Address correspondence and reprint requests to Dr. Dae Il Chang, Department of Neurology, College of Medicine, Kyunghee University, #1 Hoegidong, Dongdaemun-ku, Seoul, 130-702, Korea; e-mail: dichang@khmc.or.kr Copyright © 2003 by AAN Enterprises, Inc. References 1. Chajchir A, Benzaquen I. Fat grafting injection for soft tissue augmentation. Plast Reconstr Surg 1989;84:921–934. 2. Dreizen NG, Framm L. Sudden unilateral visual loss after autologous fat injection into the glabella area. Am J Ophthalmol 1989;107:85– 87. Transient brainstem ischemia and recurrent syncope caused by a dural arteriovenous fistula M. Lanz, MD; U. Thiemann, MD; U. Grzyska, MD; M. Ebke, MD; G. Schwendemann, MD; and J.A. Kraus, MD Dural arteriovenous fistulas (DAVFs) are direct artery-to-cerebral venous sinus shunts. Because of their relative rarity, diagnosis of DAVF is often delayed especially if symptoms are of infratentorial or spinal localization. Here we report a case of DAVF leading to transient brainstem ischemia and recurrent syncope. Case report. A 68-year-old right-handed white woman had recurrent, fully reversible episodes of diplopia and dysarthria for 1 year. These episodes were sometimes accompanied by transient acute weakness of her legs lasting up to 3 minutes and unrelated to posture or activity. She also had a history of recurrent syncope accompanied by a cessation of spontaneous breathing and palpable pulses resolving spontaneously within few seconds. There was a single episode of acute respiratory failure leading to resuscitation with no evidence of obstructive or infectious pulmonary disease or lung edema. Repeated cardiac workup did not reveal any disease. When admitted for acute dysarthria, the neurologic examination also disclosed a mild left hemiparesis, ataxia of her right upper extremity, increased reflexes of her lower limbs, and Babinski signs. The next day, she developed dysphagia and a right hemiparesis of fluctuating intensity leading to mild quadriparesis. The T1-weighted contrast media-enhanced MRI of the brainstem and cervical spinal cord revealed enlarged perimedullary veins Figure. (A) Enlarged perimedullary veins of the cervical spinal cord (arrows) on sagittal T1-weighted, contrast media-enhanced MRI. (B) Congestive edema of the brainstem at the pontomedullary junction and the upper cervical spinal cord (arrow) on sagittal T2-weighted MRI. (C) Angiography at the craniospinal junction (lateral view). The microcatheter (Target, Boston Scientific, Natick, MA) is located in the occipital branch of the middle meningeal artery (long small arrow) and the ending of the sigmoid sinus (short small arrow). The blood of the dural arteriovenous fistula (DAVF) is pathologically drained in veins of the brain stem and spinal cord (thick arrow). (D) Angiography at the craniospinal junction (lateral view). Injection of contrast media in the middle meningeal artery discloses the DAVF draining in the sigmoid sinus (small arrow) and spinal veins (thick arrow). 1152 NEUROLOGY 61 October (2 of 2) 2003 3. Egido JA, Arroyo R, Marcos A. Middle cerebral artery embolism and unilateral visual loss after autologous fat injection into the glabellar area. Stroke 1993;24:615– 616. 4. Valdatta L, Thione A, Buoro M, Tuinder S. A case of life-threatening sepsis after augmentation by fat injection. Aesthetic Plast Surg 2001;25: 347–349. 5. Tettenborn B, Caplan LR, Sloan MA, et al. Postoperative brainstem and cerebellar infarcts. Neurology 1993;43:471– 477. 6. Müller C, Rahn BA, Pfister U, Meining RP. The incidence, pathogenesis, diagnosis, and treatment of fat embolism. Orthop Rev 1994;23:107–117. 7. Nornes H, Sorteberg W, Nakstad P, et al. Hemodynamic aspects of clinical cerebral angiography. Acta Neurochir (Wien) 1990;105:89 –97. (figure, A), and the T2-weighted images demonstrated congestive edema of the brainstem at the pontomedullary junction and the upper cervical spinal cord (figure, B). There was no evidence of an ischemic lesion on the T1-weighted MRI of the brainstem. Angiography disclosed a DAVF supplied by the left middle meningeal and mastoid meningeal arteries. The DAVF emptied into the proximal left sigmoid sinus, which drained into the brainstem and medullary veins (figure, C and D). There were no flow signals in the distal part of the left sigmoid sinus, left transverse sinus, left superior and inferior petrosal sinuses, and left proximal internal jugular vein on magnetic resonance angiography, suggesting atresia or thrombosis of these parts of the venous drainage system. The DAVF was successfully managed by endovascular embolization with isobutylcyanoacrylate. On clinical follow-up evaluation, no further episodes of diplopia or dysarthria as symptoms of transient brainstem ischemias or syncopes occurred. On neurologic examination, there was no evidence of quadriparesis, but increased reflexes of the lower limbs were observed. Discussion. The etiology of DAVFs remains controversial. Initially thought to be congenital, now it is thought that cerebral sinus thrombosis