Keisuke Sasaki1, Tomoo Inoue2, Yasuo Nishijima3, Takashi Inoue3, Shinsuke Suzuki3, Toshiki Endo1, Masayuki Ezura3, Hiroshi Uenohara3, Teiji Tominaga4. 1. Department of Neurosurgery, National Hospital Organization, Sendai Medical Center, Miyagino-ku, Sendai, Miyagi, Japan; Department of Neurosurgery, Tohoku University, Graduate School of Medicine, Aoba-ku, Sendai, Miyagi, Japan. 2. Department of Neurosurgery, National Hospital Organization, Sendai Medical Center, Miyagino-ku, Sendai, Miyagi, Japan; Department of Neurosurgery, Tohoku University, Graduate School of Medicine, Aoba-ku, Sendai, Miyagi, Japan. Electronic address: tomoo49@gmail.com. 3. Department of Neurosurgery, National Hospital Organization, Sendai Medical Center, Miyagino-ku, Sendai, Miyagi, Japan. 4. Department of Neurosurgery, Tohoku University, Graduate School of Medicine, Aoba-ku, Sendai, Miyagi, Japan.
Abstract
BACKGROUND: Spinal dural arteriovenous fistulas (DAVFs) are usually associated with neurologic dysfunction adjacent to the shunt point; however, the symptoms are uncommon far from the site of the fistula. To our knowledge, this is the first report of a patient with rapidly progressive isolated pseudobulbar palsy because of thoracic DAVF. CASE DESCRIPTION: We report a patient with thoracic DAVF presenting with remote symptoms of brainstem congestion. The patient was a 36-year-old man who presented with a sudden history of vomiting, dysphagia, and flaccid weakness in the 4 limbs. Intracranial magnetic resonance (MR) imaging at a local hospital demonstrated T2 signal hyperintensity within the medulla, and he was referred to our hospital for a suspected brainstem lesion. However, cervical MR imaging revealed a dilated and tortuous perimedullary venous plexus, and spinal angiography revealed DAVF in T5-6 with a feeding artery from the intercostal artery. After obliteration of the fistula, the progression of the disease was stopped and the symptoms improved. CONCLUSIONS: Although rare, thoracic DAVFs may present symptoms resembling brainstem infarction. Prompt surgical intervention is necessary for patients with thoracic DAVF presenting with rapidly progressive pseudobulbar palsy.
BACKGROUND:Spinal dural arteriovenous fistulas (DAVFs) are usually associated with neurologic dysfunction adjacent to the shunt point; however, the symptoms are uncommon far from the site of the fistula. To our knowledge, this is the first report of a patient with rapidly progressive isolated pseudobulbar palsy because of thoracic DAVF. CASE DESCRIPTION: We report a patient with thoracic DAVF presenting with remote symptoms of brainstem congestion. The patient was a 36-year-old man who presented with a sudden history of vomiting, dysphagia, and flaccid weakness in the 4 limbs. Intracranial magnetic resonance (MR) imaging at a local hospital demonstrated T2 signal hyperintensity within the medulla, and he was referred to our hospital for a suspected brainstem lesion. However, cervical MR imaging revealed a dilated and tortuous perimedullary venous plexus, and spinal angiography revealed DAVF in T5-6 with a feeding artery from the intercostal artery. After obliteration of the fistula, the progression of the disease was stopped and the symptoms improved. CONCLUSIONS: Although rare, thoracic DAVFs may present symptoms resembling brainstem infarction. Prompt surgical intervention is necessary for patients with thoracic DAVF presenting with rapidly progressive pseudobulbar palsy.