OBSERVATION Functional Brain Imaging in

David A. Kareken, PhD; Frederick Unverzagt, PhD; Karen Caldemeyer, MD; Martin R. Farlow, MD; Gary D. Hutchins, PhD

Background: An extensive literature describes struc- sults of the neurological examination were normal. There tural lesions in apraxia, but few studies have used func- was of both hands (command, imita- tional neuroimaging. We used positron emission tomog- tion, and object) and buccofacial apraxia. The patient raphy (PET) to characterize relative cerebral glucose could recognize meaningful gestures performed by the metabolism in a 65-year-old, right-handed woman with examiner and discriminate between his accurate and awk- progressive decline in ability to manipulate objects, write, ward pantomime. The magnetic resonance image showed and articulate speech. moderate generalized atrophy and mild ischemic changes. Positron emission tomographic scans showed abnormal Objective: To characterize functional brain organiza- fludeoxyglucose F 18 uptake in the posterior frontal, tion in apraxia. supplementary motor, and parietal regions, the left af- fected more than the right. Focal metabolic deficit was Design and Methods: The patient underwent a neu- present in the angular , an area hypothesized to store rological examination, neuropsychological testing, mag- conceptual knowledge of skilled movement. netic resonance imaging, and fludeoxyglucose F 18 PET. The patient’s magnetic resonance image was coregis- Conclusions: Greater parietal than frontal physiologi- tered to her PET image, which was compared with the cal dysfunction and preserved gesture recognition are not PET images of 7 right-handed, healthy controls. Hemi- consistent with the theory that knowledge of limb praxis spheric regions of interest were normalized by calcrine is stored in the dominant parietal cortex. Gesture com- cortex. prehension may be more diffusely distributed.

Results: Except for apraxia and mild grip weakness, re- Arch Neurol. 1998;55:107-113

