Transcript DEMETIA

DEMETIA
MEMORY IS THE
RECORDING,
RETENTION &RETRIEVAL OF
KNOWLEDGE
DISORDER OF MEMORY
• DEMENTIA: acquired and persistent
compromise in multiple cognitive
domains that is severe enough to
interfere with everyday functioning
• IN ADDITION TO
MEMORY
IMPAIRMENT AT LEAST ONE OF
FOLLOWING MUST OCCURE:
• APHASIA
• AGNOSIA
• DIORDER OF EXECUTIVE FUNCTION.
DEMENTIA
• COGNITIVE DEFICIT MUST BE
SUFFICIENTLY SEVERE TO CAUSE
IMPAIRMENT IN
• OCCUPATIONAL OR
• SOCIAL ACTIVITY OF DAILY LIVING.
2-DEMENTIA
CLINICAL SYNDROME OF MEMORY DISFUNCTION
• DEMENTIA:
• 1-LOSS OF MULTIPLE ACQUIRED
COGNITIVE &EMOTIONAL ABILITY
SUFFICIENT TO INTERFER WITH DAILY
ACTIVITY.
• 2- INCIDIOUS IN ONSET ,PROGRESSIVE
• 3- AGE ASSOCIATED 1% IN 60 and doubles
every 5 years 30-50% in 85 years
HX
• Few definitive tests are available for
diagnosing dementing disorders,
• so reliable historical information regarding
the :
• onset,
• course, and
• associated features of cognitive
dysfunction is the cornerstone of
differential diagnosis.
Historical Components of the
Dementia Evaluation
INITIAL MANIFESTATIONS
• Impaired recent memory (repeats self, forgets what was heard or
read, misplaces things)
• Poor decision-making, judgment, or problem-solving; decreased
organizational skills
• Difficulty learning new tasks or performing routine tasks
• Problems managing money (balancing checkbook, forgetting to
• pay bills) or household
• Difficulties expressing self (word finding) or participating in
• conversation
• Getting lost in familiar areas, forgetting known routes while driving
• Change in personality (apathetic, disinhibited), mood (sad,
• irritable), or behavior (odd or bizarre)
Historical Components of the
Dementia Evaluation
 TEMPORAL FACTORS
• Mode of onset (acute , subacute, insidious)
• Course (static progressive, improvement over time,
fluctuating)
 INDIVIDUAL FACTORS
• Cultural background
• Educational level
• Social/occupational demands (and changes incurred by
• symptoms)
• Life circumstances (social, financial, occupational, living
arrangements)
• Premorbid personality characteristics
Historical Components of the
Dementia Evaluation
HEREDITARY FACTORS
• Familial risk factors (stroke, hypertension,
diabetes mellitus)
• Genetic: family history suggesting
autosomal dominant inheritance or
multiple cases in family suggesting nonmutation associated familial disease (e.g.,
apolipoprotein E4-associated Alzheimer's
disease)
Historical Components of the
Dementia Evaluation
 MEDICAUNEUROLOGICAL CONDITIONS
• General medical conditions (hypothyroidism,
hypertension, diabetes mellitus , heart disease)
• Neurological conditions (transient ischemic attacks,
strokes, seizures , syncope, head trauma)
• Associated motor features (tremor, gait difficulties,
speech/swallowing disturbance, ataxia)
• Sleep disturbances (sleep apnea, insomnia, sleepassociated movement disorder)
Evaluation
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Cognitive Assessment
Neuropsychiatric Assessment
Laboratory Evaluation
Neuroimaging
EXAMINATION OF AMEMORY
• MINI MENTAL STATUS EXAMINATION(MMSE)
• Detection of IMMEDIATE , LONG TERM ,
REMOTE Memory
• GENERAL &NEUROLOGIC EXAM
EVALUATION OF MEMORY
• Problem in IMMEDIATE MEMORY:
• Attention ororintation cause immediate
memory deficit
• IMMEDIATE: repeat aseries of random
digit.
• Spared in SEVER AMNESIA & mild
DEMENTIA
EXAM OF MEMORY
• LONG TERM MEMORY(SHORT TERM
MEMORY): remember and recall after 3
MINUTES
• Remote MEMORY(LONG TERM
MEMORY): RECALL OF PAST
PERSONAL , GEOGRAPHICAL OR
HISTORICAL TEST.
