Světlana SKUTILOVÁ
University Hospital Brno
 De (without) - Mens (sense)
COGNITIVE DISTURBANCE
 (memory,orientation,speech, attention,
delusions, hallucination )

 BEHAVIOUR DISTURBANCE
 (personality, emotion-
apathy,aggression,depression)
 LOSSOF SELF-SUFFICIENCY
 (employment …..take care about himselfs)
 silent epidemic of the 21. century
 illness of the all familly – objectiv anamnesis!
 150.000 patients now in the Czech republic
 (10 million inhabitants…)

 A. PRIMARY DEGENERATIVE DEMENTIA

 B.VASCULAR DEMENTIA

 C. SECONDARY DEMENTIA
 WHO makes diagnosis of dementia in CZ ?
 Neurologist +psychiatrist +geriatrist
 in cooperation with neuropsychologist
 TARGET:
 - examinate of all the brain lobes
 The most exact tests for F lobe
 The less exact tests for T lobe
 The least exact test for O+P lobe
 Standardization (comparison each other)
 Senzitivity (detection of minimum deficit)
 Repeatable ( result during time)
 Quantification (score)
 Absence of neuropsychologist
 Examination takes o few hours
 Bed – side tests
 Short, orientational assessment of cognitive
function ever by general practitioner or
ambulatory specialist
 The typ of dominant lobe impairment help us with
 diferential daignosis of dementia kind
F ( behavior function) FTD
T….P (memory function) AD
P + O (visuallconstructive function) DLBD
multiple impairment VD
 The most used test (1975)
 WHY??
 ADVANTAGE:
 - quick
 - easy administration
 - requirement of Czech insurance company
 - monitoration of dementia progression
 DISADVANTAGE:
 Not enough sufficient for
 - early stage of dementia ( MMSE oft normal)
 - diagnosis of FTD (no examination of F lobe)
 - diagnosis of DLBD ( no examination of
 O + P lobe)
 Mild 25 - 18 p.
 Medium 17 – 6 p.
 Serious 5 – 0 p.

 Standard score 28-30 p.
 ?? IMPORTANCE – therapeutic strategy !
 SCORE
 Maximum 100 points ( MMSE is a part of )
 Less than 82 p.
 : senzitivity of dementia 84%
 : specifity of dementia 100%
 MMSE ACE

 10-15 min 25 -30 min

 - EARLY dementia
 - FTD, DLBD
 -MONITORATION
 of developed
 dementia





 1. ACE:
 Suspicion of the dementia
 Expected another kind of dementia than AD
orVD
 2. MMSE:
 Already developed dementia
 Monitoration of dementia
 The causes of dementia : about 60 various diseases
A/ Primary NEURODEGENERATIVE Dementia
 B/VASCULAR Dementia
 C/ SECONDARY Dementia attending basic
 NEUROLOGIC or INTERNAL diagnosis
 (disturbance of metabolism,nutrition, endocrinopaty, toxic
brain disturbance)
 Exclude SECONDARY Dementia (TRETABLE)
 EVERY!! NEUROIMAGING (CT,MRI,PET MRI)
 - BloodTests : blood count, renal/liver
biochemistry, vitamine B12, thyroid function
tests (Cu +ceruloplasmin, serology HIV +
syphilis)
 - CSF (basic, triplet, protein 14-3-3)
 - (EEG)
 - (genetic)
 1. Minimal cognitive impairment (MCI)
 10-15% transformation to AD
 2. DEPRESSION (pseudodementia)
 - therapeutic test with antidepressant
 3. DELIRIUM
 - sudden starting, fluctuating,duration days
 + quantitative consciousness failure
 4. Side effects of FARMAKOTREATMENT
 in old age
 Anticholinergic ( Akineton, tricyclics antidepressant)
 opiates
 hypnotic
 HISTOLOGIC CRITERIA:
 1. AMYLOIDOPATHIES - ALZHEIMER´S D.
 Amyloid plaques… deposits of B amyloid
 2.TAUOPATHIES - FRONTOTEMPORAL D.
 - CBD, PSP
 Pick bodies …. deposits ofTau protein
/ubiquitin protein/
 3. SYNUKLEINOPATHIES – DLBD, PDD
 Lewy bodies… deposits of synuklein
MOST OFTEN!!
 60% of all the kinds of dementia
 Preclinic stadium even 15 years
 Neuropsychologic exam : The first sign 
DISTURBANCE OF RECENT MEMORY
 T-P lobe
 HISTOLOGY: AMYLOIDOPATHIES
AGE !!!! 65 years - 5%
 85 years - 50%
 Low education
 Low intelect and physic activity
 Social izolation
 female gender (3,1 x)
 genetic factors (early onset)
 1. AD with early onset ( to 60 years age) 5%
 - genetic risk faktors …APO E4 ( alela E4 for
apolipoprotein E )
 2. AD with late onset (most of patients) !
 -sporadic form
 1.PROBABLY
 - disturbance 2 or more cognitive function,
progression between 60-90 years, depression,
anxiety, delusion,hallucination, emotion instability,
incontinency
 Possible neurologic signs (epilepsy, parkinsonism)

