Dystonia in corticobasal degeneration

Dystonia in Corticobasal Degeneration

Zeba Vanek, MD and Joseph Jankovic, MD*

Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine,Houston, Texas, USA

AbstractOBJECTIVE: To characterize the clinical features, particu-

larly dystonia, in patients with clinically diagnosed or patho-logically proven corticobasal degeneration (CBD).

BACKGROUND: Although dystonia has been reported inmany neurodegenerative disorders, it has not been studied inCBD. Dystonia, often accompanied by painful rigidity andfixed contractures, is one of the most disabling features ofCBD.

METHODS: The medical records, imaging studies, and vid-eotapes of 66 patients who satisfied the clinical criteria of CBD,evaluated between 1988 and 1998, were reviewed. The occur-rence, nature, and distribution of dystonic features were ana-lyzed and correlated with other features of CBD.

RESULTS: Of the 66 patients with CBD, 39 (59.0%) haddystonia. The mean age at onset of initial symptoms was 63.9years (range 44–75). In 20 (51.0%) patients, dystonic symp-toms began in one arm, while 13 (33.0%) patients had initial

leg involvement. At least one arm was affected in 36 (92.0%)dystonic patients. Although 11 (28.0%) patients had leg dys-tonia, the leg was the predominant site of involvement in only1 patient. Only 12 (31.0%) patients had dystonia involving thehead, neck, or trunk in the course of the disease. The diagnosisof CBD was confirmed in all 4 patients who had autopsies.

CONCLUSION: In this large series of CBD patients wefound that asymmetric limb dystonia, particularly affecting onearm, is a common manifestation of CBD; dystonia may be theinitial manifestation of this neurodegenerative disorder. Axialor leg dystonia, without significant involvement of an arm, israre. There is no effective treatment for this relentless disorder,except for temporary relief of dystonia and pain, with localbotulinum toxin injections. © 2001 Movement DisorderSociety.

Key words: corticobasal degeneration; dystonia; botulinumtoxin

Since its first description in 1968 by Rebeiz et al.,1

corticobasal degeneration (CBD) has emerged as a dis-tinct, progressive neurodegenerative disorder with char-acteristic clinical and pathological features. The classicalclinical syndrome consists of a chronic and progressive,initially asymmetric, akinetic-rigid syndrome withapraxia. Supportive clinical features include dystonia,myoclonus, dementia, cortical sensory loss, the alienlimb phenomenon, and levodopa refractoriness. Al-though dystonia is believed to be a common occurrencein CBD, the true frequency, nature, and extent of dys-tonic manifestations in CBD have not been well-documented. Furthermore, some of the clinical observa-

tions on dystonic manifestations in CBD are either im-precise or not supported by post-mortem confirmation ofthe diagnosis.

CBD at times is difficult to distinguish clinically fromother parkinsonian syndromes, particularly at onset.2

Early in the course of disorders such as Parkinson’s dis-ease (PD), progressive supranuclear palsy (PSP), mul-tiple system atrophy, Alzheimer’s disease,3 and hemi-atrophy-hemiparkinsonism, misdiagnoses have resultedfrom misinterpretation of poor performance on manualtasks as due to apraxia, when in fact severe bradykinesia,rigidity, or dystonia was primarily responsible for themotor impairment. Clinical-pathologic studies also sug-gest that CBD is often under-diagnosed.2,4 We describethe characteristics of dystonia, found in 39 of our 66(59.0%) patients with clinically diagnosed CBD, 4 ofwhich were proven at autopsy.

METHODSThe medical records of 66 clinically diagnosed pa-

tients with CBD, and videotapes of 53, evaluated be-

A videotape accompanies this article.*Correspondence to: Joseph Jankovic, MD, Professor of Neurology

and Director, Parkinson’s Disease Center and Movement DisordersClinic, Department of Neurology, Baylor College of Medicine, 6550Fannin, Suite 1801, Houston, TX 77030.

Received 24 January 1999; Revised 5 July, 25 July, 8 August 2000;Accepted 9 August 2000

Published online 1 March 2001

Movement DisordersVol. 16, No. 2, 2001, pp. 252–257© 2001 Movement Disorder Society

252

tween 1988 and 1998 at the Parkinson’s Disease andMovement Disorder Center at the Baylor College ofMedicine, Houston, Texas, were reviewed. All 66 pa-tients met the following clinical diagnostic criteria forCBD5: (1) chronic progressive course, (2) asymmetriconset, (3) higher cortical dysfunction in the form ofapraxia, cortical sensory dysfunction, or alien limb, and(4) movement disorders in the form of dystonia, or my-oclonus, with an akinetic-rigid syndrome, resistant tolevodopa.

