Vascular Dementia
Frontotemporal
Dementia
Other
Neurodegenerative
Disorders
CJD
Subcortical Dementia
Parkinsons Disease
DLB
Huntington Disease
HIV /AIDS complex
MND
Shanker Waghray
Consultant
Old Age Psychiatry
Vascular dementia
 No definite definition
 A preferred term now is Vascular Cognitive impairment
 Second most common single cause of Dementia.
 Prevalence rates vary from 9-14% depending on criteria
used .
Aetiology
Large artery disease
Cardiac embolic events
Small vessel disease,
Haemorrhages
Specific arteriopathies
Risk factors
Vascular
HTN, MI, Diabetes, Lipid abnormalities ,smoking
Demographics
Increased Age , Low education
Genetic
family history
Stroke related factors .
Type of CVD .
Site and size of infarcts
C/F of VaD
 Insidious onset , often stepwise deterioration,
maybe relatively abrupt.
Neurological findings indicating focal lesions ,bulbar
signs, Gait disorders,
Psychomotor slowing ,abnormal executive functioning .
Depression , anxiety ,emotional labiality
Relatively preserved personality with insight in mild and
moderate cases
Investigation and Treatment
 CT scan ----Focal infarcts , WML
 SPECT ---Reduction of regional blood flow and
decreased white matter flow
 No specific lab test
 ACHEI have been used in trials but not licensed
 Non Cognitive features similar to Alzheimer's and
treatment is same
Fronto temporal Dementia .
 Insidious course
 Onset between 35-70 years
 Personality and behaviour changes occur early
rather than cognitive changes.
 Aetiology Unclear.
Frontotemporal dementia
 Lund Manchester criteria
Behaviour disorder Insidious onset, slow progression,
early loss of insight, loss of social and personal awareness
,mental rigidity ,disinhibition ,lack of judgement, impulsivity
, stereotyped repetitive behaviour, Impulsivity.
Affective symptoms Depression ,Hypochondriasis,
emotional bluntness, lack of empathy,
Speech disorder reduction of speech ,stereotypy ,echolalia .
Receptive speech preserved, late mutism.
Physical signs –Early incontinence ,Rigidity, Tremor, Low
and labile blood pressure.
 CT and MRI show frontal and /or temporal cortical
atrophy and deep white matter lesions .Functional
Imaging is helpful
 Neuropsychological tests show slow verbal
production and relatively intact reasoning and
memory while intellectual and motor speed is
reduced .
 Memory disturbances are found at an early stage but
LTM is less affected compared to Alzheimers.
Creutzfeld-Jacob Disease
 Rare and rapidly progressive
 Onset between 45-75 years.
 Affects 0.3-5 cases/million
 Subtypes
Sporadic
Genetic
Iatrogenic
New Variant CJD
CJD
 Sporadic
Rapidly progressive Dementia
Myoclonus
Ataxia
Visual Disturbances
Multifocal CNS failure
Death due to Bronchopneumonia
Mean duration is 8 months
.Only 4% survive upto 2 years
CJD
 Genetic
Autosomal Dominant
Occurs a decade earlier than sporadic
Features are much the same as sporadic
CJD
 Iatrogenic
Fortunately rare Due to inadvertent inoculation with
human prions during medical procedures
First described in a patient receiving corneal graft
Contaminated instruments are other causes
Rapidly progressive dementia
 New Variant
First described in 1996
Age is lower(29)
Causal link to BSE Risk to humans minimal.
Depressed ,withdrawn, fleeting delusions ,behaviour disturbances,
Cognitive impairment ,involuntary movements
,incontinence,aggression,auditory and visual hallucinations .
increased dependency, mutism .
progresses rapidly
Investigations
EEG –2HZ generalised bi/triphasic periodic
complexes .Serial EEGs may be required
 MRI shows atrophy of brain and Increased signal in
basal ganglion
Treatment
 No treatment can halt the progress of CJD
 Myoclonus can be treated with Na Valproate
 Mg, Amantidine ,Interferon have been tried
 Patients can be treated in an open ward
 Barrier nursing is not required
 All samples can be treated as normal
 Mean duration is 8months
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Subcortical Dementias
Cortical
Subcortical
 Aphasia early
 No Aphasia
 Recall and Recognition
 Recall impaired,
Impaired
 Calculation Involved
early
Euthymic mood
 Motor speed normal
 Personality unconcerned
Recognition better
 Calculation preserved until
late.