 2. DEFINITIVE
 Histologic verification by brain biopsy (post
mortem)
 (BRAIN CT ):
 - atrophy T lobe (P ), extension of lateral
ventricles
 BRAIN MRI:
 - hippocampal atrophy (high specifity)
 i.v.aplication of radioactive isotopes
 - FDG (fluorodeoxyglukoza) – decreased
glukose metabolism MEDIOTemporal LOBE
 (gyrus cinguli, precuneum)…late T-P lobe
 University Hospital Brno 2017
 AMYLOID BRAIN PET MRI :
 In vivo detection of amyloid plaques
 Prague 2015 expanzive /1800 dollars/
 ADVANTAGE: MCI….start of therapy
 early diagnostic
 negative result exclude,no risk
 clinical trials
 NO: asymptomatic patient with + genetic
 + familial history
 NORMAL PATOLOGY
 TRIPLET : 3 biomarkers, proteins
 BETA-AMYLOID decreased
 TAU-PROTEIN increased
 P/TAU-PROTEIN increased - most exact
 Negativ result exclude AD
 University Hospital Brno (2019)
 SYMPTOMATIC : NO CURE, NO STOP
 BUT SLOW DOWN
 A/ ACETYLCHOLINESTERASE INHIBITORS
 I: Mild and medium stage of AD (MMSE 25-13 )

 - Donepezil 10mg 1x1
 - Rivastigmin 6mg 2x1 (9,5 mg patch 1x1)
 - Galantamin
Side effects: impaired digestion, parkinsonism

 B/ MEMANTIN – influence on NMDA receptors
 Medium stage of AD (MMSE 17-6 )
 A+B/ DUALTHERAPY ( MMSE 17-13)
 Mild stadium .. Memory Late stadium… Behaviour
 DO NOT Prescribe : nootropics, vasodilatans
 Parallel therapy !!: cognitive training
 physical training
 (only to swallow a pill is insufficiently)
 We have got yet no new drug since 2004
 New trials : biologic therapy - monoclonal antibody against
amyloid
TREATMENT of DEPRESSION + ANXIETY:
SSRI, SNRI BZD
 cave tricyklics (AMT, Prothiaden ) –
anticholinergic side effect
 TREATMENT PSYCHOTIC SYMPTOMS:
 Neuroleptics
 HISTOLOGY: SYNUKLEINOPATHIES
20% of dementia! underdiagnosticed
HISTOPATOLOGY: Lewy bodies (brain stem, limbic
cortex, neocortexT-F
DAT scan – asym. hypofunction in striatum
PET MRI – sym cortical hypoperfusionT-P-O
Neuropsychologic exam: vizuokonstructive
dysfunction T-P-O
CLINICAL F.: fluctuate cognitive disturbance
visual hallucination
parkinsonism
TREATMENT:
- CAVE neuroleptic hypersenzitivity ( rapid
deterioration of parkinsonism)
- Only atypic neuroleptic
- Acetylcholinesterase inhibitors
- L-Dopa in early stadium
 HISTOLOGY:TAUOPATHIES
(ubiquitinopaties)
 Neuropsychologic exam. : 1. SYMPTOM –
 BEHAVIOUR OR LANGUAGUE DISTURBANCE
 F +T lobe
 YOUGER AGE of onset - (45 -65 )
 FAMILIAR OCCURENCE ( 30-50% )
 RAPIDLY PROGRESSION
 1. BEHAVIOURAL (Frontal) -55%
 2. LANGUAGE (PPA)
 And combination of both
 DOMINANT symptoms:
 Early change of behaviour ( perseverative,
stereotyp)
 Early change of personality
 Early emotional changes (apathy, verbal or
physical impulsivity)
 LATE somatic signs :
 Parkinsonism, MND (10-15 % )
 NEUROIMAGING ( MRI, PET MRI) :
- sym atrophy F + frontT lobe
 TREATMENT :
 Deficit of serotonin and dopamin transmiter
system
 - SSRI (Triticco)
 - atypic neuroleptic
 ( cognitiv drugs rather no)

