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Dysphasia

What is dysphasia?

Dysphasia (or aphasia) is the impairment of language skills due to damage to the brain.
Dysphasia results in changes to some or all of the following areas of communication:
understanding, talking, reading and writing.

The main centers that control speech and language are on the left side of the brain and dysphasia
most often results from damage to these areas.

Dysphasia will differ from person to person depending on which parts and how much of the
brain is affected.

Conditions that can cause dysphasia include:

• Stroke; blockage of blood supply or bleeding in the brain
• Trauma to the brain; usually as a result of an accident, infection, or toxic substance
• Degenerative diseases; e.g. dementia
• Brain tumors.
Effects of dysphasia

A person with dysphasia may experience difficulty:

• Concentrating and remembering information
• Understanding what people are saying in conversation
• Understanding and using gestures, such as pointing, waving or facial expressions
• Understanding written information such as newspapers, books and signs
• Finding the word they want to use when talking or writing. People may get stuck on a word
• Mixing up the sounds in words or meanings of words
• Using correct words (some may be nonsense words)
• Recognizing specific sounds or words
• Controlling automatic language (e.g. swearing).
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TYPES OF APHASIA
Aphasia is the partial or total loss of the ability to communicate with an individual who
previously had normal ability. Aphasia can affect one’s capacity to speak, write, calculate, and in
some cases, to understand a written or verbal message.

Aphasia is not a mental illness, but rather a consequence of damage suffered to the parts of the
brain that control language.

A person with aphasia is neither confused nor amnesic, as they have full knowledge of his or her
difficulties. People with aphasia need to be received with an open mind, and need time to speak.

The different types of aphasia:

▪ Broca’s aphasia
▪ Conduction aphasia
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▪ Global aphasia
▪ Mixted aphasia
▪ Progressive aphasia
▪ Wernicke’s aphasia
Broca’s aphasia

This form of aphasia named after Paul Broca (1824-1880), French surgeon and anthropologist
known especially for his discovery of the language centre in the human brain, is recognized by a
reduction in expression, often accompanied by hemiplegia(paralysis of the one side of the body)
and motor speech problems: the individual speaks little, speaks slowly, and has trouble finding
their words. He/she may have similar difficulties when trying to write. These writing difficulties
are comparable to the difficulties in spoken language and cannot be simply attributed to any
weakness or paralysis of the dominant right hand. Comprehension is relatively well-preserved.
It is also called aphasia of speech or expressive aphasia.

Conduction aphasia

Conduction aphasia is manifested by hesitant speech caused by a difficulty in finding words and
especially by numerous paraphasias, which means the individual mixes the sounds in words.
Being aware of this, he/she makes successive attempts to correct it with trial and error.
Sometimes, paraphasias are so abundant that they become a sort of jargon in the person’s
language. Contrary to Wernicke’s aphasia which can sometimes resemble conduction aphasia,
there are usually no major disturbances of comprehension. Conduction aphasia may be the result
of a Wernicke’s aphasia that has evolved positively
Global aphasia

The individual with global aphasia has almost no communication abilities and major problems in
comprehension. This is the most severe form of aphasia, and is applied to patients who can
produce few recognizable words and understand little or no spoken language. Persons with
Global Aphasia can neither read nor write. Global aphasia may often be seen immediately after
the patient has suffered a stroke and it may rapidly improve if the damage has not been too
extensive. However, with greater brain damage, severe and lasting disability may result.
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Mixted aphasia

Mixed aphasia implies both a reduction in expression and significant comprehension

problems. Mixed non-fluent aphasia applies to persons who have sparse and effortful speech,
resembling severe Broca’s aphasia. However, unlike individuals with Broca’s aphasia, mixed
non-fluent aphasia patients remain limited in their comprehension of speech, similar to people
with Wernicke’s aphasia. Individuals with mixed non-fluent aphasia do not read or write beyond
an elementary level.
Progressive aphasia

The language problems in people with progressive aphasia arrive insidiously, the first symptom
usually being a gradual loss of vocabulary. The problems are generally lexical and phonological,
whereas in Alzheimer’s disease, the language difficulties are semantic.

The clinical portrait in terms of language is varied, and can be grouped into two broad
categories: fluent and non-fluent aphasia. Primary progressive aphasia (PPA) is a condition
where language capabilities become slowly and progressively worse, leading to a gradual loss of
the ability to:

● read

● write

● speak

● understand what other people are saying.

Deterioration can happen slowly, over a period of years. Other mental functions such as memory,
reasoning, insight and judgement are not usually affected. It's important to get an accurate
diagnosis for PPA. This is to rule out other degenerative brain disorders like Alzheimer's disease
where language and memory and reason are affected.

