J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.5.430 on 1 May 1981. Downloaded from

Journal of Neurology, Neurosurgery, and Psychiatry, 1981, 44, 430-432

Short report Pure agraphia and unilateral optic ataxia associated with a left lesion

S H AUERBACH AND M P ALEXANDER From the Neurobelhavioral Unit, Department of Neurology, Boston Veterans Administration Medical Center and Boston University School of Medicine

SUMMARY Impairment in visually guided reaching and pure agraphia are described in a patient with a left superior parietal embolic infarction. Identification of these disorders required specific but simple test procedures. Both disorders may reflect disruption of specific integrative functions of the superior parietal lobule. Protected by copyright. Unilateral impairment of visually guided hand noted difficulty inserting his car key into the ignition, movements (optic ataxia) and pure agraphia have difficulties manipulating food on to his fork, and each been described in association with posterior difficulty directing his hand and fork precisely. All of parietal lobe lesions. Optic ataxia probably results these problems occurred with his right hand only. He had no weakness, and he experienced no clumsiness from a lesion in pathways projecting anteriorly when using his right hand in non-visually related from the visual association areas to pre-motor tasks such as buttoning his clothes. Over the next centres.' Pure agraphia has been described with few days he experienced problems writing, particu- , posterior parietal lobe and other more larly in signing his name. He tended to swerve when diffuse brain lesions (reviewed by Hecaen and driving and had difficulty parallel-parking, but he Marie2; Hecaen and Albert3). We describe a could not identify a specific cause. He was admitted to patient with both unilateral optic ataxia and pure the Neurology service to evaluate these complaints. agraphia who had a discrete left posterior parietal On admission, general medical examination showed lobe lesion. The association of these disorders with no abnormality. Speech, auditory comprehension and repetition were normal. He could follow a 4-step such a small lesion suggests that they may share a pointing sequence. Reading aloud and for compre- common mechanism. hension was intact. He had great difficulty with writing http://jnnp.bmj.com/ and copying. Right-left orientation, finger naming, Case report and calculations were normal. Cranial nerve exam- ination was normal, including intact visual fields to The patient is a 56-year-old, right-handed mail-carrier confrontation. There was a mild upward drift of his with an eighth grade education. The patient was in outstretched right upper extremity with some pro- his usual state of health until several days prior to nation; tone co-ordination and strength otherwise were admission when he began to have difficulty sorting normal. Rapid alternating movements performed his mail. He usually did this by placing the letters under visual guidance with his right hand were poor. into their appropriate slots with his right hand accord- Deep tendon reflexes were bilaterally symmetrical on September 27, 2021 by guest. ing to the name on the address label. He was able to with flexor plantar reflexes. Sensory examination was read the name, but was not able to place the letters normal for pinprick, temperature and vibration. He reliably into the correct slot. His sorting errors went had minimal difficulties with position sense testing in to both sides of the correct slot. He was aware of his the right hand; on double simultaneous tactile testing errors and made attempts to correct them. He also he occasionally mislocalised on the right hand. An electroencephalogram was normal. A computed tomo- gram showed a small enhancing infarct in the left Address for reprint requests: Dr MP Alexander, Neurobehavioral Unit, Boston VA Medical Center, 150 South Huntington Avenue, superior parietal lobule, corresponding to Brodman's Boston, Mass 02130 USA. area 7 (fig 1). Carotid arteriography showed a distal Accepted 21 January 1981 branch occlusion of the left posterior parietal artery. 430 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.5.430 on 1 May 1981. Downloaded from

Pure agraphia and unilateral optic ataxia associated with a left superior parietal lobule lesion 431 designated hand. Each hand was used individually, pointing into both the right and left hemi-fields. There were no errors pointing into the left hemi-field with his left hand. There was some difficulty accurately reaching with either hand into its contralateral hemi- field. By comparison, reaching with his right hand into the right hemi-field was greatly impaired. When the task was repeated without fixation of gaze, the patient had no difficulty accurately touching the targets. Discussion Fig 1 Contrast enhanced computerised tomographic scan showing discrete enhancing lesion in left superior Our patient presented with two prominent prob- parietal lobule. lems: agraphia without other language disturb- ances and an impairment in visually-guided hand When re-examined two weeks later he reported con- siderable improvement in his co-ordination although his writing difficulties persisted. Motor and sensory examination showed no abnormality, but the patient had developed a marked preference for his left hand for common daily activities (holding a cup of coffee, pointing to objects, etc). Two weeks after admission, he was alert and oriented. Digit span was seven for- q

