Clinical/Scientific Notes Takayasu’s arteritis with arteriographic evidence of intracranial vessel involvement Kevin Klos, MD; Kelly D. Flemming, MD; George W. Petty, MD; and Harvinder S. Luthra, MD Takayasu’s arteritis (TA) is an idiopathic granulomatous vasculitis that affects the aorta and its main branches.1 Approximately 10 to 15% of patients with TA will have ischemic stroke or transient ischemic attacks.2,3 These strokes have mainly been attributed to stenotic extracranial vessels. At least one case report has described intracranial arteritis in a patient with TA discovered at autopsy.4 We report two cases of TA with clinical and arteriographic involvement of the intracranial arteries. Case report. Case 1. A 32-year-old woman sought treatment for left forearm pain and cyanosis of the fingertips associated with use and absent left radial pulse. The left subclavian and axillary arteries were completely occluded with abundant collaterals as demonstrated by angiogram. Erythrocyte sedimentation rate (ESR) was 108. The findings were consistent with TA, and the patient was prescribed prednisone, followed 3 months later with methotrexate. Seven months after initial diagnosis of TA, the patient sought treatment for abdominal pain, severe frontal headache, and transient right leg heaviness. An initial MRI of the head was normal. MR angiography (MRA) of the neck revealed 60% stenosis of the left extracranial vertebral artery and 30% stenosis of both extracranial carotid arteries. Intracranial MRA was normal. MRA of the abdomen demonstrated a long tapered stenosis of the superior mesenteric artery (SMA) and proximal stenosis of the celiac artery. During hospitalization, the patient developed recurrent right lower extremity weakness. Repeat MRI revealed a left frontoparietal infarct. CSF examination revealed no abnormalities. A cerebral angiogram confirmed previous extracranial vessel findings but also revealed extensive abnormalities consistent with arteritis of the intracranial internal carotid, vertebral, and basilar arteries (figure, A and B). The patient developed worsening right hemiparesis and aphasia. Follow-up MRI revealed extension of the infarction in the left centrum semiovale. New infarcts were noted in the right parietal lobe and left cerebellum. Blood pressure remained stable during this time. The steroid dose was increased, methotrexate was discontinued, and cyclophosphamide, infliximab, and warfarin were initiated. One month later, the patient had no further headache or abdominal pain and was walking with a cane. A repeat MRA demonstrated improvement of the intracranial stenoses. Case 2. An 18-year-old woman sought treatment for increasing fatigue, new cardiac murmur, and bruits over the right carotid and subclavian arteries. ESR was normal. Aortography demonstrated bilateral subclavian artery stenosis and diffuse wall thickening of the descending thoracic aorta extending into the abdominal aorta with marked narrowing of these vessels. The celiac artery and SMA were stenotic at their origins. She was diagnosed with TA based on the distribution of lesions. One month later, she sought treatment for generalized seizures, severe headache, and peripheral scintillating scotoma. She denied using sympathomimetics or triptan medications. Neurologic examination was normal. CSF was normal, and there was no xanthochromia. MRI of the head demonstrated focal areas of T2 signal changes in the cerebellum and cerebrum predominantly at the gray-white junctions and deep white matter. A cerebral angiogram demonstrated segmental narrowing of some cerebral blood vessels (see figure, C). The patient was treated with high-dose steroids, valproate, and cyclophosphamide. During the following 6 months, the patient’s clinical symptoms improved, and repeat MRI scan of the head normalized. Discussion. We describe two patients who fulfilled the American College of Rheumatology criteria for the diagnosis of TA and who developed clinical and radiographic evidence of intracranial arterial involvement.1 Cases 1 and 2 illustrate extensive, diffuse intracranial involvement, including the intracranial internal carotid arteries, distal basilar artery, and the more distal branches involving the middle, anterior, and posterior cerebral artery. Arteriographic changes within the intracranial small vessels resulted in cerebral infarcts within the respective distributions. These cases demonstrate that our knowledge of TA continues to expand. The main limitation to this report is lack of histologic confirmation of cerebral vasculitis. Angiography is not 100% sensitive or specific for detection of