AUTHOR(S): Lorenzana, Luis, M.D.; Cabezudo, Jose M., M.D., Ph.D; Porras, Luis F., M.D.; Polaina, Manuel, M.D.; Rodriguez-Sanchez, Jose A., M.D.; Garcia-Yagüe, Luis M., M.D., Ph.D. Seccion de Neurocirugía, Hospital Universitario "Infanta Cristina," Universidad de Extremadura, Badajoz, Spain Neurosurgery 31; 1108-1112, 1992 ABSTRACT: THE CASE OF a young woman with focal dystonia of the hand due to a cavernous angioma of the basal ganglia is presented. The lesion involved the anterior third of the lentiform nucleus and a large portion of white matter anterior to this nucleus and lateral to the head of the caudate, as shown by magnetic resonance imaging; it was completely removed through a computed tomographyassisted stereotactic craniotomy by microsurgical technique, resulting in the cure of the patient. These facts support the pathophysiological hypothesis of a disruption of the striatopallidothalamic projection to the premotor cortex as the cause of symptomatic dystonia. A review of the reported cases of cavernous angiomas of the deep cerebral gray nuclei shows that this is the first case of cavernous angioma associated with movement disorder. KEY WORDS: Basal ganglia; Cavernous angioma; Focal dystonia; Magnetic resonance imaging; Stereotactic craniotomy Cavernous angiomas, cavernous malformations, or cavernomas constitute a significant percentage of the angiographic occult intracranial vascular malformations (11,13,21,22,29,31). Although devoid of a connective capsule, they are well-circumscribed, vascular hamartomas composed of sinusoid vascular spaces lined with a single layer of endothelial cells and devoid of an elastica or muscularis lamina that are separated by thin septa of fibrous tissue without intervening neural or glial tissue (16,27). In the past, they were considered a rare entity occurring predominantly in adulthood. With the advent of new diagnostic tools, computed tomography (CT) and magnetic resonance imaging (MRI), it has now become evident that they are more prevalent than previously thought and may occur at any age (14,21-23, 28) . Cavernomas are usually located in the supratentorial compartment, preferably in the white matter of the frontal and temporal lobes, but they also have been reported in other locations such as the cerebellum, brain stem, spinal cord, cavernous sinus, dura mater, cerebellopontine angle, pineal region, cranial nerves, and spinal roots (13,15,27,30). Although
there are asymptomatic cases (7,15,23), most of them are symptomatic. Clinical manifestations, in order of frequency, are epilepsy, acute neurological deficit secondary to hemorrhage, and space-occupying lesion syndrome characterized by progressive focal neurological deficits or by intracranial hypertension (13,28,30) . Although it has been stated that the basal ganglia are a favorite location of cavernous angiomas (27), only 24 cases occurring in the cerebral gray nuclei have been reported to date, 18 of which were histologically confirmed after surgical removal (1-3,11, 14,15,20-22,24,25,28,30,32) . In this report, we present a case of cavernous angioma of the basal ganglia in which the only clinical manifestation was a focal dystonia that resolved after surgical removal. To the best of our knowledge, this is the first case of cavernous angioma associated with extrapyramidal movement disorder. CASE REPORT This 19-year-old, right-handed woman was in good health until December 1990, when she started complaining of involuntary movements affecting her right hand both at rest and during voluntary movements. The movements were irregularly distributed during the day, lasting between 1 and 2 hours, and disappeared spontaneously. She did not complain of any other symptoms. She was admitted to our hospital on January 8, 1991. A neurological examination was normal except for a dystonia of the right hand, consisting of abduction and extension of the fingers and flexion and ulnar deviation of the wrist (Figure 1). The dystonia appeared during both rest and voluntary movements but was aggravated by actions such as grasping objects or writing, as well as when the patient closed her eyes. It disappeared during sleep. A CT scan of the head (Figure 2) showed a welldefined and slightly hyperdense nonhomogeneous lesion in the left basal ganglia region without surrounding edema or mass effect that enhanced moderately after intravenous contrast injection. MRI (Figure 3) showed that the lesion, measuring 3 × 2.5 × 2 cm, involved the anterior third of the lentiform nucleus and a large portion of the white matter anterior to this nucleus and lateral to the head of the caudate. It was a well-defined lesion with a central area of mixed-signal intensity surrounded by a rim of hypointense signal both in the T1- and T2-weighted sequences, characteristic of cavernous angioma. A left digital carotid angiography was normal. An electroencephalogram showed sporadic τ slow waves on the left temporal region. The patient underwent a left, frontal CT-assisted stereotactic craniotomy, with the use of the CRW stereotactic system, (Radionics, Burlington, MA), with total removal of the lesion through the second frontal sulcus. The neuropathological diagnosis of the specimen was cavernous angioma (Figure 4). Postoperatively, the dystonic movements immediately disappeared. Postoperative CT scan and MRI confirmed the completeness of the resection (Figure 5). The patient was discharged on the eighth postoperative day. One year after surgery, she