Dystonia was defined as a syndrome of involuntarymovements dominated by sustained muscle contractionscausing twisting and repetitive movements or abnormalpostures.6 Clinical aspects of the dystonic manifestationswere reviewed including the location, nature, accompa-nying clinical features, and response to treatment. Wethen compared patients with dystonia to those withoutdystonia to define any features of CBD that were typi-cally associated with dystonia. Demographic and clinicalinformation obtained at the initial visit as well as in thesubsequent follow-up visits was entered into a database.

Statistics:The statistical difference between the occurrence of

features such as myoclonus, tremor, chorea, alien limb,cortical sensory signs, dementia, depression, pyramidalsigns, speech, eye movement and gait abnormalities, be-tween patients with or without dystonia was analyzedusing the Pearson’s chi-square method. For continuousvariables such as age at symptom onset and duration ofsymptoms we used two-samplet-test assuming equalvariables.

RESULTS

DemographicsAmong the 66 patients with CBD, there were 44

(66.7%) women; 27 of 39 (69.2%) with dystonia werewomen (Table 1). The age at onset of CBD symptoms inour series ranged from 44–76 years (mean 64.7). There

was no significant difference in the ages of symptomonset between patients with and without dystonia. Theduration of symptoms in patients with dystonia versusthose without dystonia in our series, before the initialvisit was 4.2 years (range: 0.5–12) and 3 years (range:1–6 years), respectively (P<0.01). There was no familyhistory of a disorder resembling CBD, although 6(15.4%) patients gave a history of a family member witha parkinsonian disorder.

DystoniaAlthough accurate information on the onset of dysto-

nia, in relation to the onset of the other manifestations ofthe disease was not always available, all patients haddocumentation about dystonia before the initiation oflevodopa or other dopaminergic therapy. One patient hadbeen on trifluoperazine, given for psychotic depression,for 11 years before the onset of her symptoms, and an-other patient had taken haloperidol briefly, a few yearsbefore the onset of dystonia. While it is possible thatneuroleptic therapy increased the risk of dystonia inthese patients, we included the patients in our series be-cause they had focal dystonia and had no other featuressuggestive of tardive dyskinesia.

During the course of the disease, 37 of 39 (95.0%)patients with dystonia had dystonia affecting one or morelimbs, with an arm affected in 36 (92.0%) patients. Al-though 11 (28.0%) patients developed leg dystonia, inonly one patient, the leg was the predominant site ofinvolvement. Three (8.0%) patients had dystonia in all 4limbs. The typical dystonic posture in our series con-sisted of the affected arm adducted at the shoulder, theelbows and wrists flexed and the arm extending in frontor behind the body, with the dystonia becoming morepronounced while walking. The fingers were typicallyflexed at the metacarpo-phalangeal (MCP) joints, ex-tended or flexed at the proximal inter-phalangeal (PIP)and distal inter-phalangeal (DIP) joints, exhibiting vari-able degrees of fixed postures, with or without associated

TABLE 1. Corticobasal degeneration: demographics

Patients with dystonia Patients without dystonia Total

No. of patients 39 (59.09%) 27 (40.90%) 66 (100%)Gender Women 27 (69.23%) Women 17 (62.96%) 44 (66.66%)Handedness Right 37 (94.87%) Right 26 (96.29%) 63 (95.45%)Race Caucasians 35 (89.74%) Caucasians 26 (96.29%) 61 (92.42%)

Black 2 (5.12%) 2 (3.03%)Hispanic 2 (5.12%) Hispanic 1 (3.70%) 3 (4.54%)

Irish/Scottish/English descent 14 (35.89%) 13 (48.14%) 27 (40.90%)Family history of parkinsonism

(among first degree relatives) 6 (15.38%) 7 (25.92%) (19.69%)Mean age at symptom onset (years) 63.97 65.40 64.68 P < 0.23Mean duration of symptoms before

initial visit (years) 4.20 (0.5–12) 3 (1–6) 3.70 (0.5–12) P < 0.01

DYSTONIA IN CORTICOBASAL DEGENERATION 253

Movement Disorders, Vol. 16, No. 2, 2001

contractures. Contractures mainly affected the joints ofthe upper extremity in 22 (56%) patients, mostly thewrist and the fingers (19 patients, 49%). Eleven (28%)patients also had contractures in the shoulder joints, and6 (15%) had contractures affecting the ankles. Pain ac-companying dystonia was seen in 16 (42%), and thismostly occurred in the dystonic hand. Variable degreesof rigidity, particularly affecting the dystonic limb, wasseen in all patients.

