Dementia

Dementia : is defined as a syndrome consisting of chronic, generalized and

1-progressive
2-impairment in two or more areas of cognition:
A-memory:often forget things but never remember them ,ask same question over and over that already answered .
B-speech:Word finding difficulties or use the wrong words
C-praxis:functional difficulties, initially instrumental, subsequently basic activities of daily living
D-gnosis:
E-Visuospatial orientation:(getting lost in familiar places,on their own street, and how to get back home
G-Executive function: problem-solving , Poor judgment: As might forget all about the child and just leave the house for the
day ,abstract thinking Problems (put things in the wrong places put an iron in the freezer or a wristwatch in the sugar bowl.
, Difficulty performing familiar tasks : No cooking or shopping or gardening, ↓insight, dysorganised
J-personality and behavior: drastic changes : They might become , suspicious or fearful. Or withdrawn, (not want to go
places or see other people) Loss of initiative ,passive. Loss of concern or impulsivity, dys inhibition
(new onset irritability, unusual habits or activities) fast mood swings, going from calm to tears to anger in a few minutes
3-sufficient to interfere with social activities, relationships and work
4- absence of impairment of arousal (no disturbance of consciousness)
Severity of dementia
Mild cognitive impairment: Itis a transient state between normal cognitive function and
dementia.

Common complaints are difficulty remembering names and appointments or solving

complex problems. Their memory is worse than age-matched controls.
Epidemiology Dementia is, predominantly, a disorder of later life.
The prevalence increase with advancing age
• Age 80+ >20%
 Classifications:
Cortical and subcortical dementia has proved useful for distinguishing between two variants of
dementia syndrome. The term does not imply that pathological changes are limited to cortical
or subcortical structures. The division between those diseases that affect primarily the cerebral
cortex and those that have their major pathological impact on subcortical structures (i.e., the
basal ganglia, thalamus, and deep white matter).
Characteristic features :
subcortical dementia cortical dementia
Damage of basal ganglia and disruption of frontal Damage of cortical association area
connection
DLB,Parkinsoism, Normal-pressure hydrocephalus and , , Ex. Alzheimer's disease,FTD, and AIDS-dementia complex
Huntington’s disease ,Vascular dementia, Creutzfeldt-
Jakob disease
changes in emotions as Emotional flatness, loss of Aphasia,Apraxia ,agnosia,Visuospatial disorientation
motivation
problems with thinking and social behavior.
slowness in cognitive processing with impaired attention
Executive dysfunctional(Planning, organizing, prioritizing,
Personality typically apathetic and inert. ,Social
problem-solving, initiation disproportionately impaired
withdrawal, Disinhibition
from onset(lost interest in hobbie)

Memory impairment mildly initially Severe amnesia Memory early effected

Pychiatric manifestations early as depresion and psychosis Late

CAUSES OF DEMINTIA
Primary dementia: (that does not result from any other disease).
1-Degenerative Alzheimer’s disease (most common 60-80 %) ,
A very large number of neurological diseases can cause dementia, but most of these are very rare
Huntington’s disease (most common inherited disorders present as dementia) ,Fronto-temporal dementia,
Leukodystrophies ,,Wilson’s disease ,Dystrophia myotonica ,Lewy body dementia
Secondary dementia: (dementia that occurs as a result of a physical disease or injury).
2-Vascular (2nd most common 10-20 %) vascular dementia cause multi-infarct disease resemeble number
of separate disorders include Diffuse small-vessel disease(the most common), Amyloid angiopathy
,Multiple emboli ,Cerebral vasculitis ,Systemic lupus erythematosus
3-Neoplastic Secondary deposits, Primary cerebral tumour ,Paraneoplastic syndrome (limbic encephalitis)
4-Inflammatory Multiple sclerosis, Sarcoidosis
5-Traumatic Chronic subdural haematoma ,Post-head injury ,Punch-drunk syndrome.
6-Hydrocephalus Communicating/non-communicating ,‘normal pressure’ hydrocephalus
7-Toxic/nutritional Alcohol, Thiamin deficiency, Vitamin B12 deficiency ,Anoxia/carbon monoxide
poisoning, Heavy metal poisoning .
8-Infective – Syphilis ,HIV, Post-encephalitis ,Whipple’s disease ,Subacute sclerosing panencephalitis
9-Prion diseases Sporadic Creutzfeldt–Jakob disease (CJD) ,Variant CJD ,Kuru .
10-AIDS-dementia complex is Primary infection of the central nervous system , HIV is now the most
common cause and the sole manifestation of early-onset dementia in some parts of the world , which
develops early in the course of the disease.
Reversible causes of dementia

1. Subdural hematoma. This treatable complication of head injury can present with
dementia, often associated with symptoms of raised intracranial pressure.
2. Normal pressure hydrocephalus. The classical presentation of normal-pressure
hydrocephalus is a triad of cognitive impairment, gait disturbance, and incontinence.
3. B12 deficiency
4. Hypothyroidism
5. Infection, e.g., syphilis, HIV

6. Benign tumors
Assessment of suspected dementia (Approach)

There are two questions to be asked when assessing patients with suspected dementia:
1- Does the patient fulfill the criteria for a diagnosis of dementia?
2- If so, Dementia, 1r y or 2ry cause? Clarified by the presence other signs of nervous system involvement Or
clues suggesting a systemic disease that cause cognitive disorder.

