Slowly progressive limb-kinetic apraxia with
a decrease in unilateral cerebral blood
flow
Okuda B, Tachibana H, Kawabata K, Takeda M, Sugita M. Slowly
progressive limb-kinetic apraxia with a decrease in unilateral cerebral blood

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We report two patients with slowly progressive motor disorders, whose
principal manifestations were asymmetric limb-kinetic apraxia and muscle
rigidity. In both patients MRI revealed no responsible lesion, whereas single
photon emission computed tomography (SPECT) showed a decrease in
cerebral blood flow (CBF) in the unilateral hemisphere. One patient with
mainly right-sided apraxia had a decreased CBF in the left central region
between the frontal and parietal cortices, and the other patient with left-sided
apraxia in the right parietal cortex. In agreement with asymmetric clinical
symptoms, the regional CBF decrease in the unilateral cortical areas including
the frontal and parietal cortices may suggest a degenerative disease,
presumably diagnosed as having corticobasal degeneration.

Case report
Case 1

A 72-year-old right-handed man without a personal
or familial history of neurologic disease was admitted to our hospital due to progressive limbclumsiness. He initially noticed difficulty in using the
right hand, especially in manipulating objects, three
years before admission. One year later clumsiness
developed in the right lower limb, and he suffered
from gait disturbance: Movement disturbance grad-

76

B. Okuda, H. Tachibana, K. Kawabata,
M, Takeda, M, Suaita
"
Fifth Department of Internal Medicine, Hyogo
College of Medicine, Japan

Neurol Scand 1992: 86: 76-81.

Limb apraxia is divided into three subgroups consisting of limb-kinetic apraxia (LKA), ideomotor
apraxia and ideational apraxia (1). While ideomotor
apraxia and ideational apraxia have been well explored, LKA remains to be elucidated (2, 3). We
present two patients with slowly progressive limb
apraxia, mainly LKA, in combination with extrapyramidal symptoms. CBF measurement by using
SPECT and N-isopropyl-p[ '231]iodoamphetamine("'I-IMP) as a tracer in both patients showed a
decreased perfusion in the frontal cortex and/or the
parietal cortex on the side contralateral to the greater
apraxia. The unique combination of asymmetric
LKA and unilateral CBF decrease is distinct from
the dementing diseases such as Alzheimer's disease
or Pick's disease, and appears to be the early manifestations of a degenerative disease, called corticobasal degeneration.

I

Key words: slowly progressive apraxia;
limb-kinetic apraxia; cerebral blood flow;
corticobasal degeneration

B. Okuda, Fifth Department of Internal
Medicine, Hyogo College of Medicine,
Nishinomiya 663, Japan
Accepted for publication December 24, 199 1

ually progressed, and then he became unable to write
letters or pick up an object, and frequently fell to the
right side. Recently, he found clumsiness in the left
hand. Since he was diagnosed as having cervical
spondylosis, he underwent laminectomy of the cervical spine two months before admission to our department. Limb-clumsiness, however, persisted. On
admission, he was neither demented nor aphasic
(Mini-Mental State Score 28/30, WAIS verbal IQ
99). He presented with bilateral limb apraxia predominating on the right side and constructional
apraxia. H e had difficulty making fine finger movements such as tapping, picking up a coin and imitating finger patterns, especially with the right hand
(Fig. 1 A,B). He had also clumsiness in gestures
such as waving goodbye in response to verbal command and imitation, and clumsiness in object manipulation such as using scissors. Voluntary movements of the right leg were also difficult when
initiating gait and putting on shoes. Alien hand sign
was not seen. Eye movements were preserved except
for saccadic pursuit. He was mildly bradykinetic,
and muscle tone was rigid in the right upper limb and
paratonic in the other limbs. Muscle strength was
normally preserved in all limbs. Involuntary movements were undetected. Deep tendon reflexes were
brisk on both sides without Babinski reflex. Limbcoordination was clumsy on the right side, but not
ataxic. Superficial and deep sensations were normal.
Cortical sensation such as two-point discrimination

Slowly progressive apraxia

Ffg. I . Imitation of finger patterns in two patients. Patient I has bilateral apraxia predominating on the right side (A, B). Patient 2 had
apraxia in the left hand, but imitation is correctly performed by the right hand (C,D).

and stereognosis was preserved. He walked with
small steps, but neither pulsion phenomenon nor
kinesie paradoxale was observed. T2-weighted MRI
showed atrophic change in the left hemisphere, and
a high intensity spot in the right subcortical white
matter (Fig. 2). SPECT using 12’I-IMP( ‘231-IMP
SPECT) demonstrated decreased *231-uptakein the
left hemisphere, especially in the central region between the frontal and parietal cortices (Fig. 3). Intravenous digital subtraction angiogram (IV-DSA)
denoted no remarkable change of the main vessels.
EEG revealed no significant abnormality except sporadic theta waves in the left frontal leads. Levodopa
was administrated, but motor disorders remained
unchanged.
Case 2