APRAXIA IS a monly responsible for apraxia in both hands. Because right hemisphere lesions disorder of skilled movement not caused by are not known to induce apraxia,5,6 the weakness, akinesia, deafferentation, abnor- left hemisphere appears dominant for the mal tone or posture, movement disorders, in- tellectual deterioration, poor comprehension, storage and execution of learned move- or uncooperativeness.1 ments. Although both anterior and pos- terior lesions in the left hemisphere pro- Patients with ideomotor apraxia are un- duce apraxia, patients with parietal able to pantomime on command sym- lesions often have difficulty compre- bolic gestures (eg, saluting) or use of tools, hending gesture, and discriminating and their movements are characterized by between accurately and poorly per- errors in posture, spatial orientation, and formed pantomime gestures.7,8 Occipital joint coordination.2-4 Analogous move- lesions that disconnect visual association ment defects in the face, lips, tongue, areas from the may lead to From the Departments cheeks, larynx, and pharynx (eg, blow- impaired gesture imitation, but other- of (Drs Kareken ing out a match or whistling) are termed wise normal praxis.9 Although still and Farlow), buccofacial apraxia.1 Although a number unable to execute gesture, patients with (Drs Kareken and Unverzagt), of studies have described the structural le- frontal lesions are better able to accu- and Radiology (Drs Caldemeyer and Hutchins), Indiana sions that result in apraxia, little work has rately discriminate good from bad panto- University School of Medicine, described the functional network of brain mime, and comprehend gesture in oth- 7,8 7,10 and the Richard L. Roudebush regions that become compromised. ers. Some authors therefore propose Veterans Affairs Medical Center Lesion research suggests that left that the left supramarginal and angular (Dr Kareken), Indianapolis. intrahemispheric lesions are most com- gyri contain visuokinesthetic motor pro-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 SUBJECTS, DESIGN, AND glucose uptake was measured with 2-fludeoxyglucose F 18 (10-mCi intravenous bolus), and images were recon- METHODS structed to 9-mm spatial resolution. The PET images for all subjects were spatially registered in the horizontal plane PATIENT AND SUBJECTS along the anterior commissure–posterior commissure line. A whole brain proton density–weighted MRI scan of the The patient was a 65-year-old, high school–educated, right- patient’s head was acquired in the axial plane, and coreg- handed woman who presented to the Indiana Alzheimer istered with her PET image volume using an iterative pro- Disease Center in Indianapolis. Approximately 4 years ear- cedure that minimizes the least-squared difference be- lier, she experienced the insidious onset of difficulty speak- tween the MRI and PET image volumes. Magnetic resonance ing and writing. These symptoms gradually progressed, and images were unavailable for the controls. The regions of she became unable to properly manipulate silverware and interest analyses were performed on the PET images by one small items (eg, buttons or jewelry). She gradually be- of us (D.A.K.), normalizing hemispheric regions of inter- came unable to perform her household chores, including est by the average of left and right calcrine cortex. management of family finances. At the time of her clinic Five regions of interest were delineated: anterior fron- visit, she had difficulty finding and articulating words, but tal (FA) (corresponding to the dorsolateral prefrontal cor- continued to read and understand television programs. tex), posterior frontal (FP) (corresponding to premotor and Seven healthy control subjects from the Indiana Alz- primary motor regions), parietal, caudate-putamen (CP), heimer Disease Center–PET database were selected for im- and (SMA). All regions were di- aging comparison (mean age, 69 years; age range, 56-79 vided into left (L) and right (R). The central was ap- years). The controls had no evidence of cerebral or neu- proximated by a line just posterior to the thalamus as viewed ropsychological abnormality. Informed consent was ob- on the midsagittal image (Figure 1, A, plane a). Al- tained from all subjects after the nature of the procedure though this landmark confounds slightly the actual divi- had been fully explained. sion between frontal and parietal cortex, it was a reliable landmark. The division between FA and FP was defined MATERIALS AND PROCEDURE on the axial image with a line through the center of the 2 caudate heads. This was an easily identifiable, reliable land- Clinical Examinations mark, and constitutes the approximate division between ( 6) and the cortex ante- The patient underwent neurological and neuropsychologi- rior to it28 (Figure 1, A, plane c). was ex- cal examinations (Table 1 and Table 2). Apraxia exami- cluded from FP and from FA. For the pa- nation involved testing gesture production and compre- rietal regions of interest, the and lateral occipital hension. To test production, the patient was asked to gyri were excluded in slices inferior to the parieto- pantomime on command 10 intransitive gestures (waving occiptial sulcus (Figure 1, A, plane d). Tracing of frontal goodbye, hitchhike, salute, beckon “come here,” signal stop, and parietal regions of interest began on an axial plane just finger to lips for “shsh,” finger tapping, “ok” sign, 2 “ok” dorsal to the thalamus (Figure 1, C, plane e). On the coro- signs linked together, and the “peace” or “V” for victory nal images this plane passed through both sylvian fissures sign), 8 transitive gestures (opening a door with a key, flip- (Figure 1, B, plane e). Tracings were made of the cortical ping a coin, opening a ketchup bottle, stirring coffee with ribbon with a computer mouse on the axial images at each a spoon, and using a screwdriver, hammer, scissors, and of 5 planes dorsal to the thalamus, with an interplane in- handsaw), and 8 buccofacial movements (cough, sniff, puff terval of 5 pixels (Ϸ10 mm) (Figure 1, C). out cheeks, wink, stick out tongue, blow a kiss, blow out a The SMA was approximated with a right triangle on match, and suck on a straw). To test gesture comprehen- the sagittal image (Figure 1, C, shaded area). The vertical sion, the patient was asked to discriminate 4 target ges- line of the right angle (posterior boundary) was placed at tures from foils (using a hammer, waving goodbye, shoot- the anterior-posterior division line, and the horizontal line ing a basketball, and flipping a coin) and 4 precise target (ventral boundary) was placed at the midpoint between the gestures from the same gestures performed clumsily and and the vertex of the . The hy- with a body part as object (brushing teeth and using a ham- potenuse followed the cortex along the edge of the inter- mer, scissors, and key). hemispheric sulcus. As defined, SMA probably includes most of Brodmann area 6 and at least some of Brodmann area 8 Imaging (see reference 28). Two planes of SMA (one on the mesial surface of the interhemispheric sulcus, one 4-mm lateral) The PET studies were performed on a whole body PET to- were traced in each hemisphere and averaged. Tracings of mograph (Siemens 951/31R, 6-mm full-width half- CP were performed at a single plane of the axial image that maximum intrinsic resolution) in a dimly lit room with sub- traversed the midpoint of the caudate heads as visualized jects supine, resting, and eyes open. Regional cerebral in the coronal images.