Overall 17 of the 39 with dystonia (44.0%) cases ex-hibited adduction of the arm at the shoulder, 25 (64.0%)had flexion at the elbow, 20 (51.0%) ha flexion at thewrist, and 18 (46.0%) had flexion at the MCP joints.Seven (18.0%) patients also had the thumb, index andmiddle fingers extended, and the 4th and 5th fingersflexed. Eleven (28.0%) patients in our series had foot orleg dystonia. Of these 7 (17.9%) had unilateral leg in-volvement, and 4 (10.0%) had dystonia in both legs. Wefound the hips to be rotated in 3, and flexed in 2 patients.The knees were dystonically flexed in 4 and extended in1 advanced case. The dystonic posture in the feet resultedin foot inversion in 10 (26.0%) patients, but none hadfoot eversion. The foot was flexed in 8 (21.0%), ex-tended in 1 patient; the toes were flexed in 4 and ex-tended in 4 other patients.

Only 12 (31.0%) patients had dystonia involving thehead, neck, or trunk during the course of the disease; 10of these patients had associated limb dystonia. The cer-vical dystonia consisted of laterocollis in 7 (18.0%), ret-rocollis in 2, anterocollis in 1, and torticollis in 2 pa-tients. Only two patients had blepharospasm, none hadoro-mandibular dystonia, and 9 (24.0%) patients hadscoliosis, lordosis or kyphosis.

In the 39 patients with dystonia, 29 (74.0%) developedfixed postures mainly affecting the upper extremities, but6 (15.0%) had contractures affecting the ankles. Painaccompanying dystonia was present in 16 (42.0%), andthis mostly occurred in the dystonic hand. Variable de-grees of rigidity, particularly affecting the dystonic limb,was seen in all patients.

Other Features

In addition to dystonia, we found myoclonus in 39 ofthe 66 (59.1%) patients with CBD; 24 (36.4%) patientshad co-existent dystonia and myoclonus, usually involv-ing the same distribution. Total of 20 patients (30.3%)had tremor and 4 (6.1%) exhibited chorea. Other featuresincluded the alien limb phenomenon in 34 (51.5%), cor-tical sensory signs in 39 (59.1%), dementia in 15(22.7%), depression in 26 (39.4%), dysphagia in 11(16.7%), dysarthria in 49 (74.2%), oculomotor abnor-malities in 44 (66.7%), gait abnormalities in 62 (93.9%),

and pyramidal signs in 24 (36.4%). Statistical compari-son showed that the frequency of these associated fea-tures was similar in patients with and without dystonia.

Imaging StudiesAlthough all patients in our series had an MRI of their

brain, the results were available to us in 48. We foundmild-to-moderate generalized atrophy in 23 (82.1%) of28 dystonia patients, and in 15 (75.0%) of the patientswithout dystonia whom we evaluated. In 9 (32.1%) pa-tients with dystonia the atrophy was asymmetric and wasmore pronounced the side contralateral to the affectedside. Mild to moderately severe subcortical white matterchanges were similar in both groups (47.9% verses40.0%). Overall, MRI scans were not different in thosewith and without dystonia.

Response to TreatmentOverall, 32 patients received levodopa and 12 were

treated with dopamine agonists, but there was little or noobserved benefit. Additionally, 12 patients received clo-nazepam, 10 selegiline, and 6 received local botulinumtoxin injections during the course of their illness. Twopatients had transient improvement of their myoclonuswith clonazepam. Of the 6 patients who were treatedwith local botulinum toxin injections into dystonic andpainful areas, two had marked improvement of the dys-tonia and pain, and 4 had mild to moderate improvement.There were no complications attributed to botulinumtoxin therapy.

