Chapter 15

Neuro-cognitive Disorders
 Perspectives on Neurocognitive Disorders

 In this chapter, we examine the exciting new work related to the brain
disorders that affect cognitive processes such as learning, memory,
and consciousness.
 Most neurocognitive disorders develop much
later in life, whereas intellectual disability and
specific learning disorder manifest early in life.
 Neurocognitive disorders can be said to
comprise global cognitive impairment and focal cognitive impairment.
 In focal cognitive impairments, lesions or dysfunction in localized parts
of the brain, that typically sub-serve a predictable set of functions, are
responsible for pathology affecting for example language, praxis,
gnosis and many neuro-integrative functions.
 By contrast global cognitive deficits affect all central processing.
 Global neurocognitive disorders can be acute: they arise suddenly and
run a short course; or chronic: they follow a relentless course
dominated by cognitive impairments.
 Perspectives on Neurocognitive Disorders

 Brain failure involves the failure of the organ that is tasked with taking in
information, integrating it with motivations and other states and
producing an appropriate behavior in response. The brain fails at its
core task.
 Acute brain failure is what we refer to as delirium and chronic brain
failure and major neurocognitive disorder, or what we used to call
dementia.
 The label “neurocognitive disorders” in DSM-5 reflects a shift in the way
these disorders are viewed.
 In early editions of the DSM, they were labelled “organic mental
disorders,” along with mood, anxiety, personality, hallucinosis (an
abnormal mental state involving hallucinations), and delusional
disorders.
 The word organic indicated that brain damage or dysfunction was
believed to be the cause.
 Perspectives on Neurocognitive Disorders

 The concept organic proved to be problematic. Once the term organic

was dropped, attention moved to developing a better label for delirium,
dementia, and the amnestic disorders.
 The label “cognitive disorders” was used in DSM-IV to signify that their
predominant feature is the impairment of such cognitive abilities as
memory, attention, perception, and thinking.
 In DSM-5, neurocognitive disorders is the new category name for the
various forms of dementia and amnestic disorders, with “major” or “mild”
subtypes; DSM-5 retains the “delirium” label.
 As with certain other disorders, it may be useful to clarify why
neurocognitive disorders are discussed in a textbook on abnormal
psychology. Because they so clearly have organic causes, we could
argue that they are purely medical concerns.
 You will see, however, that the consequences of a neurocognitive
disorder often include profound changes in a person’s behaviour and
personality.
 Perspectives on Neurocognitive Disorders

 Intense anxiety, depression, or both are common, especially among

people with major neurocognitive disorder.
 Families and friends are also profoundly affected by such changes.
Imagine your emotional distress as a loved one is transformed into a
different person, acting irrationally and even forgetting your name.
 The far-ranging psychological impact of neurocognitive disorders are
very much the concern of psychologists.
There are three classes of neurocognitive disorders:
 Delirium (an often temporary condition displayed as confusion and
disorientation).
 Mild neurocognitive disorder and
 Major neurocognitive disorder ( both
progressive conditions marked by gradual
deterioration of a range of cognitive abilities).
 Delirium

 Impaired consciousness is the distinguishing

feature of delirium, while impaired arousal
and attention typify it. Without this, the
condition is not a delirium.
 Impaired consciousness is characteristically
accompanied by other cognitive deficits, such
as mood, affective, perceptual, sleep-wake cycle and
behavioural disturbances, that vary in intensity over the course of
hours or days.
 Behaviourally, delirium involves disorganization or pointless behavior.
 Delirium can also be characterized by decreased motor activity,
increased activity or a mixture of the two.
Clinical Description and Epidemiology
 People with delirium appear confused, disoriented, and out of touch
with their surroundings.
 Delirium

 They cannot focus and sustain their attention on even

the simplest tasks.
 There are marked impairments in memory and
language.
 The symptoms of delirium do not come on gradually,
but develop over several hours or a few days, and they
can vary over the course of the day.
 Delirium is estimated to be present in approximately
20% of older adults who attend hospital casualty departments.
 It is most prevalent among older adults, people with acquired immune
deficiency syndrome (AIDS), cancer patients and people undergoing
medical procedures.
 Delirium

 Delirium subsides relatively quickly.