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Neurosurgery 1992-98 December 1992, Volume 31, Number 6 1108 Focal Dystonia Secondary to Cavernous Angioma of the Basal Ganglia: Case Report and Review of the Literature Case Report
DISCUSSION According to the Scientific Advisory Board of the Dystonia Medical Research Foundation, dystonia is a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures (6). When only a single area of the body is affected, it is called focal dystonia. Etiologically, dystonias can be divided into two major divisions: idiopathic (or primary) and symptomatic (or secondary). The idiopathic group is subdivided into familial and nonfamilial (sporadic) patterns. The symptomatic group is subdivided into dystonia caused by neural degeneration of known or unknown enzymatic defect, psychogenic dystonia, and dystonia caused by identifiable mechanisms that do not involve degenerative pathology, such as perinatal cerebral damage, infarction, demyelinating disease, arteriovenous malformations, tumors, trauma, encephalitis, drugs, and toxins (5,8,10,12,17,26). In a recent comprehensive review of symptomatic dystonia caused by focal cerebral lesions, Obeso and Gimenez-Roldan (18) found five cases of hand dystonia. A lesion in the thalamus was the cause in three cases (two infarctions and one cystic lesion), and an arteriovenous malformation of the putamen was the cause in the remaining two cases (4,9,12,17,18). Hand dystonia has been related to lesions of the posterolateral thalamic nuclei and, more rarely, to lesions of the caudate nucleus, putamen, and parietal cortex (12,18). Hemidystonia has been related to large caudatocapsulolenticular lesions and also to small lesions in the putamen (12,17-19). Because of the variable location of lesions causing focal dystonia, Marsden et al. (12) and Pettigrew and Jankovic (19) have suggested that symptomatic dystonia may be produced by abnormal thalamic-mediated input to premotor and ultimately motor cortex from the basal ganglia. This inappropriate information delivered to the premotor area can be produced by a lesion anywhere in the striatopallidothalamic connections with the premotor cortex. Dystonia therefore may be viewed as a consequence of the release of premotor cortical activity from thalamic control (12,18,19,26). Our patient had a hand dystonia associated with a cavernous angioma involving the anterior third of the lentiform nucleus and the white matter anterior to this nucleus and lateral to the head of the caudate. Dystonia was most probably produced by mass effect of the angioma. That the cavernous angioma was the cause of her hand dystonia is supported by the resolution of symptoms after the cavernous angioma was removed. In addition, our findings strongly support the anatomophysiological correlation mentioned in the preceding paragraph. As far as we know, this is the first case of dystonia secondary to cavernous angioma. Table 1 summarizes the 20 reported cases of cavernous angiomas located in the cerebral gray nuclei in which enough information is provided (1-3, 11,14,15,20,24,25,28,30,32) . On the basis of their clinical presentation at the time of diagnosis, 13 had focal neurological deficits, three had hemorrhage, one had
seizures, and one had intracranial hypertension. In two patients, clinical findings at the time of admission were not stated. Ours is the only one seeking treatment for movement disorder. When the surgical results are taken into account, it can be seen that of the 17 patients undergoing operations, only one improved, whereas 11 did not benefit and one died. In four cases, the outcome was not stated. In view of these poor results, some authors have recommended a conservative management of these lesions (25). It is possible that an inadequate intraoperative localization of the lesion with the resulting forceful retraction or unwanted brain disruption may be responsible for the disappointing results obtained in the surgical treatment of deep cavernous angiomas. Thus, it has recently been suggested that localization of the lesion by means of intraoperative ultrasound or CT-guided stereotactic craniotomy may yield better results (15). In our patient, stereotactic localization and removal of the cavernous angioma through the second frontal sulcus was followed by a complete disappearance of the symptoms. It is our belief that treatment of cavernous angiomas of the basal ganglia can be rewarding when refined microsurgical technique is coupled with modern localization systems such as CT-guided stereotaxis, as in our case. ACKNOWLEDGMENT The authors acknowledge Dr. E. Alejo of the Pathology Department for the histological preparations. Received, January 21, 1992. Accepted, June 5, 1992. Reprint requests: Dr. Jose M. Cabezudo, Apartado de Correos 255, 06080 Badajoz, Spain. REFERENCES: (1-32) 1.
