The Differentiation of Frontotemporal Dementia ... - McLean Hospital

McLean Annals of Behavioral Neurology (2007), 2:5-10
©2007
www.mclean.harvard.edu/patient/adult/ncs/nbn-annals.php
The Differentiation of Frontotemporal Dementia from
Catatonia in a 43-Year-Old Woman: Case Report
Jacob Kagan 1 , R Andrew Sewell 2 , Bruce Price 3 , and Miles G Cunningham 3
1 Adult Psychiatry Residency Program, Cambridge City Hospital/Harvard Medical School,
Boston, MA; 2 Alcohol and Drug Abuse Research Center, McLean Hospital/Harvard Medical
School, Belmont, MA; 3 Department of Neurology, McLean Hospital/Harvard Medical School,
Belmont, MA
Differentiation between frontotemporal dementia (FTD) and catatonia can sometimes be
challenging, as symptoms overlap. We present a case illustrating this diagnostic dilemma, and
review the literature on techniques used to differentiate between the two.
Key words: Frontotemporal dementia, catatonia
INTRODUCTION
The diagnosis of dementia in a young
individual is devastating for both patient
and family. Diagnostic certainty is
therefore particularly important. Recent
epidemiological studies have suggested
that frontotemporal dementia (FTD) is
the second most common cause of
dementia in the under-65 age group. 1 It
is a subtype of frontotemporal lobar
degeneration (FTLD), which
encompasses a range of clinical and
pathological presentations, typified by
focal atrophy of the frontal and anterior
temporal regions. The two other
subtypes of FTLD are progressive
nonfluent aphasia (PA) and semantic
dementia (SD). 2
________________________________________
Correspondence: Miles Cunningham, MD,
PhD, Department of Neurology, McLean
Hospital/Harvard Medical School, 115 Mill
St., Belmont, MA 02478, USA.
Tel: (617)855-2051
E-mail: mcunningham@mclean.harvard.edu
Received 23 August 2006, Revised 8 April
2007, Online publication 16 April 2007
FTD is the most common, characterized
by profound changes in personality and
social conduct, ranging from
disinhibition, distractibility, purposeless
overactivity, and socially
inappropriateness to apathy, avolition,
mental rigidity, and speech that is
economical but grammatically correct. 3
Verbal perseveration, stereotypic and
compulsive behaviors, hyperorality, and
overeating are associated symptoms.
Cognitive deficits affect abstraction,
attention, problem solving and planning.
Memory deficits may also occur, but are
not thought to be a primary amnesia but
rather stem from frontal executive
impairment. 4 Extrapyramidal symptoms
of akinesia, rigidity, and gait disturbance
are relatively common; tremor occurs
less frequently and is typically postural. 5
PA consists of a progressive isolated
deficit of speech production and gestural
apraxia with preserved comprehension,
memory, and other cognitive skills until
late in the disease course. In SD, speech
production remains fluent, grammatical,
5

The Differentiation of Catatonia from Frontotemporal
Dementia in a 43-Year-Old Woman
J Kagan et al.
and free of paraphrasias but
comprehension deteriorates, face
recognition disappears, and patients also
exhibit difficulty in recognizing the
significance of objects.
Although catatonia is historically
considered a symptom of schizophrenia,
it actually occurs more frequently during
the course of a mood disorder, 6 and the
DSM-IV-TR reflects this newer thinking
about catatonia by adding catatonia as a
specifier in mood disorders and as a
separate syndrome occurring secondary
to a general medical disorder. 7 Catatonia
shares a number of characteristics with
FTD (see Table 1)—paucity of speech,
stereotyped or repetitive behaviors,
purposeless and excessive motor
activity, and echolalia. Differentiation
between catatonia and FTD may
therefore be challenging. The following
case illustrates this clinical overlap and
underscores the critical importance of
diagnostic certainty for accurate
prognosis and delineation of patient and
caregiver psychosocial burden.
CASE REPORT
A 43-year-old married white woman
presented with what her family noted to
be a two-year history of progressive
behavioral changes, including flattened
affect, social withdrawal, compulsions,
increased appetite, decreased speech,
depression, and complaints of memory
loss. A year prior to admission, a
psychiatric nurse practitioner had noted
that the patient had complained of
depressed mood, had a significantly
flattened affect, and spoke largely using
“yes” and “no” answers. She had also
become increasingly socially withdrawn,
had recently been fired from her position
as a kindergarten teachers’ aide, and had
begun compulsively showering and
using the dishwasher and clothes washer.