Evaluation or Assessment by:

A- History (onset, duration, and tempo of progression).
1-Rapid onset and rapid progression symptoms: shift the attention to etiologies other than
degenerative which include:vascular,infectious,Trauma toxic,metabolic ,inflammatory and neoplastic
• Vascular dementia :history of stroke with irregular stepwise progression suggests Vascular dementia. It is also
commonly seen in the setting of hypertension, atrial fibrillation, peripheral vascular disease, and diabetes.
• CJD : Rapid progression with motor rigidity and myoclonus
• High-risk sexual behaviours or intravenous drug user should trigger a search for central nervous system (CNS)
infection, especially HIV or syphilis.
• A history of recurrent head trauma could indicate chronic subdural hematoma, chronic traumatic encephalopathy (a
in contact sport athletes such as boxers .
• Drugs that impair cognition ( anticholinergics, psychotropic medications and sedative hypnotics) should besought •
• Alcohol abuse creates risk for malnutrition and thiamine deficiency.
• Others Veganism, bowel irradiation, an autoimmune diathesis, a remote history of gastric surgery, and chronic
antihistamine therapy for dyspepsia or gastroesophageal reflux predispose to B12 deficiency.
• A mood disorders, the recent death of a loved one, or depressive signs, such as insomnia or weight loss, raise the
possibility of depression-related cognitive impairments.
2-Slowly progressive symptoms : AD ,FTD , DLB
AD memory loss over several years, other early symptoms include difficulty with managing money, driving,
shopping, following instructions, finding words, or navigating
• FTD : Personality change, disinhibition, weight gain or compulsive eating, prominent apathy, compulsivity, loss
of empathy for others, or progressive loss of speech fluency or single-word comprehension and by a relative
sparing of memory and visuospatial abilities.
• DLB : early visual hallucinations; parkinsonism; fluctution of concsiousness or sensitivity to psychoactive
medications.
B- PHYSICAL AND NEUROLOGIC EXAMINATION
COGNITIVE AND NEUROPSYCHIATRIC EXAMINATION :
Cognitive assessment (a careful mental status examination) By Screening tools suh as :
1. Mini mental state examination
Maximum score on the MMSE is 30 points • Score of <24 points is suggestive of dementia or delirium
2. Montreal cognitive assessment
Compared with the MMSE ,the MoCA is more sensitive for the detection of mild cognitive impairment
C- Investigation in dementia
In most patients
1-Imaging of head (computed tomography and/or magnetic resonance imaging)
2- Blood tests: Full blood count, ESR , Urea and electrolytes, glucose, Calcium, liver
function tests , Thyroid function tests,Parathyroid and adrenal function ,Vitamin B12
,Syphilis serology, ANA, anti-dsDNA, Toxicology screen
3- Chest X-ray
4-Electroencephalography
In selected patients
1-Lumbar puncture
2-HIV serology
3-Brain biopsy
4-Autoantibodies screen
5-Functional brain imaging
-SPECT: single photon emission computed tomography (↓Perfusion to Tempo
parietal lobe In AD, occipital lobe in DLB)
PET: Fluorodeoxyglucose positron emission tomography
Treatment
Unfortunately, curable disease still accounts for only a small minority of cases.
1-Treatment for treatable causes
2-providing support for patients and caregiver:
Preparing lists, schedules, calendars, and labels can be helpful in the early stages. It is also
useful to stress familiar routines, walks, and simple physical exercises.
3-Effective management of risk factors may slow deterioration, as in hypertension ,DM in
vascular dementia
4-Abstinence in toxic and vitamin replacement in nutritional dementias.
5-Alzheimer-type dementia
A-Cholinesterase inhibitors (donepezil, rivastigmine, galantamine): has been shown to be of some benefit
at slowing progression of cognitive impairment in the early stages of the disease
B-Memantine slightly enhances learning and memory in early disease proves for moderate to severe AD.
C-Vitamin E: A recent study suggested that 2000 IU daily delayed the need for institutionalization of
patients with AD
D-Recent work has focused on developing antibodies against Aβ42
E- Psychotropic drugs may have a role in alleviating symptoms, such as (should be used with care because
of an increased mortality in patients who have been treated long-term with these agents).
1-disturbance of sleep , agitation, hallucinations, delusions, and confusion are difficult to treat and
represent major causes for nursing home placement and institutionalization.
-1st aggressively search for modifiable environmental or metabolic factors like  Hunger,  lack of exercise,
 toothache,  constipation,  urinary tract or respiratory infection,  electrolyte imbalance,  drug toxicity
that can be remedied without psychoactive drugs
- second generation antipsychotics such as quetiapine (starting dose, 12.5–25 mg daily) although the risk
profile for these compounds is significant.
2.depression: a low dose of an SSRI (e.g., escitalopram, starting dose 5 mg daily, target dose 5–10 mg daily)
while monitoring for efficacy and toxicity.
Sometimes apathy, visual hallucinations, depression, and other psychiatric symptoms respond to the
cholinesterase inhibitors, especially in DLB, obviating the need for other more toxic therapies. .
Alzheimer’s disease
• Alzheimer disease (AD) is a neurodegenerative disorder featuring gradually
progressive cognitive and functional deficits as well as behavioral changes and is
associated with accumulation of amyloid and tau depositions in the brain.
• most common form of dementia,the sixth most common cause of death
• It increases in prevalence with age and is rare in people under 45 years. AD is
Risk factors :
1- Old age 2-Head trauma 3-Low educational level 4-Down syndrome
5-Familial history: Genetic factors play an important role and about 15% of cases are
familial. These cases fall into two main groups:
1-early-onset disease with
autosomal dominant inheritance
2-later-onset group where
the inheritance is polygenic.
Cerebral cortex and hippocampus in A D
1-macroscopically: atrophic, particularly Hippocampal atrophy -increased ventricle -decreased cortical
volume