 Its frequency and importance as an indication
of serious illness and high risk of mortality have
prompted its recognition as one of the ‘vital
signs’ (along with heartbeat, breathing
rate, temperature, and blood pressure) that
physicians routinely check when seeing older adults.
Causes of Delirium – Predisposing and Precipitating
 Delirium has variable causes. Delirium itself is not a disease, but rater a
clinical syndrome. Its presence draws attention to its cause.
 Delirium is the end-product of various interacting predisposing and
precipitating factors – whether biological, psychological or social.
 Although anyone is susceptible to developing delirium, it typically affects
the frail and debilitated. Frailty often characterizes the elderly and
physically ill.
 Delirium

 In a fit healthy person, delirium may arise only in the event of severe
and multiple stressors, such as poly-trauma, overwhelming sepsis,
acute organ failure or poisoning.
 What becomes evident when examining the list
of predisposing factors (see Table 15.1), is their
contribution to frailty – advancing age, comorbid
physical illness, coexistence of neurocognitive
impairment and polypharmacy being the most
characteristic.
 Considering that advanced age correlates with increased prevalence of
physical illness, neurocognitive impairment and polypharmacy, it is not
surprising to understand why delirium is most common in the elderly.
 By the same reasoning, for a young, otherwise healthy person to
develop delirium, the precipitant or proximate cause must be
proportionally more severe.
 Delirium

 That delirium can be brought on by the improper use of medication is a

particular problem for older adults because of polypharmacy – the use
of many medications of different classes for multiple physical problems.
 The risk of problems among the elderly is increased further because
they tend to eliminate drugs from their systems less efficiently than
younger individuals.
 Also affecting the elderly is the ease with which delirium may be brought
about. Not surprisingly, infections and medications are often to blame.
 In particular, urinary tract infections precipitate delirium. This apparently
strange phenomenon is believed to be related to toxins contained in the
cell walls, or liberated by certain bacteria commonly responsible for
urinary tract infections.
 Immune responses and intracranial inflammation also play a role. In this
regard, the infective agent Proteus mirabilis is a common culprit.
 Delirium

 Legionnaire’s disease is recognized for its profound propensity to

cause delirium.
 Delirium often occurs during the course of major
neurocognitive disorder (dementia). At least
two-thirds of cases with delirium occur in patients
with pre-existing dementia.
 As many as 50% of people with dementia suffer
at least one episode of delirium. Typically, the
occurrence of delirium in a patient with dementia is a
poor prognostic indicator.
 Symptoms of delirium appear to persist much longer than previously
thought. Although, typically short lived, in certain cases symptoms
persist for months, or even longer, when it becomes difficult to
distinguish it from dementia with reversible features.
 Delirium

 Delirium is not unusual in young children. Febrile illnesses not

uncommonly cause delirium, often at night. Measles and bacterial
tonsillitis are common causes.
 Children may also react unpredictably, or idiosyncratically, to
conventional medications, with delirium ensuing.
 Delirium occurs very often after major surgery – across all age groups.
 Because many of the primary medical conditions can be treated,
delirium is often reversed within a relatively short time.
 Yet, in about a quarter of cases, delirium can be a sign of the end of life.
 Factors other than medical conditions can trigger delirium: sleep
deprivation, immobility and excessive stress can also cause delirium.
Delirium
Clinical Description and Statistics:
Delirium
Clinical Description and Statistics:
Delirium
Clinical Description and Statistics:
Delirium
 Major and Mild Neurocognitive Disorders