2.
3. 4. 5.
6.
7.
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Becker DM, Townsend JJ, Kramer RA, Newton TH: Occult cerebrovascular malformations: A series of 18 histologically verified cases with negative angiography. Brain 102:249-287, 1979. Bertalanffy H, Gilsbach JM, Eggert HR, Seeger W: Microsurgery of deep-seated cavernous angiomas: Report of 26 cases. Acta Neurochir (Wien) 108:91-99, 1991. Bicknell JM, Carlow TJ, Kornfeld M, Stovring J, Turner P: Familial cavernous angiomas. Arch Neurol 35:746-749, 1978. Bouttier H, Bertrand L, Marie AP: Sur un cas anatomo-clinique de syndrome thalamique dissocie. Rev Neurol 38:1492-1502, 1922. Calne DB, Lang AE: Secondary dystonia, in Fahn S (ed): Dystonia 2, Advances in Neurology. New York, Raven Press, 1988, vol 50, pp 9-33. Fahn S: Concept and classification of dystonia, in Fahn S (ed): Dystonia 2, Advances in Neurology New York, Raven Press, 1988, vol 50, pp 1-8. Farmer JP, Cosgrove GR, Villemure JG,
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
remains asymptomatic.
9. 10.
11.
12. 13.
14.
15.
16.
17.
18.
19.
20. 21.
22.
23.
24.
25. 26.
27. 28.
29. 30.
31.
32.
1991. Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J, Spetzler RF: The MRI appearance of cavernous malformations (angiomas) J Neurosurg 67:518-524, 1987. Rigamonti D, Hadley MN, Drayer BP, Johnson PC, Hofnig-Rigamonti K, Knight JT, Spetzler RF: Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med 319:343- 347, 1988. Rigamonti D, Spetzler RF: The association of venous and cavernous malformations. Report of four cases and discussion of the pathophysiological, diagnostic, and therapeutic implications. Acta Neurochir (Wien) 92:100-105, 1988. Roda JM, Alvarez F, Isla A, Blazquez MG: Thalamic cavernous malformation. Case report. J Neurosurg 272:647-649, 1990. Roos RAC, Bruyn GW: Symptomatic dystonias, in Vinken PJ, Bruyn GW, Klawans HL (eds): Extrapyramidal Disorders, Handbook of Clinical Neurology. Amsterdam, Elsevier, 1986, vol 49, revised series 5, pp 541-547. Russell DS, Rubinstein LJ: Pathology of Tumours of the Nervous System. Baltimore, Williams & Wilkins, 1989, ed. 5. Simard JM, Garcia-Bengochea F, Ballinger WE, Mickle JP, Quisling RG: Cavernous angioma: A review of 126 collected and 12 new clinical cases. Neurosurgery 18:162-172, 1986. Stein BM, Mohr JP: Vascular malformations of the brain. N Engl J Med 319:368-369, 1989. Vaquero J, Salazar J, Martinez R, Martinez P, Bravo G: Cavernomas of the central nervous system: Clinical syndromes, CT scan diagnosis, and prognosis after surgical treatment in 25 cases. Acta Neurochir (Wien) 85:29-33, 1987. Wakai S, Ueda Y, Inoh S, Nagai M: Angiographically occult angiomas: A report of thirteen cases with analysis of the cases documented in the literature. Neurosurgery 17:549-556, 1985. Yamasaki T, Handa H, Yamashita J, Paina JT, Tashiro Y, Uno A, Ishikawa M, Asato R: Intracranial and orbital cavernous angiomas. A review of 30 cases. J Neurosurg 64:197208, 1986.