Non-fluent progressive aphasia is generally characterized by a growing lack of vocabulary,

phonemic and articulatory distortions, as well as agrammatism where language can disappear
completely. Elements of agraphia are noted in the written language, which deteriorates gradually
as well. Comprehension difficulties generally appear in the early stages of evolution. These can
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be highlighted during comprehension testing, when the person attempts to complete complex
comprehensive tasks, and these become accentuated over time.
Fluent progressive aphasia is characterized by logorrhea and jargonistic speech, major
disturbances of oral and written comprehension, as well as reading disorders, trouble reading out
loud, and repetition.
Symptoms described may be consistent with any of the following: anomia, Broca’s
aphasia, mixed aphasia, Wernicke’s aphasia and word deafness.
People with primary progressive aphasia usually have a very good self-awareness, which
generates a lot of frustration and anxiety. They express great anxiety about the gradual
deterioration of their language and the likelihood of further deterioration in their overall
cognitive abilities.

Wernicke’s aphasia

Named after Carl Wernicke (1848 – 1905), German neurologist, who was one of the first to
describe the types of aphasia. Wernicke’s aphasia is characterized by significant difficulties in
understanding what is said and what is written. The person with Wernicke’s aphasia speaks
easily or even abundantly, yet may commit paraphasias and speak in jargon. He or she will
experience the same difficulties when writing as when speaking. Some people, at least initially,
are not always aware of their mistakes. Also called receptive aphasia, sensory aphasia, posterior
aphasia.

Recovery from dysphasia

Dysphasia may resolve quickly or there may be ongoing difficulties which require management
by a Speech Pathologist.

A Speech Pathologist will be able to advise ways to communicate effectively and discuss if
therapy is suitable.

In addition, the Speech Pathologist can advise family / carers on ways to maximize their
communication with the person with dysphasia.
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Treatment of dysphasia

Treatment of dysphasia is dependent on the specific difficulties the person is experiencing.

Treatment usually has a practical focus, helping the person communicate effectively in everyday
life.

For example, if Bob has difficulty finding the right words to say, therapy will aim to increase
Bob’s ability to use the right words or appropriate strategies when he has difficulty.

Various communication aids such as white boards, picture boards and electronic talkers may help
a person with dysphasia convey their message. These aids may be used in the short and long term
and are usually implemented with the help of a Speech Pathologist. Communicating with
someone who has dysphasia

General Tips
• Reduce background noise and distractions
• Ensure the person is wearing their glasses and hearing aid if appropriate
• Include the person with dysphasia in conversations
• Encourage and accept all attempts at communication
• Always check to see if both communication partners are talking about the same topic.

When you are listening

• Make sure you are looking at the person
• Allow the person plenty of time to speak
• Encourage the person to use gesture, such as hand movements or facial expressions.

When you are talking

• Make sure the person can see your face easily
• Use simple language - avoid complicated words
• Allow the person plenty of time to understand what has been said
• Ask questions that require a yes / no or one word response. e.g. ‘Would you like a vanilla or
chocolate ice cream?’ or ‘Would you like a chocolate ice cream?’
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• Draw, write or gesture to add more meaning to your spoken words

• Slowly repeat instructions or key words if you are concerned the other person has not
understood.

