Casebook of Clinical Neuropsychology Edited by Joel E. Morgan Ida Sue Baron Joseph H. Ricker‘ders “gen. 5A, 202), tiate ase, 52 A Case of Corticobasal Syndrome John A. Lucas Corticobasal degeneration (CBD) is a neurode- generative disease characterized by progressive asymmetric rigidity and apraxia. Fitst described in 1967 by Rebeiz and colleagues, the early descriptions of CBD focused primarily on the neurologic manifestations of the disorder. Initial studies suggested that “mental faculties” in CBD Patients were relatively well preserved, although some patients developed dementia late in the course of the disease (Rebeiz et al., 1967; Riley, Lang & Lewis, 1990; Rinne, Lee, Thompson, & Martin, 1994). Over the past decade, however, there has been incteased recognition of the cognitive and behavioral manifestations of CBD (Kertesr, Martinez-Lage, Davidson, & Munoz, 2000; Graham, Bak, & Hodges, 2003; Boeve, 2005; McMonagle, Blair, & Kertesr, 2006). Spe- cifically, recent studies have demonstrated the onset of cognitive difficulties early in the disease Course, with a subset of patients demonstrating measurable cognitive changes prior to the onset of motor symptoms, Corticobasal degeneration is a rare disorder, ‘The exact prevalence and incidence rates are uunknown, but the estimated incidence ranges from 0.02 to 0.92 per 100,000 per year. Incidence estimates of corticobasal syndrome (described Hater) are highervand range from 4.9 to 7.3 per 100,000 per year (Togesaki & Tanner, 2000), Corticobasal degeneration is reportedly more prevalent in women. Disease onset is most often seen in patients from age 60 to 80 years, although autopsy-confirmed CBD has been seen in patients as young as age 45. Medications do not appear to alter the neurodegenerative process, and the disease typically progresses to death within 6-9 years of diagnosis. Differential diagnosis of CBD in clinical set- tings is challenging because the disease shares Pathology and phenotypic expression with other closely related disorders, including progressive Supranuclear palsy (PSP) and frontotemporal dementia (FID) (Boeve, Lang, & Litvan, 2003), Corticobasal degeneration and PSP are both akinetic-rigid syndromes in the Parkinson spec- {rum and both can present with cognitive symp- toms of progressive aphasia and/or executive dysfunction similar to the clinical presentation of FID. Patients with CBD may also develop fea- tures of posterior cortical atrophy (PCA). More. over, patients without movement disorder who initially present with signs of nonfluent aphasia, PID, or PCA may subsequently develop CBD symptoms. Postmortem studies confirm that Patients diagnosed with CBD based on clinical resentation during life commonly show a wide range of etiologies, including PSP, frontotempo- ral lobar degeneration, Pick disease, Creutzfeldt Jakob disease, Alzheimer disease (AD), motor neuron disease inclusion dementia, neurofila- ment inclusion body disease, and nonspecific neurodegeneration (Dickson et al,, 2002), Given that the clinical phenotype of CBD is not disease specific, some have suggested that the diagnosis of corticobasal syndrome (CBS) be used to: describe patients who demonstrate the characteristic clinical constellation of CBD symptoms during life, and that the diagnosis of 559560 “CBD” be reserved for neuropathologically con- firmed cases. This convention will be used in the case study presented here. Neurologic Symptoms ‘The neurologic presentation of patients with CBS is strikingly asymmetric, Early in the disor- der, patients may present with asymmetric limb rigidity and bradykinesia reminiscent of idio- pathic Parkinson disease. Lack of responsiveness to levodopa and presence of additional cortical symptoms, however, typically lead to reconsid- eration of the diagnosis. A hallmark feature of CBS is the presence of unilateral ideomotor limb apraxia. Limb kinetic apraxia, ideational apraxia, and buccofacial apraxia may be present in addition to ideomotor apraxia, but they are less commonly seen, In some cases, patients may develop an “alien limb syndrome? with the affected limb performing involuntary, often semi-purposeful, movements either in addition to or instead of a planned or willed movement. The patient is usually aware of the movement but is unable to control it. Patients may complain that their arm and/or leg “misbe- haves; fails to do what is intended, or feels asif it