorder is a major component of this disorder, in which people act out their dreams. Patients will have visual hallucinations, slowness of movement, rigidity, difficulty walking, and fluctuations in attention. There will be blood pressure changes, heart and GI changes, and constipation. Mood changes like depression and apathy are often seen. The REM sleep disorder can appear decades before the dementia shows up (as many as 50 years before the dementia). MAJOR OR MILD VASCULAR NEUROCOGNITIVE DISORDER This is also referred to as vascular dementia or multi-infarct dementia, caused by a series of minor strokes. These cause a stepwise decrease in cognitive functioning. There must be a temporal relationship between a stroke or more than one stroke and cognitive deficits in order to make the diagnosis. It should be noted that Alzheimer’s dementia often coexists with vascular dementia. The key is stepwise cognitive impairment with occasional improvements between events. The changes occur over 5-10 years. Signs are similar to other dementias except that there may be focal neurological signs, depending on the location of the stroke. There will be variable deficits in cognitive functioning. Severely affected patients may have dysarthria or aphasia if the language centers are affected. Frontal lobe deficits with behavioral issues and incontinence are more common in this type of dementia. 254 MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO TRAUMATIC BRAIN INJURY There have been extensive changes in the DSM-V classification of various disorders, including dementia, amnesia, and delirium. Because of the stigma of the diagnosis of “dementia,” there have been different ways of describing these types of symptoms. The new addition includes aspects of visual spatial perception, social abilities, language, executive ability, learning and memory, and complex attention—deficits in any of these can cause functional impairment in brain injured patients and others with neurocognitive disorders. TBI (traumatic brain injury) is often seen in children under four years of age, adolescents (15-19 years of age), and adults over 65 years of age. More severe disease involves the phenomenon of abulia, which is lack of motivation, such that the patient is awake but doesn’t respond. Others will simply lack motivation and initiative, with psychomotor retardation and decreased emotional responsiveness. Some patients will perseverate and will be distractible so that it is difficult to maintain a conversation. The diagnosis of “post-concussion syndrome” is now called “minor neurocognitive disorder secondary to traumatic brain injury,” occurring in about 500 out of 100,000 individuals. It affects about 15 percent of head injured individuals after one year following their head injury. The most common complaint is headache, followed by dizziness. Other symptoms include blurry vision, diplopia, hearing loss, tinnitus, photophobia, and phonophobia. The diagnosis of major neurocognitive disorder secondary to traumatic brain injury involves a significant decline in baseline level of performance in one or more cognitive domains. It can be diagnosed from a standardized neuropsychological test, knowledgeable observer, or by clinical evaluation. The patient must have impairment in their level of independence. Delirium or other mental illness must be ruled out. Minor cognitive disorder tends not to interfere with independence but must affect one or more cognitive domains. 255 SUBSTANCE/MEDICATION-INDUCED MAJOR OR MILD NEUROCOGNITIVE DISORDER This involves neurocognitive changes that can happen during an acute drug intoxication, during withdrawal, or as a late effect after taking a drug. It can involve a slow recovery of brain function after using a drug or other substance for a long time. In some cases, it can take months or years after stopping drug use in order to function in a completely normal capacity. Ongoing problems may always exist. Minor neurocognitive disorder involves everyday independence but requiring certain compensatory strategies in order to accomplish daily tasks. Drugs that can affect neurocognition include alcohol, which will last for 1-2 months after drinking cessation. It is more likely to occur in individuals older than 50 years of age. After using inhalant drugs, it may take years before the cognition returns to normal, especially people who inhale leaded gasoline. Cocaine can have lasting neurocognitive effects, particularly in older users. The same is true of methamphetamine abusers, particularly in those who also abuse ketamine. Opioid users will have neurocognitive problems after quitting, particularly regarding learning and memory. PCP users will also have persistent neurocognitive deficits. About one-third of those using sedative/hypnotics and benzodiazepines will have problems after stopping the drugs. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO HIV INFECTION HIV not only affects the immune system, it also has major effects on the CNS. HIV disease is not as life-threatening as it once was but is now considered a chronic disease, with a normal lifespan expected. Combined antiretroviral treatment (CART) does not completely eliminate HIVrelated dementia, particularly milder forms of the disease. It can affect memory, attention, decision-making, learning, and problem-solving. Up to 50 percent of HIV-infected patients will be diagnosed with a mild neurocognitive disorder—at least partially. About 25 percent will fully meet the criteria and five percent will have major neurocognitive disorder from HIV disease. This is seen in young people in developing countries, where HIV treatments ae less available. 256 The diagnosis is made by meeting the requirements for a neurocognitive disorder with the addition of having confirmed HIV disease. The reason for the NCD must also not be from meningitis or progressive multifocal leukoencephalopathy. The major feature is struggling to complete simple tasks without significant concentration. Severe immunosuppression with a high viral load in the CNS is associated with NCD from HIV disease. Some may have ataxia, balance problems, and difficulty with coordination. Emotional control is difficult and there will be apathy in these patients. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO PRION DISEASE The major prion-related neurocognitive disorder is Creutzfeldt-Jakob disease or CJD. This is always fatal and includes memory problems, behavioral changes, visual disturbances, and poor coordination of rapid onset so that most patients die within a year of the diagnosis. Prions are infectious proteins that cause misfolding of proteins. Most cases are sporadic and infectious with 7.5 percent inherited as an autosomal dominant disease. It is spread through contact with infected brain matter rather than through casual contact or blood transfusion. There is no treatment for the disorder except for supportive measures. The onset of the disorder is around sixty years of age. This is different from mad cow disease and variant Creutzfeldt-Jakob disease. The key finding is rapidly progressive dementia with memory impairment, personality changes, and hallucinations. About 90 percent of patients will have myoclonus as well. Frank psychosis is not uncommon. Gait and posture abnormalities as well as ataxia can be seen. An EEG will be diagnostic of the disease. Death can happen within weeks but usually happens within six months of the diagnosis. A small percent will live 1-2 years after their diagnosis. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO PARKINSON’S DISEASE This is highly related to dementia with Lewy bodies and is a type of Lewy body dementia because of the finding of Lewy bodies in the brain of those with the disorder. The disorder starts as a movement disorder but progresses to include changes in mood and behavior 257 suggestive of dementia. About 78 percent of individuals with Parkinson’s disease will have dementia. There are fewer delusions than are seen in dementia with Lewy bodies and fewer hallucinations. Tremor at rest is a common feature. The diagnosis can only be proven after death with an autopsy of the brain. The diagnosis is made exactly the same way as dementia with Lewy bodies. It is called Parkinson’s dementia only if there is established Parkinson’s disease prior to the development of dementia. The onset of dementia must be about one year or more after the diagnosis of Parkinson’s disease. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO HUNTINGTON’S DISEASE This is an autosomal dominant disorder that is uniformly fatal. There are blood tests that can identify who has the disease and who doesn’t. Early detection of the disease will enhance quality of life. The patient will gradually lose cognitive function and may or may not have behavioral disturbances. Both mild and major neurocognitive disorders can be seen as part of this disorder. Learning and memory are relatively unaffected. The disease diagnosis is made around 40 years of age but there can be early cognitive and behavioral abnormalities seen much earlier. Early symptoms include anxiety, depression, impulsivity, irritability, and apathy. Physical symptoms include difficulty with motor skills and fidgeting, which progress to choreiform movements and speech/swallowing abnormalities. The blood test and an MRI of the brain can be used to diagnose the disorder. The disease is gradually progressive with death occurring about 15 years after motor symptoms manifest. MAJOR OR MILD NEUROCOGNITIVE DISORDER DUE TO MULTIPLE ETIOLOGIES In diagnosing this disorder, the first thing that must be established is that the patient has a mild or moderate neurocognitive disorder. This has already been discussed. After the diagnosis is made, a search for the etiology is undertaken. Sometimes just one etiology is found; however, multiple etiologies can coexist. Commonly seen is a combination of vascular dementia and Alzheimer’s dementia, although other combinations can exist. 258 UNSPECIFIED NEUROCOGNITIVE DISORDER (799.59) This is the diagnosis when there is a clear neurocognitive disorder but not enough information exists to make a diagnosis as to the etiology of the disorder. The patient may meet the criteria for a major or mild neurocognitive disorder but will not have a clear-cut reason as to why the patient has the symptom complex they have.