COMMENTS Cavernous angiomas are most commonly discovered in the setting of intracerebral hemorrhage or seizures or as an incidental finding. Patients with angiomas in the brain stem do not infrequently seek treatment for progressive stepwise neurological deficits. This interesting report demonstrates that
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8.
Meagler- Villemure K, Tampieri D, Melanson D: Intracerebral cavernous angiomas. Neurology 38:1699- 1704, 1988. Friedman DI, Jankovic J, Rolak LA: Arteriovenous malformation presenting as hemidystonia. Neurology 36:1590-1593, 1986. Garcin R: Syndrome cerebello-thalamique par lesion localisee du thalamus. Rev Neurol 93:143- 149, 1955. Lins MM, McDonnell DE, Aschenbreuer CA, Cancilla PA: Extrapyramidal disorder with pineal germinoma. J Neurosurg 48:108-116, 1978. Lobato RD, Perez C, Rivas JJ, Cordobes F: Clinical, radiological, and pathological spectrum of angiographically occult intracranial vascular malformations. J Neurosurg 68:518-531, 1988. Marsden CD, Obeso JA, Zarranz JJ, Lang AE: The anatomical basis of symptomatic hemidystonia. Brain 108:463-483, 1985. Martin NA, Wilson CB, Stein BM: Venous and cavernous malformations, in Wilson CB, Stein BM (eds): Intracranial Arteriovenous Malformations. Baltimore, Williams & Wilkins, 1984, pp 234-245. Mazza C, Scienza R, Beltramello A, Da Pian R: Cerebral cavernous malformations (cavernomas) in the pediatric age-group. Child Nerv Syst 7:139-146, 1991. McCormick P, Michelsen WJ: Management of intracranial cavernous and venous malformations, in Barrow DL (ed): Intracranial Vascular Malformations. Park Ridge, IL, American Association of Neurological Surgeons, 1990, pp 197-217. McCormick WF: Pathology of vascular malformations of the brain, in Wilson CB, Stein BM (eds): Intracranial Arteriovenous Malformations. Baltimore, Williams & Wilkins, 1984, pp 44-63. Narbona J, Obeso JA, Tuñon T, MartinezLage JM, Marsden CD: Hemidystonia secondary to localised basal ganglia tumour. J Neurol Neurosurg Psychiatry 47:704-709, 1984. Obeso JA, Gimenez-Roldan S: Clinicopathological correlation in symptomatic dystonia, in Fahn S (ed): Dystonia 2, Advances in Neurology New York, Raven Press, 1988, vol 50, pp 113-122. Pettigrew LC, Jankovic J: Hemidystonia: A report of 22 patients and review of the literature. J Neurol Neurosurg Psychiatry 48:650-657, 1983. Pozatti E, Giuliani G, Nuzzo G, Poppi M: The growth of cerebral cavernous angiomas. Neurosurgery 25:92-97, 1989. Requena I, Arias M, Lopez-Ibor L, Pereiro I, Barba A, Alonso A, Monton E: Cavernomas of the central nervous system: Clinical and neuroimaging manifestations in 47 patients. J Neurol Neurosurg Psychiatry 54:590-594,
dystonia may result from a strategically positioned lesion, suggesting both that appropriate imaging should be carried out in patients with unexplained movement disorders and that the indications for the surgical removal of vascular anomalies in eloquent brain areas may include amelioration of a movement disorder.