She gained over 50 pounds, frequently
binging on carbohydrate-rich foods. She
had grown increasingly less able to care
for herself and maintain safety within the
house. A few months prior to admission,
the patient accidentally lit a blanket on
fire on the stove and in her confusion,
did not know what to do with the
burning blanket. Shortly thereafter, the
patient’s 23-year-old son moved back
home in order to help care for his mother
while her husband was traveling.
Because of her bizarre behavior and
apparent depressed mood and
neurovegetative symptoms, she was
Criterion A. Any of: (two required in diagnosis of catatonic schizophrenia)
Motoric immobility as evidenced by cataplexy or stupor
Excessive motor activity (apparently purposeless)
Mutism or extreme negativism (motiveless resistance to instructions or maintenance
of rigid posture)
Peculiarities of voluntary movement (inappropriate or bizarre postures, stereotyped
movements, prominent mannerisms, or prominent grimacing)
Echolalia or echopraxia
Criterion B: Symptoms are the direct result of a general medical condition
Criterion C: Symptoms are not better accounted for by another mental disorder
Criterion D: Symptoms do not occur exclusively during the course of a delirium
Table 1: DSM-IV-TR criteria for Catatonic Disorder due to a General Medical
Condition 16.
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McLean Annals of Behavioral Neurology
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The Differentiation of Catatonia from Frontotemporal
Dementia in a 43-Year-Old Woman
J Kagan et al.
diagnosed with depression with
psychotic features, and given
unsuccessful trials of citalopram,
venlafaxine, bupropion, nortriptyline,
methylphenidate, and risperidone. She
then underwent 20 sessions of bilateral
electroconvulsive therapy (ECT), also
without response. One week following
her course of ECT, the patient was
hospitalized for suicidal ideation,
stabilized, and discharged two weeks
later, but soon after discharge again
endorsed thoughts of cutting her wrists,
so was brought into our acute psychiatric
care center for evaluation.
The patient was a high school graduate
who had completed some college-level
coursework before dropping out to begin
working full time. She had worked as an
aide in an elder care facility and in a
kindergarten class. There was no history
of dementia in the family, although the
patient’s mother had a history of
depression and the patient’s father had
died at age 45 of lung cancer.
On examination, she had a severely
flattened affect, echolalia, poverty of
speech with almost exclusive use of the
words “yes” and “no”, and mild
psychomotor agitation. On cognitive
testing she was inattentive and had
impaired recall, abstraction, and a
relative anomia, but preserved
visuospatial functioning. Neurological
exam showed a fine postural tremor in
the patient’s left great toe and foot, but
no rigidity, gait abnormalities, or
primitive reflexes. Basic blood count,
chemistries, liver function tests, and
urinalysis were normal with the
exception of a mild normocytic anemia.
Serum vitamin B 12 , folate, erythrocyte
sedimentation rate (ESR),
ceruloplasmin, serum lyme titers, and
autoimmune panel were negative. Rapid
plasma reagin (RPR) was non-reactive.
A lumbar puncture revealed a mildly
elevated opening pressure (24 mm H 2 O),
but normal glucose, protein, and cell
counts, non-reactive CSF syphilis
serology, and no cryptococcal antigens.
An electroencephalogram (EEG)
disclosed low-voltage beta activity,
intermittent low-voltage 9 Hz alpha
activity and low-voltage slowing with
drowsiness, but no epileptiform activity.
MRI revealed minimally increased extracerebral
space, suggesting mild early
involutional changes.
Neuropsychological evaluation that had
been performed four months prior to
admission had demonstrated impaired
attention and concentration, impaired
verbal and narrative memory, and
significant perseveration in her
responses to the Thematic Apperception
Test and Rorschach Test.
Visuoperceptual and organizational
functions were also below average in
unstructured situations (Rey figure
memory), but normal when structure was
applied (Hooper Test).