grams that are implemented by premotor areas in the have been found in the frontal , anterior left hemisphere. The (Brod- insula, and striatum.12,13 mann areas 5 and 7) might also play a role by translat- In contrast to the extensive study of lesion loca- ing motor programs into somatosensory cues that guide tion in apraxia, there has been little use of physiologi- limb movement.11 Buccofacial apraxia is less well stud- cal imaging to describe functional brain organization. ied than limb apraxia. Lesions related to this disorder Physiological imaging is important because structural

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Table 1. Neuropsychological Test Results* Table 2. Examination*

Test Patient’s Score Test Score Intellectual (WAIS-R19) Auditory comprehension Verbal IQ 84 Word Discrimination 71/72 Performance IQ 79 Body-Part Discrimination 19/20 Full Scale IQ 81 Commands 14/15 Executive Complex Ideational Material 12/12 Wisconsin Card Sorting Test20 Aural Comprehension (MAE) 18/18 Categories 5 Reading comprehension Perseverative responses 14 Symbol Discrimination 10/10 Category Test Errors21 78 Word Recognition 8/8 Trail Making A, B, S 8†; 234† Comprehension of Oral Spelling 3†/8 Contrasting Motor Program Tapping (errors) 2/20 Word-Picture Matching 10/10 Go No-Go Tapping (errors) 2/20 Reading Sentences and Paragraphs 9/10 Memory Reading Comprehension (MAE) 18/18 WMS-R Logical Memory I, II22 24, 19 Naming WMS-R Visual Reproduction I, II22 19, 18 Responsive Naming 19†/30 Rey Auditory Verbal Learning Test23 Visual Confrontation Naming 109/114 Trial 1 3† Visual Naming (MAE) 50/60 Trial 5 13 Boston Naming Test 51/60 Short delay 14 Fluency Long delay 13 Animal Naming 7† Recognition (false-positive errors) 15 (1) Letter Fluency (MAE) 16† Visual-perceptual Phase Length 7/7 Judgment of Line Orientation24 22/30 Melodic Line 7/7 Facial Recognition24 47/54 Verbal Agility 0/14 Visual Form Discrimination24 28/32 Repetition Tactile perception Single Word 9/10 Finger gnosis (errors) High-Frequency Phrases 5†/8 Right 15†/20 Low-Frequency Phrases 2†/8 Left 13†/20 Sentence Repetition (MAE) 3†/14 Finger tip number writing Written Right 12†/20 Block Spelling (MAE) 4†/11 Left 12†/20 Arithmetic Written (WRAT-III) 8th Percentile *WAIS-R indicates Wechsler Adult Intelligence Scale–Revised; WMS-R, Wechsler Memory Scale–Revised. *Unless otherwise noted, tests are from the Boston Diagnostic Aphasia †Impaired (Յ1.5 SDs). Examination.25 MAE indicates Multilingual Aphasia Examination 26; WRAT-III, Wide Range Achievement Test, third edition. 27 †Impaired (Յ90th percentile of patients with aphasia for Boston lesions have remote functional effects that computed Diagnostic Aphasia Examination; Յ1.5 SDs for MAE). tomography and magnetic resonance imaging (MRI) cannot detect.14 Although some investigations15-18 have found apraxia to relate to parietal defects in glucose RESULTS metabolism or blood flow, only 1 of these reports18 related regional functional activity to gesture produc- CLINICAL EXAMINATIONS tion and comprehension. Rapcsak et al18 recently stud- ied a woman with slowly progressive bilateral limb Neurological examination revealed an awake, alert, ver- apraxia using single photon emission computed bally dysfluent woman who was well oriented except for tomography. Despite having ideomotor apraxia, her the name of the hospital and the date. Finger to nose, conceptual knowledge of tool use and limb movement rapid alternating movements, and heel to shin were all was intact. Single photon emission computed tomog- intact. There were occasional random movements of her raphy, nonetheless, demonstrated severe biparietal hands. A hand dynamometer showed her grip strength hypoperfusion, a finding that appears inconsistent to be mildly weak bilaterally, but tone and strength were with the hypothesis that conceptual knowledge of otherwise normal in the upper and lower extremities. Re- limb praxis and gesture comprehension are mediated flexes were 2-3+ and symmetric, and there were no Bab- primarily by the .7,10 inski signs. Results of a sensory examination of light touch, We used positron emission tomography (PET) pain, and proprioception were unremarkable. Magnetic to map the cortical distribution of relative glucose resonance imaging revealed moderate cortical atrophy that metabolism in a 65-year-old woman with a 4-year was mildly asymmetric, the left hemisphere being more progressive decline in manipulating objects, writing, atrophic than the right. Mild small vessel ischemic dis- and speech articulation. In the absence of any clear ease was also present in the periventricular white mat- structural abnormality, we sought to determine the ter, but there were no focal lesions present. relative contribution of frontal and parietal cortical Results of neuropsychological and aphasia examina- glucose metabolism to her gesture production and tions appear in Tables 1 and 2. Verbal IQ was low average comprehension. and Performance IQ was borderline. Abstraction and men-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Thalamus Parieto-occipital Fissure a b Sylvian Anterior Frontal Fissure