Apraxia
The term apraxia refers to a wide variety of high-level motor disorders, characterised by an
impairment of purposeful voluntary movement skill.Apraxia is only indicated if aberrant motor
behaviour cannot be accounted for fully by pyramidal, extrapyramidal, cerebellar or peripheral
motor deficits or sensory loss, but may be observed in association with a number of low level
motor disorders (e.g. weakness, rigidity, tremor, dystonia). Equally, an apraxic deficit cannot be
inferred without excluding associated cognitive deficits, for example of language or perception,
as primary and sufficient explanations for the observed behaviour.
The occurrence of apraxic errors is mediated greatly by the context in which the action is elicited
(e.g. clinical setting or natural environment), the stimulus prompting the action (e.g. real object
or verbal command), the nature of the action (e.g. meaningful or meaningless gesture), the hand
with which the action is performed, and the difficulty of the action procedure (e.g. single gesture
or as part of a simple or complex action sequence). Apraxic deficits may also be body-part
specific; accordingly, a greater specification of upper limb, gait and trunk, and orofacial apraxias
is provided below. Subsequently, disorders which controversially carry the term ‘apraxia’ and
the role of praxis in naturalistic action are considered.
Upper limb apraxia
The most commonly drawn distinction in upper limb apraxia is that between ideomotor apraxia
and ideational
apraxia. Individuals with ideomotor apraxia (IM) commonly show disruption in the spatial and
temporal form of stored and novel gestures, which is associated with damage to the left inferior
parietal operculum. Patients with ideational apraxia (IA) on the other hand tend to make well-
formed movements but show a disruption of the conceptual content of action production,
resulting in tool misuse,production of complete but inappropriate gestures and disorganization of
movements in an action sequence. IA typically results from left parieto-occipital lesions (just
posterior to areas associated with IM), but the localizing value of IA has been questioned
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because the condition is rarely seen in isolation. Indeed, the clinical usefulness of the distinction
has been undermined by the frequent co-occurrence of IM and IA; in a study of apraxic left
hemisphere brain damaged patients, 60% showed symptoms of both IM and IA.1
Contemporary models of upper limb praxis mirror models of language processing, with
voluntary motor action involving a series of cognitive processing stages (e.g. input, output,
transcoding and conceptual knowledge).2 Such models support the notion that IM and IA
actually comprise a constellation of dissociable deficits. A variety of techniques may be used to
assess the integrity of differing components of the action system (for examples, see Table 1).
Furthermore, given the number of terminological difficulties in this area, upper limb apraxia may
be more accurately defined by the type and quality of action production errors (for a breakdown
of error types, see Table 2). In evaluating the significance of praxic errors however, the
specificity of gestural errors in a given context must be considered.
For example, body-part-as-object errors (e.g. using the index finger to pantomime brushing
teeth) have been shown to occur equally often in healthy controls as left and right hemisphere
brain damaged subjects, and, in left hemisphere patients, not to be associated with severity of
apraxia.
3 The modality of stimulus presentation (e.g. verbal, visual or both) for gesture production tasks
must be carefully selected to maximise the likelihood that any action production errors reflect
praxic dysfunction rather than concomitant cognitive deficits (e.g. misperceiving a complex,
meaningless hand posture demonstrated by the clinician).

Gait, leg and trunk apraxia Gait apraxia refers to an impaired ability to execute the highly
practised, co-ordinated movements of the lower legs required for walking, but remains rather
poorly specified and probably includes a number of different complex gait syndromes. 5
Disturbances of voluntary, non-routine movements of the lower limbs (leg apraxia) have also
been reported in patients with gait apraxia.6
However, it remains unclear whether leg apraxia and gait apraxia should be considered
manifestations of damage to a common lower limb praxic centre, or whether leg apraxia is more
closely related to the ideomotor apraxia more typically described in the upper limbs. A clearer
dissociation has been described between limb apraxias and axial or trunk apraxia, in which
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patients may have difficulty generating body postures (e.g. stand like a boxer), rising from a
lying position, rolling over or adopting a sitting position.
Orofacial apraxia
Patients with orofacial (or buccofacial) apraxia exhibit difficulties with performing voluntary
meaningful and meaningless movements with facial structures including the cheeks, lips, tongue
and eyebrows. Attempting to perform a pantomime to verbal command may result either in no
response or often a characteristic verbal repetition of the target action (e.g. “Could you show me
how to cough?”“Cough”). For some patients, imitation of an examiner’s pantomime may be
achieved more accurately.Orofacial apraxia may occur independently of limb apraxia, and
should also be distinguished from apraxia of speech which is a disorder of articulatory
integration associated with nonfluent aphasia. Orofacial apraxia is commonly associated with
damage in the left frontal operculum and insula, although the left hemisphere is particularly
implicated in lower face movements whilst the right hemisphere may play a role in both upper
and lower face actions.7
Controversial apraxias
In addition to the body part-specific apraxias described above, the term apraxia has also been
applied more controversially to a range of other motor disorders. Limb-kinetic (or melokinetic)
apraxia refers to an inability to make precise, smooth, fine and independent movement of the
fingers. The observation that the disorder can affect all types of gesture in any context
irrespective of hemispheric lateralization of damage has led to suggestions that limb-kinetic
apraxia is in fact primarily a deficit of the motor system. Other specialists maintain limbkinetic
apraxia is truly apractic in nature, resulting from premotor cortex damage. The appropriateness
of terms such as constructional apraxia and dressing apraxia has also been questioned, where a
combination of perceptual, spatial and motor deficits may explain at least some of the action
disorder.
Naturalistic action disorders
Naturalistic action refers to well-established sequences of movements aimed at achieving
practical goals such as food consumption or grooming activities. Naturalistic action is organized
by goal hierarchies which structure behaviour over long periods of time, and is critically
dependent upon cognitive processes largely sub-served by the frontal lobes (e.g. planning,
attention, working memory.) The term frontal apraxia describes a breakdown in this sequential
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organization of behaviour, and is characterized by object substitutions and misuse (e.g. spooning
butter into coffee, using the wrong implements to eat or stir). Although evidence of limb apraxia
is elicited typically in a clinical setting, studies of the real world behaviour of ideomotor apraxic
patients reveal a reduced frequency of tool-related action production and an increased number of
tool action errors relative to other patient groups.
Treatment for Apraxia of Speech
Most available treatments for AOS have limited data to support their efficacy (Wambaugh and
Doyle, 1994). Over the last few decades, a variety of treatment approaches have been studied,
with no one approach proving to be effective for all patients (Wambaugh, 2002). Given that AOS
is generally believed to primarily disturb articulation and prosody, many programs have focused
on remediating these specific deficits (Wambaugh, 2002). Therapy goals are typically designed
to improve communicative effectiveness. For the mildly apraxic patients, poor prosody may be
the primary speech deficit and, therefore, goals designed to improve intonation and stress may be
the most appropriate (Square et al., 1997). For the moderately or severely apraxic patient,
therapy might focus on relearning oral postures for individual speech sounds (Square et al.,
1997).
General techniques that have been employed include: traditional articulation therapy (repetitive
exercises involving imitation of speech sounds and words) (Rosenbek et al., 1973), finger
tapping or pacing (using a metronome) (Dworkin et al., 1988), singing and electromagnetic
(EMG) feedback to reduce tension (McNeil et al., 1976; Wambaugh, 2002). Alternative or
augmentative communication devices have also been prescribed for patients with severe AOS, as
well as the use of compensatory strategies to replace speech (e.g., gesturing, writing, drawing,
communication books, etc.) (Wambaugh, 2002).
In a recent summary of treatments for apraxia of speech, Wambaugh noted two particular
techniques that have replication data to support initial treatment findings (Wambaugh,
2002). One such approach, PROMPT, developed by Square\ and colleagues is designed to help
patients use rate and rhythm control strategies (Square et al., 1985; Square-Storer and Hayden,
1989) to improve their speech. The second program, developed by Wambaugh and collegues,
focuses on the remediation of misarticulated consonants through modeling, repetition of
minimally contrastive words, graphic cues and phonetic placement cueing (Wambaugh et al.,
1998). Despite the fact that there are no large, randomized trials of efficacy of treatment for
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AOS, single subject studies using rigorous experimental designs have demonstrated the
effectiveness of some of the treatments for AOS in individual cases mentioned above. The
effectiveness of these treatments has been replicated.