belongs to someone else, Other common motor features include limb myoclonus, asymmetric postural limb tremor, limb dystonia, dysphagia, and dysarthria (Boeve, 2005). Vertical gaze difficulty, severe postural instability, backward falls, cerebellar signs, and features of dysautonomia are not typically seen in CBS and may help differentiate the disorder from other akinetic-rigid syndromes such as PSP and multiple system atrophy (MSA). ‘Tactile perception of light touch and pinprick sensation remains intact in CBS, but evidence of cortical sensory loss is typically present, Formal examination typically reveals unilateral extinc- tions to double simultaneous stimulation and evidence of dysgraphesthesia and astereognosis Neuroradiologic Features and Gross Pathology On magnetic resonance imaging (MRI), patients with autopsy-proven CBD typically demonstrate: (2) atrophy of the posterior frontal cortex, supe- rior parietal cortex, and middle portion of the Corpus callosum on Tl-weighted images, (9) hypointense signal changes in the putamen gp T-weighted images, and (3) hyperintense signal changes in the motor cortex or sbcortic ‘matter on T2-weighted images (Josephs et al. 2004). Asymmetric findings may be observed with greater atrophy contralateral to the affected limb, but this is not always the case. When comm. pared to PSP patients, MRI findings in CBD Patients reveal greater cortical atrophy and less subcortical involvement (Josephs et al, 2008), Asymmetric hypoperfusion and hypometabo- lism involving frontoparietal cortices (with o¢ without basal ganglia involvement) have been Teported on single-photon emission computed. tomography (SPECT) and positron emission tomography (PET), respectively (Eckert et al, 2005; Koyama et al., 2007); however, these find. ings are not considered sufficiently sensitive or specific to CBD. Upon postmortem examination, gross pathol- ogy shows narrowing of cortical gyri, most nota ble in the superior frontal and superior parietal cortices (Dickson et al, 2002). Temporal and occipital lobes are relatively spared in cases where there were no clinical signs of dementia or aphasia. Patients with significant cognitive and Ianguage symptoms, however, often demonstrate additional involvement of inferior frontal and temporal lobes on autopsy. Additional findings on gross pathology may include volume loss of the cerebral white matter in the affected areas, thinning of the corpus callosum, and attenua- tion of the anterior limb of the internal capsule. ‘There may be flattening of the head of the cau- date; the thalamus may appear smaller than usual. The substantia nigra virtually always shows significant pigmentation loss. ‘The pons ‘and medulla remain relatively well preserved on. {gross inspection, t f 1 r ‘ 1 ! 1 ( 1 ‘ 1 ‘ Histopathology Swollen “achromatic” or “ballooned” neurons are important histopathological markers of CBD and are commonly found in the superior frontal and parietal cortices. ‘Tau immunostaining reveals a range of neuronal lesions in CBD, including (a) neurofibrillary lesions (ie. “corti- cobasal bodies”) in the locus ceruleus and sub- stantia nigra, (b) glial thread-like processes in5, at ne ee Sace A Case of Corticobasal Syndrome the neuropil of affected gray and white matter, (c) argyrophilic inclusions (i.e. “coiled bodies”) in oligodendroglia, and (d) neocortical astrocytic plaques with @ distinct annular array of tau-immunoreactive processes (Dickson etal, 2002). Cognitive and Neurobehavioral Features The constellation of cognitive symptoms typically observed in patients presenting with CBS is largely consistent with the known distribution of cortical and subcortical neuropathology. Hand- writing, drawing, copying, and other visuospatial construction abilities are typically compromised due in part to apraxia, It is also common, how- ever, for patients to demonstrate impairment on visuospatial tasks that have no significant motor demands (Graham, et al, 2003). As with most parkinsonian disorders, frontal- executive dysfunction is typically observed on formal neuropsychological evaluation. Studies demonstrate impaired performances on mea- sures such as the Wisconsin Card Sorting ‘Test, Trails B, Stroop Interference, and verbal fluency tasks (Pillon et al., 1995; Van Voorst et al, 2008). ‘The frontal deficit in CBS, however, is typically not as severe as that seen in PSP. Patients with corticobasal syndrome