Event Abstract

Transcortical motor aphasia in a patient with Marchifava Bignami Disease

  • 1 Mie Graduate School of Medicine, Neurology, Japan
  • 2 Tanaka Clinic, Neurology, Japan
  • 3 VA Boston Healthcare System/ Boston University Medical School, Neurology, United States
  • 4 Sakakibara Onsen Hospital, Speech Therapy, Japan
  • 5 Sakakibara Onsen Hospital, Internal Medicine, Japan
  • 6 Mie University Hospital, Emergency and Critical Care Center, Japan
  • 7 Kato Clinic, Neurology, Japan

Background This is a case report of a patient with a corpus callosum lesion that resulted in Marchiafava Bignami Disease (MBD), which typically involves unilateral apraxia and agraphia without aphasia. The patient, however, presented also with transcortical motor aphasia, which is not very common in such cases. We review cases of aphasia following corpus callosum lesions and consider possible anatomical underpinnings of the impairments we observed here. Medical History The patient is a 49 year old, right-handed man, with a history of alcohol abuse. When found by his neighbor and a policeman in his house, he was in coma and was transferred to a hospital. On admission, he was still in coma. DWI/FLAIR imaging revealed high intensity lesions in regions of the corpus callosum, and bilaterally in motor and premotor cortex (Figure 1). IMP-SPECT done 14 days post onset revealed decreased CBF bilaterally in the frontal cortex (Figure2). Following administration of thiamine, he regained consciousness. His speech was nonfluent, as measured by the Japanese Standard Language Test for Aphasia (SLTA). On the 24th day post onset, he was transferred to a rehabilitation hospital. At that time, his neurological examination demonstrated acaluculia, ideational apraxia, perseveration, decreased attention, callosal disconnection (agraphia of left hand, left unilateral alexia, diagonistic apraxia, alien hand syndrome, constructinal apraxia of right hand) and language performance consistent with transcortical motor aphasia diagnosed by SLTA. Discussion The patient described here had typical symptoms of MBD, but, in addition, exhibited transcortical motor aphasia. Aphasia in cases of MBD is rare. In one study, only 4 of 36 patients with callosal infarcts had decreased verbal fluency, and all but one of these had significant cortical involvement (1). In another study of 48 consecutive patients with anterior cerebral artery infarctions, transcortical motor aphasia was found in 10 of 30 patients with left-sided infarcts and 1 of 16 with a right-sided infarct, all of whom had involvement of the supplementary motor area or the subcortical white matter underlying it (2). Another report described a case of expressive aphasia following infarction of the right body of the corpus callosum, associated with dysprosody and hypophonia (3). Another study described a patient with transcortical motor aphasia following infarction of the left splenium of the corpus callosum (4). Saba suggested that the etiology of the language deficit of MBD may be due to damage to fibers from the inferior frontal lobe language area which pass through the genu and rostrum of the corpus callosum (5). The SPECT study of our case revealed decreased CBF in left frontal language regions. We suggest that aphasia in this patient may be explained by hypofunction of frontal language regions linked to the corpus callosal lesions. Thus, his aphasia would be, at least in part, the consequence of a disconnection of cortical language output systems from other language systems (both cortical and subcortical). Conclusion We report the case of a patient with a corpus callosum lesion, associated with Marchiafava Bignami Disease (MBD), who showed transcortical motor aphasia in addition to the deficits traditionally associated with an anterior callosal disconnection syndrome. We attribute his language dysfunction to impaired functionality of his language network, labeling this deficit a "language network disconnection syndrome".

Figure 1
Figure 2

References

1. Alonso A, et al. Clinical and MRI patterns of pericallosal artery infarctions: the significance of supplementary motor area lesions. J Neurol 259:944–951, 2012
2 Kumral E, et al. Spectrum of anterior cerebral artery territory infarction: clinical and MRI findings. Eur J Neurol 9:615–624, 2002
3. Ishizaki M, et al. Crossed aphasia following an infarction in the right corpus callosum. Clin Neurol Neurosurg 114:161–165,2012
4. Kuriyama N, et al. Transcortical aphasia in a patient with infarction of the corpus callosum left splenium: A case report. Jpn J Stroke 16:48-54,19945.
5. Saba S, et al. Aphasia due to isolated infarction of the corpus callosum.
BMJ Case Rep. Jun 12, 2014

Keywords: Corpus Callosum, Transcortical aphasia, Disconnection syndrome, Language network, Marchiafava-Bignami Disease

Conference: Academy of Aphasia 55th Annual Meeting , Baltimore, United States, 5 Nov - 7 Nov, 2017.

Presentation Type: poster presentation

Topic: Aphasia

Citation: Shindo A, Tanaka Y, Albert M, Cahana-Amitay D, Koyama Y, Nakamura T, Noda E, Minami N, Fujioka M and Kato Y (2019). Transcortical motor aphasia in a patient with Marchifava Bignami Disease. Conference Abstract: Academy of Aphasia 55th Annual Meeting . doi: 10.3389/conf.fnhum.2017.223.00070

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Received: 20 Mar 2017; Published Online: 25 Jan 2019.

* Correspondence: MD, PhD. Yutaka Tanaka, Tanaka Clinic, Neurology, Ikoma-Gun, Nara, 636-0933, Japan, EZZ05540@nifty.com