Agnosia

Agnosia is a rare disorder whereby a patient is unable to recognize and identify objects, persons,
or sounds using one or more of their senses despite otherwise normally functioning senses. The
deficit cannot be explained by memory, attention, language problems, or unfamiliarity to the
stimuli. Usually, one of the sensory modalities is affected. For example, a patient with agnosia
may not be able to identify a cup by sight, although they may be able to tell its color and identify
it by touch by its shape and texture. It is not same as anomia. Anomia is a naming disorder in
which patients cannot name an object despite using their other sensory modalities like touch and
smell.

Classically, there are 2 forms of agnosia: apperceptive and associative.

● Apperceptive agnosia is a failure in recognition due to deficits in early stages of

perceptual processing.

● Associative agnosia is a failure in recognition despite no deficit in perception.

Associative agnosia patients can typically draw, match or copy objects while
apperceptive agnosia patients cannot.

Types of Agnosia

There are 3 main types of agnosia, based on the type of sensation involved.

1. Visual (vision)

2. Auditory (hearing)

3. Tactile (touch)

Visual Agnosia

Visual agnosia refers to an impairment in recognizing visually presented objects, despite

otherwise normal visual field, acuity, color vision, brightness discrimination, language, and
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memory. Patients can recognize objects using other sensory modalities. Sometimes impairment
is worse for certain types of objects, so a variety of objects should be tested to diagnose
precisely. Visual agnosia is the most common and better-understood agnosia.

Agnosia is further divided in 2 subtypes: apperceptive visual agnosia and associative visual
agnosia.

● Apperceptive visual agnosia refers to an abnormality in visual perception and

discriminative process, despite the absence of elementary visual deficits. These people
are unable to recognize objects, draw, or copy a figure. They cannot perceive correct
forms of the object, although knowledge of the object is intact. Apperceptive visual
agnosia is typically associated with lesions to the parietal, occipital cortex

● Associative visual agnosia refers to difficulty with understanding the meaning of what
they are seeing. They can draw or copy but do not know what they have drawn. They
correctly perceive the form and know the object when tested with verbal or tactile
information, but cannot identify the object. They are unable to link the fully perceived
visual stimulus to prior experience to help them recognize the stimulus. Associative
visual agnosia is usually associated with damage to the bilateral inferior occipitotemporal
cortex.