On admission, the patient was taking
methylphenidate, venlafaxine, and
risperidone. Lorazepam was added and
titrated to 8 mg a day in the first week of
admission, but the patient’s presentation
varied little for the remainder of
hospitalization. She remained minimally
communicative, perseverative, and
echolalic, with flattened affect, persistent
purposeless activity and compulsive
grooming behaviors. Three weeks later,
she was discharged with a presumptive
diagnosis of FTD, with plans to followup
in a partial hospitalization/day
program. Venlafaxine was discontinued
with plans to initiate treatment with a
SSRI.
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McLean Annals of Behavioral Neurology
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The Differentiation of Catatonia from Frontotemporal
Dementia in a 43-Year-Old Woman
J Kagan et al.
DISCUSSION
While an age of onset younger than 45 is
unusual in FTD, it can not be used as an
exclusion criterion, as FTD has been
documented in the fourth and even third
decade of life. 1, 8 Similarly, while current
hallucinations or delusions are not
typical of FTD, they may also be absent
in schizophrenia with predominantly
negative symptoms or a catatonia from a
mood disorder. 8 Delusions and
hallucinations may likewise occur in a
small percentage of FTD patients,
although they occur less frequently than
other behavioral symptoms. 9
Multiple imaging modalities have been
used to examine FTD and catatonia,
although none have compared the two
directly. MRI of patients with FTD
typically reveals severe frontal, anterior
temporal, and anterior parietal atrophy
with a characteristic asymmetric pattern
of atrophy. 10, 11 Specific patterns include
disinhibition associated with
predominant atrophy of the orbitomedial
frontal and anterior temporal regions,
apathy associated with extensive frontal
lobe and dorsolateral frontal lobe
atrophy, and semantic impairment
associated with volume loss throughout
the left anterior temporal lobe, right
12, 13
temporal pole and subcallosal gyrus.
While there is some evidence to suggest
prefrontal volume loss in schizophrenic
patients, the evidence is inconclusive, 14
and no studies have reported
morphological abnormalities specific to
catatonia. Single photon emission
computed tomography (SPECT) imaging
has revealed severe reductions in frontal
rCBF in FTD patients, 10 but similar
reductions in rCBF have been found in
catatonic patients in the right lower and
middle prefrontal regions and parietal
cortical regions. 15 Positron emission
tomography (PET) imaging of FTD has
revealed glucose hypometabolism
predominantly in the frontal and anterior
temporal lobes and abnormalities in the
cingulate gyri, uncus, insula, and
subcortical structures including basal
17, 18
ganglia and medial thalamic regions.
PET studies in catatonic patients have
been more limited, however, one
examination of three patients with
speech-sluggish catatonia revealed
bitemporal and bifrontal glucose
hypometabolism and bi-thalamic
hypermetabolism. 19 To date, multiple
imaging modalities therefore show
significant overlap in findings between
catatonia and FTD—perhaps related to
common frontal neurocircuitry—but
there are no studies directly comparing
the two groups.
CONCLUSION
We describe a patient with the early
onset of a number of disabling,
progressive symptoms including
prominently flattened affect, paucity of
speech with maintained grammatical
structure, verbal and physical
perseveration, purposeless motor
activity, and deficits in attention,
concentration, abstraction, and memory.
Laboratory investigations for infectious,
metabolic and inflammatory processes
were unrevealing and MRI suggested
early frontal lobe atrophy. While the
differential diagnosis of an early onset
dementing process is large, this patient’s
presentation was highly suggestive of a
mood-disorder-associated catatonia or
FTD. We are unaware of prior case
reports describing this diagnostic
challenge. The importance of correct
diagnosis of FTD in a young, formerly
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McLean Annals of Behavioral Neurology
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The Differentiation of Catatonia from Frontotemporal
Dementia in a 43-Year-Old Woman
J Kagan et al.
high-functioning individual can not be
overestimated, given the rapid
progression and limited life expectancy
in this disorder. 20 Age of onset, clinical
presentation, and neuroimaging are not
helpful in differentiating catatonia from
FTD, but our patient’s non-response to
both ECT trials and lorazepam challenge
proved diagnostic in this case, as
catatonia has been shown to be
remarkably responsive to both treatment
modalities. 7, 21, 22 Future research directly
comparing catatonia and FTD might
further delineate the diagnostic utility of
MRI, PET, SPECT and fMRI, but until
then, clinical judgment and judicious
trials of lorazepam and ECT are the most
appropriate tools available to clarify this
difficult diagnostic challenge.
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Dementia in a 43-Year-Old Woman
J Kagan et al.
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