Summary
Twenty-six patients with myelographic signs suggestive of a spinal arteriovenous malformation (AVM) were examined neurologically and neurophysiologically. By selective spinal angiography it was possible to differentiate between dural arteriovenous fistulae (dAVF 20 patients) and intradural AVMs (iAVM, 6 patients). Initial complaints were nonspecific and variable, mainly consisting of sensory disorders and muscle weakness. Later, patients suffered involvement of both the upper and lower motor neurons. There was a high percentage of lower motor neuron lesions (95%), especially in dAVF patients, which were mostly of widespread distribution and included several myotomes. Electrophysiological examination regularly revealed lower neuron involvement, frequently with pathological spontaneous activity in several myotomes, pathological sensory-evoked potentials after tibial nerve stimulation, but normal sensory conduction velocities of the sural nerve, indicating sparing of the sensory ganglion. Frequently there was a discrepancy between the localization of the dural fistula or angioma and the spinal level responsible for clinical symptoms. This suggests that it may be the inadequacy of the venous drainage system to cope with the blood volume rather than the AV-shunt that is responsible for the symptoms. An early diagnosis is essential, as removal of the shunt before there has been progression to severe neurological deficits (paraplegia) is the only way to ensure a satisfactory outcome.
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Aboulker J, Bar D, Mursault L, Khoudja F, Redondo A, Gard L, Nahum H (1977) L'hypertension veineuse intrarachidienne par anomalies multiples du système cave: une cause majeure de souffrance médullaire. Chirurgie 103:1003–1015
Aminoff MJ, Logue V (1974) Clinical features of spinal vascular malformations. Brain 97:197–210
Assouline E, Gelbert F, Dormont D, Reizine D, Merland JJ (1988) MRI study of dural arteriovenous fistulae draining into the external spinal veins. J Neuroadiol 15:1–12
Bradac GB, Pöll W, Merland JJ (1984) Extradurale Angiome mit venöser perimedullärer Drainage. Neurochirurgia 27:136–140
Burguet JL, Dietemann JL, Wackenheim A, Kehr P, Buchheit F (1985) Sacral meningeal arteriovenous fistula fed by branches of the hypogastric arteries and drained through medullary veins. Neuroradiology 27:232–237
Di Chiro G (1972) Development of spinal cord angiography. Acta Radiol [Diagn] (Stockh) 13:767–770
Di Chiro G, Wener L (1973) Angiography of the spinal cord. A review of contemporary techniques and applications. J Neurosurg 39:1–29
Djindjian R (1978) Angiography in angiomas of the spinal cord. In: Pia HW, Djindjian R (eds) Spinal angiomas. Springer, Berlin Heidelberg New York, pp 98–136
Djindjian R, Hurth M, Houdart R (1970) L'angiographie de la moelle épinière. Masson, Paris
Endo S, Kusho K, Suzuki J (1979) Spontaneous regression of posterior fossa dural arteriovenous malformation. J Neurosurg 51:715–717
Hansen JH, Sogard JD (1976) Spontaneous regression of an extra-and intracranial arteriovenous malformation. J Neurosurg 45:338–341
Jellinger K, Minauf M, Garzuly F, Neumayer E (1968) Angiodysgenetische nekrotisierende Myelopathie (Bericht über 7 Fälle). Arch Psychiatr Nervenkr 211:377–404
Kendall BE, Logue V (1977) Spinal epidural angiomatous malformations draining into intrathecal veins. Neuroradiology 13:181–189
Merland JJ, Rich%'e MC, Chiras J (1980) Les fistules arterioveineuses intra-canalaires, extra-médullaires `a drainage veineux médullaire. J Neuroradiol 7:271–320
N'Diaye M, Chiras J, Meder JF, Barth MO, Koussa A, Bories J (1984) Apport de la myélographie aux hydrosolubles dans les fistules artério-veineuses dural rachidiennes à drainage veineux perimédullaire. J Neuroradiol 11:327–339
Oldfield EH, Di Chiro G, Quindlen EA, Rieth KG, Doppmann JL (1983) Successful treatment of a group of spinal cord arteriovenous malformations by interruption of dural fistula. J Neurosurg 59:1019–1030
Pia HW (1978) Symptomatology of spinal angiomas. In: Pia HW, Djindjian R (eds) Spinal angiomas. Springer, Berlin Heidelberg New York, pp 48–74
Rosenblum B, Oldfield EH, Doppmann JL, Di Chiro G (1987) Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM's in 81 patients. J Neurosurg 67:795–802
Schneider A (1980) Kreislaufstörungen und Gefäßprozesse des Rückenmarks. In: Doerr W, Seifert W (eds) Spezielle pathologische Anatomie. Vol 13/I, Springer, Berlin Heidelberg New York, pp 511–649
Symon L, Kuyama H, Kendall B (1984) Dural arteriovenous malformation of the spine. J Neurosurg 60:238–247
Terwey B, Becker H, Thron AK, Valdiek G (1989) Gadolinium — DTPA enhanced magnetic resonance imaging of spinal dural arteriovenous fistulas. J Comput Assist Tomogr 13:30–37
Thron A (1988) Vascular anatomy of the spinal cord. Neuroradiological investigations and clinical syndromes. Springer, Wien New York
Thron A, Koenig E, Peiffer P, Rossberg C (1987) Dural vascular anomalies of the spine — an important cause of progressive radiculomyelopathy. In: Cervos Navarro J, Ferszt R (eds) Stroke and microcirculation. Raven Press, New York, pp 159–165
Voigt K, Sauer M, Dichgans J (1971) Spontaneous occlusion of bilateral caroticocavernous fistula studied by serial angiography. Neuroradiology 2:207–211
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Koenig, E., Thron, A., Schrader, V. et al. Spinal arteriovenous malformations and fistulae: clinical, neuroradiological and neurophysiological findings. J Neurol 236, 260–266 (1989). https://doi.org/10.1007/BF00314453
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DOI: https://doi.org/10.1007/BF00314453