Keele University MBChB Module 3 Applied Clinical Science

Cognitive Disorders

Case 1

a. Ian is a 55 year old plumber who lives with his wife, Pat. Pat noticed that her
husband seemed unwell while watching the TV on Saturday night. Ian had lost
consciousness in his arm chair but was awake by the time the ambulance
arrived. Ian was unable to speak for 3 days but began to slowly improve while in
hospital. However, his speech was clearly abnormal.

Listen to audio clip

Tasks

1. Describe Ian’s speech.

Speech is effortful, non fluent with many approximations and errors in naming
Content is simple but comprehension is preserved

2. Define this abnormality and summarise how it arises.

This is an expressive or motor dysphasia indicating impaired ability to

generate normal speech, naming is disproportionately affected. However, as
comprehension is preserved, the individual recognizes their errors and
attempts to correct the speech, hence the effortful and non fluent nature.

3. What brain area/s are likely to be affected?

The dominant cerebral hemisphere (usually the left) is the hemisphere

specialized for speech and language.
Broca’s area is located in the frontal lobe, immediately above the fissure
separating the frontal lobe from the temporal lobe (Sylvian fissure). Broca’s
area is involved in the production of speech (with adjacent areas being
involved in other language expression eg; writing)

4. List possible causes of this presentation

CVA
Cortical dementias (especially Alzheimer’s Disease and FTD)
Head injury
Space occupying lesions
Herpes encephalitis
 5. How would you test for this disorder in clinical practice?
Naming components of MMTS and ACE-R. Less frequently used words are
often affected first.

b. Patrick is a 35 year old gentleman with Marfan’s Disease. While clearing his
mother’s garden, he experienced a sudden severe headache associated with
vomiting and slurred speech. He was rushed to hospital and underwent a
number of investigations. Nursing staff noticed that he seemed to not hear
instructions and would do what was asked.

Read the transcript

Interviewer; Hello Pat, how are things today?

Pat; It could be revurded and spectdad but aren’t you carey
Interviewer; We said that we were going to assess you in the kitchen, yes?
Could you show me how to make a cup of tea?
(Pat continues to sit at the kitchen table, without moving to the kettle)
Pat; possible query at the moment. I think all kisty the right shape for erskip
and brody.. Why and how is this type of curl.
Interviewer; Perhaps you could boil some water in the kettle first?
(Pat remains sitting)
Pat; I’ve not cooperized that this morning, where is mine gope or this tajet and I
do think lots of that and hers
(Interviewer mimes sipping a cup of tea. Pat moves over to kettle and begins to
make cup of tea very normally)

Pat; Could it be now for this notty coowd yes.

Tasks

1. Describe Patrick’s speech

Speech is fluent, rapid but nonsensical with many meaningless words.

Patrick’s responses convey little understanding or comprehension of
conversation.

2. Define the abnormality and summarise how it arises

This is a receptive or sensory dysphasia, indicating impaired comprehension

of speech. As the individual is unable to recognize errors in speech, there is
no effort to correct. Therefore, speech remains fluent and effortless but
contains many errors.
As received speech is not understood, instructions are not comprehended
and cannot be correctly interpreted and acted on. Patrick demonstrates that
non speech language comprehension is understood, suggesting a fairly
 discrete lesion.

3. What brain area/s are likely to be affected?

Like Broca’s area, Wernicke’s area is also found in the dominant hemisphere.
Wernicke’s area is located in the posterior, superior temporal gyrus.
Wernicke’s area is involved in speech comprehension with adjacent areas
being involved in other language comprehension eg; reading)

4. List possible causes of this presentation

CVA
Cortical dementias
Head injury
Space occupying lesions

5. How would you test for this disorder in clinical practice?

3 stage command test in MMTS

6. Define conduction aphasia and summarise how it arises

Conduction aphasia is the inability to repeat a phrase while other expressive

and receptive language functions are retained.
Therefore, naming and 3 stage command tests are normal, while repeating
“no ifs, ands or buts” is impaired.
In order to be able to repeat a phrase, when instructed to do so, requires
intact receptive function (so that the instruction is understood),
communication between receptive areas and expressive areas and intact
expressive function so the correct words can be generated.
In conduction aphasia, receptive and expressive function is intact but the
communication between them is impaired. The adjoining tract is called the
arcuate fasciculus and this is where the lesion must be.

