Dementia, NOS

Dementia, NOS

Articledementia
Brain & Nerve
Behavioral & Mental Health
+3
Contributed byKrish Tangella MD, MBAAug 19, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • Chronic Brain Syndrome (Dementia, NOS)
  • Mild Cognitive Impairment (Dementia, NOS)
  • Vascular Dementia (Dementia, NOS)

What is Dementia, NOS? (Definition/Background Information)

  • Dementia, NOS is a type of disorder accompanied by a collection of symptoms that indicate brain impairment and loss of brain function. It leads to a progressive decline of all cognitive aspects such as behavior, memory, attention, analytical ability, thinking, language skills, etc.
  • The onset of Dementia can be gradual or sudden. There can be a gradual deterioration of the brain’s abilities, due to age factor; or a sudden event like an accident or illness, might cause Dementia
  • It is usually irreversible and degenerative. However, in certain cases, with early detection, full recovery of the cognitive functions may be possible

Who gets Dementia, NOS? (Age and Sex Distribution)

  • Dementia generally occurs past the age of 60-65 years. It is infrequent in individuals below this age range. When it occurs below this age group, it is termed as ‘early onset Dementia’
  • Generally, females are slightly more prone to this condition, than males
  • No racial or ethnic preference has been observed. However, a majority of Dementia cases seem to be observed in geographical regions with low to mid-level incomes

What are the Risk Factors for Dementia, NOS? (Predisposing Factors)

There are a host of factors that could potentially increase the risk for Dementia. Some of these factors may be controlled, while others are unchangeable, and often relentless:

  • Age: This factor along with other brain-related conditions may enhance the risk
  • Genetic causes, in individuals having a family history of Dementia
  • Down syndrome: An early onset of Dementia might take place due to Down syndrome
  • Food and lifestyle related: Factors include regular alcohol use/abuse, smoking of tobacco, cholesterol (high levels of LDL), atherosclerosis due to fat build-up, and high blood pressure
  • Any factor that increases one’s risk for Alzheimer’s disease, like diabetes (type 2), high blood levels of the amino acid homocysteine (homocystinuria), depression, lifestyle habits
  • High estrogen levels in women
  • Metabolic conditions, like high blood sugar, salt, calcium levels; vitamin B3, B12, folic acid deficiency
  • Brain hemorrhages (subdural hematoma, stroke), trauma (prolonged, or due to repetitive head injuries while boxing, playing football), brain tumors/cysts (like lymphoma, glioma), and fluid in the brain (hydrocephalus – normal pressure)
  • Certain drugs and medications used to lower cholesterol levels, treat stomach acidity, and anticonvulsants can increase the risk of Dementia
  • Failure of kidney and liver

Direct or indirect contributory risk factors for Dementia include the following diseases, infections, and disorders (some of these maybe inherited):

  • Adrenoleukodystrophy (X-linked)
  • AIDS/HIV infections
  • Alexander disease
  • Antiphospholipid syndrome
  • Binswanger's disease
  • Canavan disease
  • Cerebrotendinous xanthomatosis
  • Cerebrovascular disorder (Moyamoya)
  • Creutzfeldt-Jakob disease
  • Dentatorubral-pallidoluysian atrophy
  • Epileptic seizures (prolonged)
  • Fatal familial insomnia
  • Fragile X-associated tremor/ataxia syndrome
  • Frontotemporal lobar degeneration
  • Gaucher's disease type 3
  • Glutaric aciduria type 1
  • Hereditary stroke disorder (CADASIL)
  • Huntington's disease
  • Hypothyroidism
  • Krabbe's disease
  • Lyme disease
  • Maple syrup urine disease
  • Meningitis/Encephalitis
  • Metachromatic leukodystrophy
  • Mitochondrial disorder (MELAS)
  • Multiple sclerosis
  • Neuroacanthocytosis
  • Neuronal ceroid lipofuscinosis
  • Niemann-Pick disease type C
  • Organic acidemia
  • Pantothenate kinase-associated neurodegeneration
  • Parkinson's disease
  • Pelizaeus-Merzbacher disease
  • Pick’s disease
  • Progressive supranuclear palsy
  • Sanfilippo syndrome type B
  • Spinocerebellar ataxia type 2
  • Spinocerebellar ataxia type 17
  • Syphilis
  • Tay-Sachs disease
  • Urea cycle disorders
  • Whipple's disease
  • Wilson's disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dementia, NOS? (Etiology)

  • Much of Dementia is not yet clearly understood
  • The influence of certain disorders/inherited conditions on Dementia development is being studied
  • The causative factors for Dementia are varied and numerous. These are classified according to the rate of progression of the condition, part of the brain that is affected, similarities between the symptoms, and whether Dementia is reversible or irreversible

Following are examples of some Dementia types and their causal factors:

Dementia based on brain-part location affected:

  • Fixed cognitive impairment:

This may occur as a result of any traumatic injury to the brain, affecting specific parts (or all parts of the brain), where the damage takes place. These may be due to external injuries or internal injuries (like a stroke).