may ini- tially present with anomia and progressive non- fluent aphasia, including slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, phonological substitutions, agramiatism, and/or telegraphic language output (McMonagle et al, 2006). A recent study by Murray and colleagues (2007) reported that nearly one-half of autopsy-proven CBD patients demonstrated effortful speech upon initial examination and one-third demon- strated evidence of word-finding problems on formal testing. Nearly 90% of all patients in this series developed signs of anomia and nonfluent aphasia during the subsequent course of illness, Patients with CBS may also demonstrate pro- gressive spelling impairments that are unrelated to handwriting difficulty. Early in the disease, spelling errors tend to be phonologically plausi- ble, whereas later in the illness errors are more Likely to be phonologically implausible (Graham et al, 2003). There are also several reports in the 561 literature regarding the presence of calculation deficits. Ina series of autopsy-proven CBD cases, approximately one-fourth of patients presented with acalculia at the time of diagnosis (Murray et al, 2007). Those without initial difficulty, how- ever, did not go on to develop calculation prob- lems later in the illness. Basic attention span and episodic memory remain relatively well preserved in CBS, When memory deficits are observed, they are typically seen later in the disease process and are gener- ally mild in comparison to those of patients with AD. The nature of memory problems in CBS appears to reflect poor use of strategic processes during encoding and retrieval, and thus tend to bbe more suggestive of frontal-subcortical system involvement rather than medial temporal lobe dysfunction (Graham et al, 2003). Depression, apathy, irritability, and agitation are common features of CBS, A recent archival study of patients with pathologically confirmed CBD found evidence of three distinct neurobe- havioral syndromes, including depression, obsessive-compulsive behaviors, and a frontal- type behavioral disorder characterized by impul- sivity and disinhibition (Geda etal., 2007). Visual hallucinations were not present in any of the Patients studied and can help differentiate CBS from Lewy body dementia, where visual halluci- nations are common The Case of D. L. A 58-year-old, right handed, white female with 12 years of formal education was referted by her primary care physician to evaluate complaints of memory and expressive language dificulties The patient and her husband had been noticing a mild progression of these difficulties for 2 years, Her words would often “come out wrong” either in a stutter or with incorrect pronunciation, Although initially infrequent and limited to long or low-frequency words, by the time she pre- sented for evaluation the problem was occurring more often and affecting more common words. She also reported feeling less attentive and less able to remember things that people told her. In the year following the onset of her cognitive symptoms, the patient also began to notice the gradual onset of motor difficulties. She complained of occasional lack of control of the right hand,562 stating that it would “misbehave” and do some- thing other than what she intended it to do. Her handwriting had also become progressively slower and less fluid. By the time she presented for evaluation, she had begun to use her left hand for tasks that she would have normally per- formed with her right hand. She felt slower and less steady in her gait, but had not suffered any falls. She reported no tremor, urinary inconti- nence, or visual hallucinations at the time of the evaluation, With regard to activities of daily living, the Patient reported giving up some of her responsi- bilities in managing. the household finances because of difficulty writing checks and perform- ing math calculations. She was managing her medications independently and driving without significant difficulty, although her husband had noticed some slowing in her reaction time. Appetite was unchanged and she reported normal nighttime sleep, with no evidence of apneic episodes, dream enactment behavior, or abnormal movements during sleep. She reported having good energy in the morning, but she would typically become fatigued as the day pro- sgtessed. Her symptoms worsened in parallel with increased fatigue, but there were