Paranoia
and Memory Loss/Frontal Lobe Dementia
By Piero G. Antuono, MD, Professor,
Department of Neurology and Pharmacology, Director, Memory Disorders Clinic,
Medical College of Wisconsin.
Case Presentation
Alzheimer's disease is the most common neurological disease of adult life.
It afflicts 4 million people in the United States, and it's incidences
increase with age. The early symptoms are characterized by an insidious
onset and gradual progression of memory loss.
Some dementias, however, do not follow this pattern. Vascular dementias
have recently been characterized by specific diagnostic criteria. They
can be summarized by sudden onset of dementia and step-wise progression
with focal neurological findings and positive brain imaging. Lewy body
dementia is characterized by early appearance of symptoms in the course
of dementia, accompanied by progression and hallucinations. Primary progressive
aphasia includes early dissolution of speech in an otherwise cognitively
intact individual. They can become globally aphasic in spite of being able
to continue to run a household or even work. Eventually 20% per year progress
to dementia, which appears similar in the advanced states to Alzheimer's
disease.
Frontal lobe dementias are characterized by early psychiatric symptoms
followed later by cognitive impairments. Frontal lobe syndrome is therefore
the presenting symptomatology: apathy, poor social judgment, and bizarre
behavior. Histologically, the frontal lobe dementias are proven to be characterized
by Pick's cells (Pick's disease). At the present time, these diverse histological
types are clinically indistinguishable.
When degenerative neurological diseases affect the subcortical structures,
it may cause apathy and slowness of thinking which can be mistaken for
dementia. Examples of such conditions are Parkinson's disease, olivo-ponto
cerebellar atrophy (OPCA), and hydrocephalus.
The following cases will illustrate a patient with frontal lobe dementia
and OPCA as examples of uncommon dementias.
A 74-year-old
white woman was noted to have undergone a drastic personality change over
the last year. She was described as becoming much quieter with several
instances of paranoia and suspiciousness. The onset of these changes was
described as slow with a gradual progression. She was preoccupied with
her own safety. She always locked the house, even when her husband went
to mow the lawn. She has been known to prop the back door closed with a
shovel. When driving in the evening, she would disguise herself as a man,
putting her hair up in a black knit cap. The patient's daughter removed
a handgun from the home. Within one week the patient purchased another
one. She looks for the gun every morning to make sure she knows where it
is. At the present time the patient has hidden the gun and the bullets.
She has accused her husband of having an affair. The patient, who was quite
neat in the past, has become disheveled, not paying attention to her dressing
and hygiene. Her ability to cook and clean the house has significantly
deteriorated. In particular, she has developed hiding and hoarding behaviors.
She was resistant to throwing anything away, including spoiled food. She
tended to "over shop" and pack the freezer and refrigerator with
food. Recently, she had become obsessed with winning the sweepstakes, and
has ordered many books in order to enhance her chance of winning.
The husband denied his wife has significant memory problems. There is no
noted difficulty with memory loss or ability to recognize people or objects.
She has shown no difficulty with driving a car, getting lost, or using
household appliances and the telephone. The patient has a masters degree
in education and has maintained her interests and hobbies, which include
learning a foreign language.
The neurological
examination was normal. There was no evidence of dyspraxia or dysnomia
during the evaluation. Visual spatial skills appeared to be intact. The
Mini Mental State Examination score was 29/30. There was no depression
and mood and affect were congruent. Some difficulty was present with abstract
thinking.
Neuropsychological testing showed intact remote memory, fluent speech,
and non-labile affect. The patient was oriented to time, place, and person.
The performance on tests of memory was in the average range. Her constructional
and organizational skills were normal. The patient's response to a self-reported
depression inventory were also within normal limits. However, on the test
of verbal abstraction and reasoning, the patient performed in the low average
range. Her ability to generate words was poor, also in the low average
range. There was evidence of perseveration. Similarly, there was impairment
on tasks which required shifting strategies. Concept formation and mental
flexibility was moderately impaired.
In summary, the neuropsychological testing was consistent with normal range
for attention, visual spatial processing, and naming. In contrast, the
patient had difficulty with executive functioning tasks such as verbal
fluency, verbal abstraction, concept formation, and mental flexibility
suggesting a predominantly largely frontal lobe dysfunction.
MRI showed a soft tissue lesion in the fourth ventricle (Fig. 1). This
was consistent with a probable subependymoma which was thought to be an
incidental finding. There was evidence of frontal lobe atrophy (Fig. 2).
Figure 1. MRI T-2 study showing a fourth ventricle lesion likely an ependymoma. |
Figure
2. MRI T-1 study showing predominantly frontal lobe atrophy. |
The findings
are suggestive of a degenerative disease of the frontal lobes. The presence
of impaired judgment and executive skills with normal visual spatial and
mnemonic function seem to suggest this condition. These "frontal lobe"
dementias histologically have been proven to be consistent with Pick's
disease, subcortical gliosis, or simple neuronal loss. The history of normal
cognitive performance in the face of poor judgment, poor executive function,
and the imaging consistent with frontal lobe involvement are the basis
of this diagnosis.
In the later stages as the dementia progresses involving posterior cerebral
functions, it resembles Alzheimer's disease. Management is more challenging
in the early stages than in Alzheimer's disease due to poor judgment and
safety risks these patients represent. Because of this, they tend to be
institutionalized earlier than patients with Alzheimer's disease.