The authors have achieved successful surgical management of a deep-seated, large cavernous malformation with postoperative resolution of disabling focal dystonia. Cavernous malformations in or near eloquent locations may cause symptoms by slow oozing of blood into the surrounding brain parenchyma with resulting irritative phenomena, by the sudden thrombosis of one or more caverns with expansion of these compartments and focal mass effect, or by frank exsanguination into surrounding brain parenchyma. There is evidence of all of these phenomena in pathological specimens of larger cavernous malformations. We agree with the authors that mass effect is the most probable pathophysiological phenomenon in this particular case, because resection of the cavernous malformation resulted in the prompt resolution of the dystonia. One cannot exclude, however, focal irritative phenomena causing dystonia by an excitatory mechanism. This hypothesis is appealing in light of the frequent dystonic posturing associated with intractable partial complex seizures. Intracranial monitoring confirms the coupling of dystonic posturing with ictal epileptiform activity spreading to the basal ganglia. Resection of the cavernous malformation could conceivably result in elimination of such subcortical irritative activity (similar to the arrest of seizures after the resection of cortical lesions). As the authors point out, lesions in a wide variety of subcortical locations have been associated with focal dystonia. This would rule out a focal and highly specific anatomical substrate for this particular symptom. It is more likely that lesions causing dystonia do so by disrupting one or more pathways involved in the subcortical control of motor activity. The lesion in this particular case is relatively large and may have disrupted any one of many possible extrapyramidal projections or connections between the thalamus, basal ganglia, and premotor cortex. It is interesting that dystonia was so focal in such a large lesion, involving the upper extremity and sparing the trunk and lower limb. This is consistent with a somatotopic distribution of these extrapyramidal control pathways. Surgical excision of most deep-seated cavernous angiomas can be carried out safely by microsurgical techniques, by transsulcal dissection, and in some cases, by the assistance of computer-guided or stereotactic localization. In most instances, dramatic symptomatic relief can be accomplished. Symptomatic cavernous malformations in eloquent locations are frequently associated with progressive
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Steven L. Giannotta Los Angeles, California
Figure 2. CT scan showing a high-density lesion in the left basal ganglia before (A) and after (B) intravenous contrast injection.
Figure 3. MRI showing a lesion characterized by a core of mixed signal intensity and a rim of decreased signal intensity involving the anterior third of the lentiform nucleus and surrounding white matter. A, axial T1-weighted image. B, axial T2-weighted image. C, coronal T1-weighted image.
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Figure 1. Photograph showing dystonia of the right hand.
Figure 5. Postoperative T1-weighted MRI showing completeness of the resection.
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Figure 4. Photomicrographic appearance of the removed specimen showing vascular channels composed of collagenous walls and lined by a single layer of endothelium, without intervening brain tissue (hematoxylin and eosin stain, ×40).
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Table 1. Summary of Reported Cases of Cavernous Angiomas of the Cerebral Gray Nucleia
there are asymptomatic cases (7,15,23), most of them are symptomatic. Clinical manifestations, in order of frequency, are epilepsy, acute neurological deficit secondary to hemorrhage, and space-occupying lesion syndrome characterized by progressive focal neurological deficits or by intracranial hypertension (13,28,30) . Although it has been stated that the basal ganglia are a favorite location of cavernous angiomas (27), only 24 cases occurring in the cerebral gray nuclei have been reported to date, 18 of which were histologically confirmed after surgical removal (1-3,11, 14,15,20-22,24,25,28,30,32) . In this report, we present a case of cavernous angioma of the basal ganglia in which the only clinical manifestation was a focal dystonia that resolved after surgical removal. To the best of our knowledge, this is the first case of cavernous angioma associated with extrapyramidal movement disorder. CASE REPORT This 19-year-old, right-handed woman was in good health until December 1990, when she started complaining of involuntary movements affecting her right hand both at rest and during voluntary movements. The movements were irregularly distributed