otherwise no reported fluctuations in her cognitive abilities. She reported being in good spirits at the time of the assessment, but her husband noted a 1-2 year history of increased apathy, He stated that the patient had once been “the life of the party” but was no longer initiating conversation or activities. Although she would interact with others at their initiation, she no longer seemed motivated to seek out interpersonal contact. ‘The patients past medical history included hypertension and high cholesterol, both of which were well controlled. Medications included Plavix and Lipitor, Family history was negative for demen- tia, movement disorder, or vascular disease Prior to her neuropsychological assessment, the patient was evaluated by a staff neurologist who found evidence of mild, largely asymmetric ‘motor and sensory deficits in the right upper extremity, including limb rigidity, bradykinesia, action myoclonus, agraphesthesia, astereogno- sis, and extinctions to double simultaneous stimulation. No resting tremoi, gross miuscle weakness, axial rigidity, postural instability, or gaze abnormality was noted. The radiologist read Neurodegenerative Disorders the MRI as unremarkable; however, the neurolg. Sist noted evidence of left greater than right Pos: terior frontal lobe atrophy. On the day of her neuropsychological evalua. tion, the patient presented as a neatly dressed well groomed, alert, and fully oriented woman who appeared her stated age. Verbal output during conversation was mildly slow and hei. fant, with occasional imprecision in articulation (eg. “fish” for “fist”). Casual Sait revealed reduced arm swing on the right, with the arm held extended at the elbow. Mild lack of coordi. nation was observed in the right hand daring writing, drawing, and manual tasks and at times she would switch to using her lefthand. Response times were slow. Vision and hearing appeared adequate to perceive test stimuli. She was pleas. ant throughout the session and did not appear concerned by cognitive or motor difficulties, She appeared to put forth adequate effort and results were considered valid estimates of her current cognitive status, Examination Results General intellectual functioning was low average for the patients age, as measured by the third edition of the Wechsler Adult Intelligence Scale. (WAIS-IDG Table 52-1). Verbal intellectual abil ity was average for her age and consistent with premorbid estimates based on demographic variables and word reading skill. Nonverbal intellectual ability was impaired and represents cognitive weakness, with the magnitude of the observed 19-point discrepancy between VIQ and PIQ being found in less than 10% of the WAIS-II standardization sample. Examination of WAIS-III factor scores revealed average verbal knowledge, low-average working memory, bor: derline perceptual organization ability, and impaired processing speed. ‘The remainder of the neuropsychological test results are presented in Table 52-2. On language assessment, the patient was able to follow single and multiple-step auditory verbal commands accurately on the Token Test. Expressive vocabu- lary was average on the WAIS-IIl. Mild slurring, hesitancy, and phonemic substitutions were observed on repetition of multisyllabic words and longer sentences from the Boston Diagnostic Aphasia Examination (BDAE-3). Performance on wA Case of Corticobasal Syndrome Table 52-1. Premorbid and Intellectual Functioning Wechsler Test of Adult Reading (WTAR) Standard Score Demographics-Predicted Score Estimated VIQ WAIS-IIL VIQ=91 PIQ=72 FSIQ= 80 Vocabulary 563 Age SS —_—-Ww™——_Asess_ 9 Similarities 8 Arithmetic 6 Digit Span u Information 10, Letter-Number Sequencing 7 7 Picture Comp 16 8 Digit Symbol 4 3 Block Desi 13 5 Matrix Reasoning 5 6 Symbol Search 2 5 the Boston Naming Test was low average for her age and education (51/60), with seven additional correct responses produced when phonemic cues were provided. Occasional phonological substitution/transposition errors were noted in her BNT responses (eg, “agraspagrus” for aspara- 84s, “omnicron” for unicorn), On both repetition and naming tasks, the patient would readily rec- ognize when she was making errorsand she would spontaneously attempt to correct them. Genera- tive naming to phonological cues and semantic categories was impaired, She demonstrated low-average performance on the WAIS-III Picture Completion