EXAM OF MEMORY
motor , reflex, cerebellar, gait
• (CHOREA
HUNTINGTON
• TREMOR , RIGIDITY, POSTURAL REFLEX
IMPAIRMENT
PARKINSON
• MYOCLONUS
POST ANOXIC, PRION
• PRIMITIVE REFLEX
BILATERAL
CORTICAL , SUBCORTICAL AND BRAINSTEM
• ATAXIA & CEREBELLAR
WERNIKE
• KORSAKOFE
• CRANIAL NERVE &HEMIPARASIS STROKE
EXAM OF MEMORY
• SENSORY EXAM IS DIFFICULT
• DEFICIT IN HIGH CORTICAL FUNCTION
(AGNOSIA, NEGLECT EXTINCTION
:,CORTICAL LESION
• VIBRATION &POSITION SENSE IMPAIRMENT
VIT B12 DEFICIENCY
• LOSS OF PAIN & TEM. WITH PAIN CRISIS
SYPHILITIC
ASSOCIATED MEDICAL FINDING
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THYROID DYSFUNCTION
VIT B12 DEFICIENCY
TOXICITY OF ANTICHOLINERGIC
WITHDRAWAL OF ALCHOL&SEDATIVE
HEPATIC DISEASE
TRAUMA
MENINGITIS, SAH, NPH,
INTRACRANIAL MASS
EVALUATION OF MEMORY
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CT SCAN
MRI, functional MRI(DETECT SOL,
DIFFUSE ATROPHY
ALZHEIMER,
FOCAL ATROPHY
PICK,S
PET (positron emission tomography, SPECT
(single positron emission computed tomography)
asses global metabolic patterns
• diffuse neuronal loss on degenerative
EVALUATION OF MEMORY
• ELECTROPHYSIOLOGY USE FUL TO
DETECT :
• 1-EPILEPSY,
• 2-SLEEP DISORDER THAT RESULT TO
MEMORY PROBLEM
• 3-DIFFUSE &NONSPECIFIC SLOWING
• 4- PERIODIC PATTERN
• 5-TRIPHASIC, OR BURST
SUPPERATION
in PRION
EVALUATION
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FLUID &TISSUE ANALYSIS
1-cbcdiff
2- LFT
3- TFT,
4-DRUG ANALYSIS IN TOXICATION,
5-TEST FOR CHRONIC MEMINGITIS,
CARCINOMA
• 6- RECTAL BIOPSY
AMILOIDOSIS
EVALUATION
• 7-BRAIN BIOPSY (VASCULITIS,
PRION, INFECTION …)
• 8-CSF
EVALUATION OF MEMORY
• GENETIC TEST :
• HUNTINGTON , ATYPICAL ALZHEIMER,
DEMENTIA
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ALZHEIMER
70%0F DEMENTIA
VASCULAR
10 -20% OF dementia
ALCHOL DEMENTIA
INTRACRANIAL TUMOR
• NORMAL PRESSURE
HYDROCEPHALUS
• CHRONIC DRUG INTOXICATION , METABOLIC
• INFECTION (PRION)
• OTHER NEUROLOGIC (PARKINSON, HUNTIONGTON,
SUPRANUCLEAR PALSY
• PSEUDODEMENTIAI
DEMENTIA(reversible)
1-inflammatory: sarcoidosis , SLE,
vasculitis(primary &secondary)
2- meningitis due to fungi, TB, listeria
monocytosis, lyme. Syphilis, whipple
3 -VIT B12 deficiency
4 - toxics(drugs)
5- mass lesion (tumor, hematom
,communication hydrocephalus
6-coplex partial STATUS epilepticus
DEMENTIA(irreversible)
• 1- DEGENERATIVE (Alzeheimer, PICK,S
huntington, parkinson, lewy body…)
• 2-VASCULAR(multiinfarct dementia, intra
vascular coagulation)
• 3- METABOLIC: storage disease,
leukodystrophy)
• 4- NEOPLASTIC( meningeal metastasis
,gliomatosis cerebri)
NEUROTRANSMITTER OF
MEMORY
• CHOLINERGIC SYSTEM: has a critica l
role in longterm memory
• SCOPOLAMINE(cholinergic antagonist)
impaire memory
• PHYSIOSTIGINE(cholinergic agonist)
faciliate memory performance
• CATHECHOLAMINE : important role in
working memory
ALZEHEIMER( risk factor)
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Age(Prevalance): >60 years
5%
>85years
20-50%
Life time of developing AD is 12-17%
Alzheimer is sporadic , genetics basis 5%
Familial Alzheimer is AD : mutation in the APP (
chromosome 21) presenilin 1 ( chromosome 14 )
, presenilin 2 , APoe €4
• Others:Limited education, depression,gender,
strogen replacement, head trauma, history of
thyroid disease.