A larger and more generalized area of the brain may be involved if the causal factors are infections, excess alcohol consumption, severe forms of hydrocephalus, or long-standing epileptic seizures.

In one form of Dementia (the Frontotemporal Lobar Dementia), the focal area of brain is affected. The affected brain area includes the frontal and temporal lobes. It is thought to be caused due to reasons that have not been understood, or are yet to be established.

Based on rate of progression of Dementia:

  • Slowly Progressive Dementia:

Neurodegenerative conditions and disorders cause slowly progressive Dementia. In this type, the nerve cells lose their function over many years. A few examples are Dementia that occur due to Alzheimer’s disease, Huntington’s disease, Dementia due to Lewy bodies, untreated hypothyroidism, Vascular Dementia, Dementia Pugilistica (caused when a boxer suffers prolonged head injuries), nutrition-related, brain infections, etc.

  • Rapidly Progressive Dementia:

An example of rapidly progressive Dementia includes Creutzfeldt-Jakob disease that causes a sudden advancement of the condition. Many of the ‘slow and gradual progress’ Dementia, such as  Alzheimer’s disease related, Huntington’s disease, Dementia due to Lewy bodies, can have periods where Dementia develops rapidly.

Following are the types of Dementia based on causative factors:

  • Alzheimer’s disease
  • Huntington’s disease
  • Creutzfeldt-Jakob disease
  • Niemann-Pick disease type C
  • AIDS/HIV related

Following are some examples based on reversibility of Dementia:

  • Dementia due to hormonal, metabolic, nutritional insufficiency causes, can be reversible
  • Dementia due to brain infections, such as syphilis, encephalitis, meningitis, Lyme disease, may be reversible
  • Reactions to drugs causing Dementia, may be reversible
  • Cardiovascular and pulmonary issues that causes significant oxygen shortage affects the brain irreversibly
  • Tumors and brain hemorrhages, may cause irreversible Dementia

What are the Signs and Symptoms of Dementia, NOS?

Signs and symptoms associated with Dementia are influenced by a variety of factors, such as causal factor(s), age, onset conditions, comorbidities present, etc. The presentations may be mild or severe:

  • Progressive deterioration of the senses, memory loss, communication difficulties, incoherence
  • Loss of basic motor (physical coordination) skills, such as difficulty walking, maintaining balance, swallowing difficulties
  • Changes in overall personality, such as regression or absence of reasoning/rationale, agitation, delusions, depression
  • Extremely irrational mental/emotional/physical behavior (may be completely inappropriate for the situation) resulting in loss of social abilities and social awkwardness

How is Dementia, NOS Diagnosed?

A variety of tests are usually conducted to evaluate Dementia. The evaluation is also based on the set of signs and symptoms, and other disorders that may be present. The main diagnostic tools include:

  • Physical exam with a comprehensive evaluation of medical history, including collecting details on any underlying conditions/disorders and their progression
  • Neurological and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
  • Psychiatric examination
  • Imaging studies performed are: MRI scan of the central nervous system (brain and spine), CT scan (head), PET imaging of brain
  • Blood analysis: Total blood count, blood chemistry, glucose, ammonia, blood gas analysis, electrolytes, vitamin levels
  • Urine analysis: Screening for toxic substances, alcohol, determination of vitamin deficiencies
  • Cerebrospinal fluid analysis
  • Liver and kidney function tests
  • Thyroid function tests, such as TSH, T3, and T4
  • Electroencephalogram (EEG)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dementia, NOS?

The complications of Dementia (NOS) depend on many factors, such as the severity of the condition, the underlying causal factor, age, and overall health of the individual, etc. The complications may include:

  • The main complication, which occurs on account of memory loss and neurological function impairment, is that institutionalized care might be required for prolonged periods, or for the rest of an individual’s life. This is due to complete degeneration of mental and emotional health, since there is a progressive inability of individuals to take care of themselves
  • There may be a loss of inhibition, spontaneous incontinence (involuntary bowel or bladder movement), loss of sleep
  • Complete loss of personality, causing paranoia, depression, irrational aggression/anxiety, and delirium
  • The individual may become susceptible to opportunistic infections due to a reduced sense of hygiene, decreased intake of food/nutrition. This often leads to a reduced lifespan
  • An individual may have persistent pain, which is often under-treated due to lack of good communication between the individual and their healthcare providers
  • Feeding and swallowing problems resulting in poor intake of food
  • Treatment medication may have significant side effects

How is Dementia, NOS Treated?