subtest and mild difficulty judging the spatial orientation of Tines. Clock drawing was intact, both to command and copy. Her copy of a complex figure, however, ‘was impaired, with evidence of poor organization and mild spatial distortion, Cognitive processing speed was variable, Speeded word reading and color naming on the Color-Word Interference subtest of the Delis- Kaplan Executive Function Scales (I0-KEFS) were within normal limits, whereas speeded word gen- eration on verbal fluency tasks was impaired. Performances on WAIS-III Symbol Search and Digit Symbol subtests were impaired. On the D-KEFS Trail-Making subtest, visuomotor sequencing speed fell in the low-average to mildly impaired range Auditory-verbal attention span was average. Her ability to mentally arrange sequences of num. bers and letters was low average. In contrast, she demonstrated impairment on measures of mental calculation, divided attention (D-KEF$ ‘Trail Making Condition 4), and inhibition of a prepo- tent response (D-KEFS Color-Word Interference Condition 3). She demonstrated mild learning inefficiency on the California Verbal Learning ‘est (CVLT-ID She freely recalled 8/16 words following a 20- minute delay, which was also mildly impaired, but reflected relatively intact retention as com- pared to her performance on the final learning trial (9/16) and her short-delayed free recall (8/16). On recognition testing, she correctly iden- tified 11/16 target words, with no false-positive errors. Immediate and delayed memory for Logical Memory stories and Visual Reproduction figures on the Wechsler Memory Scale (WMS-III) Were average for her age, with above-average retention over time.1 564 Table 52-2. Neuropsychological Test Data eae Raw ‘Token Test Boston Naming Test COWAT Semantic Fluency Judgment of Line Orientation Clock Drawing Rey-Osterreith copy DKEFS Trail Making Condition 1 Condition 2 Condition 3 Condition 4 Condition 5 DKEES Color-Word Interf, Condition 1 Condition 2 Condition 3 Condition 4 ‘Wisconsin Card Sorting Perseverative Responses Categories = 4 Set Losse: Frontal Assessment Battery Similarities Lexical Fluency Motor Series Programming Conflicting Instructions Go-No-Go Environmental Control Total Wechsler Memory Scale Logical Memory I Logical Memory IL Percent Retention Visual Reproduction 1 Visual Reproduction I Percent Retention, cviT-2 ‘Trial 1-5 Short Delay Free Short Delay Cued Long Delay Free Long Delay Cued Recognition Hits False Positives 44/44 51/60 20 29 15/30 22/36 33 60 55 164 51 35 25 80 19 R 3/3 13 U3 3/3 3/3 3/3 ug 37 2 85 76 6 Percentile 75 18 5 9 4 wal NIA NIA NIA NIA NIA NIA NIA Neurodegenerative Disorders = Verbal abstract reasoning on WAIS-IIT Simi: lavities was low average for her age, Nonverbal conceptualization as measured by WAIS.IH Matrix Reasoning was mildly impaired, She scored in the borderline range on the Frontal. Assessment Battery, with points lost for reduced lexical fluency and difficulty acquiring Lurigs 3-step hand sequence. She demonstrated diff culty maintaining cognitive set on the Wisconsin Card Sorting test. s ‘The patient denied clinically significant symp. toms of depression, obtaining a score of 3 on Beck Depression Inventory (BDI-1), Discussion Results of this neuropsychological examination, reveal a pattern of cognitive deficits that are largely consistent with subcortical and frontal system dysfunction, Namely, there is evidence of psychomotor slowing, retrieval difficulties on naming and list-learning tasks, and executive deficits on D-KEFS subtests, Wisconsin Card Sorting Test, and Rey-Osterrieth copy (ie, poor organization). Behaviorally, the patient’ hus- band reported reduced spontaneity and initia: tion, and the patient did not appear distressed by. her problems during the test session, suggesting some degree of anosognosia. A In addition to her frontal-subcortical deficits, the patient also demonstrates language expres: sion difficulties, both during conversation and on repetition and naming tasks. The quality.of her impairments suggests a mild apraxia’ of |” speech and/or progressive nonfluent aphasia. The patient's cognitive dysfunction was of insidious onset and progressed gradually for 2 years prior to her neuropsychological evalua: tion, and she developed an asymmetric extra: pyramidal and cortical sensory disorder over this period of time. Taken together, the patient’ clini- cal presentation, neurologic