c e e

Posterior a Frontal

Parietal Thalamus b

Thalamus A d d B C

Figure 1. A through C, Region of interest definition in 1 healthy control. Axial image A shows the planes approximating the (a), interhemispheric fissure (b), the division between anterior and posterior frontal region (c), and the plane that excludes the occipital regions from the parietal region (d) in sections below the parieto-occipital fissure. Coronal image B is taken at plane a. Sagittal image C depicts the approximate location of the 5 axial sections, beginning with the base plane e, that were averaged to create the frontal and parietal regions of interest. The shaded area in the sagittal image represents the approximate region of the supplementary motor area.

Controls Letter (C, F, and L) and category (animals) fluency

) 1.3

x Patient were defective. Although visual confrontation naming was e t

r Controls’ Mean and SD o 1.2 normal, the patient had difficulty retrieving the name of C

e n i an object when given its description. Spelling was also r 1.1 c l a impaired, even when using plastic block letters and C / 1.0 n o

i spelling aloud (both of which circumvented her motor g

e 0.9 R apraxia). The patient was able to produce slowly (

n o i 0.8 handwritten block letters, with occasional letter mal- t a z i l

i formation. t 0.7 U

e s

o 0.6 c

u APRAXIA TESTING l G 0.5 R-FA L-FA R-FP L-FP R-P L-P R-CP L-CP R-SMA L-SMA Region On command, the patient performed successfully only 2 of 10 intransitive gestures. She improved only mini- Figure 2. Fludeoxyglucose F 18 uptake in regions of interest, normalized by the mally when asked to imitate the examiner. Pantomime average of left and right calcrine cortex (patient vs healthy elderly controls, n=7). R indicates right; L, left; FA, frontal-anterior; FP, frontal-posterior; P, of transitive movement (use of tools) was likewise im- parietal; CP, caudate-putamen; and SMA, supplementary motor area. paired in both hands (1 of 8 correct). There was no im- provement with imitation, and only mild improvement tal flexibility in novel problem solving were normal on the with actual objects. During intransitive gestures, the pa- Wisconsin Card Sorting Test. Category Test errors were tient’s most frequent problem was her inability to inter- average for age and education (T score, 4520). Visuomo- nally configure the fingers on her hand. She was acutely tor tracking (Trail Making) was mildly slow (T scores, 32 aware of her errors, and many times used 1 hand to try and 35 for parts A and B, respectively29). Memory and vi- (unsuccessfully) to force her fingers into the correct con- sual perception were intact. Tactile perception (finger gno- figuration. In both intransitive and transitive gestures, sis and graphesthesis) was impaired bilaterally. Right-left she additionally experienced trouble orienting her hand orientation was normal. The patient performed simple ad- and arms correctly toward the imagined tool or object dition and subtraction with variable accuracy, and her score (eg, pointing at her mouth when making the shsh ges- on the mathematics section of the Wide Range Achieve- ture). In 6 of the 8 transitive gestures, her movements ment Test, third edition, was in the eighth percentile. were themselves incorrect. These configural and move- The Boston Diagnostic Aphasia Examination re- ment errors persisted with imitation. Content errors were vealed no deficit in auditory or written language com- also evident. When pantomiming the shsh gesture, the prehension, but showed marked motor language impair- patient blew on her finger as if blowing out a match. When ment. Speech was halting, labored, and marked by pantomiming scissors, she moved her mouth synergis- frequent literal and semantic paraphasias. Asked about tically to the rhythm of opening and closing the scissors her work, she offered the following response: (which she could not make her hand do). There was only 1 perseverative error in which she carried the move- Well, I was, ah re. . . tired before that, but ah, I worked, ah ments of one action into the movements of another. Her be . . . ah . . . at a . . . ah . . . let me think, at . . . ah . . . I . . . ability to recognize movements in the examiner, and to place where people, ah . . . a . . . cars . . . ah . . . in Michigan. I did I didn’t . . . I was in the . . . ah office, and ah working in discriminate between his accurate and poor perfor- a . . . on a . . . c . . . on a cc . . . cc . . . con . . . con . . . con- mance, was unaffected (4 of 4 and 4 of 4, respectively). tuter. And before that it was on a . . . I... before . . . I got The patient also had difficulty pantomiming and imitat- that mon . . . I was working in a . . . s . . . savings an a ing buccofacial gestures, although her performance was loa... loan. better than her limb movements (4 of 6 correct, most of