A 64-year-old right-handed woman without a personal or familial history of neurologic disease was
admitted to our hospital because of progressive gait
disturbance. She started having difficulty in moving
the left leg three years before admission. She often
fell to the left side. She became incapable of going
up the stairs or putting on shoes. Several months
later she found clumsiness in the left hand. She had
difficulty in getting up and picking up an object with

the left hand. A family member noticed a decrease
in her speech a year before admission. She was diagnosed as having Parkinson’s disease in another hospital. On admission to our hospital, she was not
aphasic but moderately demented (Mini-Mental
State Score 16/30, WAIS was not performed). Limb
apraxia was revealed on the left side. She had difficulty making precise finger movements of the left
hand such as tapping and imitating finger patterns
(Fig. 1 C,D). Manipulating objects of the left hand
was difficult when picking up a coin, putting on
gloves and buttoning. Gestures such as waving goodbye and saluting were clumsy on the left side in response to verbal command and imitation. Movements of the right hand were slow but correctly
performed. Voluntary movements of the left leg were
also difficult when initiating gait and kicking a ball.
Constructional ability was disturbed. Alien hand sign
was absent. Cortical sense such as two-point discrimination, stereognosis and graphesthesia was preserved. She presented with bilateral upward gaze
palsy with preserved Bell’s phenomenon. Muscle
strength was normal in all limbs. She was bradykinetic, and muscle tone of the limbs was rigid predominating on the left side. Deep tendon reflexes
were normal without Babinski reflex. Neither involuntary movements nor cerebellar ataxia was dell

Okuda et al.

Fig. 2. T2-weighted MRI (Spin echo, TR 2000 ms, TE 120 ms) in Patient 1. A high intensity spot in the right subcortical white matter is seen, and there is atrophic change in the left hemisphere.

tected. T2-weighted MRI showed high intensity
spots in the periventricular region, but no cortical
lesion was seen (Fig. 4). IMP-SPECT disclosed a
decrease in '*'I-uptake in the right parietal cortex
(Fig. 5 ) . IV-DSA revealed no remarkable change of
the main vessels. EEG gave normal findings.
Levodopa was administered, but motor disorders
did not improve.
Discussion

ldeomotor apraxia and ideational apraxia are generally defined as a disorder of symbolic action such
as gesture and a disorder of a series of acts such as
object handling, respectively (1,4). Unlike ideomotor apraxia and ideational apraxia, LKA is defined
as a breakdown of previously skillful movements,
manifested by difficulty making fine precise movements (3,4). The disorder is more obvious when
testing finger movements and can be evident when
making gestures, imitating postures, and using objects (1). Liepmann postulated that a lesion in the
sensory motor cortex induced LKA on the contralatera1 side ( 3 ) . While the status of LKA remains disputable, several cases have been reported to have

motor clumsiness of this type (4-6). The responsible lesion is considered to be located in the central
region between the frontal and parietal cortices (precentral gyrus and postcentral gyrus) (5,6). It is hypothesized that LKA is induced by impaired organization for motor control involving interaction
between the motor cortex and the somatosensory
cortex (7, 8). Our two patients had disorders of gesture and object handling, but more prominent disorder was difficulty making fine finger movements.
Accordingly, the apraxic disorders in these patients
were mostly ascribed to LKA rather than ideomotor
apraxia or ideational apraxia. Muscle rigidity may
play a role in difficulty in performing voluntary movements. Patients with parkinsonism often have slowness in limb movements but can correctly perform
purposeful movements or finger imitation until becoming severely rigid and akinetic. Thus, motor
clumsiness in our patients cannot be attributed to
muscle rigidity. Patient 1 had bilateral LKA with
predominance on the right side, and Patient 2 had
left-sided LKA. A decrease in CBF suggested by
IMP-SPECT, in the left central region in patient 1
and in the right parietal region in Patient 2, may
account for LKA on the contralateral side respec-

Slowly progressive apraxia

Fig. 3. '"I-IMP SPECT axial view performed 30 min after administration of "'1-IMP (early image) in Patient 1. In comparison to the
right hemisphere, '2311-uptakein the left hemisphere is decreased, especially in the central region (arrow).

