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The Nature of Apraxia in Corticobasal Degeneration

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The Nature of Apraxia in Corticobasal Degeneration Journal ofNeurology, Neurosurgery, and Psychiatry 1994;57:455-459 455 The nature of apraxia in corticobasal J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.4.455 on 1 April 1994. Downloaded from degeneration R Leiguarda, A J Lees, M Merello, S Starkstein, C D Marsden Abstract shows a combination of parietal and frontal Although apraxia is one of the most fre- hypometabolism and an asymmetrical quent signs in corticobasal degeneration, decrease of fluorodopa uptake in the striatum the phenomenology of this disorder has and medial frontal cortex.378 not been formally examined. Hence 10 When fully developed, the disorder has a patients with corticobasal degeneration distinctive clinical picture, but the initial were studied with a standardised evalua- manifestations of basal ganglia dysfunction tion for different types of apraxia. To may be confused with other akinetic-rigid minimise the confounding effects of the syndromes. Therefore, findings such as primary motor disorder, apraxia was apraxia are essential for the clinical assessed in the least affected limb. diagnosis.' 57 Whereas none of the patients showed The pathological process in corticobasal buccofacial apraxia, seven showed degeneration affects many brain structures deficits on tests ofideomotor apraxia and involved in praxic functions (parietal and movement imitation, four on tests of non-primary motor cortex). Apraxia has been sequential arm movements (all of whom described in about 80% of patients, but the had ideomotor apraxia), and three on nature of this deficit has not been systemati- tests of ideational apraxia (all of whom cally examined.'- We have tested 10 patients had ideomotor apraxia). Ideomotor with corticobasal degeneration for the pres- apraxia significantly correlated with ence of buccofacial apraxia, ideomotor deficit in both the mini mental state apraxia, and ideational apraxia, and have cor- examination and in a task sensitive to related these phenomena with other cardinal frontal lobe dysfunction (picture features of the disorder. One important limi- arrangement). Two of the three patients tation in the evaluation of limb apraxia in cor- with ideomotor apraxia and ideational ticobasal degeneration is how to assess this apraxia showed severe cognitive impair- disorder in the presence of an akinetic-rigid ments. The alien limb behaviour was syndrome and involuntary movements. To present only in patients with ideomotor minimise this problem we only examined apraxia. In conclusion, ideomotor praxic functions in the less affected limb. apraxia is the most frequent type of apraxia in corticobasal degeneration, http://jnnp.bmj.com/ and may be due to dysfunction of the Patients and methods supplementary motor area. There is a Ten patients with corticobasal degeneration subgroup of patients with corticobasal were studied (table 1). Criteria for diagnosis degeneration who have a severe apraxia were the insidious onset and gradual progres- (ideomotor and ideational apraxia), sion of an asymmetric levodopa-resistant aki- which correlates with global cognitive netic-rigid syndrome, with or without other impairment, and may result from addi- basal ganglia features (dystonia, tremor), on September 27, 2021 by guest. Protected copyright. tional parietal or diffuse cortical dam- associated with signs of cortical dysfunction Raul Carrea Institute ofNeurological age. such as cortical sensory loss, alien limb, or Research, FLENI, apraxia. Buenos Aires, (7 Neurol Neurosurg Psychiatry 1994;57:455-459) Apraxia was evaluated by a neurologist Argentina R Leiguarda with a structured assessment. Gestures were M Merello scored as apraxic only when: (1) no abnormal S Starkstein Corticobasal degeneration is a slowly pro- involuntary movements (for example tremor, Institute ofNeurology, gressive disorder characterised by an asym- dystonia, myoclonus) coincided with the National Hospital for metrical akinetic-rigid syndrome, often motor response; (2) comprehension of the Neurology and Neurosurgery, Queen accompanied by other involuntary move- command was normal; (3) patients could Square, London, UK ments, particularly myoclonus, and associ- properly name all the objects tested; and (4) A J Lees ated with lateralised cortical signs such as the abnormal response was not corrected M Merello C D Marsden alien limb behaviour and sensory loss, as well after verbal instructions were repeated once. Correspondence to: as apraxia. Cognitive decline, when evident, Dr Ram6n Leiguarda, usually develops late in the course of the BUCCOFACIAL APRAXIA FLENI, Ayacucho 2166, ( 112) Buenos Aires, disease, and language disturbances are absent Patients were asked to perform 10 gestures