Alzehiemer(clinical features)
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1-Memory loss (recent involved.
2- aphasia (wernike)
3- apraxia
4- agnosia (a nosognosia,
prosopagnosia,
• a topoghraphagnosia)
Alzeheimer(clinical symptomes)
• 5-Psychiatric symptoms
•
•
(depression,psychotic symptom
Paranoid delusion , and hallucination.
• 6-Vegetative symptoms(sleep
disturbance, incontinence)
• 7-Later bed ridden
Alzeiheimer(pathogenesis)
• 1- CORTICAL ATROPHY
• 2- NEUROTIC (senile)PLAQUES, that is
amyloid surroded by dystrophic neuritis
• 3- NEUROFIBRILLARY TANGLE(paired
hellicalfillaments.)
• Filaments are major constituent of NFT, ,
neuropil treads, dystrophic neuritis.
Alzeheimer(pathogenesis)
• AD disrupts cortical input to hippcampus
from association and limbic structure
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&
• DISRUPTS HIPPOCAMPAL OUT FLOW
FROM Amomonis , subiculum to
association cortex , diencephalon, basal
forebrain and amygdala.
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Alzeheimer( pathogenesis)
• ALZEHEIMER disconnect the
hippocampus from its major
• INPUT & OUT PUT pathways,
•
BECAUSE
• HIPPOCAMAL FORMATION ARE
INVOLVED EARLY IN AD.
Alzeheimer (management)
• 1-MEMORY TONIC: central
acetylcholinestrase inhibitor:
TACRIN, EXELON MILD &MODERATE
STAGE OF DISEASE
• 2-HALLOPERIDOL FOR DELUSION
• 3-SEROTONIN REUPTAKE INHIBITOR
FOR DEPRESSION
MCI
• The most studied form of MCI is that of isolated
recent memory loss, or amnestic MCI.
• Diagnostic criteria are as follows:
 1-a subjective or objective impairment in recent
memory,
 2-relatively preserved cognitive functions in
other domains,
 3- and presence of normally performed everyday
activities of daily living..
MCI
• Persons who present with amnestic MCI have an increased risk of
developing diagnosable AD at rates of 12% to 15% per year, in
contrast with I% to 2% per year in age-matched normal subjects.
•
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Increased risk of more rapid transition or "conversion“ to AD
includes:
significant hippocampal atrophy on MRI,
worsened performance on recent memory testing,
Elevated CSF tau and reduced A~-42 in the CSF, and
the presence of an apoE4 allele
Alz PET
FTD
• onset typically between the ages of 50 and
60 years
• and featuring insidious personality change,
disinhibition, and subsequent gradual loss
of speech output. Approximately 50% of
the cases were familial.
• Amyloid PLAQUES and
NEUROFIBRILLARY TANGLE not seen.
• FTD as a tauopathy
• No treatment.
NPH
NPH
• Clinical triad ( gait apraxia , dementia ,
incontinency )
• Ivestigation : LP, Imaging
• Tretment : Shunt
• Complication of shunt
Creutzfeldt-Iakob Disease
• clinical presentation includes:
• rapidly progressive dementia, myoclonus,
• Other clinical sign : involvement of
cerebral cortex , cerebellum , basal
ganglia brainstem and splinal cortex
• Psychiatric symptom
• Pathogenesis : proteinaceous infectious
paticle ( prion )
CJD
• Investigation studies :
EEG
LP ( 14-3-3 pr )
MRI
Biopsy ( Pr Psc)
• treatment : no treatment
DLB
• Second most common cause of dementia
• Clinical :
cogition decline without prominent memory
impairment
Visual hallucination
Parkinsonism
Note : well respond to anticholinestrase but
sensetive to antipsychotic drugs
Dementia with NEUROLOGIC sign
• HUNTINGTON:dominant inheritence
•
chore athetosi
•
dementia
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parkinson:
tremor
rigidity
bradykinesia
Dementia (10-15%)-
Dementia with neurologic sign
• Progressive supra nuclear palsy:
•
falling
•
Axiall dystonia
•
pseudobulbar palsy
•
supra nuclear ophthalmoplegia
DEMENTIA WITH NEUROLOGIC SIGN
• AIDS DEMENTIA COMPLEX
•
DEMENTIA
•
ATAXIA
•
SACCADIC &PURSUIT MOVEMENT
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upper motor sign
•
Incontinence
• NEUROSYPHLIS (GENERAL PARESIS