A case of fibromuscular dysplasia presenting lateral medullary syndrome

We reported a case of fibromuscular dysplasia (FMD) presenting lateral medullary syndrome accompanied with left truncal ataxia, left Horner's syndrome and superficial sensory deficit in the right extremities. He was 46-year-old man and had no remarkable risk factors for the cerebrovascular disease such as hypertension, diabetes mellitus and valvular heart disease. Cerebral angiography was performed and there was the string-of-beads-like shadow in the left vertebral artery, revealing a localized FMD. Wedge-shaped area of low signal intensity in the left lower medulla was recognized in magnetic resonance imaging (spin echo Tr/Te 600/30). It was suggested that the lesion of the lower medulla caused the patient's several symptoms. In Japan, there have been a few case reports of cervical or intracranial FMD, but we cannot find any report of FMD localizing in the unilateral vertebral artery. In general, it has been said that the etiology of lateral medullary syndrome under fifty years old differs from those of geriatric patients. The authors pointed out the significance of FMD as one of those risk factors causing lateral medullary syndrome and also discussed the mechanism of the sensory disturbance sparing face.     

Bilateral medial medullary infarction due to bilateral vertebral artery dissection

We describe a 52-year-old woman who experienced transient motor weakness and numbness of the left extremities and presented 2 days later with severe hemiparesis and sensory impairment of the right extremities and right lingual palsy. Magnetic resonance imaging (MRI) revealed bilateral upper medial medullary infarction, primarily in the left ventral portion. The findings of both three-dimensional (3D) computed tomographic and conventional angiography suggested dissection of both intracranial vertebral arteries (VAs). Medial medullary infarction is generally caused by atherosclerosis within a VA or anterior spinal artery. This is the first report of bilateral medial medullary infarction due to dissection of both intracranial VAs.     

A case of brain stem infarction with bilateral hearing impairment and tinnitus at the onset

We reported a 49-year-old male with brain stem infarction who had bilateral hearing impairment and tinnitus at the onset and subsequently developed various neurological symptoms, including bilateral lateral inferior pontine syndrome, one and a half syndrome and upward gaze palsy. Although CT scan failed to reveal any abnormalities initially, MRI revealed symmetrical foci bilaterally from the lateral inferior pons to the middle cerebellar peduncle, as well as in the paramedian portion of the mid-pons. Cerebral angiography: The left vertebral artery (VA) occluded at the 4th segment. The right VA showed severe stenosis at the 4th segment. The basilar artery (BA) was found to be occluded in the lower 1/3 below the clivus. Furthermore, CAG demonstrated upper portion of the BA, bilateral superior cerebellar artery and posterior cerebral artery via the posterior communicating artery, but the bilateral anterior inferior cerebellar arteries (AICAs) were absent or occluded. Neuroradiological findings suggested ischemia in the bilateral AICA and the middle portion of the BA. Bilateral hearing impairment rarely accompanies cerebrovascular disorders. This case of bilateral hearing impairment, tinnitus at the onset, followed by bilateral lateral inferior pontine syndrome was considered to be an extremely rare pathological condition.     

Fibromuscular dysplasia of the basilar artery presenting as cerebral infarction in a young female]

We reported a 20-year-old woman with fibromuscular dysplasia (FMD) of the basilar artery presenting multiple cerebral infarctions. A sudden onset of consciousness disturbance and right hemiparesis was experienced. A neurological examination on day 2 revealed an absence of light and corneal reflexes on the left side, homonymous left upper quadrant anopsia and right hemiparesis with Babinski sign: she was also somnolent. On head MRI, multiple high signal intensity lesions were seen in the right occipital lobe, bilateral thalami and left pons on T2- and diffusion weighted images. Brain angiogram revealed the string of beads sign of the basilar artery, suggesting FMD. Neurological deficits gradually improved in the 2 months that followed, leaving slight hemiparesis and homonymous left upper quadrant anopsia In the following 3 years, no recurrence was seen with aspirin (81 mg/day). FMD in the head and neck usually affects extracranial segments of the carotid and vertebral arteries, while FMD of the basilar artery is extremely rare. To the best of our knowledge, 12 cases with FMD of the basilar artery have been reported; of these, 11 were symptomatic and 5 died. Since FMD of the basilar artery has poor prognosis, attention needs to be paid for FMD in young adults as a differential diagnosis of cerebral infarction in the territory of the basilar artery.     