exam, neuroimaging results, and frontal-subcortical features of the |) | neuropsychological profile suggest a neurodegen- erative disorder in the Parkinson spectrum. ‘The constellation of symptoms and the report of speech/language disturbance as an initial feature are consistent with a clinical diagnosis of CBS. The diagnosis wouldbe further supported ifthe patient proves untesponsive toa trial of levodopa.or A Case of Corticobasal Syndrome With regard to other Parkinson-spectrum dis- orders outside the corticobasal syndrome, the absence of hallucinations, fluctuating alertness, REM-sleep behavior disorder, or characteristic visual spatial impairments on neuropsychologi- cal testing argues against a diagnosis of Lewy body dementia. Likewise, the absence of promi- nent cerebellar or autonomic symptoms lowers the likelihood of MSA. Patients with frontotem- poral dementia with parkinsonism associated with mutation on chromosome 17 (FTDP-17) ‘may present with apathy and (less commonly) signs of dystonia such as demonstrated by this Patient; however, the hallmark behavioral change of marked disinhibition is not present. Moreover, the absence of FTD-type dementia and/or par- Kinsonism in the patient’ first-degree relatives effectively rules out this hereditary disease. Given the clinical features of this case, it is tempting to go beyond the “CBS” nomenclature and consider a more specific diagnosis of “CBD” With the extrapyramidal signs being asymmet- ric rather than symmetric and the absence of vertical supranuclear gaze palsy or backward falls, one might feel confident in ruling out PSP as an etiology. Similarly, the patients parkin- sonism, intact memory retention, and absence of muscle wasting may be considered sufficient evidence to rule out disorders such as fronto- temporal lobar degeneration, Alzheimer disease, and motor neuron disease, respectively. As noted earlier, however, there is significant neuropatho- logic heterogeneity underlying the classic “CBD” phenotype, resulting in poor positive predictive Value for underlying CBD pathology when the diagnosis is based on clinical presentation alone As such, we cannot confidently rule out PSP or other disorders within the CBS spectrum, as any number of pathologies may ultimately be identi- fied upon postmortem examination, Beyond diagnostic decision making, the neu ropsychological assessment provides valuable information for the practical management of the Patient's symptoms. ‘The patient’s slowing and executive dysfunction suggest that driving safety may be compromised. If she is reluctant to vol untarily abstain from driving, an objective driv- ing evaluation would be recommended through a local rehabilitation facility or department of motor vehicles office 565 With regard to medication managernent, she should be supervised to ensure that she is taking her medications in accordance with her prescribed regimen. She may also benefit from changes in her medication from pills to elixirs if she develops swallowing difficulty as her disease progresses. Referral for evaluation by a speech and lan- guage therapist may help attenuate the patients deteriorating communication skills. Recent stud- ies of patients with progressive nonfluent aphasia suggest that speech and language therapies may provide some protective benefit and slow the Progression of anomia over time. ‘Treatment efforts are most beneficial early in the disease, when semantic knowledge and episodic memory are relatively well preserved and can support new learning. As the disease progresses, speech and Janguage therapies may be implemented to teach the patient alternate means of functional com- munication (e.,, augmentative communication devices, functional communication boards). ‘The need for positive supportive measures should also be evaluated. Assessment of the home environment, evaluation of physical and occupational therapy needs, benefits of exercise programs, and other measures to optimize the Patient’ level of functioning should be explored through appropriate referrals, Given the relatively poor prognosis associated with a diagnosis of CBS, counseling is recom- ‘mended for the patient’ family and care providers ‘The patient’ apathy and possible anosognosia, as reported by her husband and observed during the neuropsychological assessment, can be beneficial to her, but her husband and family will likely need help adjusting to and