vasculitis. Conditions that may mimic cerebral vasculitis include vasospasm, Fleming–Call syndrome, noninflammatory vasculopathies, and use of certain medications.5 Both of our patients had severe headache, but neither had evidence of CSF xanthochromia nor had used sympathomimetic or triptan medications. We treated both patients based on our clinical suspicion that intracranial involvement represented extension of vasculitis. Based on these cases, we believe that intracranial involvement may be under-recognized, and cerebral an- Figure. Conventional angiography of the posterior circulation of Patient 1 reveals a 60% stenosis of the midbasilar artery (open arrow; A). Segmental narrowing of the posterior inferior cerebellar artery is also noted (closed arrow). In the same patient, the left supraclinoid carotid artery revealed a 60% stenosis (open arrow), and the distal anterior and middle cerebral arteries demonstrated tapered segmental stenoses (closed arrows; B). Conventional angiography of Patient 2 demonstrates segmental narrowing of vessels, including the posterior cerebral arteries and superior cerebellar arteries (C). The distal basilar artery is mildly narrowed. 1550 NEUROLOGY 60 May (1 of 2) 2003 giography should be considered for patients diagnosed with TA who have severe headache, strokelike episodes, or seizures. From the Departments of Neurology (Drs. Klos, Flemming, and Petty) and Rheumatology (Dr. Luthra), Mayo Clinic, Rochester, MN. Received October 2, 2002. Accepted in final form January 10, 2003. Address correspondence and reprint requests to Kelly D. Flemming, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail: Flemming.Kelly@mayo.edu Copyright © 2003 by AAN Enterprises, Inc. A French accent after corpus callosum infarct D.A. Hall, MD; C.A. Anderson, MD; C.M. Filley, MD; J. Newcombe, MA, CCC-SLP; and R.L. Hughes, MD An accent is a feature of speech that characterizes certain people or regions. The foreign accent syndrome (FAS) is a rare disorder in which persons develop a speech pattern perceived by listeners to be foreign.1,2 FAS has typically been observed after anterior cortical or subcortical lesions in the hemisphere dominant for language,1,2 and a disruption of linguistic prosody has been implicated.1 We report a patient in whom a French accent appeared after an ischemic infarct in the corpus callosum (CC). Case report. A 53-year-old right-handed (⫹4 on the modified Annett handedness inventory3) English-speaking office worker had the sudden onset of difficulty speaking. Examination on the day of the event showed fluent speech with a staccato quality, and several observers then and later noted that her speech had acquired an accent in English resembling that of a native French speaker. There was a prominent disturbance of melodic line, with altered intonation, stresses, and pauses that imparted the impression of a French accent. Otherwise, her examination revealed normal fluency, repetition, and naming, but auditory comprehension was impaired. No facial or limb apraxia was noted. Reading comprehension was impaired, and there was mild agraphia. The production and understanding of affective prosody was preserved, and there was neither left-hand apraxia nor tactile anomia. The remainder of her neurologic examination was normal. Initial MRI showed an ischemic infarct in the body of the CC; the lesion was primarily in the midline but also extended slightly to the left (figure, A). After discharge, speech therapy produced no substantial improvement. Her speech remained strikingly altered during the ensuing months because of the French accent. On her return to the United States after a visit to Canada, border guards who heard her speak believed she was French Canadian. MRI performed 6 months after onset showed a small infarct in the body of the CC where the initial lesion had been observed (see figure, B). One year after symptom onset, neurobehavioral deficits persisted with the exception of a diminution in staccato speech. She was unable to work because of the language deficits associated with the foreign accent. She was fluent, had no dysarthria, and had normal repetition. Confrontation naming was also normal: she earned a score of 60/60 on the Boston Naming Test4 with only mild hesitancy in responding. She had difficulty, however, with commands involving two or more steps and with reading comprehension of complex sentences and paragraphs.5 In subse- References 1. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu’s arteritis. Arthritis Rheum 1990;33:1129 –1134. 2. Kerr GS, Hallahan CW, Girando J, Leavitt R, Fauci A, Hoffman G. Takayasu’s arteritis. Ann Intern Med 1994;120:919 –929. 