Rotational vertebral artery syndrome due to compression of nondominant vertebral artery terminating in posterior inferior cerebellar artery

Rotational vertebral artery syndrome (RVAS) is characterized by recurrent attacks of paroxysmal vertigo, nystagmus, and ataxia induced by head rotation. We report on a patient who developed atypical RVAS due to compression of the vertebral artery (VA) terminating in the posterior inferior cerebellar artery (PICA). A 59-year-old man suffered from vertigo and nystagmus induced by leftward head rotation and oculography showed right beating horizontal-torsional and downbeat nystagmus. Cerebral angiography showed hypoplastic right VA terminating in PICA without connection to the basilar artery. The basilar artery received its flow from the left VA only and branched out both anterior inferior cerebellar arteries. Cerebral angiography revealed a complete occlusion of the right distal VA at the level of the C1–2 junction when the head was rotated to a leftward position. In contrast, the blood flow through the left vertebral and basilar arteries remained intact while turning the head to either side. The hemodynamic compromise observed in our patient with RVAS indicates that isolated vertigo and nystagmus may occur due to transient ischemia of the inferior cerebellum or lateral medulla.     

Hemifacial spasm developed after contralateral vertebral artery ligation

Although the mechanism of hemifacial spasm (HFS) is not yet well established, vascular compression of the facial nerve root exit zone and hyperexcitability of the facial nucleus have been suggested. We report a case of HFS in the setting of coinciding intracranial hemorrhage (ICH) of the pons and proximal ligation of the contralateral vertebral artery (VA) for the treatment of a fusiform aneurysm of the distal VA and discuss the possible etiologies of HFS in this patient. A 51-year-old male with an ICH of the pons was admitted to our hospital. Neuroimaging studies revealed an incidental fusiform aneurysm of the right VA distal to the origin of the posterior inferior cerebellar artery. Eight months after proximal ligation of the VA the patient presented with intermittent spasm of the left side of his face. Pre- and post-ligation magnetic resonance angiography revealed an enlarged diameter of the VA. The spasm completely disappeared after microvascular decompression.     

Bilateral vertebral artery dissection and its chronological changes detected by MR angiography: a case report]

A case of intracranial bilateral vertebral artery (VA) dissection presenting with ischemic symptoms which following unilateral dissection is presented. A 42-year-old male with an occipital headache was pointed out right vertebral artery stenosis with magnetic resonance (MR) angiography 8 day before admission. He admitted to our hospital complaining of severe vertigo and tinnitus. MR images and cerebral angiograms revealed bilateral VA dissection with infarcts in light lower surface of cerebellum perfused by posterior inferior cerebellar artery and right hypothalamus. Conservative therapy was adopted and serial MR angiography was performed. His symptoms were improved gradually and MR angiograms obtained 2 months later revealed improvement of bilateral VA stenosis. It is generally accepted that VA dissection presenting ischemic symptoms has good outcome by conservative therapy only. However, its pathological process of progression is still unknown. Based on the serial MR findings, we discuss the mechanisms of bilateral VA dissection. In this case, we consider that unilateral VA dissection extended to contralateral vertebral artery through the vertebrobasilar junction. Frequent MR angiography in acute phase could be of great use for monitoring the progression of dissection.     

Babinski-Nageotte syndrome on magnetic resonance imaging

A 70-year-old woman developed left hypoglossal nerve palsy, a right hemiparesis sparing the face, and a typical left Wallenberg's syndrome. These symptoms resulted from a lesion in the left half of the medulla oblongata, suggesting Babinski-Nageotte syndrome, a rare cerebrovascular disease. This is the first case of ischemic infarction in the territory of the left vertebral artery and posterior inferior cerebellar artery demonstrated on magnetic resonance imaging. Severe bilateral lesions of the distal vertebral arteries demonstrated on digital subtraction angiography may have contributed to the development of this syndrome.     