 Previously and still widely called dementia, the DSM-5 disorder major
neurocognitive disorder involves global impairment in brain
functioning that affects attention, memory, language, praxis, gnosis,
executive functioning and changes in personality, mood reality testing
and behavior.
 Major neurocognitive disorder is typically
chronic, progressive and irreversible and
can be likened to chronic brain failure.
 Mild neurocognitive disorder is a new
DSM-5 disorder that was created to focus
attention on the early stages of cognitive decline.
 Here the person has modest impairments in cognitive abilities but can,
with some accommodations (for example, making extensive lists of
things to do or creating elaborate schedules), continue to function
independently.
 Major and Mild Neurocognitive Disorders

 The causes of neurocognitive disorders include many

neurodegenerative and other medical conditions as well as the abuse of
substances, alcohol and certain medications that impair cognitive
functioning.
 Many attempts at classifying neurocognitive disorders have been
proposed, all with their own difficulties.
 However, one useful classification considers them as reversible and
irreversible. In this approach, the neurocognitive deficits encountered in
conditions such as major depression, certain infections, reversible organ
failure, nutritional deficiencies and endocrine and metabolic conditions
can be reversed with the restoration of cognitive functional integrity.
 By contrast, irreversible neurocognitive disorders arise from irreversible
brain damage (traumatic, stroke, neoplastic, infection or otherwise) and
the so-called primary neurodegenerative conditions such as Alzheimer’s
and Pick’s diseases.
Major Neurocognitive Disorder
Mild Neurocognitive Disorder
 Major and Mild Neurocognitive Disorders

Clinical Description and Statistics

 Depending on the individual and the cause, the gradual progression of
neurocognitive disorder may have somewhat different symptoms,
although all aspects of cognitive functioning are eventually affected.
 In the initial stages, memory impairment is
typically seen as an inability to register ongoing
events.
 In other words, a person can remember how to
talk and may remember events from many years
ago but will have trouble remembering what
happened in the past hour.
 They might not find their way home because visuospatial skills are
impaired among people with neurocognitive disorder.
 Agnosia, the inability to recognize and name objects, is one of the
most familiar symptoms.
 Major and Mild Neurocognitive Disorders

 Facial agnosia, the inability to recognize even familiar

faces, can be extremely distressing to family members.
 A general deterioration of intellectual function results
from impairment in attention; memory of recent events
and information; language functioning;
recognition; ability to perform previously
learnt complex motor tasks; as well as
the conceptualisation of situations, and abstract reasoning, cognitive
flexibility, planning, execution and self-monitoring impacting real-life
problem solving.
 In short, neurocognitive disorders, or dementia, are
defined by memory impairment with aphasia
(language disturbance), agnosia (failure to recognise
or identify objects despite intact sensory function),
apraxia (inability to carry out motor activities despite
intact motor function) and executive functioning (such as planning,
organising, sequencing and abstracting).
 Major and Mild Neurocognitive Disorders

 Perhaps partly because people suffering from

neurocognitive disorder are aware that they are
deteriorating mentally, emotional changes often
occur as well.
 However, it is accepted that emotional problems
and personality change arise from primary brain
pathology, accompanying the cognitive
impairments that define neurocognitive disorders.
 Other disturbances include apathy and aberrations in reality testing,
often evidenced by suspiciousness and frank delusions of persecution
and infidelity.
 Patients with neurocognitive disorder often wander, seemingly aimlessly.
 Sometimes, they exhibit a tendency to return to an earlier place of
familiarity, like a first home. This phenomenon is called peregrination.
 Major and Mild Neurocognitive Disorders

