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Prezentace aplikace PowerPoint


(Cognitive and Behavioral Neurology) - is dealing with disorders of higher nervous functions ... field of study neurology, psychiatry, neuropsychology ... – PowerPoint PPT presentation

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Title: Prezentace aplikace PowerPoint

Behavioral Neurology
Behavioral Neurology(Cognitive and Behavioral
  • - is dealing with disorders of higher nervous
  • resulting from structural brain damage
    (directed attention,
  • mood, gnostic functions, cognitive functions,
    memory, )
  • investigates a relationship between brain and
  • between brain and mind
  • relatively young, interdisciplinary field of
    study neurology,
  • psychiatry, neuropsychology
  • in the past BN dealt mainly with dementias and
  • currently BN is rather focused on frontal and
    temporal lobe
  • syndromes, consciousness (awareness) and
  • agnosias, and many other aspects of HNF.

Phineas Gage
  • 1848, New England
  • PG, 25 years, efficient and faithful foreman on
    the railroad construction through Vermont.
  • When preparing the rock shooting PG mistakenly
    ram down powder and detonator with iron rod
    (needful sand was missing).
  • During the explosion the iron rod (length 1 m,
    diameter 4 cm, 6 kg) threw open Gages left
    cheek, bashed in the base skull, passed over the
    ventral part of the brain and catapulted through
    crown of the head.
  • Personality change he started to be volatile,
    impolite, time to time he was extremely
    foul-mouthed and stubborn. For incredibility he
    lost a good job, rotated with many work places
    (incl. career in circus), he became an alcoholic
    and desperate. The neurological status was
    otherwise normal.
  • He died in 38 years because of status

H. Damasio, 1992
Patient H.M.
  • Intractable temporal lobe epilepsy from the age
    of 16 yrs
  • 1953 W. Scoville bilateral resection of
    mesiotemporal regions.
  • Postoperatively persisted serious anterograde
    amnesia (impairment of storage)

Examination of mental functions
  • It should be a requisite part of standard
    neurologic examination at least Mini Mental
    State Examination should be performed in
    neurologic pts.
  • It has to be systematic and hierarchic
  • (level of consciousness ? directed attention ?
    cognition, mood, speech)
  • Golden neurologic rule to localize a lesion
    should be applied for mental functions too
    (neuronal networks).
  • Extremely important is thorough history taking
    (changes in pts behavior) and focusing on the
    pts behavior during the examination (evaluation
    of his/her appearance, cooperation, attention,
    memory, mental flexibility, social adaptability,
    ability of nonverbal communication, depressive
    symptomatology, etc.).

Bedside tests of attention
  • Luria (fist-palm-side) test
  • Luria sketch (visual completion test)
    (alternating square and pointed figs.)
  • Continuous performance test
  • After registering target digit in presented
    digit chain a subject has to knock on a table
  • 4-9-1-7-5-4-0-7-9-2-4-3-7-5-0-2
  • Digit span test (3-7) subject has to learn and
    repeat long digit chains, also test on short-term

Large-scale neural network for directed attention
(Mesulam MM)
Neglect syndrome a failure to report,
respond, or orient to contralateral novel stimuli
that is caused by damage of large-scale neural
network for directed attention and not by an
elemental sensorimotor deficit. It is a form of
selective unawareness. Pts with neglect
syndrome often appears to be unaware of
contralateral stimuli, they ignore these items,
and do not react to them.Within neglect there
can be hemiakinesia (motor neglect movement
deficiency pseudohemiparesis), anosognosia
and/or anosodiaforia (absence of concomitant
emotions for serious functional deficit).
Bedside memory testing is limited

  • episodic m.
  • (autobiographic data)
  • long-term m. (gt 1 min)
  • Explicite memory semantic m.
  • (declarative) (encyclopedic

  • (visual x verbal,
    recall x recognition)
  • short-term
    (working) m. (30-40 s) (digit span)
  • procedural m. (completing word
    fragment, m. for movements)
  • Implicit memory
  • demonstrated by completion priming
  • of tasks that do not require
  • conscious processing
  • the ability to acquire a motor skills or
    cognitive routines by experience

(mesiotemporal regions hipp,entorh, perirh, GP)
(more extensive reg. MTLT,P,O)
(DLPFC associative visual and auditory areas)
(subcortical circuits BG, cerebellum ctx
visual, motor,..)
H O S P - - - -
Disorders of symbolic functions
  • Dysphasia - disorders of speech (Motor or
    expressive /Broca?s/ dysphasia Sensory or
    receptive /Wernicke?s/ dysphasia Global
  • Aprosodia impairment of affective component of
    speech (speech melody, intonation, voice timbre,
    use of pauses, etc.) recove nedominantní
  • Dressing apraxia - difficulties in dressing, e.g.
    Getting arm into pyjamas,
  • Constructional apraxia innability to copy
    geometrical pattern
  • Alexia - disturbance of reading (angular or
    lingual g. within dominant hemisphere).
  • Agraphia - disturbance of writing (GFM or PO
    junction of the dominant hemisphere).
  • Acalculia - disturbance of calculation (dominant
    hemisphere, also within the Gerstmann?s syndrome
    angular g.).