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Middle Frontal Gyrus Inferior Frontal Gyrus Inferior Frontal Gyrus Insula

Supramarginal Gyrus Angular Gyrus Angular Gyrus

Superior Parietal Superior Parietal Lobule Lobule A B C

Figure 3. Three sections (A through C) of the patient’s positron emission tomographic and coregistered proton density magnetic resonance images showing regions of relative hypometabolism. The inferior and middle frontal gyri fall in the anterior-frontal regions defined on the positron emission tomographic scan. In image B, note hypometabolism falling in the region of the angular and supramarginal gyri.

which were awkward and slow). She was, however, un- rietal lobule, in particular the supramarginal and angu- able to perform any of the oral agility items on the Bos- lar gyri, contain the visuokinesthetic motor programs ton Diagnostic Aphasia Examination to the minimum for skilled movement.7,10 number of trial repetitions (purse lips, open and close There are several explanations for why gesture com- mouth, retract lips, tongue to alternate corners of mouth, prehension might be preserved with parietal lobe dys- protrude tongue, and tongue to upper and lower teeth). function. First, gesture recognition and discrimination may simply be an easier task, and visuokinesthetic en- FUNCTIONAL IMAGING grams stored in the left inferior parietal lobule may not have degraded sufficiently to impair gesture comprehen- Positron emission tomography showed the patient to have sion.18 Alternatively, a more detailed examination of ges- fludeoxyglucose F 18 uptake in the distribution of the ture comprehension might have revealed comprehen- controls for R-FA, L-FA, R-FP, R-P, and CP bilaterally. sion defects. However, the imaging results are similar to The fludeoxyglucose F 18 uptake fell outside the distri- those of another case, described by Rapcsak et al.18 These bution of the controls in L-FP, L-P, and SMA bilaterally. authors also described a patient with a slowly progres- The left-right discrepancy in frontal and parietal re- sive ideomotor apraxia, severe biparietal atrophy, and gions appeared greater in the patient (Figure 2). Thus, widespread parietal hypoperfusion demonstrated with the patient’s left hemisphere showed greater loss than the single photon emission computed tomography. Despite right, and reduced fludeoxyglucose F 18 uptake was dis- this patient’s extensive reduction in parietal lobe per- tributed in a posterior frontal and parietal distribution. fusion, a comprehensive examination revealed well- Visual examination of the patient’s coregistered PET and preserved gesture comprehension and conceptual knowl- MRI scans showed focal reductions of fludeoxyglucose edge of limb movements. F 18 uptake in the region of the left angular and supra- Gesture comprehension may therefore be more dif- marginal gyri (Figure 3). Focal reductions were also pres- fusely distributed. Evidence for this comes from Bonda ent in the left insular cortex and the inferior precentral et al,30 who studied the regional cerebral blood flow of gyrus (Figure 3, A). healthy individuals observing meaningful human ges- tures. In addition to increased regional cerebral blood flow COMMENT in the , they found that regional ce- rebral blood flow increases in the ventral temporo- This 65-year-old woman with progressive speech and occipital cortex and prestriate cortex in the right hemi- motor difficulty had significant limb ideomotor and sphere, and the caudal , ventral buccofacial apraxia, but spared gesture comprehension. temporo-occipital cortex, and prestriate cortex of the left. A comprehensive neuropsychological examination This corresponds to nonhuman primate studies of re- showed that this occurred in the context of normal ciprocal connections between the inferior parietal lob- memory, mental flexibility, abstraction, language com- ule and visual areas (eg, TPO-2, TPO-3, prehension, and visuospatial perception. With absent TPO-4, MT, superior temporal sulcus),31-34 where cells focal structural lesions and global cognitive impair- respond selectively to forms of movement.35-39 ments to complicate interpretation of regional meta- Another account for the observed findings and pa- bolic dysfunction, PET revealed asymmetric reduced rietal lobe mediation of gesture comprehension could be fludeoxyglucose F 18 uptake, with greater loss in the that this patient’s apraxia results from dys- left FP and parietal regions. Focal metabolic loss spe- function, but reduced parietal fludeoxyglucose F 18 up- cifically involved the angular and supramarginal gyri, take is secondary to loss of afferent projections to the pa- which corresponded with the patient’s impaired tactile rietal lobe from premotor and supplementary motor perception, , and dyscalculia. These findings areas. 