 Cognitive functioning continues to deteriorate until the

person requires almost total support to carry out
day-to-day activities.
 Ultimately, death supervenes: terminal events include
pneumonia, bedsores, injuries from falls, dehydration,
cardiac arrhythmias, stroke and multiple organ failure.
 Suicide is also a recognized cause of death.
Statistics
 Globally, it is estimated that one new case of major neurocognitive
disorder is identified every 7 seconds.
 Major neurocognitive disorder can develop at almost any age, although
this disorder is more frequent in older adults.
 Estimates in the United States suggest a prevalence of a little more
than 5% in people older than 65; this rate increases to 20%–40% in
those older than 85.
 Major and Mild Neurocognitive
Disorders: Statistics
 Among the eldest of adults, research on
centenarians (people 100 years and older)
indicates that up to 100% showed signs of
neurocognitive disorder.
 Neurocognitive disorder due to Alzheimer’s
disease rarely occurs in people under 45 years of
age. Rates doubles with every 5 years after the of age 75.
 Almost 10% of those over 70 had mild neurocognitive disorder and
11.6% met the criteria for mild amnestic neurocognitive disorder.
 Race also seemed to be a factor with black men and women at higher
risk for mild neurocognitive disorder than white men and women.
 Since the proportion of people aged 66-80 years in sub-Saharan Africa is
higher and those 81 years and older lower, the overall prevalence of
neurocognitive disorder is lower than in Europe.
 However, in sub-Saharan Africa, the prevalence of 3-19% among people
older than 65 is similar to that encountered in other parts of the world.
 Major and Mild Neurocognitive
Disorders: Statistics
 The UFS in conjunction with the 10/66 Dementia
Research Group, demonstrated a higher than
expected prevalence of dementia (6%) in people
over 65 among an urban black community.
 The 2011 national census estimated that up to 2.2
million people in South Africa may be suffering from neurocognitive
disorders.
 In addition to the human costs of neurocognitive disorder, the financial
costs are staggering.
 Estimates of the costs of caring for people with neurocognitive disorder
due to Alzheimer’s disease are often quoted to be about $100 billion
per year in the United States.
 One estimate indicates that the total worldwide societal cost of major
neurocognitive disorder is more than $315 billion.
 DSM-5 Classes of Neurocognitive
Disorder
 DSM-5 identifies classes of neurocognitive disorder based on
(presumptive) aetiology:
1. Alzheimer’s disease
2. Vascular disease
3. Frontotemporal degeneration
4. Traumatic brain injury
5. Lewy body disease
6. Parkinson’s disease
7. HIV infection
8. Substance use
9. Huntington’s disease
10. Prion disease
11. Other medical
conditions
 Neurocognitive Disorder Due to Alzheimer’s
Disease
Neurocognitive Disorder Due to Alzheimer’s Disease
 In 1907 the German psychiatrist Alois Alzheimer first described the
disorder that bears his name. He wrote of a 51-year-old woman who
had a “strange disease of the cerebral cortex” that manifested as
progressive memory impairment and other behavioural and cognitive
problems, including suspiciousness.
 He called the disorder an “atypical form of senile dementia”; thereafter,
it was referred to as Alzheimer’s disease.
Clinical description and Statistics
 The DSM-5 diagnostic criteria for neurocognitive disorder due to
Alzheimer’s disease include multiple cognitive deficits that develop
gradually and steadily.
 Predominant are impairment of memory,
orientation, judgment, and reasoning.
 Neurocognitive Disorder Due to Alzheimer’s
Disease

 The inability to integrate new information results in failure to learn new

associations.
 Individuals with Alzheimer’s disease forget important events and lose
objects.
 Their interest in non-routine activities narrows.
 They tend to lose interest in other people and, as a result, become
more socially isolated.
 As the disorder progresses, they can become
confused, agitated, depressed, anxious or even
combative.
 Many of these difficulties become more pronounced
late in the day, typically around dusk—in a phenomenon referred
to as “sundowner syndrome”—perhaps as a result of fatigue or a
disturbance in the brain’s biological clock.
 Neurocognitive Disorder Due to Alzheimer’s
Disease