Treatment of Dementia is based on its cause, severity of the condition, and associated signs and symptoms. Dementia management is undertaken on a case-by-case basis and there is no standard treatment. Some of the methods include:

  • Addressing general health problems that are mostly linked to mental balance and well-being. This includes the treatment of anemia, dietary or vitamin deficiencies, heart-related ailments, infections, thyroid disorders, hormonal imbalances, and regulating blood pressure
  • Treatment of the underlying illness, to prevent progression of Dementia
  • Ensuring that the individual has a nutritious diet with medical supplements (balanced diet that include proteins, fiber-rich fruits and vegetables, milk shakes, juices, and plenty of water)
  • Ensuring that the individual is well-cared for in a safe environment (at home, or outside) and is under regular (and maybe constant) care and support
  • Use of medications to control behavioral issues and prevent further loss of mental stability, like stimulants, mood stabilizers, cholinesterase inhibitors, NMDA receptor antagonists, antipsychotic drugs, sedatives, anti-depressants, etc. Many of these medications may cause side effects, like nausea, stomach problems, drowsiness, and vomiting
  • Hospitalization may be required (for short or long duration), depending on the condition of the individual. This is usually followed by regular check-ups
  • Some forms of Dementia cannot be treated and may be only managed by providing proper supportive care

A healthcare provider may utilize the following treatment measures on a case-by-case basis. These are meant to provide mental and emotional support, and to develop or retain communication skills:

  • Psychiatric treatment
  • Group therapy interactive sessions
  • Support groups

How can Dementia, NOS be Prevented?

Prevention of Dementia is related to the causative agent and other underlying ailments. Some cause factors are permanent, while others are alterable. Dementia caused by the latter (alterable factors) may be prevented. Nevertheless, in almost all cases of the disorder with irreversible factors, progression of the condition can be delayed or controlled, to some extent.

  • Dementia linked to age and other comorbidities (presence of other diseases/disorders), genetic factors (inherited conditions such as Down syndrome), may not be preventable
  • Factors associated with diet, lifestyle, and health in general; like alcoholism, smoking, obesity, cholesterol levels, stress, nutritional deficiencies, hormonal problems, use of certain medications, diabetes, etc., may be regulated and/or controlled
  • Any injuries to the brain, or the presence of brain tumors, have to be treated immediately and adequate follow-up maintained. There is a possibility that these may significantly contribute to Dementia development, in the future
  • Individuals who fall in the high-risk category (and all others over the age of 60 years) should be encouraged to be socially active and physically fit (with regular exercising). A healthy diet rich in fruits, vegetables, fish, whole grains, and low in fats and sugar, is recommended
  • High-risk individuals should constantly maintain a healthy mind, by undertaking mentally stimulating activities; like reading/writing/teaching, solving puzzles, playing video games, listening to music, learning new skills (painting, language, playing an instrument), etc.

What is the Prognosis of Dementia, NOS? (Outcomes/Resolutions)

The prognosis for an individual with Dementia is based on a combination of many factors:

  • The causative factor, underlying disorder, or inherited condition (if any):

Causative factor of this brain disorder plays an important role in determining the prognosis. Individuals with long-standing comorbidities and infections do the worst and may require lifetime medical support and personalized care (maybe in a healthcare environment). Death can occur on account of major underlying disorders.

Other prognostic factors include:

  • Age of the adult
  • Degree of severity
  • General health and medical history
  • Tolerance level/health of the individual, when subjected to various medications
  • Complications observed, if any

Normally, life-long management of the condition is necessary, since the brain is abnormally affected. This is particularly important, if other/secondary disorders are present.

In all cases, early detection and aggressive management of Dementia, with regular health checkups are essential. This will not only prevent a further and rapid deterioration of the condition, but also help sustain the quality of life.

Additional and Relevant Useful Information for Dementia, NOS:

  • There are voluntary organizations and support groups for Dementia that provides counsel, help, and understanding, to the affected individuals and their families
  • Participation in social activities (such as volunteering), getting involved in community teaching, joining online social groups, etc. are some of the other activities that should be encouraged to delay/decrease progression of the condition
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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