ward, three reverse. Spontaneous speech, auditory Protected by copyright. comprehension and repetition were intact. Reading aloud and for comprehension were normal. He could perform simple calculations. With more complex problems he had difficulty, became frustrated and re- fused to complete the problems. Right-left orientation, body part identification and praxis testing were nor- a mal. Copying of figures was sloppy, but he preserved the general configuration and many of the details. The Albert line-crossing test for neglect4 was nor- mal. More detailed tests of writing and hand use were /S performed. ii#-E~ I4/1T/fhi£ 7/S A)t1/cr- Writing (fig 2a and b) The patient's handwriting was bI untidy and poorly formed. He made frequent errors in with letter omissions, substitutions and repetition. The patient would cross lines or make -tt"-L- co loops in inappropriate places. Occasionally he would omit an entire word. When asked to spell orally these http://jnnp.bmj.com/ same words, he could usually do so correctly. Using -tj. the word list from the Wide Range Achievement Test he performed at a 6-6 grade leve'l with oral spelling and below a fourth grade level with written spelling. c He was able to spell correctly aloud many words that he coud not write correctly. These errors were present Fig 2 Samples of handwriting, showing the errors for spontaneous writing as well as for writing to described in the test. (a) Handwriting to dictation dictation and to copy. They were present in cursive taken from the Wide Range Achievement Test. Here and printed productions. The patient refused further the patient intended to write the words BETWEEN, on September 27, 2021 by guest. testing and left-handed writing samples were not CHIN, SPLIT and was able to do it correctly orally. obtained. (b) Spontaneous handwriting in response to a command Tests of visually guided reaching His initial com- to write about the weather. The patient intended to plaints suggested a disorder in visually-guided reach- write: THE WEA THER IS NICE TODA Y. He was ing. To pursue this, we administered a version of a able to spell orally all the words correctly. test described by Holmes,5 and modified by Levine, (c) Handwriting when asked to describe the Cookie Kaufman and Mohr.6 The patient was asked to fixate Theft Picture.'6 The patient intended to write: THE on the nose of the examiner. He was then asked to MOTHER IS DRYING DISHES, THE CHILDREN touch quickly the eraser tip of a pencil held some- ARE TAKING COOKIES. Again, he made no errors where in front of him. He used the index finger of a in the oral production of these sentences. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.5.430 on 1 May 1981. Downloaded from

432 S H Auerbach and M P Alexander movements (optic ataxia). The agraphia was in- lesions in the posterior superior dominant parietal itially considered a non-specific concomitant of a lobe may result in disordered written language in- clumsy hand, but further testing delineated its dependent of other language disturbances. specific nature. The optic ataxia was unusually im- portant because of his job as a mail sorter, but its References specific characterisation as impaired visually guided reaching became manifest only with special 1 Damasio AR, Benton AL. Impairment of hand testing.6 Both of these disorders might easily be movement under visual guidance. Neurology overlooked. (Minneap) 1979; 29:170-8. Pure agraphia is an infrequent result of focal 2 Marie P, Hecaen H. Agraphia: writing disorders brain lesions, but the few reported cases have associated with unilateral cortical lesions. In: areas: a small region Heilman KM, Valenstein E, eds. Clinical Neuro- involved one or two specific psychology, New York: Oxford University Press, in the left posterior frontal lobe7 8 or a small 1979. region in the left superior parietal lobe.9-11 Our 3 Hecaen H, Albert M. Human Neuropsychology. patient's lesion was in the latter area. Patients New York: John Wiley and Sons, 1978; 59-66. with pure agraphia from superior parietal lobule 4 Allbert M. A simple test of visual neglect. Neur- lesions have had the same complex writing im- ology (Minneap) 1973; 23:638-64. pairment as our patients including misspellings, 5 Holmes G. Disturbances of visual orientation. letter and word omissions, additions and repeti- Br J Ophthalmol 1918; 2:449-68, 506-16. tions, and most compellingly, oral spelling superior 6 Levine D, Kaufman KJ, Mohr JP. Inaccurate to written spelling. These cases may be uncommon reaching associated with a superior parietal lobe because isolated superior parietal lesions are rare. tumor. Neurology (Minneap) 1978; 25:556-61. 7 Pennfield W, Roberts L. Speech and Brain Mech- When caused by infarction, simultaneous involve- anisms, Princeton: Princeton University Press, Protected by copyright. ment of the will produce 1959. additional language disturbance.'2 8 Dubois J, Hecaen H, Marie P. L'agraphie pure. Impairment of hand movements under visual Neuropsychologia 1969; 7:271-86. guidance has been reviewed by Levine, Kaufman 9 Russell R, Espir MLE. Traumatic , and Mohr,6 and by Damasio and Benton.' Both Oxford: Oxford University Press, 1961. reports summarise the anatomical and electro- 10 Kinsbourne M, Rosenfield DB. Agraphia selective physiological data which support the importance for written spelling: an experimental case study. of the posterior superior parietal lobe (areas 5 and Brain Lang 1974; 1:215-26. 7) in the control of visually-guided movements in 11 Rosait G, DeBastiani P. Pure agraphia: a dis- the contralateral field. Its importance is twofold. crete form of aphasia. J Neurol Neurosurg Psy- of the superior parietal lobe chiatry 1979; 42:266-9. Firstly, this portion 12 Hier DB, Mogil SI, Rubin NP, Komros GR. has the structural capacity for cross-modal sen- Semantic aphasia: a neglected entity. Brain Lang sory integrations."3 Secondly, it immediately over- 1980; 10(l):120-31. lies the white matter pathways which connect 13 Montc'astle VB, Lynch JC, Georgepoulos A. visual association cortex (area 19) and motor Posterior parietal association cortex of the mon- http://jnnp.bmj.com/ association cortex (area 6).14 Both the impaired key: command functions for operations within supramodal spatial integration and the disconnec- extra-personal space. J Neurophysiol 1975; 38: tion of parietal and frontal association cortices 871-908. are relevant to the functional disorder in visually 14 Haxima R, Kuypers HCJM. Intrahemispheric guided reaching. cortical connexions and visual guidance of hand and Geschwind" that all cases and finger movements in the rhesus monkey. Chedru argued Brain 1975; 98:239-60. of pure agraphia are, in fact, not pure, but that 15 Chedru F, Gaschwind N. Writing disturbances in the agraphia is secondary to some other more acute confusional state. Neuropsychologia 1972; on September 27, 2021 by guest. basic deficit, commonly motor impairment or con- 10:343-53. fusion. We believe that pure agraphia in our case 16 Goodglass H, Kaplan E. The Assessment of may be partly produced by a general difficulty in Aphasia and Related Disorders. Philadelphia, visually guided hand movements and that small Lea and Febiger, 1972.