during the day, lasting between 1 and 2 hours, and disappeared spontaneously. She did not complain of any other symptoms. She was admitted to our hospital on January 8, 1991. A neurological examination was normal except for a dystonia of the right hand, consisting of abduction and extension of the fingers and flexion and ulnar deviation of the wrist (Figure 1). The dystonia appeared during both rest and voluntary movements but was aggravated by actions such as grasping objects or writing, as well as when the patient closed her eyes. It disappeared during sleep. A CT scan of the head (Figure 2) showed a welldefined and slightly hyperdense nonhomogeneous lesion in the left basal ganglia region without surrounding edema or mass effect that enhanced moderately after intravenous contrast injection. MRI (Figure 3) showed that the lesion, measuring 3 × 2.5 × 2 cm, involved the anterior third of the lentiform nucleus and a large portion of the white matter anterior to this nucleus and lateral to the head of the caudate. It was a well-defined lesion with a central area of mixed-signal intensity surrounded by a rim of hypointense signal both in the T1- and T2-weighted sequences, characteristic of cavernous angioma. A left digital carotid angiography was normal. An electroencephalogram showed sporadic τ slow waves on the left temporal region. The patient underwent a left, frontal CT-assisted stereotactic craniotomy, with the use of the CRW stereotactic system, (Radionics, Burlington, MA), with total removal of the lesion through the second frontal sulcus. The neuropathological diagnosis of the specimen was cavernous angioma (Figure 4). Postoperatively, the dystonic movements immediately disappeared. Postoperative CT scan and MRI confirmed the completeness of the resection (Figure 5). The patient was discharged on the eighth postoperative day. One year after surgery, she
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Neurosurgery 1992-98 December 1992, Volume 31, Number 6 1108 Focal Dystonia Secondary to Cavernous Angioma of the Basal Ganglia: Case Report and Review of the Literature Case Report
DISCUSSION According to the Scientific Advisory Board of the Dystonia Medical Research Foundation, dystonia is a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures (6). When only a single area of the body is affected, it is called focal dystonia. Etiologically, dystonias can be divided into two major divisions: idiopathic (or primary) and symptomatic (or secondary). The idiopathic group is subdivided into familial and nonfamilial (sporadic) patterns. The symptomatic group is subdivided into dystonia caused by neural degeneration of known or unknown enzymatic defect, psychogenic dystonia, and dystonia caused by identifiable mechanisms that do not involve degenerative pathology, such as perinatal cerebral damage, infarction, demyelinating disease, arteriovenous malformations, tumors, trauma, encephalitis, drugs, and toxins (5,8,10,12,17,26). In a recent comprehensive review of symptomatic dystonia caused by focal cerebral lesions, Obeso and Gimenez-Roldan (18) found five cases of hand dystonia. A lesion in the thalamus was the cause in three cases (two infarctions and one cystic lesion), and an arteriovenous malformation of the putamen was the cause in the remaining two cases (4,9,12,17,18). Hand dystonia has been related to lesions of the posterolateral thalamic nuclei and, more rarely, to lesions of the caudate nucleus, putamen, and parietal cortex (12,18). Hemidystonia has been related to large caudatocapsulolenticular lesions and also to small lesions in the putamen (12,17-19). Because of the variable location of lesions causing focal dystonia, Marsden et al. (12) and Pettigrew and Jankovic (19) have suggested that symptomatic dystonia may be produced by abnormal thalamic-mediated input to premotor and ultimately motor cortex from the basal ganglia. This inappropriate information delivered to the premotor area can be produced by a lesion anywhere in the striatopallidothalamic connections with the premotor cortex. Dystonia therefore may be viewed as a consequence of the release of premotor cortical activity from thalamic control (12,18,19,26). Our patient had a hand dystonia associated with a cavernous angioma involving the anterior third of the lentiform nucleus and the white matter anterior to this nucleus and lateral to the head of the caudate. Dystonia was most probably produced by mass effect of the angioma. That the cavernous angioma was the cause of her hand dystonia is supported by the resolution of symptoms after the cavernous angioma was removed. In addition, our findings strongly support the anatomophysiological correlation mentioned in the preceding paragraph. As far as we know, this is the first case of dystonia secondary to cavernous angioma. Table 1 summarizes the 20 reported cases of cavernous angiomas located in the cerebral gray nuclei in which enough information is provided (1-3, 11,14,15,20,24,25,28,30,32) . On the basis of their clinical presentation at the time of diagnosis, 13 had focal neurological deficits, three had hemorrhage, one had
