Clinical Neurology and Neurosurgery 103 (2001) 120– 122 www.elsevier.com/locate/clineuro
Case report
Multiple spinal intramedullary cavernous angioma: case report Hatice Balaban a, H. O8 zden S¸ener a,*, I: lhan Erden b, S¸u¨kru¨ C ¸ ag˘lar c, Afsun S¸ahin d, a Nezih Yu¨cemen a
Department of Neurology, Faculty of Medicine, Uni6ersity of Ankara, 06100 Ankara, Turkey Department of Radiology, Faculty of Medicine, Uni6ersity of Ankara, 06100 Ankara, Turkey c Department of Neurosurgery, Faculty of Medicine, Uni6ersity of Ankara, 06100 Ankara, Turkey d Faculty of Medicine, Uni6ersity of Ankara, Ankara, Turkey b
Received 30 September 2000; received in revised form 26 February 2001; accepted 4 April 2001
Abstract Spinal cavernous angiomas frequently accompany to cranial cavernous angiomas. Multiple spinal cord cavernous angiomas are very rare and to authors knowledge, only one case has been described having multiple intramedullary cavernous angiomas without cranial involvement until now. In this report, we present a case with acute paraplegia who had thoracic and cervical intramedullary cavernous angiomas and normal cranial magnetic resonance imaging. © 2001 Elsevier Science B.V. All rights reserved. Keywords: Cavernous angioma; Spinal cord; Intramedullary; Multiple; Hemorrhage
1. Introduction
2. Case report
Cavernous angiomas are thin walled, lobulated, sinusoidal vascular channels and no interposing neural or glial tissue [1–3]. The spinal cord is a rare site for cavernous angiomas and when they occur they are frequently found in epidural space; intramedullary ones are extremely rare [1,4]. Spinal cord cavernous angiomas frequently accompany cranial cavernous angiomas [5,6]. To the authors knowledge, only one case has been described having multiple intramedullary cavernous angiomas without cranial involvement until now [7]. We present a case with paraplegia who was diagnosed to have multiple intramedullary cavernous angiomas.
A 34-year-old woman suffering severe weakness and numbness of lower extremities and bowel –bladder incontinence was referred to the neurology department from a local hospital. There had been severe back pain and difficulty in control of micturition starting after forced physical activity two weeks before. Slight numbness and weakness in her right leg appeared within four days and progressed to complete weakness and numbness in both legs with bowel and bladder incontinence within the next five days. Neurological examination revealed flaccid paraplegia. Deep tendon reflexes were abolished in the lower extremities and decreased in right upper extremity, Babinski reflex was present bilaterally. There was anesthesia below the level of T4 dermatome, and the position and vibration senses were absent in the legs. Spine magnetic resonance imaging (MRI) on the 8th day of the symptoms showed two lesions. There was a lesion hypointense at T1 and hyperintense at T2 weighted images at the level of the fourth to sixth cervical vertebra. The spinal cord was expanded at this
* Corresponding author. Present address: I: bni Sina Hastanesi, No¨roloji Klinig˘i, 6. Kat, Samanpazarı 06100, Ankara, Turkey. Tel.: +90-312-3103333/2220; fax: +90-312-3106371. E-mail address:
[email protected] (H.O8 . S¸ener).
0303-8467/01/$ - see front matter © 2001 Elsevier Science B.V. All rights reserved. PII: S 0 3 0 3 - 8 4 6 7 ( 0 1 ) 0 0 1 2 3 - 8
H. Balaban et al. / Clinical Neurology and Neurosurgery 103 (2001) 120–122
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reaction for tuberculosis. Antibodies to herpes symplex type 1 virus, varicella zoster, cytomegalovirus, borrelia burgdorferi, and brucella were normal. A second MRI two weeks later showed hyperintense appearance of the lesions at T1 and T2 weighted images, which was suggestive of subacute hemorrhage (Fig. 2). Characteristic mixed signal intensity with surrounding ring of low signal intensity was present at the thoracic lesion at T2 weighted images. The lesions were interpreted as cavernous angiomas and the patient referred to the neurosurgery department.
3. Discussion
Fig. 1. T2 weighted MRI of the cervical and thoracic spine on the 8th day.
level. Another heterogeneous lesion at T1 and T2 weighted images at the level of the first to second thoracic vertebra was also observed (Fig. 1). Spinal angiography was normal. Cerebrospinal fluid examination was normal for cytology, protein, glycose, IgG index, immune-fixation electrophoresis, protein-chain-
Cavernous angiomas account for 5– 16% of all spinal vascular malformations [2]. With the advent of MRI, their prevalence was seen to be higher than previously believed [4]. The ages at which clinical findings appear are quite variable but most of the patients are between third and fifth decades and it is reported that females are more frequently affected [3]. Lesions are most frequently localized at the cervical and thoracic spinal cord but may be seen at any level from upper cervical cord to cauda equina [1,6]. They commonly accompany intracranial cavernous malformations [5,6]. MRI is nearly pathognomonic [1,7]. The hypointense halo caused by hemosiderin accumulation around the heterogeneous hyperintense lesion in T2 weighted images with normal angiography is highly characteristic [1,3,8,9]. Cavernous angiomas mostly appear as solitary lesions in the spinal cord. Zevgaridis et al. reviewed 116 patients with intramedullary spinal cavernous angioma, which had been published between 1903 and 1996. They
Fig. 2. T1 (left) and T2 (right) weighted MRI on the 22nd day.
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found only one patient who had two spinal intramedullary cavernous angiomas [10]. That patient had two spinal intramedullary cavernous angiomas located at thoracic and cervical levels with normal cranial MRI [7]. Later, Visteh et al. reported 17 patients with intramedullary cavernous angiomas. Only one patient had a second cavernous angioma in the spinal cord who also had a cranial cavernous angioma [6]. To our knowledge, our case is the second in the literature who has multiple spinal intramedullary cavernous angiomas with normal brain. An interesting feature in our case is the inversion of the cervical vertebral axis and cervical spondylosis. Cervical spondylosis itself is not known to cause hematomyelia. Although this accompaniment of the cavernoma and the spondylosis may be a coincidence, spondylosis could be a provocative factor for bleeding of the cervical cavernoma. In conclusion, searching the whole spinal canal is important for detecting asymptomatic malformations in patients with a spinal cavernous angioma even if the cranial MRI is normal.
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