Depressed mood
 Motor speed slowed
 Apathetic personality
J.Cummings
Pure Subcortical Syndrome
 Slowed Information Processing
 Impaired Executive Function
 Apathy
 Impaired recall with relatively persevered
recognition (retrieval not encoding deficit)
 Gait Disorder
Parkinson's Disease
 James Parkinson
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1812
Commonest degenerative disease after Dementias.
1% Lifetime risk .
Common between 50-70yrs of age rare <40. No
upper age limit .
Due to loss of pigmented cells in SN widespread
distribution so autonomic dysfunction and
degeneration is seen
Amyloid Plaques and NFTs are seen
Clinical Features
 Insidious Onset
 Tremor---more in upper limb.<by action >by
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emotion
Bradykinesia /lead pipe rigidity
Gait –festinating .
Monotonus speech.
Mask like Facies.
Micrographia ---Writing tails off towards the end
of a sentence.
Dementia in PD
Common executive dysfunction precede Motor symptoms
eg: working memory, attention ,verbal fluency.
----Detected by tests of planning ,spatial working memory.
Reflects dopaminergic dysfunction in front striatal networks
Cognitive impairment is seen in 10%.
Progresses to Dementia in 60%--same as non PD .
Predictors of Dementia at initial diagnosis
Age /Motor impairment/Impaired semantic
fluency/Impaired pentagon copying .
Dementia
 Was not considered part of the original definition
 Wide variation of prevalence---Seen in 23----41%
risk of developing Dementia is 6 times more than in non
PD.
 Risk factors –Males ,Low education, Depression
,Hallucinations ,Older age ,family history of Dementia,
Motor impairment ,Impaired verbal fluency, impaired
pentagon copying .
 Rigidity and postural disorders are more related to
subsequent Dementia than tremors .
In Early PD
 Two types of Cognitive impairment.
a) Executive deficits (due to frontostriatal dysfunction )–
likley to fluctuate with disease course but not clearly
associated with dementia
b) Posterior cortical deficits Lewy body disposition or AD
type pathology
 Maybe – there is multiple Dementia syndromes in PD
PDD
 Cognitive deficits in late PDD are similar to LBD
Marked visuospatial dysfunction ,Fluctuating cognition
,Executive dysfunction ,working and episodic memory
,Language relatively well persevered particularly object
naming .
Visual hallucinations
Sleep disturbances
Diagnosis
 MMSE not entirely diagnostic
 CAMCOG and CAMDEX are more sensitive tests
 In later stages cortical features are seen
 Diagnosis of PDD is made in patients with PD who
develop dementia >1year later
 If <1year it is DLB
Treatment
 Rivastigmine and to some extent Galantamine have
been found useful
 For non cognitive symptoms no one drug is available
Symptomatic treatment
Dementia of Lewy body(DLB)
 Now the preferred term
 Spectrum of disease
Three broad patterns
a) Nigrostriatal involvement -----motor symptoms of
Parkinsons
b) Cortical involvement producing cognitive decline
c) Sympathetic nervous system involvement producing
autonomic failure .
Accounts for just under 20% of all Dementias .
M:F ratio is 1.5:1
Dementia –Lewy Body
Consensus Diagnostic criteria
 Progressive cognitive decline and
two of the following core features
a) Fluctuating cognition with variations in attention and
alertness
b) Visual hallucinations –well formed
c) spontaneous motor features of Parkinson's
Supportive Features
falls, syncope ,sensitivity to neuroleptics, systematized
delusions,
Hallucinations in other modalities.
Imaging
 WMH and periventricular lesions are seen
 SPECT shows Dopamine transporter loss in caudate
and Putamen and is a marker of nigrostriatal
degeneration
Treatment
 For Cognitive ----ACHEI all three have ben tried
with good results
 For non cognitive need to be careful due to
sensitivity –Atypicals have been used .