 People with neurocognitive disorder due to Alzheimer’s disease also

display one or more other cognitive disturbances,
including aphasia (difficulty with language), apraxia
(impaired motor functioning), agnosia (failure to
recognize objects), or difficulty with activities such
as planning, organizing, sequencing, or
abstracting information.
 These cognitive impairments have a serious negative impact on social
and occupational functioning, and they represent a significant decline
from previous abilities.
 Cognitive deterioration with Alzheimer’s disease is slow during the early
and later stages but more rapid during the middle stages.
 The average survival time is estimated to be about 8 years, although
many individuals live dependently for more than 10 years.
 Neurocognitive Disorder Due to Alzheimer’s
Disease
 In some forms, the dis- ease can occur relatively early, during the 40s
or 50s (sometimes referred to as early onset), but it usually appears
during the 60s or 70s.
 Approximately 50% of the cases of neurocognitive
disorder are found to be the result of Alzheimer’s
disease, which is believed to afflict more than
5 million Americans and millions more worldwide.
Neurocognitive Disorder Due to Alzheimer’s Disease
 Vascular Neurocognitive Disorder

 Vascular neurocognitive disorder is a progressive brain disorder that

is a common cause of neurocognitive deficits.
 It is one of the more common causes of
neurocognitive disorder and it results from
recurrent ischaemic events in the brain.
 Put another way, this condition results
from the cumulative effects of successive,
small, often microscopic, strokes occurring
deep in the brain.
Clinical Description
 DSM-5 lists as criteria for vascular neurocognitive disorder cognitive
disturbances such as declines in speed of information processing and
executive functioning (e.g., complex decision-making).
 In contrast, those with Alzheimer’s disease have memory problems as
their initial cognitive disturbance.
 Vascular Neurocognitive Disorder

 Mood disturbances, particularly depression and excessive

emotionality, also characterize vascular neurocognitive disorder.
 Patients are also more acutely aware of their general and cognitive
decline than those with Alzheimer’s disease.
 Observation and neurological examination will reveal
so-called long-tract motor signs: limb plasticity,
hyperreflexia and a noticeable shuffling gait.
 The onset of vascular dementia is typically more
sudden than the onset for the Alzheimer’s type,
probably because the disorder is the result of stroke, which inflicts
brain damage immediately.
 The outcome, however, is similar for people with both types: Ultimately,
they will require formal nursing care until they succumb to an infectious
disease such as pneumonia.
 Other Medical Conditions That Cause
Neurocognitive Disorder
 DSM-5 identifies eight specific causes in addition to Alzheimer’s
disease and vascular damage:
 Frontotemporal degeneration
 Traumatic brain injury
 Lewy body disease
 Parkinson’s disease
 HIV infection
 Substance use
 Huntington’s disease
 Prion disease
 In addition, a final category—neurocognitive disorder due to another
medical condition—is provided for other causes.
 Other Medical Conditions That Cause
Neurocognitive Disorder

Other medical conditions that can lead to neurocognitive disorder

include:
 Normal pressure hydrocephalus (excessive water in the cranium,
resulting from brain shrinkage).
 Hypothyroidism (an underactive thyroid gland).
 Brain Tumor.
 Vitamin B12 deficiency.
 There is increasing recognition of neurocognitive
disorder among athletes who receive repeated
blows to the head.
 In the past this type of neurocognitive disorder
was referred to as dementia pugilistica (which suggested
that it was restricted to boxers or pugilists) but it is currently referred to
as chronic traumatic encephalopathy (CTE).
 Other Medical Conditions That Cause
Neurocognitive Disorder
 CTE is caused by repetitive head trauma that can provoke distinctive
neurodegeneration.
 In their effect on cognitive ability, all of these disorders are comparable
to the other forms of neurocognitive disorder we have discussed so far.
 Frontotemporal Neurocognitive Disorder

 Frontotemporal Neurocognitive Disorder is an overarching term used

to categorize a variety of brain disorders that damage the frontal or
temporal regions of the brain.
 This condition is dominated by
disturbed personality, language and
behaviour.
 DSM-5 identifies two variants of
frontotemporal neurocognitive disorder characterised by two
predominant modes of expression:
1. Behavioural disruption (e.g., socially inappropriate actions, apathy,
making poor judgments).
2. Language disturbance (e.g., problems with speech, finding the right
word, naming objects).
 One of the disorders in this category of neurocognitive disorders is
Pick’s disease, a rare neurological condition—occurring in about 5% of
those people with neurocognitive impairment.
 Pick’s Disease & Traumatic Brain Injury

