Balint’s Syndrome

 

•   Triad of:

o   Simultanagnosia

o   Ocular apraxia

o   Optic Ataxia

 

•   Individual elements:

o   Simultanagnosia

-   Inability to grasp entire meaning of a picture despite the capacity to recognise individual elements (Cant see the forest for the trees)

-   Deficit/narrowing of attention

o   Ocular apraxia

-   Unable to generate voluntary saccades despite normal involuntary, reflexive saccades

-   Disconnection of occipital lobe from frontal eye fields

-   Also referred to as “psychic gaze paralysis” or “spasm of fixation”

o   Optic ataxia (visuomotor ataxia)

-   A defect in reaching under visual guidance

-   Disconnection between occipital lobe and anterior frontal motor control centres

•   Other symptoms/signs:

o   Will not fixate on novel stimuli in visual field

o   Lack of blink to threat

o   Become visually fixated on single objects

 

•   Bilateral parietal lesions

•   Specific causes:

o   Hypotensive stroke

o   Dementia’s (Posterior cortical atrophy)

o   PRES

o   CJD

o   PML

o   Other: CNS vasculitis, adrenoleukodystrophy, perinatal hypoxic injury, RCVS, acute haemorrhagic leukoencephalitis

 

Gerstmann Syndrome

Clinical features

•   Alexia +/- agraphia

•   Acalculia

•   Left-right disorientation

•   Finger agnosia

Localisation:

•   Dominant Parietal lobe

o   If in isolation may be Inferior parietal lobule, particularly angular gyrus and surroundwhite matter

Examination:

•   Finger agnosia – ask patient to name or point to a finger (of their own or examiner)

•   Left right disorientation – ask patient to raise left/right hand

•   Combine above:

o   by asking patient to put left thumb or little finger on right ear.

o   Examiner crosses their arms and asks patient to touch a specific digit (with a specific hand) – e.g. touch my left ring finger with your right hand

Posterior Cortical Atrophy Syndrome

Consensus classification of posterior cortical atrophy. 2017

Clinical Features

•   Insidious onset

•   Gradual progression

•   Prominent early disturbance of visual ± other posterior cognitive functions

Cognitive Features

(At least three of the following must be present as early or presenting features):

•   Space perception deficit

•   Balint’s syndrome:

o   Simultanagnosia

o   Oculomotor apraxia

o   Optic ataxia

•   Object perception deficit

•   Constructional dyspraxia

•   Environmental agnosia

•   Dressing apraxia

•   Gerstmann syndrome:

o   Alexia

o   Agraphia

o   Acalculia

o   Left/right disorientation

o   Finger agnosia

•   Limb apraxia (not limb-kinetic)

•   Apperceptive prosopagnosia (

•   Homonymous visual field defect

All of the following must be evident:

•   Relatively spared anterograde memory function

•   Relatively spared speech and nonvisual language functions

•   Relatively spared executive functions

•   Relatively spared behaviour and personality

Neuroimaging:

•   Predominant occipito-parietal or occipito-temporal atrophy on magnetic resonance imaging/hypometabolism on positron emission tomography/hypoperfusion single-photon emission computed tomography

 

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Visual agnosia (inc. prosopagnosia)

•   Loss of ability to recognise despite normal visual and naming function

•   Apperceptive agnosia

o   Perceptual defect distorts the visual image so that the object is unrecognisable

o   May be able to see parts but not the whole (e.g. simultanagnosia)

o   Apperceptive prosopagnosia – able to recognise parts of face, but not put it all together

o   Lesions of bilateral parieto-occipital regions

 

•   Associative agnosia

o   Inability to associate object with past experience and memory

o   Object can be recognised using other sensory modalities

o   Associative prosopagnosia - Can describe face but unable to associate it with particular person

o   Lesions of bilateral occipitotemporal region