and elevated sinus pressure may play a role. Impaired venous outflow may cause indigenous dysplastic dural vessels within the sinus, establishing a direct artery-to-sinus communication that causes the opening of pre-existing microarteriovenous fistulas in the sinus walls.1-3 Increased resistance to activated protein C and factor V Leiden in white patients with DAVFs has also been reported, suggesting a link between thrombophilia and the development of DAVFs.4 However, other thrombophilic risk factors (e.g., prothrombin G20210A, MTHFRC677T, ␤-fibrinogenG455A, PAI-14G/5G, and FXIIIVal34Leu) are apparently not involved in the pathogenesis of DAVFs.5 Another case of a DAVF in a patient with recurrent transient brainstem ischemia with the DAVF located at the craniocervical junction has been reported.6 However, we report additional clinical features, such as recurrent syncope and a single episode of transient respiratory failure, as unique, previously undocumented symptoms of a DAVF. Branches of the external carotid artery fed the DAVF in our reported case. The abnormal draining of the proximal left sigmoid sinus into the brainstem and medullary veins was an additional prerequisite for the occurrence of clinical symptoms. Intermittent increased pressure of these engorged and congested veins is the most likely cause of the transient brainstem ischemias, which resulted in dysarthria, dysphagia, syncopes, and respiratory failure. The radiologic findings do not differentiate between atresia and thrombosis of the left venous sinuses leading to the abnormal drainage of the proximal left sigmoid sinus. However, because the patient developed clinical symptoms at age 67 years, it is possible that the abnormal drainage was the result of thrombosis. Thrombosis of the distal left sigmoid and other sinuses and consecutive elevation of the venous pressure in the proximal left sigmoid sinus may have contributed to the development of the DAVF. From the Departments of Neurology (Drs. Lanz, Ebke, Schwendemann, and Kraus) and Radiology (Dr. Thiemann), Zentralkrankenhaus Bremen Ost, Bremen, Germany; and Department of Neuroradiology (Dr. Grzyska), University of Hamburg, Germany. Received January 27, 2003. Accepted in final form June 10, 2003. Address correspondence and reprint requests to Dr. Michael Lanz, Department of Neurology, Zentralkrankenhaus Bremen Ost, Züricher Strasse 40, D-28325 Bremen, Germany; e-mail: MLanz@zkhost.bremen.de Copyright © 2003 by AAN Enterprises, Inc. References 1. Herman JM, Spetzler RF, Bederson JB, Kurbat JM, Zabramski JM. Genesis of a dural arteriovenous malformation in a rat model. J Neurosurg 1995;83:539 –545. 2. Kutluk K, Schumacher M, Mironov A. The role of sinus thrombosis in occipital dural arteriovenous malformations— development and spontaneous closure. Neurochirurgia (Stuttg) 1991;34:144 –147. 3. Mullan S. Reflections upon the nature and management of intracranial and intraspinal vascular malformations and fistulae. J Neuro- Conduction block of varicella zoster virus neuropathy T. Murakami, MD; K. Shibazaki, MD; K. Kurokawa, MD; Y. Ichikawa, MD; Y. Ohsawa, MD; and Y. Sunada, MD Motor involvement by varicella zoster virus (VZV) in the extremities is relatively rare and may be due to the extension of inflammation to the anterior horn or anterior motor roots.1 In this study we observed conduction block of the peripheral nerves and lesions in the right upper extremity on MRI in a patient with VZV infection. Case report. A 64-year-old woman noticed swelling of her right index finger followed by dysesthesia, reddening of the skin, and vesicle in the area from the right shoulder to the palm. She was diagnosed with herpes zoster and treated with acyclovir IV. She had been treated for chronic myelocytic leukemia (CML, chronic stage) since 1992. Four weeks later she noticed weakness of her right upper extremity that progressed slowly. She was admitted to our hospital 2 months later. Physical examination revealed pigmentation of skin in the right C6 and C7 dermatomes. Cranial nerves were intact. Manual muscle testing revealed weakness in the right upper extremity, which did not correspond to individual myotomes: deltoid (4/5), biceps (4/5), brachioradialis (4/5), triceps (3/5), extensor carpi radialis (3/5), extensor digitorum (3/5), abductor pollicis brevis (2/5), flexor digitorum superficialis (1/5), abductor digiti minimi (4/5), and dorsal interossei (4/5) (MRC rating scale). There was mild hypesthesia and hyperalgesia in the median-innervated area of the right palm. Deep tendon reflexes were absent in the right upper extremity. White blood count was 3,900/␮L. C-reactive protein was slightly elevated (4.9 mg/dL). Sedimentation rate was 75 mm/hour. Antinuclear antibody and p-ANCA were negative. The serum titer of anti-VZV immunoglobulin (Ig) M was negative, but that of anti-VZV IgG was elevated. Cell numbers, protein level, sugar, and IgG index in the CSF were normal. Nerve conduction study revealed conduction block and conduction velocity slowing of the right median nerve in the forearm (figure, A). Conduction block was also demonstrated between the cubitus and brachial plexus of the right radial nerve (figure, B). Needle EMG showed active neurogenic change in the right triceps muscles suggesting the involvement of surg 1994;80:606 – 616. 