硬脑膜动-静脉瘘合并髓静脉扩张的临床及影像学表现(附1例报告及文献复习)

目的报道1例硬脑膜动-静脉瘘(DAVF)合并髓静脉扩张病例的临床资料,探讨DAVF合并髓静脉扩张的临床及影像学表现。方法回顾性收集1例DAVF合并髓静脉扩张患者的临床及影像学资料,并结合文献复习进行分析。结果患者以症状性癫痫起病。头颅MRI表现为脑白质内迂曲、扩张血管影,并继发性脑梗死和脑水肿;全脑数字减影血管造影(DSA)提示右侧颈内动脉造影静脉期上矢状窦前1/3不显影,静脉晚期颅内多发髓静脉迂曲增粗,经室管膜下静脉-大脑内静脉回流,右侧颈外动脉造影示DAVF形成,大脑上静脉逆向回流,左侧颈内动脉造影示左侧发育性静脉畸形。经血管内介入栓塞DAVF治疗后患者病情改善。结论DAVF合并髓静脉扩张少见,可继发脑实质病变进而出现神经功能缺损症状与体征;影像学表现为头颅MRI提示髓静脉扩张涉及的疾病谱较多,需仔细鉴别;MRI和DSA联合检查能更好评价脑实质和血管情况。     

Embolic internal auditory artery infarction from vertebral artery dissection

A 51-year-old man developed sudden vertigo, right hearing loss and dysphagia. Examination revealed right Horner syndrome, spontaneous torsional-horizontal nystagmus, right central type facial palsy, dysarthria, reduced soft palate elevation without gag reflex, left hypesthesia, right dysmetria and imbalance. Audiometry and bithermal caloric tests documented right sensorineural hearing loss and canal paresis. Brain MRI and cerebral angiography documented right lateral medullary infarction from vertebral artery dissection, without involvement of other parts of the brainstem supplied by the anterior inferior cerebellar artery (AICA). This case suggests artery-to-artery embolism as a possible mechanism of isolated vertigo or hearing loss from labyrinthine infarction.     

Rotational vertebral artery syndrome

Whether the rotational vertebral artery syndrome (RVAS), consisting of attacks of vertigo, nystagmus and tinnitus elicited by head-rotation induced compression of the dominant vertebral artery (VA), reflects ischemic dysfunction of uni- or bilateral peripheral or central vestibular structures, is still debated. We report on a patient with bilateral high-grade carotid stenoses, in whom rightward headrotation led to RVAS symptoms including a prominent nystagmus. Three-dimensional kinematic analysis of the nystagmus pattern, recorded with search coils, revealed major downbeat nystagmus with minor horizontal and torsional components. Magnetic resonance angiography demonstrated a hypoplastic right VA terminating in the posterior inferior cerebellar artery, a dominant left VA, and a hypoplastic P1-segment of the left posterior cerebral artery (PCA) that was supplied by the left posterior communicating artery (PCoA). The right PCA and both anterior inferior cerebellar arteries were supplied by the basilar artery. The right PCoA originated from the right internal carotid artery. Color duplex sonography showed severe reduction of diastolic blood flow velocities in the left VA during RVAS attacks. The nystagmus pattern can be best explained by vectorial addition of 3D sensitivity vectors of stimulated right and left anterior and horizontal semicircular canals with slightly stronger stimulation on the left side. We hypothesize that in RVAS, compression of dominant VA leads to acute vertebrobasilar insufficiency with bilateral, but asymmetric ischemia of the superior labyrinth. With regard to RVAS etiology, our case illustrates a type of pure vascular RVAS. Severity of attacks markedly decreased after successful bilateral carotid endarterectomy.     

The medial medullary infarction (Dejerine syndrome) following chiropractic neck manipulation]

A-38-year-old man suddenly developed nausea, vomiting and vertigo during chiropractic neck manipulation. This was followed by right hemiplegia, right deep sensory disturbance and left hypoglossal nerve palsy, consistent with the medial medullary infarction (Dejerine syndrome). The MRI revealed infarction at left medial part of the medulla. The vertebral angiogram and MRA showed marked narrowing of the left vertebral artery. X-rays of the cervical spine showed no spondylosis, dislocation nor osteolysis of the odontoid process. The serological studies, including lupus anticoagulant, protein C, and protein S gave normal results. Although vascular accidents involving the brain stem after chiropractic neck manipulation have been reported since Pratt-Thomas and Berger, previous reports are still rare. In them lateral medullary infarction (Wallenberg syndrome) is probably the most common case. On the other hand, medial medullary syndrome (Dejerine syndrome) is absolutely rare. To our knowledge, the only one report has been made by Watanabe and his colleagues before our present case. The mechanism was suggested that rotation and tilting of the neck stretches and compresses the vertebral artery at the cervical joint causing injury to the vessel, with an intimal tearing, dissection, and pseudoaneurysm formation. Consequently, the present case may be caused by injury to the left vertebral artery with an intimal tearing during neck manipulation sufficient to cause disection and subsequent infarction of the brain stem.     

