Dural arteriovenous fistula (D-AVF) at the foramen magnum is an extremely rare disease entity. It produces venous hypertension, and can lead to progressive cervical myelopathy thereafter. On the other hand, the venous hypertension may lead to formation of a venous varix, and it can rarely result in an abrupt onset of subarachnoid hemorrhage (SAH) when the venous varix is ruptured. The diagnosis of D-AVF at the foramen magnum as a cause of SAH may be difficult due to its low incidence. Furthermore, when the D-AVF is fed solely by the ascending pharyngeal artery (APA), it may be missed if the external carotid angiography is not performed. The outcome could be fatal if the fistula is unrecognized. Herein, we report on a rare case of SAH caused by ruptured venous varix due to D-AVF at the foramen magnum fed solely by the APA. A review of relevant literatures is provided, and the treatment modalities and outcomes are also discussed.
Angiography ; Arteries* ; Central Nervous System Vascular Malformations* ; Diagnosis ; Fistula ; Foramen Magnum* ; Hand ; Hypertension ; Incidence ; Rare Diseases ; Spinal Cord Diseases ; Subarachnoid Hemorrhage* ; Varicose Veins*

PURPOSE: To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome. CASE SUMMARY: A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months. CONCLUSIONS: Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.
Adrenal Cortex Hormones ; Angiography ; Blepharoptosis ; Cavernous Sinus* ; Central Nervous System Vascular Malformations* ; Cerebral Angiography ; Diagnosis ; Diplopia ; Embolization, Therapeutic ; Exophthalmos ; Exotropia ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Inflammation ; Magnetic Resonance Imaging ; Middle Aged ; Oculomotor Nerve Diseases ; Orbit ; Pupil ; Recurrence ; Strabismus ; Tolosa-Hunt Syndrome*

Intracranial dural arteriovenous fistula (dAVF) usually results in various problems in the brain. But it can be presented as a myelopathy, which may make early diagnosis and management to be difficult. We recently experienced a case of cervical myelopathy caused by intracranial dAVF. A 60-year-old man presented with a 3-year history of gait disturbance due to a progressive weakness of both legs. Neurological examination revealed spastic paraparesis (grade IV) and Babinski sign on both sides. Magnetic resonance imaging showed serpentine vascular signal voids at C2-T1 on T2-weighted image with increased signal intensity and swelling of spinal cord at C1-C4. We performed a brain computed tomography angiography and found intracranial dAVF with multiple arteriovenous shunts. Venous drainages were noted at tentorial veins and cervical perimedullary veins. After Onyx embolization, the patient showed gradual improvement in motor power and gait disturbance. The venous drainage pattern is a well-known prognostic factor of dAVF. In our case, the intracranial dAVF drained to spinal perimedullary vein, which seemed to result in the ischemic myelopathy. Although it is rare condition, it sometimes can cause serious complications. Therefore, we should keep in mind the possibility of intracranial dAVF when a patient presents myelopathy.
Angiography ; Arteriovenous Fistula ; Brain ; Central Nervous System Vascular Malformations* ; Drainage ; Early Diagnosis ; Gait ; Humans ; Ischemia ; Leg ; Magnetic Resonance Imaging ; Middle Aged ; Neurologic Examination ; Paraparesis, Spastic ; Reflex, Babinski ; Spinal Cord ; Spinal Cord Diseases* ; Spinal Cord Ischemia ; Veins

Persistent primitive trigeminal artery (PPTA) is the most common carotid-basilar anastomosis; on the other hand, persistent primitive otic artery (PPOA) is extremely rare. PPTA is often misdiagnosed as PPOA on cerebral angiography. We present a case of PPTA that mimicked PPOA on cerebral angiography. We further describe the utility of brain computed tomography angiography for differential diagnosis of PPTA from PPOA, together with a review of previous literature.
Angiography ; Arteries* ; Brain ; Central Nervous System Vascular Malformations ; Cerebral Angiography* ; Diagnosis, Differential ; Hand

Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation, however it is still rare and underdiagnosed. Magnetic resonance imaging findings such as spinal cord edema and dilated and tortuous perimedullary veins play a pivotal role in the confirmation of the diagnosis. However, spinal angiography remains the gold standard in the diagnosis of SDAVF. Classic angiographic findings of SDAVF are early filling of radicular veins, delayed venous return, and an extensive network of dilated perimedullary venous plexus. A series of angiograms of SDAVF at different locations along the spinal column, and mimics of serpentine perimedullary venous plexus on MR images, are demonstrated. Thorough knowledge of SDAVF aids correct diagnosis and prevents irreversible complications.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Central Nervous System Vascular Malformations/*diagnosis/epidemiology/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Spinal Cord Diseases/diagnosis ; Spine/radiography

Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage.
Basal Ganglia ; Brain ; Central Nervous System ; Diagnosis ; Follow-Up Studies ; Hemangioma, Cavernous, Central Nervous System* ; Hemorrhage ; Hemosiderin ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Vascular Malformations

Diagnosis of traumatic arteriovenous fistula (AVF) is usually delayed because it takes time to be enlarged enough to emerge radiologically or because symptoms occur a few months after the trauma. A 56-year-old woman presented with a newly developed tinnitus immediately after a head trauma. Pulsatile high-frequency tinnitus was heard also by examiner and recorded using a transcanal microphone. Angiography revealed an intracranial dural AVF fed by the middle meningeal artery, draining the superior sagittal sinus on the affected side. After percutaneous transarterial embolization, tinnitus successfully disappeared. The clinical presentation, radiologic and angiographic features, and management are discussed. To our knowledge, this is the first reported case of pulsatile tinnitus caused by AVF developed immediately after a trauma. We emphasize that precise physical examination, laboratory tests, and appropriate radiographic imaging are essential for accurate diagnosis and treatment when a patient presents with pulsatile tinnitus, especially after a head trauma.
Angiography ; Arteriovenous Fistula ; Central Nervous System Vascular Malformations* ; Craniocerebral Trauma* ; Diagnosis ; Female ; Golf* ; Head* ; Humans ; Meningeal Arteries ; Middle Aged ; Physical Examination ; Superior Sagittal Sinus ; Tinnitus*

Dural ateriovenous fistula (DAVF) at the craniocervical junction is rare. We report a patient presenting with brainstem dysfunction as an uncommon onset. Brainstem lesion was suggested by magnetic resonance image study. Angiogram revealed a DAVF at a high cervical segment supplied by the meningeal branch of the right vertebral artery, with ascending and descending venous drainage. Complete obliteration of the fistula was achieved via transarterial Onyx embolization. Clinical cure was achieved in the follow-up period; meanwhile, imaging abnormalities of this case disappeared. Accordingly, we hypothesize that a brainstem lesion of this case was caused by craniocervical DAVF, which induced venous hypertension. Thus, venous drainage patterns should be paid attention to because they are important for diagnosis and theraputic strategy.
Brain Stem* ; Central Nervous System Vascular Malformations ; Diagnosis ; Drainage ; Estrogens, Conjugated (USP)* ; Fistula* ; Follow-Up Studies ; Humans ; Hyperemia ; Hypertension ; Vertebral Artery

A 68-year-old man developed mild quadriparesis 1 month prior to presentation. At 4 days before presentation, he developed dysarthria and more severe quadriparesis, predominantly on the left side of the body. MRI revealed edematous lesions in the medulla and throughout the spinal cord. Angiography showed a right tentorial dural arteriovenous fistula (DAVF). Despite successful treatment achieved by endovascular embolization, there was no improvement in the weakness of either leg. Early diagnosis of tentorial DAVF is important to prevent neurological complications such as encephalomyelopathy and hemorrhage.
Aged ; Angiography ; Central Nervous System Vascular Malformations* ; Dysarthria ; Early Diagnosis ; Hemorrhage ; Humans ; Leg ; Magnetic Resonance Imaging ; Quadriplegia ; Spinal Cord

INTRODUCTIONWe reviewed the clinical features, brain and spinal cord magnetic resonance (MR) imaging findings and associated abnormalities in six patients with spinal cavernous malformations (CMs).

METHODSLesions were defined on gradient-recalled echo (GRE) images but measured on T2-weighted images performed on 1.5- and 3-tesla clinical scanners.

RESULTSFour patients had associated multiple cranial CMs and one patient had multiple spinal CMs. All spinal CMs were predominantly hypointense on GRE images, and most were predominantly hyperintense and surrounded by hypointense edge on T2-weighted images. Other associations included asymptomatic vertebral body and splenic haemangiomas.

CONCLUSIONWe conclude that intramedullary spinal CMs typically have 'mulberry' or 'popcorn' appearances similar to those of cranial CM. The presence of associated haemangioma or familial cranial CM syndrome on MR imaging may suggest the correct diagnosis without requiring invasive investigations.

Adult ; Aged ; Brain Neoplasms ; pathology ; Central Nervous System Vascular Malformations ; pathology ; Child, Preschool ; Diagnosis, Differential ; Female ; Hemangioma, Cavernous, Central Nervous System ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplastic Syndromes, Hereditary ; pathology ; Retrospective Studies ; Spinal Cord Diseases ; pathology ; Spinal Cord Neoplasms ; pathology