 It produces symptoms similar to that of Alzheimer’s disease, but with

more pronounced emotional, interpersonal and behavioural changes.
 Pick’s disease is characterised by deposition of
abnormal extracellular proteins called Pick’s bodies.
 The course of this disease is believed to last from
5 to 10 years, and appears to have a genetic
component.
 Pick’s disease usually occurs relatively early in life—during a person’s
40s or 50s—and is therefore considered an example of early onset
neurocognitive disorder.
 Severe trauma to the head causes the brain to
sustain lasting injuries (called traumatic brain
injury or TBI) which can lead to neurocognitive
disorder.
 Traumatic Brain Injury

 Neurocognitive disorder due to traumatic brain injury includes

symptoms that persist for at least a week following the trauma,
including executive dysfunction (e.g., difficulty planning complex
activities) and problems with learning and memory.
 Those that are at greatest risk for TBI are teens and young adults,
especially accompanied by alcohol abuse or lower socio-economic
class.
 Traffic accidents, assaults, falls, and suicide
attempts are common civilian causes.
 TBI is understandable as commonplace in
combat and acts of terror.
 Lewy body Disease

 The second most common type of neurocognitive disorders (after

Alzheimer’s disease) is neurocognitive disorder due to Lewy body
disease.
 Lewy bodies are microscopic deposits of a protein
that damage brain cells over time.
 The signs of this disorder come on gradually and
include impairment in alertness and attention,
vivid visual hallucinations, and motor impairment
as seen in Parkinson’s disease.
 Patients with Lewy body disease are highly prone to falls.
 Of interest, Lewy bodies are frequently encountered in the brains of
patients with Parkinson’s disease.
 By virtue of this, and the occurrence of movement disorder symptoms in
Lewy body disease, it is often considered as one of the so-called
‘Parkinson plus syndromes’, or features of Parkinson’s syndrome
occurring with cognitive impairment.
 Parkinson’s Disease

 Parkinson’s disease is the prototypical movement disorder, first

described as the ‘shaking palsy’ or ‘paralysis agitans’ by James
Parkinson in 1827.
 It is a neurodegenerative disorder that affects about 1 in every 1,000
people worldwide.
 Quintessentially a movement disorder, Parkinson’s disease is defined
by its motor, or movement abnormalities.
 Patients with Parkinson’s disease demonstrate
cell loss in the substantia nigra of the midbrain.
 Dopaminergic fibres radiate from the substantia
nigra to terminate on the corpus striatum of the
basal ganglia. Loss of this innervation gives rise
to productive and deficit symptoms.
 Lewy bodies are found in brain specimens of patients with Parkinson’s
disease.
 Parkinson’s Disease

 Movement abnormalities manifest as follow; resting tremor, that looks

like a pill-rolling movement if it affects the fingers;
muscle rigidity, that, when superimposed with
tremor, creates a feeling of moving a cog –
hence its description as ‘cogwheel
rigidity’; loss of responsivity in facial
muscles so that the face looks expressionless or
‘mask-like’; slowing of gait and ever-
decreasing length of stride – when
combined with a tendency to lean forward while
walking; the gait becomes rushed, or ‘festinant’, as
if the patient is attempting to keep up with a body
wanting to topple forward; reduced or absent arm
swing, and indeed all spontaneous movements,
stooped posture; varying degrees of postural instability,
increasing their risk of falls.
 Patients with Parkinson’s disease also talk very softly and
 Parkinson’s Disease

 Often, difficulty writing is one of the earliest signs, along with

clumsiness.
 Parkinsonian motor symptoms can be summarized as productive and
deficit symptoms: productive symptoms are ‘release phenomena’ or
abnormal motor programs released by the striatum and include tremor
and rigidity.
 By contrast, deficit symptoms are slowness, or bradykinesia, and lack
of spontaneous muscle movements, affecting arm swing and affective
expression.
 Anxiety is a common presentation. As the disease progresses,
depression often supervenes, as does, more rarely, psychosis.
 With time, the movement disorder becomes very limiting. Often,
choreic movements emerge with sudden violent jerks.
 Initially, a minority of patients undergo cognitive decline.
 Parkinson’s Disease