seizures, and one had intracranial hypertension. In two patients, clinical findings at the time of admission were not stated. Ours is the only one seeking treatment for movement disorder. When the surgical results are taken into account, it can be seen that of the 17 patients undergoing operations, only one improved, whereas 11 did not benefit and one died. In four cases, the outcome was not stated. In view of these poor results, some authors have recommended a conservative management of these lesions (25). It is possible that an inadequate intraoperative localization of the lesion with the resulting forceful retraction or unwanted brain disruption may be responsible for the disappointing results obtained in the surgical treatment of deep cavernous angiomas. Thus, it has recently been suggested that localization of the lesion by means of intraoperative ultrasound or CT-guided stereotactic craniotomy may yield better results (15). In our patient, stereotactic localization and removal of the cavernous angioma through the second frontal sulcus was followed by a complete disappearance of the symptoms. It is our belief that treatment of cavernous angiomas of the basal ganglia can be rewarding when refined microsurgical technique is coupled with modern localization systems such as CT-guided stereotaxis, as in our case. ACKNOWLEDGMENT The authors acknowledge Dr. E. Alejo of the Pathology Department for the histological preparations. Received, January 21, 1992. Accepted, June 5, 1992. Reprint requests: Dr. Jose M. Cabezudo, Apartado de Correos 255, 06080 Badajoz, Spain. REFERENCES: (1-32) 1.
2.
3. 4. 5.
6.
7.
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Becker DM, Townsend JJ, Kramer RA, Newton TH: Occult cerebrovascular malformations: A series of 18 histologically verified cases with negative angiography. Brain 102:249-287, 1979. Bertalanffy H, Gilsbach JM, Eggert HR, Seeger W: Microsurgery of deep-seated cavernous angiomas: Report of 26 cases. Acta Neurochir (Wien) 108:91-99, 1991. Bicknell JM, Carlow TJ, Kornfeld M, Stovring J, Turner P: Familial cavernous angiomas. Arch Neurol 35:746-749, 1978. Bouttier H, Bertrand L, Marie AP: Sur un cas anatomo-clinique de syndrome thalamique dissocie. Rev Neurol 38:1492-1502, 1922. Calne DB, Lang AE: Secondary dystonia, in Fahn S (ed): Dystonia 2, Advances in Neurology. New York, Raven Press, 1988, vol 50, pp 9-33. Fahn S: Concept and classification of dystonia, in Fahn S (ed): Dystonia 2, Advances in Neurology New York, Raven Press, 1988, vol 50, pp 1-8. Farmer JP, Cosgrove GR, Villemure JG,
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
remains asymptomatic.
9. 10.
11.
12. 13.
14.
15.
16.
17.
18.
19.
20. 21.
22.
23.
24.
25. 26.
27. 28.
29. 30.
31.
32.
1991. Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J, Spetzler RF: The MRI appearance of cavernous malformations (angiomas) J Neurosurg 67:518-524, 1987. Rigamonti D, Hadley MN, Drayer BP, Johnson PC, Hofnig-Rigamonti K, Knight JT, Spetzler RF: Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med 319:343- 347, 1988. Rigamonti D, Spetzler RF: The association of venous and cavernous malformations. Report of four cases and discussion of the pathophysiological, diagnostic, and therapeutic implications. Acta Neurochir (Wien) 92:100-105, 1988. Roda JM, Alvarez F, Isla A, Blazquez MG: Thalamic cavernous malformation. Case report. J Neurosurg 272:647-649, 1990. Roos RAC, Bruyn GW: Symptomatic dystonias, in Vinken PJ, Bruyn GW, Klawans HL (eds): Extrapyramidal Disorders, Handbook of Clinical Neurology. Amsterdam, Elsevier, 1986, vol 49, revised series 5, pp 541-547. Russell DS, Rubinstein LJ: Pathology of Tumours of the Nervous System. Baltimore, Williams & Wilkins, 1989, ed. 5. Simard JM, Garcia-Bengochea F, Ballinger WE, Mickle JP, Quisling RG: Cavernous angioma: A review of 126 collected and 12 new clinical cases. Neurosurgery 18:162-172, 1986. Stein BM, Mohr JP: Vascular malformations of the brain. N Engl J Med 319:368-369, 1989. Vaquero J, Salazar J, Martinez R, Martinez P, Bravo G: Cavernomas of the central nervous system: Clinical syndromes, CT scan diagnosis, and prognosis after surgical treatment in 25 cases. Acta Neurochir (Wien) 85:29-33, 1987. Wakai S, Ueda Y, Inoh S, Nagai M: Angiographically occult angiomas: A report of thirteen cases with analysis of the cases documented in the literature. Neurosurgery 17:549-556, 1985. Yamasaki T, Handa H, Yamashita J, Paina JT, Tashiro Y, Uno A, Ishikawa M, Asato R: Intracranial and orbital cavernous angiomas. A review of 30 cases. J Neurosurg 64:197208, 1986.
