Disorders of Higher Cortical Visual

Function
D Lelli, MD
Feb 6, 2015
 Cortical Organization
To be efficient, sensory processing in the brain is done in stages:
“Primary Cortex”
 Closest cortex to the peripheral sensory pathways
 Primary sensory cortex – FIRST step in processing after thalamus
“Association Cortex” – the next steps in processing sensory
information:
 Unimodal association cortex - secondary processing of a single sensory modality
 Multimodal association cortex:
 integrates information from multiple modalities,
 many connections with hippocampus (memory)
Processing of Visual Information
Processing of Visual Information
Higher order visual deficits
If you lesion any particular processing area – you get deficits
related to that function
e.g.: lesion V4 -> color anomia

Disconnection syndromes:
Dysfunction from disconnecting visual cortex from higher
processing areas (ie: cut the wires)

Simultaneous Occurrence:
These areas are close to one another – so often multiple deficits
will be present in one patient
Clinical Presentation
Symptoms:
Often vague – “blurred vision”, “trouble seeing”
Often have seen many specialists with normal examinations
Occasionally brought in by family (unaware of deficits)

Examination (easy at the bedside):

VA and ophthalmic exam usually normal
Careful testing of fields, especially for inattention
Color vision
Reading / writing / naming
Magazine (face recognition, complex scenes)
Quick Word on Etiologies

Common pathologies are stroke, tumor, encephalitis

Etiology mostly separated by temporal course (ie:

sudden -> stroke, subacute -> tumor, chronic ->
degenerative)

Some syndromes require bilateral lesions, adjust

differential accordingly (i.e.: stroke -> watershed
infarcts, bilateral emboli, vasculitis, etc.)
 Disorders of V1,V2,V3
Typically difficult to lesion separately
Homonymous visual field defects

Dominant occipital lobe lesion when

combined with a splenium lesion gives rise to
a specific syndrome

ALEXIA without AGRAPHIA

Clasically a disconnection syndrome
Intact R occipital lobe can’t communicate with
left sided language areas
Writing intact -> but can’t read what they
wrote
However, lesions also likely affect adjacent
visual word form area – so possible to see pure
alexia without a splenium lesion
Must differentiate from hemianopic reading
Ventral Pathway Lesions
Achromatopsia (V4, lingual gyri):
See world in grayscale, in less severe lesions poor distinguishing of colors
Symptoms -> gray vision, world looks dirty, tinted vision (less severe lesion)
Unilateral lesions can give hemi-achromatopsia (clue – reading only one side
of the color plates)
 Better test – move colored object from affected to intact hemifield
Bilateral lesions -> achromatopsia or dyschromatopsia
 Best test – Farnsworth 100 or D15 (color sorting)
Often associated deficits: superior homonymous field defects, prosopagnosia,
topographagnosia
 Ventral Pathway Lesions
Color anomia
Intact color perception, but can’t name the color
Most often with left occipital lesions and a corresponding
right homonymous hemianopsia, +/- alexia without agraphia
Ventral Pathway Lesions
General visual agnosia (left > bilateral fusiform, lingual, and
parahippocampal gyri):
Difficulty recognizing objects by sight, intact via touch
Worse for 2D (drawings) than for 3D objects
Must have enough intact afferent visual function
Apperceptive visual agnosia – can’t form the visual representations
in the brain (can’t copy objects)
 Integrative agnosia – can copy simple shapes, but can’t do more complex ones
 Transformation agnosia – can’t recognize object from unusual viewpoints
Associative visual agnosia can form the visual representation, but
can’t match it to memory of objects (can copy objects but not name
them)
Ventral Pathway Lesions
Prosopagnosia (right or bilateral mesial occipitotemporal
lesions – fusiform gyri; also seen in anterior temporal lobe):
Inability to recognize familiarity to faces (not just naming)
Patients usually use voice, gait, other cues to recognize people
Bedside test:
 Confirm knowledge of celebrities, then show pictures
Ventral Pathway Lesions
Topographagnosia:
Getting lost in familiar surroundings
Often associated (and possibly caused) by landmark
agnosia (inability to recognize landmarks) – due to lesion
in right medial occipitotemporal areas
Dorsal Pathway Lesions
Akinetopsia (lateral occipito-temporal lesions, V5)
VERY rare…, unilateral lesions only detectable by
computerized testing, localized to contralateral hemifield
Bilateral lesions - loss of motion perception (only 2
cases):
 Water pouring from a pitcher seems frozen
 Objects seem to jump instead of move
Dorsal Pathway Lesions
Balint Syndrome (bilateral occipito-parietal):
Classically the combination of optic ataxia, oculomotor
apraxia, and simultanagnosia
However, often the above deficits occur independently,
so no need to have the entire syndrome
Dorsal Pathway Lesions
Simultanagnosia (medial occipitoparietal junction):
Poor spatial attention, lack of awareness of more than
one object at a time
Marked functional limitations (bump into objects, can’t
drive, difficult to read, hard to search for objects)
A problem of shifting attention from local to global
structures
Dorsal Pathway Lesions
Optic Ataxia (inferior parietal lobule):
Inaccurate reaching to targets under visual guidance
Due to poor representation of position of objects in space
Unilateral lesions – may impair reaching with
contralateral arm or only in the contralateral hemifield
Dorsal Pathway Lesions
Acquired Ocular Motor Apraxia (bilateral parietal eye field or bilateral
frontal eye fields):
Defect in initiation and guidance of saccades to a visual target
 Blinking can often help generate the saccade
 Often multiple searching saccades (inaccurate representation of object’s location in space)
Reflexive saccades often spared (suddenly appearing object)
Unilateral lesions cause contralateral saccadic deficits (eye movement
recordings)
Often pursuit affected as well (pursuit centres adjacent to PEF), reduced pursuit
to targets moving ipsilaterally and excessive pursuit to targets contralaterally
Dorsal Pathway Lesions
Astereopsis (bilateral occipitoparietal lesions)
Can be tested with routine stereopsis tests
Wrap Up
Visual processing is done in a hierarchichal manner, as
is most other sensory processing in the brain
Lesions of primary cortex cause field defects
In the ventral pathway – lesions in intermediate areas
damage color perception and higher level lesions cause
impaired object recognition
In the dorsal pathway, lesions in intermediate areas
damage motion perception and higher level lesions
cause impaired spatial awareness, localization and
guidance
Questions?