Case 2

Case 2

a. Martin is a 29 year old landscape gardener. While traveling home from work
he was involved in a head on collision with another car. He was unconscious for
3 days and made a slow recovery, complicated by complex orthapaedic injuries.
Martin needed to return to living with his parents, who helped him to maintain his
recovery. However, Martin’s mum began to notice a lot of problems. Martin
appeared to get very frustrated and irritable with frequent explosive outbursts
where he would threaten his parents and wreck their possessions. Martin was
also very intolerant of change and did not cope well when his dad went back to
work. He began to avoid his old friends and seemed unable to concentrate for
any length of time. He was unable to cope with returning to work – he could not
complete jobs and was unable to organize his diary, frequently turning up to work
with the wrong equipment. He eventually stopped work altogether after he cut
down every tree in a client’s orchard. He had been asked to prune 1 tree only.

b. The following was wriiten by Dr Harlow, an American GP, about his patient
Phineas Gage

“The equilibrium or balance, so to speak, between his intellectual faculties and

animal propensities, seems to have been destroyed. He is fitful, irreverent,
indulging at times in the grossest profanity (which was not previously his
custom), manifesting but little deference for his fellows, impatient of restraint or
advice when it conflicts with his desires, at times pertinaciously obstinate, yet
capricious and vacillating, devising many plans of future operations, which are no
sooner arranged than they are abandoned in turn for others appearing more
feasible. A child in his intellectual capacity and manifestations, he has the animal
passions of a strong man. Previous to his injury, although untrained in the
schools, he possessed a well-balanced mind, and was looked upon by those
who knew him as a shrewd, smart businessman, very energetic and persistent in
executing all his plans of operation. In this regard his mind was radically
changed, so decidedly that his friends and acquaintances said he was "no longer
Gage."

Tasks

1. List the symptoms of cognitive impairment present in both cases.

Impaired planning
Impaired sequencing
Impaired judgement
Perseveration
Impaired concentration
Disinhibition

2. Define the abnormality and summarise how it arises

This is referred to as the Dysexecutive Syndrome. In health, the normal brain

is able to inhibit primitive urges (fight, flight. feed and f**k). When damaged,
this inhibition may be lost with much challenging behaviour resulting from
impulsivity and poor judgement.
The brain also allows us to be rapidly adaptive to new situations or change
and when damaged this adaptability is impaired.
 The brain is able to filter out unwanted information so that we may focus our
attention on matters of interest (divided attention and dual task interference).

3. What brain are/s are likely to be affected?

Dorsolateral prefrontal cortex.

4. List possible causes of this presentation

Brain injury
Cortical dementias (especially FTD and Alzheimer’s Disease)

5. How would you test for this in clinical practice?

Stroop Test
Wisconsin Card Sort Test
Trail making test
Traffic Jam
ACE-R, verbal fluency test
ACE-R – clock drawing
Luria 3 step hand test
Go, no go test
Primitive reflexes

Case 3.

Ethel is a 75 year old lady who recently lost her husband, Dennis, after 55 years
of marriage. Ethel’s daughter, Sharon, began to worry that her mum wasn’t
coping with the death of Dennis. Ethel seemed to lose her confidence and not
look after herself very well. Ethel seemed to constantly lose things and accused
her daughter of stealing things she had lost. Sharon noticed that Ethel asked the
same questions several times and would leave the gas cooker on and not lock
the doors at night.

Tasks

1. Identify the symptoms of cognitive impairment.

Ethel appears to have poor short term memory and may be unaware of, or
unable to meet her day to day care needs.

2. Define the abnormality and how it is caused

Poor short term memory is often the first sign of cognitive impairment that is
obvious to family/ carers. Individuals cannot recall recent events and are unable
to make new memory (anterograde amnesia) and can therefore not learn new
information.

3. What brain area/s are likely to be affected

Temporal lobe atrophy, particularly involving the hippocampi and adjacent

diencephalic structures, correlates with memory impairment
4. Outline how memory is organized

Memory can be divided into explicit and implicit.

Explicit memory may be further divided into short term and long term.
Short term memory may be additionally subdivided into anterograde and
retrograde.

Long term memory may be considered as having two subtypes, semantic

(localizing to the left temporal lobe) and episodic (less well localized but certainly
involving the hippocampi and limbic system).

Implicit memory consists of procedural memory and conditioned reflexes (and

appear to localize to the basal ganglia).

5. List possible causes of this presentation and summarise their differing

pathologies
Senile Dementia of Alzheimer’s Type – STM impairment is often the first sign of
illness. Other cognitive functions are also affected (language, praxis, frontal lobe
functions, recognition). There is widespread cortical atrophy with particular
neuronal loss in the hippocampal region. Histological findings include amyloid
plaques and neurofibrillary tangles in the cerebral cortex.

Lewy Body Dementia – characterised by dementia, parkinsonism, fluctuating

consciousness and psychosis, this disease is caused by cortical and subcortical
neuronal loss with alpha synuclein being deposited in Lewy Bodies.

Frontotemporal Dementias – complex group of disorders with pathological

changes confined to the cortex.

Vascular Dementia
Alcohol Induced Dementia
Korsakoff’s Disease – profound anterograde amnesia with preservation of other
cognitive functions. Cause is thiamine depletion (usually due to alcohol use)that
often causes demyelination of the mammillary bodies .

6. How would you test for this in clinical practice?

MMTS – 3 minute recall

ACE-R – anterograde memory test (address)