A case of fibromuscular dysplasia presenting with Wallenberg syndrome, and developing a giant aneurysm of the internal carotid artery in the cavernous sinus]

A 25-year-old man developed Wallenberg syndrome (WS). At that time his carotid angiography was normal. When he was 28 years old, he suffered from retinal artery embolism in the left eye. At the age of 30 years, he had an acute onset of abducens nerve palsy in his right eye. The carotid angiography showed a giant aneurysm at the cavernous sinus portion in the right internal carotid artery. At his age of 38, the right oculomotor, trochlear and trigeminal nerves were involved. A vertebral angiography revealed a bead-like formation, and a diagnosis of fibromuscular dysplasia (FMD) was made. An intensive angiographic examination revealed many stenotic or dilated lesions in the carotid, vertebral, coronary, renal, and hepatic arteries. A sural nerve biopsy specimen revealed that the sural vein was involved. In Japan only one case of FMD presenting with WS is known. FMD should be under consideration as an underlying disease, when WS occurred in younger patients with few risk factors. In this patient an angiography revealed no abnormality in the cavernous sinus portion of the internal carotid artery, when he suffered from WS. However, eight years later he was proved to have a giant aneurysm in the cavernous sinus portion. In conclusion, we support the hypothesis that aneurysm may originate from angiographically normal arterial wall in FMD.     

A Case of Posterior Circulation Embolism Due to a Subtype of Bow Hunter's Syndrome Diagnosed by Non-Invasive Examination

Bow hunter's syndrome is the mechanical compression of the vertebral artery due to cervical rotation, resulting in ischemic symptoms in the vertebrobasilar artery territory. However, some cases present without typical symptoms and exhibit compression of the non-dominant side of the vertebral artery. We encountered a case of posterior circulation embolism due to a subtype of bow hunter's syndrome in a 74-year-old man. Although the right vertebral artery was not visualized on time-of-flight magnetic resonance angiography in the neutral position, duplex ultrasonography and time-of-flight magnetic resonance angiography in the left cervical rotation position showed blood flow in the right vertebral artery. In this case, blood flow in the contralateral vertebral artery was normal, and typical bow hunter's syndrome symptoms did not occur. In a case of posterior circulation embolism with undetermined etiology, wherein the routine duplex ultrasonography and time-of-flight magnetic resonance angiography results were inconclusive, additional testing with head positioning led to the diagnosis of a subtype of bow hunter's syndrome.     

Sphenoid Ridge Meningioma Presenting as Acute Cerebral Infarction

A previously healthy 52-year-old man presented to the emergency room with acute onset left hemiparesis and dysarthria. Brain computed tomography and magnetic resonance examinations revealed acute cerebral infarction in the right middle cerebral artery territory and a sphenoid ridge meningioma encasing the right carotid artery terminus. Cerebral angiography demonstrated complete occlusion of the right proximal M1 portion. A computed tomography perfusion study showed a wide area of perfusion-diffusion mismatch. Over the ensuing 48 hours, left sided weakness deteriorated despite medical treatment. Emergency extracranial-intracranial bypass was performed using a double-barrel technique, leaving the tumor as it was, and subsequently his neurological function was improved dramatically. We present a rare case of sphenoid ridge meningioma causing acute cerebral infarction as a result of middle cerebral artery compression.     

A case of bilateral cerebellar infarction in the distribution of the bilateral superior cerebellar artery]

We reported a case of bilateral cerebellar hemorrhagic infarction in the distribution of the bilateral superior cerebellar artery. A 58-year-old man suddenly developed dizziness and transient loss of consciousness. The neurological examination revealed left hearing disturbance, left sensory disturbance involving face, dysarthria and bilateral ataxia. This patient was considered to be classic clinical syndrome of right superior cerebellar artery. CT and MRI revealed hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery. The right posterior cerebral artery was filling through the right posterior communicating artery on the right carotid angiography taken 2 hours after the onset. Bilateral vertebral angiography on the 18th day demonstrated no occlusions in the basilar artery and the bilateral superior cerebellar artery. Hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery, sparing other territories as the present case, is extremely rare. In this case, cerebral embolism (top of the basilar syndrome) was suggested because of existence of atrial fibrillation and sudden onset.     