 It is estimated that about 75% of people who survive more than 10

years with Parkinson’s disease develop neurocognitive disorder;
conservative estimates place the rate at 4 to 6 times that found in the
general population.
 The neurocognitive disturbances of Parkinson’s disease are
prototypical of the so-called ‘sub-cortical dementia’ – slowness,
memory deficits that respond to cueing and affective disturbances.
 Other sub-cortical dementias include vascular neurocognitive disorder
and HIV-neurocognitive disorder.
 Human Immunodeficiency Virus-Type 1
(HIV-1)
 The Human Immunodeficiency Virus type 1 (HIV-1)
which causes AIDS, can also cause neurocognitive
disorder (called neurocognitive disorder due to HIV
infection).
 This impairment seems to be unrelated to the
opportunistic infections that accompany HIV; in other
words, the HIV infection itself seems to be responsible for the
neurological impairment.
 The early symptoms of neurocognitive disorder resulting from HIV are
cognitive slowness, impaired attention, and forgetfulness.
 Affected individuals also tend to be clumsy, to show
repetitive movements such as tremors and leg weakness,
and to become apathetic and socially withdrawn.
 Cognitive impairment is an important factor in ensuring
treatment compliance in patients with HIV.
 Human Immunodeficiency Virus-Type 1
(HIV-1)
 People with HIV seem particularly susceptible to impaired thinking in
the later stages of HIV infection, although significant declines in
cognitive abilities may occur earlier.
 Cognitive impairments were highly common among those infected with
AIDS, but with the introduction of new medications (highly active anti-
retroviral therapies, or HAARTs) less than 10% of patients now
experience neurocognitive disorder.
 Like neurocognitive disorder from Parkinson’s disease and several
other causes, neurocognitive disorder resulting from HIV is sometimes
referred to as subcortical dementia.
 Huntington’s Disease
 Huntington’s disease is a monogenetic disorder,
characterized by the emergence of chorea – often
violent, unexpected jerks of the limbs. These jerks have
a dance-like quality, hence the name, chorea, which
means dance (cf. choreography in ballet).
 Early on in the disease, subtle cognitive deficits are
demonstrable by sensitive neuropsychological testing
well before motor signs emerge.
 Huntington’s disease is one of the few psychiatric disorders where a
specific aetiology is known. The condition is inherited in an autosomal
dominant fashion and involves a single genetic lesion – the occurrence
of multiple end-repeats on chromosome 4 resulting in the production of
an abnormal protein, appropriately named huntingtin.
 As a result, cells in the body of the caudate nucleus of the corpus
striatum of the basal ganglia die, causing the characteristic
appearance on coronal sections with neuroimaging where the
third ventricle appears like a box.
Huntington’s Disease
 Huntington’s Disease

 People with Huntington’s disease can live for 20 years after the first
signs of the disease appear, although skilled nursing care is often
required during the last stages.
 Just as with Parkinson’s disease, only a portion of people with
Huntington’s disease go on to display serious neurocognitive impairment
– between 20% and 80%.
 However, some researchers believe that all patients with Huntington’s
disease would eventually display neurocognitive impairments if they
lived long enough.
 Neurocognitive disorder due to Huntington’s disease also follows the
subcortical pattern.
 Mood and psychotic disturbances are common. Huntington’s disease
also carries an unusually high risk of suicide.
 Prion Disease

 Neurocognitive disorder due to Prion disease is rare and it is a

progressive neurodegenerative disorder caused by prions – biological
molecules that are encountered in normal tissue, but, when slightly
modified, insert themselves into cells to produce cell death.
 They can reproduce or clone themselves,
like living organisms. Prions are much
smaller than the smallest of viruses and are
so-named because they are proteinaceous in
nature.
 The name prion refers to proteinaceous
particle. Unlike other infectious agents such as bacteria or viruses,
prions are thought by some to have no DNA or RNA that can be
destroyed by chemicals or radiation.
 As a result, there is no known treatment for prion disease and the
course of this disorder is always fatal.
 Prion Disease

