Pure apraxic agraphia: a disconnection syndrome after left subcortical stroke

Introduction: The dissociation between speech and writing was first described in the 19th century by Marce [1], Ogle [2] and Pitres [3]. Hodges described a patient with pure apractic agraphia without limb praxis or language disorder caused by a left frontal cyst [4]. The patient's writing was characterized by poor forms of the graphemes in the absence of spelling errors. Spontaneous writing and copying were equally impaired. The lesion of the patient extended from the superior surface of the left frontal lobe to the anterior horn of the lateral ventricle.

Case report: A 72-year-old right-handed female patient presented with difficulty of speaking and handwriting which had developed acutely 3 days prior to admission. She had previously had fast fluent handwriting, but now complained about having problems to initiate the correct writing movements. She now needed several attempts to write single words that looked untidy compared to her premorbid handwriting. She did not make spelling or syntactic errors, but her writing tended to become smaller towards the end of each word.

The patient was asked to copy, write to dictation and spontaneously. The tests were performed with the right-hand. Letter formation was poor, but when legible, letters were in most cases correctly selected and the spelling was unimpaired. Words, pseudo-words and numbers were equally affected. Drawing and reading were adequate. We diagnosed pure apraxic agraphia.

Discussion: Croisile et al. reported a patient with a haematoma in the left centrum semiovale who suffered from impaired grapheme production in the absence of limb apraxia [5]. In contrast to the patient described by Hodges [4] the lesion spared both frontal and parietal cortex. The lesion of our patient was located in the centrum semiovale in the vicinity of the caudate head, but did not involve it. Accordingly, our findings confirm the assumption presupposed by Croisile et al. [5], that apractic agraphia (like other forms of apraxia) can result from a selective disconnection of cortico-cortical (fronto-parietal) or cortico-subcortical pathways between left frontal cortex and the left basal ganglia.

1. Marcé LV. Paris: Compte-rendu de la Société de Biologie, 1856; 3: 93–115.

2. Ogle JW. Report of the MRC of St George's Hospital, London 1867; 83–122.

3. Pitres A. Revue de Médecine 1884; 4: 855–73.

4. Hodges JR. Cortex 1991; 27:469–73.

5. Croisile B, Laurent B, Michel D, Trillet M. JNNP 1990; 53:263–5.