Natural HistoryOf the 39 patients with dystonia in our series, 15 had

follow-up evaluations ranging from 3 months to 9 years(average: 3.1 years). Seven (18.0%) patients progressedto an akinetic-rigid-dystonic stage. Of the 6 cases ofclinically diagnosed CBD who died, 4 underwent a post-mortem evaluation that confirmed their diagnosis. Ofthese, 3 had dystonia.

DISCUSSIONDystonia as the initial presentation of PD is well rec-

ognized, but this movement disorder often heralds theonset of other neurodegenerative diseases, includingCBD.7-11 Dystonia was first reported in the original de-scription of CBD by Rebeiz et al in 1968.1 Subsequentreports suggested that dystonia is commonly associatedwith CBD, but there have been no epidemiological stud-ies addressing the incidence or prevalence of CBD ingeneral, or dystonia in CBD, in particular.12 In most suchseries, the occurrence or description of dystonia is eithermentioned very briefly, or omitted altogether. Overall,the frequency of dystonia in CBD in most clinical series

Z. VANEK AND J. JANKOVIC254


has been reported to range between 43–83.0%.1,6,7,13–20

Our findings confirm that dystonia is a common mani-festation of CBD; 39 of 66 (59.0%) patients had at leastone dystonic feature at some point during their illness.

The commonest initial symptom reported by patientswith CBD is an asymmetric progressive clumsiness anddifficulty using one limb, usually an arm,6,7,8although inone clinical-pathological study dementia was the mostcommon presentation.21 On further evaluation, thisclumsiness is usually due to ideomotor apraxia, brady-kinesia, or dystonia. In our series 59 (89.4%) patients hadonset of symptoms in one limb, with difficulty using anarm in 35 (53.0%). We did not find a statistically sig-nificant difference in the ages of symptom onset betweenpatients with and without dystonia, but in those withdystonia the duration of symptoms was on an average 1.2years longer than those without dystonia (P< 0.01) (Seetable). This suggests that dystonia is more common in themore advanced cases. We found no difference in anyother features when the two populations of CBD patients,with and without dystonia, were compared.

One of the chief limitations of our retrospective studyis that the diagnosis was confirmed at autopsy in only 4patients. It is well recognized that some patients withclinical features of CBD are later found to have otherneurodegenerative disorders, most notably PSP. Our re-sults, however, are comparable to a pathological series,although the sample sizes of these latter studies are con-siderably smaller. In one study of 30 patients, only 6 ofwhom were examined at autopsy, Rinne et al.7 foundearly asymmetric limb dystonia in 12 of 30 (40.0%) pa-tients, and subsequent dystonia in 25 of 30 (83.0%) pa-tients. In the largest series of pathologically establishedCBD cases reported by Wenning et al.,4 dystonia wasfound in 7 of 14 (50.0%) of the patients. Kompoliti et al.9

reported the clinical presentation and treatment outcomeof 147 patients from multiple centers diagnosed withCBD by movement disorder experts with no predeter-mined diagnostic criteria, of which 7 had autopsy con-firmation of the diagnosis. In this retrospective meta-analysis, dystonia was found in 105 (71.0%) patients.None of the reported series provided a detailed descrip-tion of the dystonia. One or more limbs was the mostcommon site for dystonia in our CBD population, withan arm affected in 36 (92.0%); only 12 (31.0%) patientshad dystonia involving the head, neck, or trunk duringthe course of the disease, 10 of these had associated limbdystonia. Although a common, and often the most dis-abling, symptom, there is little or no information in theliterature on the occurrence of contractures in CBD pa-tients. In our patients with dystonia, 29 (74.0%) hadfixed postures. The data presented above apply only to

those patients diagnosed clinically with the classicalmovement disorder of CBD. It is unknown how oftendystonia and contractures are seen in patients with thedementia, behavioral, and dysphasic predominant pheno-types.21