tively. Furthermore, a decreased CBF in the left central region in Patient 1 may be related to bilateral
LKA, because the dominant hemisphere lesion can
induce bilateral clumsiness (9).
Since Mesulam's report (lo), diseases causing
progressive impairment of higher cortical function
have been discussed in connection with Pick's disease, Alzheimer's disease, or Creutzfeldt-Jacob disease (1 1- 13). Slowly progressive apraxia combined
with extrapyramidal signs has been reported in patients with degenerative diseases, called corticobasal
degeneration (14) or cortical-basal ganglionic degeneration (CBD) (1 5). In these cases reported, the usual
age of onset was in the sixth and seventh decades,
and limb-clumsiness gradually developed with asymmetric features. Cerebral cortical signs consisted of
apraxia, cortical sensory loss, constructional disability and alien hand signs, suggesting dysfunction of
the frontal and parietal cortices. The other main
clinical features were extrapyramidal signs such as
akinesia, rigidity and involuntary movements. Dementia, pyramidal signs and supranuclear oculomotor palsy were sometimes seen. The common neuropathological findings of autopsied cases were
degenerative changes Consisting of gliosis and neu-

ronal achromasia in both the cerebral cortex, mainly
the frontal and parietal cortices, and the basal ganglia, mainly the substantia nigra.
The clinical features of our two cases appear to be
compatible with CBD. Patient 1 had typical clinical
features common to CBD described above. The patient had cervical spondylois, but no improvement of
the motor disorders could be obtained following
laminectomy. The apraxic disorders with rigidity
cannot be ascribed to cervical spondylosis. Since
Patient 2 was demented, this case raises a possibility
of the other dementing diseases, including Pick's
disease, Alzheimer's disease, progressive supranuclear palsy, Parkinson's disease, or diffuse Lewy
body disease. The patient's initial and principal
symptoms, however, were motor disturbance comprising left-sided apraxia and parkinsonism, which
were unusual in Pick's disease or Alzheimer's disease. Furthermore, cortical signs such as limb apraxia
and constructional disability cannot occur in progressive supranuclear palsy, Parkinson's disease or
diffuse Lewy body disease. To our knowledge, LKA
has not been reported in the above diseases. The
other possibility is that a vascular disorder may play
a role in the motor disorders. While MRI revealed
79

Okuda et al.

Fig. 4 . T2-weighted MRI (Spin echo, TR 2000 ms, TE 120 ms) in
Patient 2. High intensity spots in the periventricular area are seen,
but no cortical lesion is noted.

no responsible lesion, IMP-SPECT suggested the
regional hypoperfusion in both patients. A vascular
insufficiency may be associated with a degenerative
process. A few cases of CBD presented with mild
vessel disorders in pathological examinations (15).
Our two patients, however, had no risk factors for
stroke, and IV-DSA showed no significant changes
of the main vessels in both patients. Above all, a
vascular insufficiency can hardly cause the slow progression of both higher cortical and basal ganglionic
dysfunctions. The unique combination of asymmetric LKA and extrapyramidal signs favor the diagnosis of CBD. Therefore, we presented two cases as
slowly progressive LKA, presumably diagnosed as
having CBD. However, the possibility of a distinct
entity with focal cortical degeneration cannot be
excluded (16, 17). Moreover, CBD has not been recognized fully as a clinical entity. Further clinicopathological studies are required to make a distinction between CBD and the other disorders causing
progressive deterioration of cerebral cortical or subcortical functions such as Pick's disease, Alzheimer's
disease or progressive supranuclear palsy.
In the present series, a hemispheric decrease in
CB F corresponded to neuropathological findings of
CBD, which revealed degenerative changes in the
cerebral cortex, mainly in the frontal and parietal

80

cortices. Sawle et al. reported positron emission
tomography (PET) findings in clinically diagnosed
CBD (18). PET showed that fluorodopa uptake was
asymmetrically reduced in the striatum and that cortical oxygen metabolism was significantly depressed
in the superior and posterior temporal, inferior parietal and occipital association cortices as well as in
the posterior frontal cortex. In the present study,
IMP-SPECT showed a unilateral decrease in '231uptake in the basal ganglia in Patient 1, but asymmetric uptake in the basal ganglia was undetectable
in Patient 2. The cortical hypometabolic areas demonstrated by PET are larger than the cortical regions
where '231-uptake was decreased in the present
study. The inconsistent results may be explained by
the difference in duration of patient's illness studied
in both series. Both patients in the present study had
a history of three years, whereas most patients studied by Sawle et al. had a history of more than four
years. Moreover, patients of the latter groups presented with supranuclear gaze palsies, myoclonus,
or alien limb sign together with clinical features seen
in the former group. These findings suggest that either the frontal or parietal cortex or both cortices
may be unilaterally involved in the early stage of
CBD, accompanied by asymmetric involvement of
the basal ganglia, and that the degenerative process
may extend to the other cortical areas in the later
stage. Thus, a regional CBF decrease in the unilateral frontal or parietal cortex or both cortices may
aid the clinical diagnosis of CBD, especially in the
early stage.

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Slowly progressive apraxia

Fig. 5. ‘”I-IMP SPECT axial view performed 30 min after administration of ‘231-IMP(early image) in Patient 2. ”’1-uptake is reduced
in the right parietal region (arrow).

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