Gnosis greek cognition
  • Gnostic function an ability to know
    (recognize) individual objects
  • __________________________________________________
  • AGNOSIA (without recognition) def. impaired
    recognition of an object which is sensorially
    presented while at the same time the impairment
    cannot be reduced to sensory defects (intact
    primary sensory cortex), mental deterioration,
    disorders of consciousness and attention, or to a
    non-familiarity with the object.
  • The term agnosia is from S. Freud (1891)
  • Finkelnburg 1870 asymbolia
  • Jackson 1876 imperception
  • Munk 1881 seelenblindheit(mind blindness) /X
    cortical blindness/
  • Affected individuals behave as seeing () the
    object for the first time in their life.

Beware of erroneous diagnosis of agnosias! -
darkness or very rapid object presentation -
unfamiliar objects (e.g. tuning fork) -
insufficient instructions - overlooking another
disease (polyneuropathy, cataract,
otosclerosis,) - aphasic phenomena - apraxic
phenomena Agnosias are related to the lesions
within associative cortices and their very
surrounding but also with disconnections
(impairment of the corpus callosum or long fibers
within the white matter). Unfortunately in the
practice agnosias are often associated with other
neurologic deficits (aphasia, apraxia, behavioral
disorder)! Resulting clinical manifestation is
therefore highly individual.
Visual agnosiaSpecific impairment of
recognition of visually presented objects pt is
well seeing but he/she is not able to identify
these items. Clinical classification according
to the character of impairmentApperceptive
visual agnosia patient is neither able to
recognize objects visually nor their form, and is
not able to describe it correctly.Associative
visual agnosia patient is not able to recognize
objects but he/she can describe the form or even
is able to draw the object correctly.According
to the type of affected stimuli - Agnosia for
objects - Agnosia for colors - Akinetopsia -
Prosopagnosia - Simultanagnosia - Pure alexia
- ..
- Agnosia for objects (by definition pts are not
able to recognize objects when they are solely
presented visually. Usually visual object agnosia
was considered as the classical example of
agnosias, but frankly it is very rare type of
visual agnosia. The most frequently it arises
from bilateral (rarely just left-sided) damage of
lateral parts of occipital lobes (strokes). -
Agnosia for colors (coloragnosia) the loss of
ability to recognize colors as an acquired
disorder. Pt is unable to recognize colors, but
he/she understands colors and is able to
correctly name e.g. the color of banana, orange,
etc. (lesion within left occipital lobe
prestriatal cortex ventral visual stream). It
needs to be differentiated from from inability to
name colors!- Hemiagnosia for colors the
inability to recognize colors confined to one
half of the visual field maybe attention defect
(similarly to unilateral spatial agnosia)? -
Akinetopsia selective impairment of visual
perception of motion (motion blindness), whilst
there is a normal recognition of colors or object
forms. Lesion within extrastriatal cortex (dorsal
stream, lateral TPO region).- Prosopagnosia
(not as rare as visual object agnosia) loss of
ability to recognize familiar faces. It can be
highly specific (for human faces, for own face,
for animal faces). Most often there is a lesion
within right-sided occipitotemporal or
parietooccipital cortical regions (ventral
Auditory agnosia Very rare, usually
is resulting from the lesion within the
left-sided lateral temporal neocortex.
Auditory agnosia for non-linguistic sounds
(psychic deafness) (the inability to recognize
concrete sounds as animal noises, the sound of a
stream of water, of a sounding bell, of a ticking
of a clock, etc.) - Phonagnosia (auditory
analog of prosopagnosia) impairment of voice
recognition and discrimination the inability to
recognize familiar voice (lesion in lower and
lateral parts of the right parietal lobe) and to
discriminate between unfamiliar voices
(impairment of the temporal lobes independently
of the side). De facto 2 anatomical systems 2
distinct clinical syndromes. - Sensory amusia
the inability to recognize music, melody or
rhythms (lesion within the non-dominant
Tactile agnosia
astereognosia A condition in which objects
tactually are not recognized. The sensation had
to be intact. Primary astereognosia patient
is neither able to tactually recognize objects
nor their forms or materials from which they are
made.Secondary astereognosia Patient does not
recognize tactually objects but he/she recognizes
well the form, size or material.The most
commonly lesion can be found within the parietal
lobe behind the postcentral gyrus (incl.
supramarginal gyrus ). The disorder can be