Types of Visual Agnosia

● Prosopagnosia is the inability to recognize familiar faces. Patients can often identify other
aspects like gender, hair, emotions. Prosopagnosia results from damage to fusiform face
area (located in the inferior temporal cortex in fusiform gyrus). People with apperceptive
prosopagnosia cannot perceive facial expression and cues but can recognize non-facial
clues like hair and clothing. Associative prosopagnosia patients can derive some facial
information like gender and age. Mr. P in Oliver Sack’s book The Man Who Mistook His
Wife for a Hat presents a classic case of prosopagnosia.

● Simultanagnosia is the inability to recognize and sort out objects when they appear
together, but they can recognize them when they appear alone. Patients are unable to
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perceive the overall meaning of a picture or multiple things together, although they can
describe isolated elements. Two forms of simultagnosia have been described.

1. Dorsal simultagnosia: Patients cannot see more than one object at a time. For example,
when presented with a picture with table, chair and flower vase, they may report only one
thing at a time. When their attention is diverted to the other thing, they can then identify
only that thing; other things disappear to them. They often have difficulty reading as it
involves viewing more than one word at a time. They often bump into objects that are
close together. Dorsal simultagnosia is typically associated with lesions in the bilateral
occipitotemporal cortex.

2. Ventral simultagnosia: These individuals also cannot identify more than one object or
complex objects at one time, although they can see more than one object at a time. They
are unable to perceive the whole picture as one and derive a meaning out of it. For
example, in a night sky picture with stars and full moon, they may identify moon as a
ball, unable to derive the meaning of the whole picture. Ventral Simultagnosia is
associated with lesions in the left inferior occipital area.

● Color agnosia is the inability to identify and distinguish colors, despite intact basic color
vision and brightness discrimination mechanisms. It is very difficult to diagnose this type
of agnosia as colors can only be appreciated visually. Usually, these patients have a
lesion in the left occipitotemporal region of the brain.

● Topographical agnosia is the inability to orient to surroundings because of inability to

interpret the spatial information. These patients have a good memory of the layout and
specifics of the places well known to them, but they are unable to navigate their way
through. They are unable to use the visual cues to guide them in the right direction.
Associated with a lesion in the right posterior cingulate area of the brain.

● Finger agnosia is a difficulty in naming and differentiating among the fingers of either
hand as well the hand of others. It does not refer to the inability to identify a finger as a
finger, as the name may suggest. It is a part of the constellation of symptoms often
referred to as Gerstmann syndrome which includes acalculia, agraphia, finger agnosia,
and left-right disorientation.
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● Akinetopsia refers to the inability to perceive motion.

● Agnostic alexia refers to the inability to recognize words visually (they can still write and
talk without difficulty).

Auditory Agnosia

● Auditory agnosia is an inability to recognize sounds despite intact hearing. Auditory

agnosias are typically associated with right side temporal lesions.

Types of Auditory Agnosia

● Phonagnosia is the inability to recognize familiar voices. They can recognize words
spoken by others. It is caused by damage to certain parts of sound association region.

● Verbal auditory agnosia or pure word deafness is the inability to comprehend spoken
words but can read, write, and speak in a relatively normal manner.

● Nonverbal auditory agnosia is the inability to comprehend nonverbal sounds and noises,
with sparing of speech comprehension.

● Amusia is the inability to recognize the music. They are unable to comprehend that
certain types of sounds represent music and therefore cannot distinguish music from other
sounds.

Tactile Agnosia

Tactile agnosia refers to the inability to recognize objects by touch. They can name objects by
sight.

● Amorphognosia is the inability to identify the size and shape of objects by touch, for
example, a triangle or square.

● Anosognosia is the inability to identify distinctive qualities like texture and weight, for
example, a piece of wood, cotton or metal.

● Tactile asymbolia is impaired recognition by touch in the absence of amorphognosia and

ahylognosia.
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Causes

Agnosia can result from various neurological conditions like strokes, tumors, infections,
dementia, hypoxia, toxins such as carbon monoxide poisoning, head injury, developmental
disorders, or other neurological conditions. It can present suddenly, for example, in stroke or
head injury, or gradually, for example, in tumor, dementia. Symptoms depend on the area
involved. People with agnosia usually retain their other cognitive abilities.

Agnosia happens when there is brain damage along pathways that connect primary sensory
processing areas. These areas typically include the posterior parietal cortex and occipitotemporal
regions.

