Eur Neurol 1997:37:193-194 Crossed Mixed Transcortical Aphasia with Hypernomia Toskikatsu Fujiia , Atsushi Yamadoria . Reiko Fukatsuh, Tatsuji Ogawac, Kyoko Suzuki3 “Section ofNeuropsychology. Division of Disability Science. Tohoku University Graduate School of Medicine, Sendai, bDepartment of Neurology, Miyagi National Hospital. Miyagi. ‘ Department of Neurology. Sendai City Hospital. Sendai, Japan corpus callosum. A Tl-weighled MRI in the sagittal plane demonstrating hypointensity of the middle portion of the corpus callosum, the genu and splenium (D). Fig. 2. A-D Cranial MRI on May 9. 1994. Slight hypcrintcnsity on T2W1 in the sagittal image was observed at the middle portion of the corpus callosum (B) . 3 Truffcrt A, Dumas J-J, Dandclot J-B: Dvsconncxion interhcmisphcrique syndrome de Balint et troubles arthritiques persistants: maladie de Marchiafava-Bignami avec hémorragie de la substance blanche. Rev Neurol (Paris) 1996;152:174-180. 4 Bottomley P. Hart IIR Jr. Edelstcin WA.Schenck JF. Smith LS, Leuc WM. Mueller OM. Redington RW: Anatomy and metabolism of the normal human brain studied by magnetic resonance at 1.5 tesla. Radiology 1984; 150:441-446. Kazuya Yamashita. Third Department of Internal Medicine. Shimane Medical University. 89-1. Enya-cho, l/.umo. Shimanc (Japan) Short Reports 193 Downloaded by: King's College London 137.73.144.138 - 9/30/2018 1:09:05 PM Fig. 1. Cranial MRI on March 3, 1994. A T2-wcighted MRI in the axial (A) , the sagittal (B). and the coronal image (C) revealing hypcrintcnsity of the Mixed transcortical aphasia is a syndrome characterized by reduced speech, severe comprehension deficits, preserved ability of repetition and naming difficulty [1]. We report here a patient with crossed mixed transcortical aphasia who showed preserved naming and unique naming behavior. The patient was a 75-year-old, strongly right-handed [2] house­ wife with an eighth-grade education. She had no left-handed rela­ tives. She suddenly developed left hemiplegia and aphasia. Neurolog­ ical examination on day 27 revealed left homonymous hemianopsia with intact remaining cranial-nerve functions. She had a hemiplegia, hyperreflexia and pathological reflexes on the left side. We could not obtain reliable data of sensory function because of the patients' poor comprehension. CT scan on day 60 revealed a large infarct in the territory of the right middle cerebral artery' (fig. 1). EEG on day 67 showed generalized slowing throughout the right hemisphere. On neuropsychological examination, she was alert, but disori­ ented to tim e and place. She showed mild left unilateral neglect and constructional impairment. On testing of apraxia, she could not understand the examiner's commands. She seldom spoke sponta­ neously and tended to speak only when she was spoken to. Further­ more, when she spoke, she utilized the speech which had been spoken to her. She showed completion phenomenon. Her speech was fluent with normal rhythm but lacked affective elements. She repeated words perfectly and sentences fairly well with normal intonation. She showed occasional omitting of postpositional particles and verbal paraphasia. She showed little evidence of comprehending conversa­ tional speech and simple commands. Contrary to poor comprehen­ sion, her naming ability was quite good. Whenever objects, pictures or color tokens were presented, she started naming these stimuli one after another without waiting for the examiner’s instructions. She named most of these stimuli correctly. Some errors were mainly semantic paraphasic without category specificity. Naming on defini­ tion and generating words (verbal fluency) was not feasible because of the patient’s extremely poor comprehension. Reading aloud was excellent but reading comprehension was poor. She correctly wrote 3 names out of 8 visually presented stimuli in kanji on first examina­ tion. and 5 out of 5 on second examination. On both occasions, she never wrote in kana despite our commands. On writing to dictation, she could write words in kanji but never in kana. However, on anoth­ er occasion, she wrote down sentences correctly mixing kanji and kana scripts. The Standard Language Test of Aphasia [3] was admin­ istered twice. Table 1 summarizes the results. The most interesting feature of this crossed mixed transcortical aphasia was a preserved naming ability and its compulsive nature. There have been a few reports regarding spared naming without com­ prehension [4, 5]. These cases including ours have a