3. Takano K, Sadoshima S, Ibayashi S, Ichiya Y, Fujishima M. Altered cerebral hemodynamics and metabolism in Takayasu’s arteritis with neurological deficits. Stroke 1993;24:1501–1506. 4. Molnar P, Hegedus K. Direct involvement of intracerebral arteries in Takayasu’s arteritis. Acta Neuropathol 1984;63:83– 86. 5. Call GK, Fleming MC, Sealfon S, Levine H, Kristler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke 1988;19:1159 –1170. quent months, many English- and French-speaking listeners who heard a recording of her speech generally concurred that her accent was best characterized as French or French Canadian. Discussion. After an infarct in the body of the CC, this woman developed a French accent. A disturbance of melodic line, with altered intonation, stresses, and pauses, was prominent in her spontaneous speech, suggesting that linguistic aprosody was fundamental to her new accent. She had mild fluent aphasia and alexia but no deficits involving affective prosody or callosal integration of motor or sensory functions. This patient is unique because the neuropathology was confined to the CC. FAS is thought to reflect a disorder of speech production related to damage in motor regions subserving speech.1,2 Some patients are initially mute, and others have a staccato speech rhythm, as in our patient, or a “sing-song” phrasal intonation.2 Structural lesions causing FAS typically involve regions of the anterior cerebral cortex, basal ganglia, or white matter in the language-dominant hemisphere. Many authorities assert that deficits in linguistic prosody underlie this syndrome.1 The French accent in our patient was apparently caused by the callosal infarct. The unusual lesion location remains puzzling because motor structures subserving speech production were not directly involved, but the localization adds the CC to the cerebral regions implicated in FAS. The CC helps mediate prosodic elements of language; deficits in affective and linguistic prosody have been documented in a patient with damage to the anterior fourfifths of the CC.6 Because linguistic aprosody also occurs after lesions of the right7 and left cerebrum,5 the CC appears to participate in a distributed network for linguistic prosody involving both hemispheres. In our patient, we believe the CC lesion accounted for the FAS by disrupting a bihemispheric distributed network for linguistic prosody. Her speech was characterized by impaired linguistic prosody that produced the French accent. Although further verification is necessary, we conclude that disturbance of interhemispheric connectivity may contribute to the pathogenesis of FAS. Acknowledgment The authors thank William Wagner, MD for referral of the patient and Kenneth M. Heilman, MD for thoughtful comments about the case. From the Departments of Neurology (Drs. Hall, Anderson, Filley, and Hughes), Psychiatry (Drs. Anderson and Filley), Emergency Medicine (Dr. Anderson), and Speech Pathology (J. Newcombe), University of Colorado School of Medicine; Denver Veterans Affairs Medical Center (Drs. Anderson and Filley); and Denver Health Medical Center (Dr. Hughes), CO. Figure. (A) Fast spin-echo inversion recovery coronal MRI showing an acute ischemic infarct in the corpus callosum (TR, 128; TE, 8002). (B) T1-weighted sagittal MRI showing a discrete area of infarction in the body of the corpus callosum (TR, 400; TE, 14). May (1 of 2) 2003 NEUROLOGY 60 1551 Takayasu's arteritis with arteriographic evidence of intracranial vessel involvement Kevin Klos, Kelly D. Flemming, George W. Petty, et al. Neurology 2003;60;1550-1551 DOI 10.1212/01.WNL.0000058758.47170.AE This information is current as of May 13, 2003 Updated Information & Services including high resolution figures, can be found at: http://www.neurology.org/content/60/9/1550.full.html References This article cites 5 articles, 2 of which you can access for free at: http://www.neurology.org/content/60/9/1550.full.html##ref-list-1 Citations This article has been cited by 1 HighWire-hosted articles: http://www.neurology.org/content/60/9/1550.full.html##otherarticles Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): All Imaging http://www.neurology.org//cgi/collection/all_imaging All Immunology http://www.neurology.org//cgi/collection/all_immunology Vasculitis http://www.neurology.org//cgi/collection/vasculitis Permissions & Licensing Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions Reprints Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus Neurology ® is the official journal of the American Academy of Neurology. 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