Demências, parkinsonismo e tauopatias

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Transcript Demências, parkinsonismo e tauopatias

Parkinsonism and other
movement disorders
PRM de Bittencourt
www.unineuro.com.br
The concept of Parkinson’s
disease
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1977: started classical
neurology training
1982: first started with
a large amount of
clinical work
1985: depression
definitely associated
with Parkinson’s,
imipramine replaced
other anticholinergics
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It was thought
Parkinson’s evolved
without dementia, with
depression, perhaps
dementia at the end
Initial surprise at the
great number of cases
“cured” of Parkinson’s
disease
The most frequent cause of parkinsonism in Curitiba in
the 80s: “labirintitis”
 1988: Chouza e MeloSouza, parkinsonism
due to cinarizine and
 Letter to CD Marsden in
flunarizine
1986
 100. Cunha CA,
 1988: Dr Marsden:
Bittencourt PRM,
diagnosis is really
Kohlscheen KL, Mercer
difficult
LM. Reversible
 Recommended a
parkinsonism induced
number of criteria
by cinarizine and
flunarizine. Revista
Médica do Paraná
50:13-16, 1993
Present vision (Litvan 2003)
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Pathologically,
patients with
parkinsonism and
dementia may be
classified as
tauopathies or
sinucleinopathies,
based on their
aggregates of
abnormal proteins
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There are no
biologic markers at
the moment that
allow the diagnosis
of the various
disease that start
with parkinsonism or
dementia, and their
clinical diagnosis
may be a challenge
Parkinsonism with dementia
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tauopathies (PSP, Pick disease)
synucleinopathies (Parkinson, dementia
with Lewy bodies
Drug-induced (combination of drugs,
anti-cholinergics, or dopaminergics)
Infeccious (Creutzfeldt-Jakob, HIV)
Vascular
Parkinsonism with dementia
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Toxic (Wilson, manganese)
Tumoral (primary, secondary, chronic
subdural hematomas)
Normal pressure hydrocephalus
Post-traumatic (dementia pugilistica)
Sleep apnoea
synucleinopathies
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Parkinson’s
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akynesia; postural disturbance with axial
involvemente, rigidity, response to L-PODA
Lewy body disease
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Demential more proeminent
More rapidly progressive
N response to l-DOPA
tauopatias
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Familial
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Frontotemporal lobe
dementia with
parkinsonism
associated with
chromosome- 17
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Frontal behaviour
(disinhibition,
isolation, disfunction
executive aphasia)
parkinsonism
Typical Parkinson’s patient
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60 year-old, male, non-smoker, brought
by family or refered by clinician due to
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Slowness
Lack of volition, apparent sadness
Motor difficulty with every day activities
Sleep disturbances
Typical Parkinson’s
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Consults other physicians because
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Labyrinth (dizzines, postural instability, gait
difficulty, apparent lack of balance)
Vertebral column: lumbar pain, difficulty
moving legs
Physical Examination
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Posture: parkinsonian
Gait: parkinsonian
Slowness of movemento: rigidity
Lack of movement: akinesia
Tremor
Asymetric signs
On physical exam
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Cardiovascular, respiratory, abdominal,
head, neck, limbs: normal
Movement + thought: slow
Diagnosis: therapeutic test
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Response to l-DOPA
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Immediate
Dose-dependent
3/3h
¼ de 250mg
Medical diagnosis
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Systemic investigation normal
Neuroimaging normal
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CT
MRI
Diagnosis functional
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Neuroimaging functional: normal
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SPECT
PET
EEG with mapping of alpha at low
normal
Functional diagnosis
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IQ + Memory normal
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WAIS
Weschler Memory Scale
Minimental
Natural history until 80s
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1-2 years before diagnosis
5 years good response to L-DOPA
5 years partial incapacity with multiple
drugs
2-3 years with terminal incapacity
Dysphagia + aspiration
Natural history after the 80s
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1-2 years before diagnosis
5+ years good response to post-DA
stimualtors : pramipexole
5+ years good response to small doses of LDOPA given at short intervals or SR +
multiple drugs
5 years partial incapacity with multiple drugs
2-3 years with terminal incapacity
Dysphagia + aspiration
História natural após ano 2000
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12 anos de diagnóstico, resposta a
estimuladores pós sinápticos : pramipexole,
pequenas doses de L-DOPA ou SR +
múltiplos medicamentos
5 anos de incapacidade parcial com múltiplos
medicamentos ou estimulador de gânglios da
base com retorno quase ao estado inicial, em
pacientes com menos de 70 anos
2-3 anos de incapacidade terminal
Disfagia + broncoaspiração
Multiple drugs
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Tricyclics, venlafaxine, bupropione
entacapone
quetiapine
Avoid anticholinergic effect
Avoid depressive effect
Environmental treatment
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Collection of cars versus mechanic
Ballroom dancing, snooker, tricot
Wedding invitations, model ships and
airpplanes
Physical exercise
Extremely healthy life
Repetitive routine with novel fine and
physical motor and mental acitvities
Essential tremor
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Familial ou episodic
Rapid, action, symetric, diffuse
Propranolol, alcohol, phenobarbitone
Caffeine, dopaminergic substances
Benign
Cigarrete
Dystonias
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Tardive dyskinesia
Psychogenic dyskinesia
Focal dystonia
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Facial hemispasm
Generalized dystonia
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Dystonic cerebral palsy
Choreas
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Sydenham
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pregnancy
Huntington
Drug induced
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Antipsychotic
Metochlopramide
Fluoxetine
L-DOPA
Chorea, dyskinesia, dystonia
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Botox
Anticholinergics
Mood stabilizers
DA blockers
Benzodiazepines