theoretical importance. The fact that both auditory and reading comprehension were impaired, with preserved repetition, writing to dictation and Fig. 1. CT scan obtained on day 60 showing a large infarct in the territory of the right middle cerebral artery. Table 1. Results on subtestsofSLTA Day 27 - 34 Day 49 - 56 Auditory comprehension Words Sentences O' NE O' NE Repetition Words Sentences 10/10(100) 6/10(60) 10/10(100) 7/10(70) 18/20(90) 16/20(80) Naming of visually presented stimuli Reading aloud Kana words Kanji words Sentences 10/10(100) 10/10(100) 4/5 (80) 10/10(100) 10/10(100) 5/5(100) Reading comprehension Kana words Kanji words Sentences O' O' NE O' O' NE Writing the name of visually presented stimuli Kana words Kanji words 02 3/8(37.5) 02 5/5(100) Writing to dictation Kana words Kanji words Sentences 02 2/5 (40) 2/5 (40) O2 5/5(100) 3/5 (60) reading aloud, indicate that the phonological and orthographic word form systems were intact, but could only activate the phonological and orthographic output systems and not gain access to the seman­ tics. On the contrary, she could name and write the names of visually presented stimuli in kanji, suggesting that the visual form system remained accessible to the phonological and orthographic output sys­ tems. This patient’s profile provides evidence supporting the multi­ ple semantic systems hypothesis or the direct visual-lexicon route hypothesis [6]. She started naming without any command whenever objects, pic­ tures or color tokens were presented to her. We propose using the term ‘hypemomia’ for this compulsive and indiscriminate naming behavior. Like our patient, Heilman et al. [4] reported a case of mixed transcortical aphasia with intact naming who spontaneously named objects. Jacome [7] reported a mixed transcortical aphasic who showed repetitive, spontaneous whistling, and whistling in response to questions. He speculated that the symptom resulted from the release of the nondominant musical function by damage to the dominant hemisphere. Yamadori ct al. [8, 9] described two symp­ toms they termed ‘hypergraphia’ and ‘hypcrlalia’ as a right hemi­ sphere syndrome. They hypothesized that the exaggerated writing and talking activity might result from disinhibition of the left hemi­ sphere speech area from the right hemisphere. Hypemomia seen in the present case may be explained by a similar disinhibition hypothe­ sis because she had a large right hemisphere infarct which might have released the left hemisphere activity. References 1 Benson DF. Ardila A: Aphasia: A Clinical Perspective. Oxford. Oxford University Press. 1996. 2 Ollcr-Daurella L. Maso-SubiranaE: Scmiologiaclinica y EEG dc la latcralidad prefcrencial. 8th Congr Nacional Neuro-Psiquiatr Madrid. Libro de Ponencias, 1965. 3 Takeda K: Standard Language Test of Aphasia (SLTA): Detailed descrip­ tion of construction of aphasia test in Japanese (in Japanese). Neurol Sci (Tokyo) I977;21:t002-I()l3. 4 Heilman KM, Tucker DM. Valenstein E: A case of mixed transcortical aphasia with intact naming. Brain 1976;99:415-426. 5 Krcmin H: Naming and its disorders; in Bolter F. Grafman J (eds): Hand­ book of Neuropsychology. Amsterdam, Elsevier. 1988. vol 1. pp 307-328. 6 Shuren J, Gcldmachcr D. Heilman KM: Nonoplic aphasia: Aphasia with preserved confrontation naming in Alzheimer's disease. Neurology 1993: 43:1900-1907. 7 Jacome DE: Aphasia with elation, hypermusia. musicophilia and compul­ sive whistling. J Neurol Neurosurg Psychiatry 1984:47:308—310. 8 Yamadori A. Mori E. Tabuchi M. Kudo Y. Mitani Y: Hypergraphia: A right hemisphere syndrome. J Neurol Neurosurg Psychiatry 1986:49: 1160-1164. 9 Yamadori A, Osumi Y, Tabuchi M. Mori E, Yoshida T. Ohkawa S, Yoncda Y: Hypcrlalia: A right cerebral hemisphere syndrome. Behav Neurol 1990; 3:143-151. Toshikatsu Fujii. MD, Section of Neuropsychology, Division of Disability Science. Tohoku University Graduate School of Medicine. 1-1. Seiryo-machi, Aoba-ku. Sendai 980-77 (Japan) Phone: +81-22-717-7357, Fax: +81-22-717-7360 e-mail: fujii@mail.cc.tohoku.ac.jp 194 Short Reports Downloaded by: King's College London 137.73.144.138 - 9/30/2018 1:09:05 PM SLTA: Standard Language Test of Aphasia: NH: not examined: Kana: Japa­ nese characters for syllabic writing; Kanji: Chinese characters used in Japanese writing. Percentages in parentheses. 1 The patient could not understand the task and named all the pictures. 2 We asked the patient to write with Kana character, but the patient contin­ ued writing with Kanji.