Churg-Strauss syndrome presenting with subarachnoid hemorrhage from ruptured dissecting aneurysm of the intracranial vertebral artery

Churg-Strauss syndrome (CSS) represents a rare systemic vasculitis that is almost invariably accompanied by bronchial asthma and eosinophilia. We report a case of a 36-year-old woman with previously diagnosed CSS presented with subarachnoid hemorrhage (SAH) from dissecting aneurysm in a vertebral artery (VA). Two months before onset of SAH, the patient had presented with numbness on her right lower leg due to peripheral neuropathy. On admission, angiography revealed dissecting aneurysm of the right intracranial VA and stenosis of the basilar artery. Hematological examination revealed an increased percentage of eosinophils. Ruptured dissecting aneurysm of the intracranial VA was diagnosed. Emergent coil embolization of the dissecting aneurysm and occlusion of the parent artery was performed to prevent repeated hemorrhage from the dissecting aneurysm. Then pharmacotherapy with prednisone was initiated for CSS. The patient recovered well and was discharged without any neurological deficit. As far as we know, this is the first reported case of CSS presented with SAH from dissecting aneurysm on posterior circulation.     

Cerebellopontine angle lipoma with extracranial extension

Lipomas of the cerebellopontine angle (CPA) are rare. A recent literature review identified only 98 reported cases of CPA lipoma. We present here a case of CPA lipoma in a 28-year-old woman who was admitted to our hospital with hearing loss in her left ear. Computed tomography scan and magnetic resonance imaging revealed a CPA mass lesion with extracranial extension around the left internal carotiol artery. The patient was operated on in the sitting position via a right suboccipital craniectomy. The intracranial part of the mass was partially removed. Histopathological examination resulted in a diagnosis of lipoma. Surgical treatment of CPA lipomas is rarely indicated, and the aim of surgery must be decompression of neural structures.     

Isolated limb sensory disturbance accompanied with sudden deafness from vertebral artery dissection: a case report

A 52-year-old male presented with right sudden deafness and left isolated limb sensory disturbance following posterior neck pain. An examination revealed a mild degree of right sensory neural hearing loss and decreased pain and thermal sensation in the left side below the L3 level. Brain MRI showed a small lesion in the midlateral portion of the right middle-rostral medulla. The combination of the image findings suggests a right lateral medullary infarction caused by a vertebral artery (VA) dissection. In our case, the fibers carrying the modalities of the lateral spinothalamic tract restricted to below the L3 level, sparing other sensations, suggest a selective involvement. This finding suggests that sensory dermatomal representation in the modality may be preserved at least up to the level of the medulla oblongata. Moreover, in our case, the small emboli originating from the dissected VA possibly caused a small selective cochlear infarction. This type of embolism may give rise to various combinations of symptoms depending on the branches involved. For patients with an acute onset of headache or neck pain who present with isolated limb sensory disturbance accompanied by sudden deafness, even without the classical brainstem signs, a VA dissection and medullary infarction should therefore be considered.     

Transbrachial Stent-Assisted Coil Embolization of Right Posterior Inferior Cerebellar Artery Aneurysm: Technical Case Report

OBJECTIVE AND IMPORTANCE: We describe a case of stent-assisted coil embolization of a wide neck right posterior inferior cerebellar artery (PICA) aneurysm via a transbrachial artery approach. CLINICAL PRESENTATION: A 73-year-old woman with a past medical history of hypertension, hyperlipidemia, colon cancer, and osteoporosis presented with a throbbing occipital area headache. Magnetic resonance angiography revealed a wide neck right PICA aneurysm. She presented for a planned endovascular stent-assisted coil embolization. TECHNIQUE: The initial transfemoral approach was complicated by persistent guide catheter instability due to marked tortuosity of the right vertebral artery (VA). The left VA could not be accessed due to left subclavian occlusion with steal syndrome. Neuroform-2 (Boston Scientific; Natick, MA) stent-assisted Matrix (Boston Scientific; Natick, MA) coil embolization was successfully performed through a 6-French guide catheter using a transbrachial approach. CONCLUSION: Current advances in technology produce neuroendovascular devices that are smaller with increased flexibility, allowing nonfemoral vascular access to the cerebral circulation via alternate arterial routes. This patient case demonstrates transbrachial access is a viable approach for endovascular aneurysm coil embolization, with or without stent assistance, in cases with an unstable guide catheter due to tortuous vessels or abnormal anatomy when femoral access is complicated or contraindicated.