observed in lesions within both dominant and
non-dominant hemispheres.
Multisensorial agnosias
very rare
Disorders of somatognosia
- impairments of the recognition of the body
scheme.- Autotopagnosia patient does not
recognize parts of his/her own body. Disorder is
not related to the dominant/non-dominant
hemisphere, it results from impairment of
contralateral parietal lobe.- Hemisomatagnosia-
Finger agnosia difficulty in distinguishing
fingers on hand (this condition can be seen in
Gerstmann?s syndrome).- Mirror asomatognosia
mirror-induced disorders of the body image.
Right-sided lesions. - Agnosia of pain pain
asymbolia (Schilder-Stengel syndrome) emotional
reactions to the pain are absent in the patient.
Disorder is caused by the dysfunction of parietal
Anosognosia inability tp recognise and to
understand own physical disability (especially
motor deficit - hemiplegia) that is actually
denying by the patient. Typically anosognosia can
be seen in pts with left-sided hemiplegia. In
fact the awareness of own deficit is lacking
disorder of focused attention! Antons
syndromesimultaneous occurrence of cortical
blindness and anosognosia (pt denies truthfull
loss of vision) Neglect syndrome unilateral
spatial agnosia attentional hemideficit
selective unawareness of contralateral stimuli.
Practically pts with neglect syndrome ignore
contralateral stimuli and do not react to
them.Within neglect also there can be
hemiakinesia (movement deficiency) and/or
anosodiaforia (absence of concomitant emotions
for serious functional deficit). damage of
large-scale cortico-subcortical neurocognitive
network for directed attention (right-sided
inferior parietal lobule, right-sided prefrontal
and orbitofrontal cortex, right-sided thalamus
and basal ganglia)
(No Transcript)
Unconscious perception in neglect syndromeThere
is increasing evidence that some pts with neglect
may covertly perceive the contralateral stimuli
and that may at least partially react to these
stimuli (Volpe et al. 1979 Berti et al. 1992
Wallace 1994) - Covert recognition of faces in
prosopagnosia In some cases of prosopagnosia,
there has been a dramatic dissociation between
the loss of face recognition ability on the one
hand, and the apparently preserved ability to
recognize faces, when that is assessed indirectly
skin resistance (Bauer 1984 Bruyer 1992
DeHaan et al. 1987, 1992), ERP (Renault et al.
1989)- Implicit shape perception in
apperceptive visual agnosia- Implicit object
identification in associative visual agnosia
(Taylor and Warrington 1971 Goodale et al. 1991
Jankowiak et al. 1992 Farah and Feinberg 1997)
- Blindsight the best known syndrome
preserved ability of some patients to respond to
certain aspects of visual stimuli in the areas of
their visual fields that are blind on
conventional clinical testing (lesions of prim.
visual cortex). (Riddoch 1917 Weiskrantz et al.
1974, 1977, 1996, 1998 Perenin 1987 Ptito et
al. 1991 Stoerig and Cowey 1992 Tomaiuolo et
al. 1997 Sahraie et al. 1997 Zeki and ffytche
1998) - Inverse Antons syndrome - pt. with
spared central island of vision denies visual
sensation and he/she is behaving as the blind
selective impairment of awareness for visual
stimuli in complete visual field (covert vision).
(Walsh and Hoyt 1969 Hartmann et al. 1991
Brázdil et al. 2000)
Dissociations between perception and
consciousness after brain damage (conscious
perception and unconscious /implicit, covert/
Determination of hemispheric dominance
  • Interview about writing, eating with spoon,
    throwing a ball, kicking, step tapping domin.
    hand 50/min, nondomin. hand 45/min.
  • Left hemisphere is dominant in 95 right-handers
    and 60 left-handers!
  • Left hemisphere dominant for speech and motor
    functions, reading, writing, counting,
    recognition of colors, verbal memory, important
    for linguistic thinking, ...
  • Right hemisphere dominant for attentional
    functions, prosopognosia, prosodia (affective
    component of speech), nonverbal communication
    (ability to read from face), visuo-spatial
    perception, visual and topographical memory,
    recognition of music,

Drug-induced mental disorders
  • Quite frequent, especially in elderly
    patients (mostly they are caused by
    pharmacological polytherapy)
  • Depression, delirium, psychosis, agitation,
  • Digitalis
  • Corticosteroids
  • Indomethacine
  • Phenacetine
  • Phenylbutazone
  • Cimetidine
  • Benzodiazepines
  • Captopril
  • Propranolol
  • Niphedipin
  • PNC
  • Cephalosporines
  • Oral contraceptives
  • Vincristine
  • Carbamazepine
  • Phenytoine
  • Primidone
  • Topiramate
  • Clobazam
  • Phenobarbital
  • Levodopa
  • Amantadine
  • Anticholinergics
  • Thyroxine
  • Interferone
  • ..

score gt 24 normal lt 24 suggests dementia