History and Physical Examination

Careful patient history is very important in cases of suspected agnosia. Clues to diagnosis and
etiology often involve a detailed interview with the patient, family members, and caregivers. It is
harder to diagnose when the patient lacks the insight of his or her deficits. History will reveal
clues pointing to the type of agnosia. For example, a patient with visual agnosia will
specify problems in day-to-day life, for example, getting dressed or inability to differentiate fork
and a knife because of inability to recognize certain types of objects visually. Patients with
topographical agnosia will present with getting often lost, findings directions. Patients with
akinetopsia often bump in things, get confused how things got moved around, have difficulty in
crossing roads and driving and are accident prone. Patients with simultagnosia will often have
trouble reading and writing and interpreting multiple things at a time.

Other important points to elicit in history can confirm that other sensory modalities, memory,
and cognition are not affected.

Initial assessment involves asking patients to identify common objects through sight, touch, and
smell. A thorough physical examination is done to detect for any primary deficits in individual
senses (visual fields, acuity, reading, drawing, hearing tests, among others) or in the ability to
communicate that may interfere with identifying the objects (memory, cognition, attention,
aphasia).
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An important aspect of diagnosing agnosia is ruling out alternative explanations. Recognition

disturbance can occur in many other neurological conditions, but agnosia can be diagnosed only
in the absence of aphasia, generalized dementia, delirium, or any deficit that may impair some or
all of the information-processing steps involved in object recognition.

It is very important to rule out genuine unfamiliarity with stimuli. It is important to consider
cultural and life experience and other factors that may lead to unfamiliarity. Testing of common
objects and constructing a balanced set of items that are determined individually should be done.

For example, to assess for visual agnosia, show the patient several familiar items like keys, a
pen, and a banana and ask to name them. Differentiating visual agnosia and aphasia is important.
For prosopagnosia, show the patient pictures of famous people, their family members, and
themselves. For color agnosia, show solid color cards and ask them to name the colors. For
simultagnosia, present pictures with several objects or people and ask them to identify what they
see. For tactile agnosia, make sure patient is unable to see, then put common things in each hand
one by one and ask the patient to identify by touch alone. Inability to correctly identify at least
50% of stimuli tested will suggest the diagnosis of agnosia.

Evaluation

Diagnosis is mainly clinical, based on clinical history and physical examination including a
thorough neurologic exam, psychological exam, and certain standardized tests of brain function.
It is important to make sure that the abnormality cannot be explained in another way, for
example, genuine unfamiliarity with the stimuli, poor instructions, overlooked disease like
neuropathy and cataracts, or other neurologic conditions like dyspraxia or dysphasia.

Referrals to ophthalmology, speech pathology, audiology, psychiatry, and other professionals

may be needed for detailed evaluation of sensory and neurobehavioral status. Once a tentative
diagnosis of agnosia is made, a thorough neuropsychological examination is done to test
intelligence, memory function, linguistic competence, and assess sensory-perceptual processing.

Various standardized tests are available to test for memory and cognition (MMSE, Montreal
Cognitive assessment, ADAS-cog) and aphasia (Boston naming test, western aphasia battery).
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Diagnosis is supplemented by neuroimaging tests such as head CT and brain MRI to look for
etiology. More testing may be needed based on clinical judgment and suspected etiology.
Auditory or visual evoked potentials may be used to determine whether a deficit exists in the
sensory projection area as opposed to primary sensory or association cortex.

Treatment and Management

Agnosia can considerably limit the everyday functioning of the patients. It can also significantly
affect the lives of family and caretakers. There is no direct cure. Treat the underlying cause,
when possible. For example, treating and preventing stroke, antibiotics and/or surgery for brain
abscess, and surgery and/or radiation for brain tumors.

Agnosia is managed supportively. Rehabilitation, speech, and occupational therapy play an

important role in the treatment of agnosia and mainly focus on teaching patients to use the intact
sensory modalities to compensate. Restorative training has limited utility. Interventions tend
to focus on helping these patients and their family and caregivers cope and adapt to the
condition, and furthermore, to help patients function independently within their context.
Counseling of family members and helping them with behavioral changes is equally important.

Rehabilitation approaches need to be individualized and should focus on the specific deficit by
developing compensatory strategies.

Alternative Cues and Strategies

Some general strategies that can be used are alternative cues, for example, teaching people with
prosopagnosia learn to identify any facial scars or hairstyles. For patients with visual agnosia,
teaching alternative strategies like learning to identify everything by touch; in patients
with prosopagnosia, teaching voice recognition for people; and teaching patients with auditory
agnosia to lip read and write everything.

Organizational Strategies

● Provide a predictable environment and consistency in performing a task, for example,

placing things in the same place every day. Routines help develop consistency in
performing a task.
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● Declutter, for example, things patients use every day should be kept out in one place.
This may help patients with visual agnosia.