Huntington's Disease
 First described in 1872
 Prevalence is 5.5/100000
 Occurs world wide
 Males>Females
Genetics
Single autosomal gene with high penetrance
---on short arm of chromosome 4
Family history not always forthcoming
New mutations can occur but rare.
HD
 Occurs in 4th to 5th decades so prevention is difficult
 Prenatal testing is now available by molecular
genetic testing .
 Reduced level of GABA in Basal Ganglia and
substantia Nigra
 Cholinergic neurons are severely deficient in the
Striatum
Motor symptoms
 Chorea is the predominate feature
 Rapid non repetitive contractions involving orofacial
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,truncal areas and limbs and worsens with anxiety.
Cannot be controlled voluntarily but is early stages
patients integrate this movements with purposeful
actions
Gait abnormalities and dysarthria occurs later.
Difficulty with walking, turning ,
Falls
Dementia in HD
 Dementia is insidious and no impairment is seen in
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asymptomatic carriers.
Psychomotor slowing
Depression
Executive functioning ,language ,perceptual and spatial
skills are all affected
Verbal recognition memory ,word recognition and object
naming remain intact so communication is possible initially
Language may become impaired in later stages.
 Memory deficits are common ,
 Problem is with acquiring and retrieving memories
rather than retention.
 Appear early –can predate the motor symptoms
 Basal ganglia and fronto striatal connections are
involved in memory deficits.
Imaging and course
 CAT scan shows caudate atrophy.
 MRI shows reduced volumes of Thalamus and medain
temporal structures.
 SPECT shows reduced blood flow in basal ganglia
 Course is longer than other Dementias –15-30 years is
average
 No drug treatment ---Symptomatic treatment
C/F
 First reported in 80s.Virus isolated in 1983
 Sero conversion occurs in 50-90% of cases 2-4 weeks after
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exposure.
Mild fever ,myalgia ,sore throat ,lymphadenopathy is seen
and subsides within 2 weeks.
Asymptomatic phase lasts for 2-10years during which the
person remains infective
Constitutional symptoms eg fever ,night sweats ,weight loss
is seen.
Opportunistic infections take over.
Nervous system is an early target with viral ,fungal and
parasitic infections .
HIV/AIDS Dementia complex
 Term coined in 1986 by Navia et al
 Cognitive and behavioural changes with motor deficits are
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common
Lethargy and social withdrawal can be mistaken for
depression
Dementia is seen in 60% and occurs early with minimum
systemic manifestations
Onset can be insidious or abrupt poor concentration and
memory are early symptoms and can be mistaken for
systemic illness or depression
STM is poor but LTM is good and patients are good at
confabulating and so appear normal
 Asymptomatic HIV positive patients performed
poorly on tests of abstract thinking ,learning
,memory and speed of information processing.
 Severe global impairment is seen in half of the
cases within 2 months .
 Insight is preserved till late .
 Final stage is marked by severe global dementia.
Imaging and Treatment
MRI shows areas of increased T2 signals in white
matter
 PET shows subcortical hypo metabolism with
progression to cortical areas .
 SPECT shows perfusion deficits
 Treatment
Antiretroviral
Motor Neurone Disease
 Disorder of unknown aetiology .
 Males>females
 50-70 years
 5-6%have an affected relative. Occasional families show
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autosomal inheritance
Gene is localised to chromosome 9 also 17and 3
Insidious onset with atrophy of small muscles
No sensory change
Patients survive 2-3 years
Dementia
 Was considered rare.
 Testing may reveal deficits in memory
 Dementia is of frontal lobe type and begins 6-12 months
before wasting begins
 Personality changes with disordered conduct ,stereotyped
behaviour ,reduced verbal output .
 Progressive language difficulties with stereotyped phrase,
echolalia and finally mutism
Imaging
 Atrophy is seen in neocortices ,amygdala and basal ganglia.
 PET scan shows reduced blood flow in frontal areas .
 No cholinergic deficit
 NO SPECIFIC treatment -----symptomatic
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