In addition to dystonia, patients with CBD often mani-fest other hyperkinetic movement disorders. Stimulus-sensitive myoclonus, one of the characteristic features ofCBD, has been reported to occur in 25.0–80.5% of pa-tients with CBD.22–25 We found myoclonus in 39(59.1%) of our CBD patients. Of the 39 patients withdystonia, 24 (61.5%) had co-existent myoclonus, and in23 (96.0%) of these patients, the myoclonus was presentin the limb manifesting dystonia. Nine (23.1%) cases ofCBD with dystonia in our series also had tremor in thedystonic limb, compared to 11 (40.7%) who had tremorin a limb without dystonia. Only 2 CBD patients withdystonia exhibited choreic movements, also seen in 2patients who did not have dystonia. All CBD patientswith or without dystonia had rigidity and bradykinesiathat was most pronounced in the dystonic limb. The alienlimb phenomenon has been described to range between42.8–50.0% of all cases of CBD.8,26–28In our series thealien limb phenomenon was seen in a total of 23 (59.0%)patients who had dystonia, 18 had an alien hand, and 5had an alien leg. This is compared to 11 (40.7%) patientswith an alien limb found in patients without dystonia. Innearly all cases, the alien limb phenomenon was seen inthe dystonic limb. We found that 26 (66.7%) patientswith dystonia had cortical sensory dysfunction comparedto 13 (48.1%) patients who did not have dystonia, butthis difference did not reach statistical significance.

MRI findings do not distinguish between patients withor without dystonia. Overall, an asymmetric pericentralcortical atrophy has been reported in about half of pa-tients who have had conventional MRI imaging.29–31Wefound mild to moderate generalized atrophy in 23(82.1%) of 28 dystonia patients and in 15 (75.0%) of thepatients without dystonia. In 9 (32.1%) patients with dys-tonia, the atrophy was asymmetric and more prominenton the side contralateral to the dystonia.

Relentless progression and ultimate death resultingfrom complications of immobility characterize the natu-ral history of CBD and the presence of dystonia does notappear to influence the natural history of the disease. Amedian survival from the onset of symptoms of CBD hasbeen reported to range from 2.5–12.5 years (mean7.9).5,30–32Of the 39 patients with dystonia in our series,15 had follow-up evaluations ranging from 3 months to9 years (mean: 3.1 years). Seven (18.0%) patients pro-gressed to an advanced, akinetic-rigid-dystonic stagecharacterized by an expressionless face with open mouth



and rigidly flexed limbs. The pathological examinationin our 4 cases failed to identify any markers of dystonia.The presence of pathology at many sites in the motorpathways makes it impossible to attribute the dystonia toa lesion at any one site, or draw pathophysiologic con-clusions.

The symptoms and signs of CBD respond poorly ornot at all to any form of therapy. In the series of 147CBD cases reported by Kompoliti et al.,9 an improve-ment in dystonia was apparently seen in 4 patientstreated with benzodiazepines, 3 with anticholinergics,and 2 with baclofen. Of the 9 patients treated with botu-linum toxin injections, 6 experienced improvement oftheir dystonia and the pain associated with it. Of the 39cases of CBD with dystonia seen at our center, 17 hadadequate follow-up data to assess the results of treat-ment. There was little or no benefit in any of the symp-toms in patients who were given a trial of levodopa withor without dopamine agonists. This is consistent with ourexperience and, in contrast to the report of Frucht et al.,33

we have not observed levodopa-induced dyskinesia inour patients. Transient, though often substantial, relief ofdystonia and pain was seen with local botulinum toxintherapy, and two patients had transient improvement oftheir myoclonus with clonazepam.

Our study constitutes the largest series of CBD pa-tients reported from a single center. Although a referralbias may have caused an accrual of a higher proportionof atypical cases, asymmetric limb dystonia, particularlyaffecting one arm, is a feature commonly reported inCBD. Dystonia involving the leg, head, neck, or trunk,however, is uncommon. There were no significant dif-ferences in demographic or associated features, betweenpatients with or without dystonia. Patients with dystoniahave significantly longer duration of symptoms, suggest-ing that dystonia correlates with progression of the dis-ease. There is no effective treatment for this relentlessdisorder except for temporary relief of dystonia and painwith local botulinum toxin injections. Further clinico-pathologic studies are needed to elucidate the anatomicaland physiologic substrates of dystonia in this disorder.

Acknowledgments:The authors thank Lynn Ratkos, M.S.,R.N., for her technical assistance and the National ParkinsonFoundation for their support.

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LEGENDS TO THE VIDEOTAPE

Segment 1

This segment shows a 68-year-old woman exhibitingdystonic posturing of the left hand. She has flexion of thethumb, extension of the fingers at the DIP and PIP joints,and partial flexion at the MP joints. She also exhibits thealien limb phenomenon.

Segment 2

This segment shows a 75-year-old woman with dys-tonic posturing of the left upper extremity with develop-ment of contractures. She has prominent flexion of thewrist, extension of the index finger and flexion of theother fingers.



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Dystonia in corticobasal degeneration