coping with the diagnosis. Education regarding the cognitive and behavioral changes associated with the patient’ clinical syn- Arome, as wel as discussion of effective strategies for managing these changes will be essential in helping the patient and her family. Referral for practical education for managing physical changes associated with disease progression would also be helpfal. Information about national support orga- nizations Association for FrontotemporalDemen- tias, Worldwide Education and Awareness for Movement Disorders, etc.) and local caregiver support groups should be provided, Finally, repeating the neuropsychological stud- ies annually can help gauge the momentum ofesa coisceetRnisa coca 566 disease progression over time, revisit functional recommendations, and guide decisions regard- ing the level of care required by the patient. References Boeve, B, Lang, A., & Litvan, I. (2003). Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Annals of Neurology, 54, $15-S19. Boeve, B. (2005). Corticobasal degeneration: The syn- drome and the disease. In I. Litvan (Bd.), Atypical Parkinsonian disorders (pp. 309-334). Totawa, NJ: Humana Press, ‘Chand, P,, & Litvan, I (2008). Progressive supranuclear palsy and corticobasal degeneration: Similarities and differences. Future Neurology, 3, 299-307. Dickson, D., Bergeron, C., Chin, S., Duyckaerts, C., Horoupian, D., Ikeda, K,, Jellinger, K., Lantos, P. L., Lippa, C. F, Tabaton, M., Vonsattel, J. P, Waka- bayashi, K., & Litvan, 1, (2002). Office of rare dis- eases neuropathologic criteria for corticobasal degeneration. Journal of Neuropathology and Exper- imental Neurology, 61, 935-946. Eckert, T., Barnes, A., Dhawan, V., Frucht, S., Gordon, M. B, Feigin, A. S., Eidelberg D. (2005). FDG PET in the differential diagnosis of parkinsonian disor- ders. Neuroimage. 26, 912-921 . Geda, Y. E., Boeve, B. E, Negash, S., Graff-Radford, N, R., Knopman, D, §., Parisi, J. E., Dickson, D. W., Petersen R. C. (2007). Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. Journal of Neuropsychiatry & Clinical Neurosciences, 19, 77-80, Graham, N. L., Bak, I. H., & Hodges, J. R. (2003).Cor- ticobasal degeneration as a cognitive disorder, Movement Disorders, 18, 1224-1232. Graham, N. L., Zeman, A., Young, A.W, Patterson, K. ‘& Hodges, JR. (1999), Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action, Journal of Neurology, Newro- surgery, & Psychaitry, 67, 334-344, Josephs, K. A, ‘lang-Wai, D. E, Edland, $. D., Knopman, D. §, Dickson, D. W,, Parisi, J. E., Petersen, R. C., Jack, C. R., Jr, Boeve, B. (2004). Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration, A Neurology, 61(12), 1881-1884, Josephs, K.A.. Whitwell, J. L., Dickson, D. W, Bp B.E, Knopman, D. S, Petersen, R, C, Panay Jack, C. R. Jt: (2008). Voxel-based morpho autopsy proven PSP and CBD. Newobiaog, Aging. 29, 280-289 2 Kertesz, A.,Martinez-Lage, P, Davidson, W, 8 D. G. (2000). The corticobasal degeneration rome overlaps progressive aphasia and fy poral dementia, Neurology, 55, 1368-1375), Koyama, M., Yagishita, A., Nakata, Y, ™M,, Bandoh, M,, Mizutani, T. (2007), corticobasal degeneration syndrome, Ne 07. 49, 905-912. F McMonagle, P, Blais, M., & Kertesz, A. (3 Corticobasal degeneration and progressive ap Neurology, 67, 1444-1451. Murray, R, Neumann, M., Forman, M. S, Fa J Massimo, L., Rice, A., Miller, BL. Johnson, j Clark, C.M., Hurtig, H. L, Gomno-Tempini, Mf Lee, V. M-¥, ‘Trojanowski, J. Q. Gross ™M, (2007). Cognitive and motor assessment autopsy-preven corticobasal degeneration, Ne ogy. 68, 1274-1283. ay Pillon, B., Blin, J, Vidailhet, M, Deweer,B, Stig: As Dubois, B,, & Agid, Y. (1995). The neuropsyc logical pattern of corticobasal degeneration, ology, 45, 1477-1483. Rebeiz, J, Kolodny, E,, & Richardson, E. (1967) ticodentatonigral degeneration with neuron romasia: A progressive disorder of late adult Transactions of the American Neurological A tion, 92, 23-26, : Riley, D. E., Lang, A. E., Lewis, A. (1990) Cortical-bastl | ganglionic degeneration. Neurology, 40, 1208-12 Rinne, j.0, Lee, MS,, Thompson, RD. & Marsd CD. (1994), Costicobasal degeneration: A clinic Inik, RJ. Parisi, J. E., Ahlskog, E., Knopman,, Dickson, D. W,, Petersen, R. C., ‘Smith, G Josephs, K. A. (2008) Neuropsychological findin in clinically atypical autopsy confirmed corticobasal# degeneration and progressive supranuciear’ Parkinsonism & Related Disorders. 14, 376-378.