40,41 This does not provide a complete account of appear inconsistent with the theory that the inferior pa- the data in the present case, as the patient’s finger agno-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 sia, dysgraphesthesis, dyscalculia, and spelling impair- research suggests that the angular and supramarginal gyri ment strongly implicate dysfunction of the left inferior mediate gesture comprehension. Although the conclu- parietal lobule.42-44 Finally, represents the most fre- sions that can be drawn from a case study are limited, quently studied cause of apraxia. In both our patient and functional imaging in this and other work suggests that the one described by Rapcsak et al,18 apraxia was the re- gesture comprehension may be spared with significant sult of insidious disease. It is possible that functional brain functional disruption of the posterior parietal lobule. Fu- reorganization is different in disorders of sudden onset. ture research in apraxia should be targeted at develop- Other findings in this study are nonetheless consis- ing more systematic functional imaging studies that ana- tent with current anatomic models of praxis. Early stud- lyze extended networks of cortical and subcortical regions ies of regional cerebral blood flow and cortical record- that mediate action production and comprehension. Pa- ings show that SMA plays a key role in motor planning.45-47 tients with early symptoms of CBD may constitute an ad- In this patient, regions corresponding to the premotor vantageous population of study, since apraxic symp- area and SMA were more severely affected than the dor- toms may sometimes occur in the absence of other solateral . Buccofacial apraxia is fre- confounding cognitive disturbances.63 quently the result of frontal lesions, and in this case it may be secondary to reduced functional integrity in the Accepted for publication July 28, 1997. insular and inferior precentral cortex.12,13 In fact, This study was supported by the Physiologic Imaging Dronkers48 recently found that patients with speech Research Center of the Indiana University School of Medi- apraxia have lesions in the of the in- cine, the Indiana Alzheimer Disease Center (P30 AG10133), sula, which may coordinate oral motor planning and ar- and the Richard L. Roudebush Veterans Affairs Medical Cen- ticulation. ter, Indianapolis. The diagnosis of our patient is uncertain, but the We gratefully acknowledge the assistance of Vincent onset of symptoms and course resembles that of corti- Mathews, MD, the staff of the Indiana University PET fa- cobasal degeneration (CBD), a slowly progressive disor- cility, Marilyn M. Wagner, PhD, of Frazier Rehabilitation der whose initial symptoms include apraxia.49-55 There Center (Louisville, Ky) for portions of the neuropsychologi- are, nonetheless, differences between our patient and oth- cal test data, and Julie C. Stout, PhD, for valuable com- ers with confirmed CBD. Unlike our patient, all 30 cases ments on a previous version of the manuscript. of CBD studied by Rinne et al53 presented with limb ri- Reprints: David A. Kareken, PhD, Neuropsychology Sec- gidity 5 years after onset, and a substantial number ex- tion (RI 5999C), Department of Neurology, Indiana Uni- hibited dystonia (83%), myoclonus (57%), and alien limb versity School of Medicine, Indianapolis, IN 46202 (e- (50%). Some patients with neuropathological confirma- mail: [email protected]). tion of CBD, however, have delayed onset of the typical 56 motor features. Our patient also exhibited apparently REFERENCES normal fludeoxyglucose F 18 uptake in the caudate and putamen. 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