4. Kraus JA, Stüper BK, Nahser HC, Klockgether T, Berlit P. Significantly increased prevalence of factor V Leiden in patients with dural arteriovenous fistulas. J Neurol 2000;247:521–523. 5. Kraus JA, Stüper BK, Müller J, et al. Molecular analysis of thrombophilic risk factors in patients with dural arteriovenous fistulas. J Neurol 2002;249:680 – 682. 6. Masuo O, Ozaki F, Okita R, et al. Dural arteriovenous fistula at the cranio-cervical junction presenting transient ischemic attack: a case report. No Shinkei Geka 1999;27:1043–1046. the part between spiral groove of the upper arm and brachial plexus. MRI of the right forearm demonstrated extensive lesions in the connective tissue around the finger flexor tendons and muscles along the median nerve. There was no compressive mass in the forearm such as chloroma (local collection of blast cells), which may be seen in CML. Oral administration of prednisolone improved the strength of abductor pollicis brevis (3/5), the conduction blocks of the two nerves, and the lesions on MRI. Discussion. Our case was an immunocompromised host owing to CML, and the reactivation of VZV seemed to occur in the dorsal root ganglia, resulting in sensory disturbances and skin lesions first. After the appearance of usual symptoms of herpes zoster, motor involvement occurred at the level of peripheral nerves. Electrophysiologic study demonstrated the conduction block at uncommon sites of two different nerves. These findings suggested that the Schwann cells of the motor nerves were damaged locally by viral direct invasion or allergic mechanisms. In addition, we observed abnormal signals in the connective tissues along the median nerve on MRI. As the lesions were diminished by steroid therapy, they probably reflected the inflammatory changes. Motor involvement of herpes zoster has been reported usually as segmental zoster paresis, and rarely polyneuritis (GuillainBarré syndrome), but there is no report of conduction block of peripheral nerves associated with VZV infection presenting with mononeuritis multiplex. In segmental zoster the responsible lesions for motor involvement are considered to be anterior horn or anterior root, to where the inflammation in the ganglia extends.2,3 In respect to polyneuritis after zoster, the segmental demyelination and remyelination of the nerves was described in two patients.4 There are only a few reports of mononeuropathy,2,5 in which motor nerve demyelination was suggested in four cases by electrophysiologic findings (disproportionate conduction slowing or temporally dispersed compound muscle action potentials). Abnormal MRI findings have been reported in the cauda equina, spinal nerves, cord, cranial nerves, pons, cerebral white matter, and meninges in VZV infection.1,6 We applied MRI to examine the conduction block of our patient. Abnormally increased short-tau inversion recovery signals were observed stronger in the flexor tendon and muscle interspaces along the median nerve than the muscle itself. Although acute denervation of muscle might change the MRI signal in some part, the extensive Figure. (A) Right median compound muscle action potentials (CMAP) recorded from the thenar muscle of the patient. The sites of stimulation include 2.5, 3.7, and 5.7 cm proximal to the distal crease at the wrist and elbow. The median sensory response was absent. This study demonstrated focal conduction block and slowing in the lower forearm segment. One month later, the CMAP with stimulation 3.7 cm proximal to the wrist increased from 0.31 to 1.24 mV. (B) Right radial CMAP recorded from the extensor carpi ulnaris muscle. The sites of stimulation include the mid forearm, above the elbow, proximal to the spiral groove, and Erb point. The median and ulnar nerves were stimulated above the elbow simultaneously as a reference. Conduction block was observed between cubitus and brachial plexus. October (2 of 2) 2003 NEUROLOGY 61 1153 Transient brainstem ischemia and recurrent syncope caused by a dural arteriovenous fistula M. Lanz, U. Thiemann, U. Grzyska, et al. Neurology 2003;61;1152-1153 DOI 10.1212/WNL.61.8.1152 This information is current as of October 27, 2003 Updated Information & Services including high resolution figures, can be found at: http://www.neurology.org/content/61/8/1152.full.html References This article cites 5 articles, 0 of which you can access for free at: http://www.neurology.org/content/61/8/1152.full.html##ref-list-1 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 Arteriovenous malformation http://www.neurology.org//cgi/collection/arteriovenous_malformation MRI http://www.neurology.org//cgi/collection/mri 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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