● Label everything

Differential Diagnosis

It is important to remember that pure forms of agnosia are very rare. It is important to evaluate
and rule out dementia, aphasia, acute confusional states (delirium), disturbance of attention, and
unfamiliarity with stimuli. Also, clinicians need to confirm make sure no actual sensory
impairment such as color blindness, cataracts, hearing loss, neuropathy, among others.

Prognosis

Few patients with agnosia regain their sensory function. Most recovery occurs within the first
three months and to a variable degree may continue up to a year. Prognosis depends on - patient's
age, etiology, type, size and location of the area affected, the extent of impairment, effectiveness
of therapy.

DISCONNECTION SYNDROMES

Overview

The term “disconnection syndrome” is applied to the effects of lesions of association pathways,
either those which lie within a single cerebral hemisphere or those which join the two halves of
the brain (Geschwind,1965). Often the disconnection syndromes are talked about more generally
as Collosal Syndromes. Of course, the corpus callosum is by far the largest of the nerve fiber
bundles that directly connect one cerebral hemisphere with the other. These are generally called
cerebral commissures and include the anterior and hippocampal commissures. These syndromes
occur when the corpus callosum is partially or fully surgically divided. Syndromes of hemisphere
disconnection can also occur when there is a partial disconnection such as in naturally occurring
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diseases/condition such as stroke (thrombosis etc.) Callosal lesions are often accompanied by
damage to neighboring structures. Therefore “neighborhood signs” may overshadow signs of
callosal disconnection.

Split-Brain Effects

1. Just after complete cerebral commissurotomy patients respond well and can write letters to
simple commands. Easily confused by three or even two-part verbal commands. Seen as
mildly akinetic. A seeming imperviousness is observed. Often patients may write short and
usually one-word answers. Considerable variation from one patient to another in terms of
neurologic outcome and deficits.
2. After a few months symptoms of hemisphere disconnection are compensated for to a large
degree and seen in improved personality functioning, interactions and social situations and on
neurologic exam.
a.) Visual material: visual material presented selectively to a single hemisphere by fixing gaze
on some picture or object. Can describe or read various kinds of material in the right half-field at
the level substantially the same before surgery. When presented to left half-field usually reports
they see nothing or a flash of light.

b.) Inability to describe verbally left half-field stimuli includes hemialexia, where subject can’t
read individual words flashed to the left half-field. Some recovery occurs over a period of years
where word is recognized by right hemisphere as a symbol for something, albeit diffuse semantic
information. Semantic information transferred to the speaking left hemisphere which
approximates the stimulus word.

c.) Auditory Suppression: Right-handers identify single words if presented to one ear at a time.
If different words presented during dichotic listening tasks, right ear information will be reliably
reported. Left ear words poorly reported although occasionally appropriate actions are made by
the left hand indicating some perception. Repeated testing indicates that your asymmetry may
decrease particularly if patients attention is directed to left ear or if information load is reduced.
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d.) Motor function: left-sided apraxia to verbal commands noted. Left limb apraxia seen
because of poor comprehension by right hemisphere and poor ipsilateral control by left
hemisphere. Compensatory mechanisms may emerge including increased right-hemisphere
comprehension of words and increased left-hemisphere control of left hand.

e.) Somesthetic Effects (including touch, pressure, and proprioception). Unseen objects in right
hand are handled, named and described in normal fashion. In contrast, naming of objects held out
of sight in left hand are consistently failed. Despite inability to name the object in left hand, the
left hand is able to manipulate the item and show how it’s used ;or they retrieve the object with
the left hand from among the collection of objects screened from the patient sight (implicating
that the object is also being identified by the right hemisphere.)

f.) Cross-replication of hand postures: One patient told to make postures with one hand, can’t
mimic same posture with the opposite hand . When a specific posture is flashed in one visual
half-field, can be copied by hand on the same side but not by the other hand.

g.) Inter-manual point localization: After a complete commissurotomy, there is a partial loss of
ability to name exact points stimulated on left-side of body. Defect is least apparent in the face
and most apparent and distal parts (e.g. fingertips). To test for this, while hand is shielded, the
patient is asked to identify which finger tip was touched by examiner and to touch that finger
with their own thumb (on the same side touched by examiner). Then when asked to identify the
same fingertip on the opposite hand, (and once again to touch it with the thumb on that hand)
they are unable to do it. Cross localization can be done by split-brain patients at a level not much
better than chance.