 Prion infection leads to localized cell death in the brain, causing

multiple cavities that give the brain a sponge-like appearance.
 Prion disease in humans came to attention following the recognition of
a progressive neurodegenerative condition, resulting in death, among
the Fore people of Papua New Guinea.
 Kuru resulted from infection by prions through the
practice of cannibalism. The Fore practiced ritual
cannibalism, during which they ate the brains of
deceased members of a nearby village.
 Creutzfeldt-Jakob Disease

 Creutzfeldt-Jakob disease is a type of prion disease.

 It is believed to affect only one in every million individuals and no
cases have ever been reported in the Americas or South Africa.
 In the UK Creutzfeldt-Jakob disease and its possible
association with bovine spongiform encephalopathy or
“mad cow disease”, caused much concern.
 What was alarming was the discovery of a so-called ‘new
variant’ Creutzfeldt-Jakob, - a rapidly progressive form of
the disease. Ten cases of new variant Creutzfeldt-
Jakob were believed to be linked to bovine spongiform
encephalopathy.
 This discovery led to the fear that the disease might be transmitted
from infected cattle to humans and exports of beef from the UK were
banned for a number of years.
 Creutzfeldt-Jakob disease causes progressive cognitive decline.
 Creutzfeldt-Jakob Disease

 Initially, it manifests with mild slowness of thinking. Soon, devastating

cognitive decline becomes apparent.
 Patients are often lethargic, while bouts of aggression and
psychotic-like agitation may
occur.
 They display motoric abnormalities with gross ataxia and myoclonic
jerks. Seizures, coma and death follow. There is no treatment.
 Substance/Medication-Induced
Neurocognitive Disorder
 Prolonged drug use, especially combined with poor diet, can damage
the brain and, in some circumstances, can lead to Substance /
medication-induced neurocognitive disorder.
 This impairment unfortunately lasts beyond the period involved in
intoxication or withdrawal from these substances.
Description and Epidemiology
 As many as 7% of individuals dependent on alcohol meet the criteria for
neurocognitive disorder.
 The long-term abuse of a number of drugs can lead to symptoms of
neurocognitive disorder, including alcohol, inhalants such as glue or
gasoline (which some people inhale for the euphoric feeling they
produce), and sedative, hypnotic, and anxiolytic drugs.
 These drugs pose a threat because they create dependence, making it
difficult for a user to stop ingesting them. The resulting neural toxicity
(brain damage) can be permanent and can cause the same symptoms
as seen in neurocognitive disorder due to Alzheimer’s type.
 Substance/Medication-Induced
Neurocognitive Disorder
 The DSM-5 criteria for substance/medication-induced
neurocognitive disorder are essentially the same as many of the other
forms of neurocognitive disorder; they include memory impairment and
at least one of the following cognitive disturbances: aphasia (language
disturbance), apraxia (inability to carry out
motor activities despite intact motor function),
agnosia (failure to recognize or identify objects
despite intact sensory function), or
a disturbance in executive
functioning (such as planning,
organizing, sequencing, and
abstracting).
 Characteristics of Neurocognitive
Disorders

Characteristic Dementia of the Alzheimer’s Subcortical Dementias

Type
Language Aphasia (difficulties with No aphasia
articulating speech)
Memory Both recall and recognition are Impaired recall; normal or less
impaired impaired recognition
Visuo-spatial skills Impaired Impaired
Mood Less severe depression and More severe depression and
anxiety anxiety
Motor speed Normal Slowed
Coordination Normal until late in the Impaired
progression
Source: Adapted, with permission of Oxford University Press, from Cummings, J. L. (Ed.) (1990).
Subcortical dementia. New York, NY: Oxford University Press,
© 1990 Jeffrey L. Cummings.