Disconnection Syndrome

Dr Gulab Soni
 White matter connection

3 types of white matter fibre:

1) Commissural fibre: interhemispheric

Horizontal
2) Association fibre: intrahemispheric
3) Projection fibre- Vertical
 Disconnection syndrome:
Introduction
• Defined as symptoms and sign, which are
arises due to white matter disconnection
• 1965 - Norman Geschwind, US neurologist
• Usually two types-
1) Interhemispheric disconnection syndrome:
due to involvement of commissural fibres
2) Intrahemispheric disconnection syndrome:
due to association fibres involvement
 Disconnection syndrome
Fibre type Tract involved Symptoms

Association Arcuate fasciculus Conduction aphasia,

ideomotor apraxia

Inferior longitudinal Visual object agnosia

fasciculus Pure alexia
Prosopagnosia (B/L)

Commissural Corpus callosum Left hand apraxia, left hand

agraphia, pure alexia etc

Projection Corticospinal tract Locked in syndrome,

hemiparesis
Apraxia
 Visual object agnosia
Inability to recognize object
by sight DORSAL STREAM-VISUALY
GUIDED ACTION
• Based on lesion:
a) Lateral occipital cortex
(cortical): apperceptive
visual agnosia
VENTRAL STREAM-
b) Associative fibre VISUAL RECOGNITION

VISUAL INFORMATION
connections
(transcortical): associative
visual agnosia-
disconnection syndrome
Visual object Agnosia
 Alexia
Acquired inability to read
1) Alexia without agraphia /pure alexia / pure
word blindness/visual verbal agnosia -
disconnection syndrome
2) Alexia with agraphia- lesion at angular gyrus
3) Third alexia/ alexic agraphia- seen with
brocas aphasia
Pure Alexia
Conduction aphasia
 Conduction aphasia
• The lesions of conduction aphasia usually
involve either the superior temporal or
inferior parietal region.
1) Conduction aphasia plus limb apraxia:
parietal lesions
2) Conduction aphasia without apraxia:
temporal lesions
 Callosal Disconnection Syndrome
Verbal Disconnection Motor Disconnection

Left visual anomia Crossed optic ataxia

Left hemialexia Left unilateral motor apraxia

Left auditory anomia Agraphia of the left hand

Left tactile anomia Right unilateral construnctional

apraxia
Right olfactory anomia Alien hand syndrome

Mainly due to-Posterior callosal lesion Mainly due to-Anterior callosal lesion
 A) Verbal disconnection disorders
• Verbal output is exclusively sub served by the
left hemisphere
• So in callosal lesion patient have difficulty in
communicating from right hemisphere
 1. Left visual anomia
• Image seen in right visual field (left
hemisphere) are normally named or
described, while those shown in the left hemi
field (right hemisphere) cannot.
• Lesion site: Posterior and dorsal portion of
splenium
 2. Left hemialexia
• Special case of anomia for words shown in the
left visual field, patient unable to report aloud
or write down those presented on the left side
• Lesion site: Ventral and anterior portion of
splenium
 3. Left auditory anomia
• Repetition of verbal material (syllables, words
and numbers) or naming of auditory stimuli
(sound and voices) are normal when presented to
each ear separately- due to the bilateral
projection of cortical auditory pathway
• Bilateral simultaneous stimulation of both ear,
results in deficit from left ear i.e right hemisphere
• Lesion site: Posterior inferior trunk/isthmus
 4. Left tactile anomia
• Patient can describe and name objects
palpated with the right hand (without
vision)but not with the left hand
• Also k/a pseudoastereognosis
• Lesion site: posterior trunk
 5. Right olfactory anomia
• Odors presented to right nostril (right
hemisphere) are not named, while those
presented to left nostril (left hemisphere)
are—olfactory inputs project ipsilaterally.
• No anosmia: patient winces in response to
unpleasant odours
 B) Motor disconnection disorders
• Difficulty in coordinating bimanual gesture
due to lack of sensory motor transfer, ex-
difficulty in tie shoelaces in absence visual
inspection
• Rapid alternating movement of both hand
also impaired, ex-hand clapping/tapping
• Lesion: anterior corpus callosum
 1. Right unilateral constunctional
apraxia
• Difficulty in drawing and constructive abilities
when using right hand
• Due to disconnection of the left motor cortex
from right hemisphere visuospatial skills
 2. Alien hand syndrome

• “Feeling that one limb is foreign or “has a will

of its own,” together with observable
involuntary motor activity
• Depending upon the site of lesion, 3 types of
alien hand syndrome described
 Alien hand syndrome
Frontal variant Callosal variant Sensory variant

Clinical features Reflexive grasping, Intermanual Levitation

groping and compulsive conflict
manipulation of tools
Lesion site Medial prefrontal cortex Anterior corpus Parietal cortex
callosum
Affected side of Dominant hand Nondominant Nondominant hand
hand hand
Associated finding Transcortical motor Sensory ataxia,
aphasia, Optic ataxia,
neglect
Cause ACA territory stroke CBGD
Callosal surgery in epilepsy Posterior cortical
Head injury atrophy (AD)
 Geschwind’s disconnection syndromes
Disconnections between Disconnections between Disconnections between Disconnection between
sensory areas and limbic sensory areas and Wernicke’s sensory areas and motor the hemispheres
cortex area cortex

Pain asymbolia (no Tactile aphasia/anomia/agnosia: Conduction aphasia/central Callosal disconnection

response to pain in the (the inability to name a held aphasia: disconnections of syndrome
presence of normal tactile object in the presence of Broca’s area from
discriminatory function) preserved speech and naming Wernicke’s area
in other sense modalities

Verbal learning impairment Pure word deafness(auditory Apraxia: disconnections of

examples- autism, verbal agnosia): inability to the hand motor
schizophrenia understand spoken words in cortex from posterior
the presence of preserved sensory area
hearing

Pure word blindness: (visual

verbal agnosia or alexia)
 Thank-u
References:
• Adams and victors-text book of neurology, 10th edition
• Bradleys text book of neurology, 6th edi
• Stroke Syndromes, 3ed . By Louis R. Caplan, Jan van Gijn
• Marco Catani and Dominic H. The rises and falls of
disconnection syndromes. Brain (2005), 128, 2224–2239
• Developmental Neuropsychology (Otfried Spreen, Anthony H.
Risser, Dorothy Edgell)
• The American Psychiatric Publishing Textbook of Geriatric
Neuropsychiatry (Norman L. Foster)
• Limb kinetic apraxia: localises lesion at premotor cortex
or adjacent centrum semiovale/corona radiata
• Ideomotor apraxia: not follow command, pantomime
and imitation of object, how they are used but when
object given to patient the demonstrate there use
• Ideational apraxia: deficit in the execution of a goal-
directed sequence of movements of a tool
• Alien hand phenomenon: severe form of sympathetic
apraxia, characterized by additional features of motor
disinhibition on the left hand
• Dressing and construction apraxia: bilateral (or
right-sided) dorsal parietal lobe lesions,
denotes disturbance in spatial orientation
• In callosal apraxia when lesion extends
towards medially it may be associated with
trancortical aphasia