COMMENTS Cavernous angiomas are most commonly discovered in the setting of intracerebral hemorrhage or seizures or as an incidental finding. Patients with angiomas in the brain stem do not infrequently seek treatment for progressive stepwise neurological deficits. This interesting report demonstrates that
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8.
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dystonia may result from a strategically positioned lesion, suggesting both that appropriate imaging should be carried out in patients with unexplained movement disorders and that the indications for the surgical removal of vascular anomalies in eloquent brain areas may include amelioration of a movement disorder.
The authors have achieved successful surgical management of a deep-seated, large cavernous malformation with postoperative resolution of disabling focal dystonia. Cavernous malformations in or near eloquent locations may cause symptoms by slow oozing of blood into the surrounding brain parenchyma with resulting irritative phenomena, by the sudden thrombosis of one or more caverns with expansion of these compartments and focal mass effect, or by frank exsanguination into surrounding brain parenchyma. There is evidence of all of these phenomena in pathological specimens of larger cavernous malformations. We agree with the authors that mass effect is the most probable pathophysiological phenomenon in this particular case, because resection of the cavernous malformation resulted in the prompt resolution of the dystonia. One cannot exclude, however, focal irritative phenomena causing dystonia by an excitatory mechanism. This hypothesis is appealing in light of the frequent dystonic posturing associated with intractable partial complex seizures. Intracranial monitoring confirms the coupling of dystonic posturing with ictal epileptiform activity spreading to the basal ganglia. Resection of the cavernous malformation could conceivably result in elimination of such subcortical irritative activity (similar to the arrest of seizures after the resection of cortical lesions). As the authors point out, lesions in a wide variety of subcortical locations have been associated with focal dystonia. This would rule out a focal and highly specific anatomical substrate for this particular symptom. It is more likely that lesions causing dystonia do so by disrupting one or more pathways involved in the subcortical control of motor activity. The lesion in this particular case is relatively large and may have disrupted any one of many possible extrapyramidal projections or connections between the thalamus, basal ganglia, and premotor cortex. It is interesting that dystonia was so focal in such a large lesion, involving the upper extremity and sparing the trunk and lower limb. This is consistent with a somatotopic distribution of these extrapyramidal control pathways. Surgical excision of most deep-seated cavernous angiomas can be carried out safely by microsurgical techniques, by transsulcal dissection, and in some cases, by the assistance of computer-guided or stereotactic localization. In most instances, dramatic symptomatic relief can be accomplished. Symptomatic cavernous malformations in eloquent locations are frequently associated with progressive
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Steven L. Giannotta Los Angeles, California
Figure 2. CT scan showing a high-density lesion in the left basal ganglia before (A) and after (B) intravenous contrast injection.
Figure 3. MRI showing a lesion characterized by a core of mixed signal intensity and a rim of decreased signal intensity involving the anterior third of the lentiform nucleus and surrounding white matter. A, axial T1-weighted image. B, axial T2-weighted image. C, coronal T1-weighted image.
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Figure 1. Photograph showing dystonia of the right hand.
Figure 5. Postoperative T1-weighted MRI showing completeness of the resection.
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Figure 4. Photomicrographic appearance of the removed specimen showing vascular channels composed of collagenous walls and lined by a single layer of endothelium, without intervening brain tissue (hematoxylin and eosin stain, ×40).
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Table 1. Summary of Reported Cases of Cavernous Angiomas of the Cerebral Gray Nucleia
