Pure Agraphia: a Discrete Form of Aphasia
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.3.266 on 1 March 1979. Downloaded from Journal ofNeurology, Neurosurgery, andPsychiatry, 1979, 42, 266-269 Pure agraphia: a discrete form of aphasia G. ROSATI AND P. DE BASTIANI From the Neurological Clinic of the University of Ferrara, Ferrara, Italy SUM MARY A 62 year old, right handed man developed a pure agraphia as the result of a left temporal lobe stroke. Isolated writing disturbances persisted for seven months until he had a second cerebrovascular accident resulting in total aphasia and right hemiplegia. A CAT scan obtained four months after the first episode showed a localised dilatation of the posterior portion of the left Sylvian cistern and patchy areas of low absorption in the left temporal lobe. The report supports suggestions that localised damage to the language area can produce a pure agraphia as the sole detectable disorder of language organisation. Pure agraphia-that is, the selective impairment limited to written codification resulting from of written communication-has rarely been des- ischaemic damage to a discrete part of the lan- cribed in the literature. Before 1960, a number of guage area. authors (Wernicke, 1903; Henschen, 1922; Sinico, Protected by copyright. 1926; Morselli, 1930; Mahoudeau, 1950; Mahou- Case report deau et al., 1951) reported cases of what they called pure agraphia which was caused by a lesion A 62 year old, right handed farmer, ex-telegraphist, of the lower end of the second frontal convolution with a grade school certificate, was admitted to the (F2). More recently, the assumption of a specific Cardiovascular Division of Ferrara City Hospital centre for writing in this part of the frontal lobe in February 1977 for a recent myocardial infarc- has been rejected (Leischner, 1969; Chedru and tion. On the morning of the eighteenth day in Geschwind, 1972). Since writing is a very complet hospital on rising from the chair, he felt weakness function combining dyshomogeneous elements, it of the right arm. A neurological examination has been suggested that agraphia is of no clinical showed only a slight right sided weakness of the value for localisation of damage (Leischner, 1969). face and arm. An EEG showed slowing of activity Some doubts have also been expressed concerning over the mid-regions of the left cerebral hemi- the existence of pure agraphia as an autonomous sphere. The right sided weakness recovered within entity (Kreindler and Fradis, 1968). However, even about two hours. In the afternoon, re-examination though numerous questions on the significance of showed no evidence of a motor deficit. The next isolated writing disorders have not yet been morning, when attempting to write a letter, he re- http://jnnp.bmj.com/ answered, further evidence has been accumulated alised he was writing nonsense. At this point he was recently (Hecaen et al., 1963; Dubois et al., 1969; transferred to the Neurological Clinic where he ap- Assal et al., 1970; Aimard et al., 1975) to indicate peared alert and oriented to person, place, and that isolated writing disturbances do indeed occur time. Past and recent memory functions and in brain-damaged subjects. Unfortunately, these knowledge of contemporary events, were intact. cases are characterised by a variety of both the Forms I and II of the Weschler Memory Scale nature and site of the cerebral lesions, and the were used to assess verbal and non-verbal memory. language area was not always involved (Dubois For his command of contemporary events we on September 26, 2021 by guest. et al., 1969). asked the patient to answer questions about on- In this report we describe the case of a right going local events, current agricultural prices (rele- handed man who developed a disorder strictly vant to his work as a farmer), and the like. Pupils, visual acuity, and visual fields were all normal. Ocular movements were full, with normal pursuit Address for correspondence and reprint requests: Dr G. Rosati, Clinica Neurologica dell'Universiti, Corso della Giovecca 203, 44100 and saccadic versions. Arteriosclerotic changes in Ferrara, Italy. retinal vessels were present. The other cranial Accepted 17 August 1978 nerves were intact. Motor examination showed 266 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.3.266 on 1 March 1979. Downloaded from Pure agraphia: a discrete form of aphasia 267 normal tone, strength, and co-ordination. The 50% of the time. The alignment of writing on the right and left sided tendon reflexes were equal. paper was correct. His ability to transmit Morse Primary sensory modalities, graphaesthesia, and code was tested with a tapping instrument, and he stereognosis were intact. was severely impaired in transmitting signals with On testing higher cortical functions, a striking either the right or left hand. The only signal defect in ability to write was detected. Spoken which was correctly transmitted was the one cor- speech and reading, as well as praxic, stereognostic responding to "end of the transmission." The tap- (including fingergnosis), and perceptive functions, ping of Morse signals was normally interpreted. were found to be normal. For tests of language, On a test of block writing, no significant differ- the subject was given the Language Test con- ences were found in comparison to handwriting, structed by the Neuropsychology Centre of the except that spelling appeared to be much less Neurological Clinic of the University of Milan affected in this case. Sometimes during writing (Copyright, Association for the Development of examinations he became discouraged and was un- Neuropsychological Research, Milan, 1974). This able to complete the task. On the Token test, test includes sections on expressive, receptive, and which was administered in the short form (De graphic abilities, as well as buccofacial apraxias. Renzi and Faglioni, 1975), his score was 29 out of Examples of spontaneous writing and writing to 36. On the Wechsler Bellevue Intelligence Scale dictation are shown in Fig. 1. There were numerous his verbal IQ was 95 and performance IQ 98, paragraphias, omission of vowels and consonants, placing him at the division between normal and transposition of letters in a word, repetition of impaired in receptive ability. letters and syllables, and repetition (perseveration) No special hearing disorders were detected of words in a sentence (with omission of the first audiometrically. and more often the last syllables). Sometimes, an During the next three weeks in hospital, the Protected by copyright. excess in the number of strokes in an individual clinical picture remained unchanged. A com- letter was noted (for example, "uomini"). Ability parison between protocols showed no significant to write numbers to dictation was preserved only improvement in neuropsychological performance. for individual numbers. Copied writing was rela- The EEG was repeated five times during this tively conserved and not "imitative." Transcription period and showed a persistence of localised of capital letters into lower case was correct about damage in the region of the left temporal lobe. 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In B, patchy areas of diminishled absorption are shown in the left temporal lobe. The patient refused carotid arteriography. He was previously as an isolated defect after a localised discharged after 10 weeks in hospital. lesion in the left perisylvian region. However, On follow up examinations in each of the four agraphia combined with other disorders, linguistic subsequent months, no changes were noted in his or mixed, is usually caused by a pathological neuropsychological test scores. process involving this region. Two months after leaving hospital, computerised Writing is a very complex function combining http://jnnp.bmj.com/ axial tomography was carried out which demon- linguistic, acoustic, praxic, kinaesthetic, visual, strated a left sided dilatation of the posterior por- and motor components (Luria, 1973; Simernits- tion of the Sylvian cistern and patchy areas of kaya, 1974). On the basis of the particular writing diminished absorption in the left temporal lobe components which are disturbed, various clinical (Fig. 2). These were interpreted as the result of a forms of agraphia have been described (Leischner, remote infarct in the territory of the middle 1969; Lhermitte and Gautier, 1969). In our case, cerebral artery. the writing disturbance would have to be classified Two months later, the patient sustained a second as aphasic agraphia. However. no other disorders on September 26, 2021 by guest. cerebrovascular accident, with sudden onset, gross of language organisation could be cletected. There- right hemiplegia, and total aphasia. He became fore, when categorising agraphia, the presence or comatose and died four days later. absence of other language defects as an indicator of whether the disorder is of aphasic or other Discussion origin, cannot be accepted as a general condition.