2-Microscopically (Histologically) : histological confirmation usually occurs only after death

characterized by the presence of senile plaques and neurofibrillary tangles in the cerebral cortex. Lead to In
particular impairment of cholinergic transmission, although abnormalities of noradrenaline
(norepinephrine), 5-HT, glutamate and substance P have also been described.
Clinical features
Gradual onset ,very slowly progressive
1-Early Short or episodic memory impairment
Short- and long-term memory are both affected but defects in the former are usually more obvious.
2- then and usually in association with common other cognitive cortical dysfunctions such as language
and visuspatial affection, apraxia and executive
3- Depression is commonly present. Occasionally, patients become aggressive,
The clinical features can be made acutely worse by intercurrent physical disease.
• Investigations and Management
Mentioned in previous pages
 Vascular dementia
Usually present with
1-prominent executive deficits and relatively milder memory loss, as compared with the
pervasive memory impairment seen in Alzheimer's disease
Executive function deficits
1-difficulty in organizing thoughts, time, materials, and belongings;
2-difficulty in initiating tasks, switching flexibly between tasks, or sustaining focus on the
relevant aspects of a stimulus or task;
3-poor judgment or abstraction;
criteria sets for diagnosis of vascular dementia
1-abrupt cognitive deterioration and stepwise progression
2-focal signs Temporal relationship of stroke and dementia
3- cerebrovascular disease on brain imaging
Fronto-temporal dementia
neurodegenerative disorder; characterized by a gradual onset and
progression of changes for several years prior to presentation in:
1-behavior (frontal lobe abnormalities) such as
hyperorality, utilization behavior. and inappropriate sexuality . or
2-language deficits .
The most common presenting symptom is word-finding difficulty.
decreased fluency or hesitancy in producing speech
comprehension.
❖ Clinical subtypes. including
1- Behavioral variant of FTD (bvFTD),
2- Semantic variant primary progressive aphasia (PPA),
3- nonfluent agrammatic variant PPA,
The clinical subtypes of FTD and related disorders are defined by
the hallmark patterns of symptoms and signs observed
Oral apraxia important for diagnosis ?
Lewy body dementia
This neurodegenerative disorder is Alzheimer's-like dementia includes
-Initially prominent memory loss, aphasia, and apraxia and
-later executive deficits (eg, disinhibition, loss of initiative, incontinence).
Clinically characterized by a tetrad of symptoms:
1-Signs and symptoms of Parkinson’s disease:

Parkinsonian features in diffuse Lewy body disease are mild to moderate and start at about the same
time as the dementia

Parkinson's disease+dementia: dementia presented after Parkinsonian features by many years.

2-Fluctuation course of cognitive state

up to 81% have unexplained periods of confusion that lasts days to weeks .
3- Prominent visual hallucinations.
4- Extreme sensitivity to antipsychotic agents
Other characteristics:

1-Bradykinesia, rigidity, and falls are common, but resting tremor often is absent.

2- Madopar, Sinemet response is not nearly as robust as in Parkinson's disease .

3- prominent autonomic parkinsonism symptoms (ie, symptoms of Shy-Drager syndrome) : orthostatic

hypotension and constipation.