 Subtypes: non- fluent aphasia
 semantic
 logopenic
DOMINANT signs: APHASIA
 NEUROIMAGING: asym atrophy T lobe
(dominant)
 TREATMENT : Logopedics
 - 2o% of dementia
 - after stroke 5x higher risk of onset
 - men more disabled than women
 - diagnosis is problematic, differentiation of
AD often only histologic, oft mixed dementia
( AD +VD)
 Brain MRI (CT)
 Neuropsychologic exam.: more than 1 lobe is
 impared
 SONO cerebral vessels
 CSF: biomarkers negativ
 1. D. due to mikroangiopathy ( 90% because of HT)
 - Binswanger´s disease (subcortical leukoencefalopathy)
2. D. due to strategic lokalizated infarct (F,T )
3. Multiinfarct D. (multiply small and large infarcts)
4. D. due to difusse hypoxic-ischemic encefalopathy (KPR)
( 5.) D. familial : AMYLOID angiopathy ( frequent stroke)
CADASIL (AD, mutation on 19. chromozom)
- young age, migraine, skin biopsy

 Primary and secondary PREVENTION of
cerebrovascular disease
 Acetylcholinesterase inhibitors
 Memantin
 DO NOT Prescribe : nootropics, vasodilatans
 Very often!
 - DominantAD + vascular changes
 - DominantVD + alzheimer changes

 1. Following BASIC NEUROLOGIC DG:
 Normal pressure hydrocephalus
 Brain tumors
 Kraniocerebral injury – chronic SDHematoma
 Epilepsy
 Neuroinfection - JCD, neurosyphilis, AIDS
 SM late stadium
 Huntington´s disease
 Wilson´s disease
 Prion disease
 Incidence 1-2 per million
 100% mortality
 Incubation time more than 10 years
 The most infectious tissues: BRAIN!
 - cerebral dura, cornea, blood?
 RISK: -Transplant from deseased
 (from 2007 mandatory testing cornea donor)
 - NCH operation ( contaminated instrument)
 Disinfectant, UV radiation – DO NOT DESTROY
 Rapidly progressive dementia
 Cerebelar or visual signs (ataxia)
 Extrapyramidal signs ( myoclonus)
 Pyramidal signs
 Akinetic mutism
 AUTOPSY MANDATORY
 EEG - periodic sharp wave complexes
 CSF: 14-3-3 protein detection
 Brain MRI: high signal abnormalities in
caudate nucleus + putamen
 NO TREATMENT
 1. SPORADIC 85%
 50-70 years
 Duration 6 months
 2. GENETIC (mutation) 10-15%

 3. NEW variant (infectious) 2-3%
 19-39 years
 Duration 1-1,5 years
 Due to consumption of infectious animal (BSP)
 KURU ( kanibalism, Papua N. Guinea)
 FFI Fatal familiar insomnia
 (+ dementia)
 Gerstman-Straussler-Scheiner d.
 (dementia)
 2. Following BASIC INTERNAL DG:
 Hepatal encephalopathy
 Renal (uremic) encefalopathy
 Endocrinopathy (hypothyreodism)
 Deficiency B12,B1,B6,folat acid
 Alcohol abuse