Argentina. or mild.' Computerised axial tomography or (for example, stick out the tongue, blow out a Received 12 April 1993 MRI may demonstrate asymmetrical fron- match, sniff a flower), and were given three and in revised form 7 June 1993. toparietal cortical atrophy in the later stages points when the movement was accurately Accepted 28 July 1993 of the illness. Positron emission tomography performed; two points when correct only after 456 Leiguarda, Lees, Merello, Starkstein, Marsden Table 1 Clinicalfeatures in 10 patients with corticobasal degeneration J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.4.455 on 1 April 1994. Downloaded from Patients 1 2 3 4 5 6 7 8 9 10 Age (y) 69 61 78 78 58 78 69 72 69 61 Sex F M M F F M F F F M Handedness R R R R R R R R R R Disease duration (y) 4 3 5 4 3 3 5 5 4 6 Side of initial symptoms L L R L L R R L R R Akinesia, rigidity + + + + + + + + + + Postural instability, falls + - + + ± ± + ± + + Dystonia + + + + - + + - + + Myoclonus + - + - + + + - + Tremor (postural, action) - - - - - + + + + Cortical sensoryloss + + - - + + + - + Apraxia - - + + - + + + + + Visuoconstructive disability - - - - - - + + Alien limb - - + - - + + - + + Dysphasia - - - - - - + + Dementia - - - - - - + + Frontal dysfunction - - + + - + + + Supranuclear gaze palsy - - - + + - + - - + Hyperreflexia + + + - - - + + + + Primitive reflexes - - - - - + + + + + Babinski sign + + - - - - - - - + Dysarthria - + - - ± ± + - + Dysphagia - - - + + = present;-= absent. imitation; one point when the correct perfor- IDEATIONAL APRAXIA mance was preceded by pauses, or the overall Patients were asked to carry out three mul- gesture was acceptable though defective in tiple step tasks. For the first task, patients terms of amplitude, force and speed; and zero were given a flashlight and two batteries, and points when the gesture was incomplete or were asked to take the proper steps to turn on irrelevant, there was perseveration, or no oral the bulb. Thus patients were supposed to movements were carried out (total score 30). unscrew the bottom of the flashlight (step 1), introduce the batteries (step 2), and turn on IDEOMOTOR APRAXIA the light (step 3). For the second task, Patients were asked to perform with the least patients were given a closed padlock and the affected limb 10 intransitive movements (for corresponding key, and were asked to open example, wave goodbye, snap the fingers, the padlock with the key and close it again. hitch hike) and 10 transitive movements (for The steps were first, to introduce the key in example, use a hammer, use a screwdriver, the padlock and open it, second to take the use a key) in either verbal, visual or tactile key out, and third to close the padlock (the modalities, and on imitation. Maximum score padlock would not close with the key in). for intransitive movements was 20. For every Finally, patients were given a piece of paper, item, two points were scored for a correct an open envelope, and a stamp, and were http://jnnp.bmj.com/ performance; one point when the correct per- asked to mail the letter. The steps were first, formance was preceded by hesitation or a to fold the paper, second to put the paper repeated trial; and zero points when the into the envelope and close the envelope, and requested gesture was not or only partially third to glue the stamp to the envelope. The performed. For transitive movements, alternative sequences of glueing the stamp patients were given three points whenever the before folding the paper or putting the paper performance was appropriate for the object; into the envelope were also considered as cor- on September 27, 2021 by guest. Protected copyright. two points when the performance resembled rect. As patients had to use both hands to the correct one but included different types of perform these movements, we were not inter- errors (see later); one point when the move- ested in the correct performance of each step ment had a weak resemblance to the appro- in terms of the accuracy of individual move- priate gesture; and zero points when the ments, but in the overall performance of the gesture was so wrong or incomplete as to be task sequence. Therefore, if the patient had a unrecognisable, or there was a complete fail- motor deficit that precluded the execution of ure to carry out the command (maximum an action that required bimanual coordina- score for transitive movements: 30 points). tion (for example, unscrewing the bottom of Four types of errors were considered- the flashlight), the examiner assisted him or namely, content, temporal, spatial, and oth- her by holding the object or completing part ers.9 Delay in initiation, timing, and of the action after the sequence was pointed amplitude errors were not considered. out by the patient. Scoring was based on the Content errors, lack of response, and
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