h.) Right-hemisphere Verbal Comprehension: Auditory comprehension of words by

disconnected right hemisphere is seen in the patients ability to retrieve, with the left hand,
various objects if they are named aloud. Visual comprehension of words is accurate especially
with short, and high-frequency words. So if a word name of an object (e.g. the word key) is
flashed to the left visual half-field, patient can retrieve the item with the left hand from a group
of objects hidden from view. Typically incorrect verbal descriptions are given after the word is
presented, implicating that only the right hemisphere knew the answer. Right hemisphere
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language capabilities in right-handers are more evident when left hemisphere has been removed.
If removed during infancy these capabilities may seem nearly normal as child develops language
capabilities. Left hemisphere disease later in childhood that affects language abilities typically
shows less intact language function since right hemisphere does not compensate as well. After
split brain surgery and the left hemisphere is present and relatively intact, most linguistic abilities
of disconnected right hemisphere are largely absent or suppressed. The disconnected right
hemisphere’s receptive vocabulary can grow considerably and reach levels comparable to that of
the vocabulary of the 10-to 16 year-old. Right hemisphere language has limitations: such as poor
syntactic ability which is rudimentary at best. The rich lexical structure of language in right
hemisphere diminishes significantly.

Other Disorders Following Hemisphere Disconnection

1. Unilateral verbal anosmia:unable to named odors presentd to the right nostril even when
readily presented to the left. Not a defect of smell in right nostril.
2. Double hemianopia
3. Hemialexia and Alexia without agraphia: When printed letters or short words are presented
to left half-field patient unable to read information. Can readily read it when presented in the
right half-field. Left hemialexia when combined with right homonymous hemianopia results
in Alexia without Agraphia. For example, stroke patients can write but are unable to read
even when they have just written correctly to dictation. Occurs in about 75% of righthanders
with left posterior cerebral artery infarcts. Typically the patient has a right homonymous
hemianopia resulting from a left occipital lobe lesion. In this case nothing can be seen, much
less read in the right half-field. Visual information can reach the left hemisphere language
only from the left half-field via the right occipital cortex and spelenium. Since there is a
lesion typically in this region, it has disconnected the right occipital cortex from the left
hemisphere. The left hemisphere still retains the ability to right to dictation even though it
does no longer have access to information arriving in the right occipital lobe from the left
visual half-field.
4. Auditory Suppression
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5. Unilateral (left) ideomotor apraxia: This was the first described callosal symptom in the
literature. In response to a verbal command the right-handed individual can’t carry out with
the left hand some behavior which the right hand can do. Important to distinguish upon
examination that the problem doesn’t have to do with a loss of dexterity or mild weakness.
Also distinguished from ideational apraxia
6. Unilateral (right) constructional apraxia: The inability to organize several parts into a
configuration despite normal ability to handle or draw the individual parts. This type of
apraxia can be quite prominent in the right hand of right-handers with callosal lesions. Have
the patient copy with one hand, and then with the other hand, various geometric figures.
Hemisphere disconnection in a right-hander is strongly suggested if patient can copy designs
better with left hand.This may only be the case when there is no other right hemisphere
involvement (e.g., left hand is paretic or ataxic)…in this instance they probably do no better
with one hand vs. the other.
7. Spatial acalculia: Since hemisphere disconnection can cause right-handed disability for
spatial forms patient may have difficulty using pencil and paper to solve arithmetic problems.
This deficit typically progressively received with recovery. Sometimes they can do mental
calculations even though they cant to written ones.
Summary of Disconnection Syndromes based on the work of NORMAN GESCHWIND..

(if you want to read this these are the syndromes he covers)

1. Word blindness without agraphia: Regarded as the first example of a callosal disconnection
syndromes for which clear anatomical evidence was established.
2. Pure Word Deafness: A lesion, located subcortically in the left temporal lesion typically
destroys the left auditory radiation as well as callosal fibers from the opposite auditory region.
The lesion has the effect of preventing the speech area (that part of the auditory association
cortex that is Wernicke’s area) from receiving auditory stimulation. Although the right
primary auditory cortex could receive auditory stimuli it can’t convey them to the speech area
because the callosal connections from the right side were destroyed in the left temporal lobe.
3. Lesions of Wernicke’s Area: although verbal comprehension is impaired, speech is also
impaired. The loss of Wernicke’s area can be regarded as the destruction of a memory store as
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it was in fact regarded classically. Presumably it functions importantly as the “storehouse” of

auditory associations. Geschwind describes that “a name” passes through Wernicke’s area
then via the angular gyrus which arouses associations and other parts of the brain. Wernicke’s
area attains its essential importance and comprehension as it is also involved in the arousal of
associations. (maybe we should discuss this and talk about whether this is phenomena is
discussed in this way anymore).
4. Tactile aphasia: A disturbance characterized by an inability to name objects tactilely in the
preservation of the ability to name on the basis of visual or auditory stimulation and
spontaneous speech.