A case of brain stem infarction with bilateral hearing loss]

The study case was a 66-year-old man who had bilateral neurosensory hearing impairment due to brain stem infarctions. He noticed mild hearing loss, frequent vertigo and tinnitus. About one month later, his hearing took a sudden turn for the worse, and he suffered from dysarthria, dysphagea and abasia. Neurological examination revealed pseudobulber palsy, left hemiparesis, cerebeller ataxia, disturbance of pain and temperature sensation on the right face and left side of the body. Brain stem auditory evoked potentials (BAEPs) showed a delayed small wave V with the abscence of previous waves on the right side and no significant waves on the left side. Brain magnetic resonance images (MRI) revealed infarctions in the bilateral middle cerebellar peduncles, including in the right lateral portions of pons, and the right lower pontine base. We believe that not only peripheral, but also central auditory pathways adjacent to infarctions were damaged. Magnetic resonance angiography (MRA) showed severe stenosis or occlusion of left vertebral artery and basilar artery. We concluded that hypoperfusion of the vertebrobasilar artery territories caused ischemia of the cochlear nerve and the auditory tracts in the brain stem, which resulted in bilateral hearing loss.     

小脑后下动脉解剖变异致双侧小脑梗死2例报道并文献复习

目的 探讨小脑后下动脉(posterior inferior cerebellar artery, PICA)解剖变异致双侧小脑梗死的临床特征及发病机制。方法 对2例经颅脑MRI确诊的双侧小脑梗死患者采用CT血管造影(CTA)、磁共振血管成像(MRA)或数字减影血管造影(DSA)显示其头颈部血管,从而了解后循环血管的形态特征并复习相关文献。结果 病例1经DSA证实左侧椎动脉较细,远端管腔闭塞,通过右椎动脉代偿供血原左侧PICA供血区但欠充分,双侧PICA共同起源于右侧椎动脉。病例2经CTA证实右侧椎动脉较左侧明显细且远端显示欠清,MRA示双侧PICA共同起源于左侧椎动脉。结论 2例双侧小脑梗死患者均存在一侧椎动脉优势供血,且双侧PICA共同起源于该侧椎动脉。在该解剖变异基础上一侧椎动脉发生病变时可出现双侧小脑梗死。因此,在临床中出现双侧小脑梗死时临床医师不能忽略这一解剖变异基础。     

Repeated vertebrobasilar ischemic stroke caused by an intracranial vertebral artery dissection not detected on the initial angiogram: a case report]

Spontaneous dissection of the intracranial vertebral artery has been increasingly recognized as a cause of vertebrobasilar ischemic stroke. However, little is known about its natural history and clinical course. The authors report a young patient with repeated cerebral infarction in the posterior cerebral circulation caused by dissection of the vertebral artery, which was not detected on the first angiogram. The otherwise healthy 22-year-old male suddenly developed visual disturbance. Neuroradiological examination revealed right occipital infarction, but cerebral aniography revealed no caliber change or other pathological findings. He was treated with antiplatelet therapy. Two years later, he suffered vertigo, nausea, and dysarthria due to newly developed left cerebellar and medullary infarction. Cerebral angiography revealed left vertebral artery occlusion. T1-weighted MR image demonstrated Gadlinium-enhanced intramural hematoma in the occluded left vertebral artery, which was compatible to the arterial dissection. The present case argues serial neuroimaging studies especially in young patients with vertebrobasilar stroke in order to rule out the arterial dissection, even if initial angiography failed to demonstrate any radiographical evidence.     

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.     

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.     

Contralateral Cerebral Infarction after Stent Placement in Carotid Artery: An Unexpected Complication

Stenting is a useful alternative treatment modality in carotid artery stenosis patients who are too high-risk to undergo carotid endarterectomy (CEA). We report a case of contralateral cerebral infarction after stenting for extracranial carotid stenosis. A 78-year-old woman was admitted to the hospital with left-sided weakness. Based on magnetic resonance imaging (MRI) of the brain and conventional angiography, she was diagnosed with an acute watershed infarct of the right hemisphere secondary to severe carotid stenosis. Stenting was performed for treatment of the right carotid artery stenosis after a one-week cerebral angiogram was completed. Thirty minutes after stent placement, the patient exhibited a generalized seizure. Four hours later, brain MRI revealed left hemispheric cerebral infarction. Complex aorta-like arch elongation, tortuosity, calcification, and acute angulation at the origin of the supra-aortic arteries may increase the risk of procedural complications. In our case, we suggest that difficult carotid artery catheterization, with aggressive maneuvering during stenting, likely injured the tortuous, atherosclerotic aortic arch, and led to infarction of the contralateral cerebral hemisphere by thromboemboli formed on the wall of the atherosclerotic aorta.     

A case of medial medullary infarction with persistent primitive hypoglossal artery]

A 66-year-old woman was admitted to our hospital because of vomiting, dizziness and vertigo. Neurological examination on admission revealed only upbeat nystagmus without cranial nerve symptoms, paresis, cerebellar signs or sensory disturbances. Magnetic resonance(MR) images demonstrated a new T 2 high intensity and T 1 iso-intensity signal lesion in the right upper medial medulla. This medial medullary infarction caused central vestibular dysfunction. MR angiography and digital subtraction angiography demonstrated a persistent primitive hypoglossal artery (PPHA) originating from the right internal carotid artery to the vertebrobasilar artery associated with the stenosis of the right internal carotid artery at the level of the cervical bifurcation. This is the first report of medullary infarction with persistent carotid-basilar anastomosis. We suspected this medullary infarction was caused by artery to artery embolism in the branch of the right vertebral artery through the PPHA distal originated from the stenosis of the right internal carotid artery.     

"Fou rire prodromique" associated with simultaneous bilateral capsular genu infarction.

Pathological laughter is exaggerated, uncontrollable, and inappropriate laughter usually unrelated to a true emotion or a congruent mood. "Fou rire prodromique" is a rare form of prodromal pathological laughter of uncertain pathophysiology that heralds an ischaemic neurologic deficit. We report a case of prodromal pathological laughter marking the onset of bilateral capsular genu infarction. T2-weighted cranial magnetic resonance imaging (MRI) showed bilateral capsular genu infarction. There was also a diffusion defect in the same areas on diffusion-weighted imaging (DWI). Although it is known that bilateral subcortical lesions can cause pathological laughter, this is the first demonstration of simultaneous associated bilateral capsular genu infarction on cranial DWI MRI.     

Vertebral artery occlusion following neck trauma: report of two cases]

We are reporting two cases of vertebral artery occlusion resulting from cervical spine trauma. A 41-year-old man experienced vertigo and nausea 6 hrs after chiropractic manipulation. On admission, he was alert and demonstrated nystagmus, hypalgia of left leg, and right Horner sign. A MR image revealed infarction in the right cerebellar hemisphere. A MR angiogram did not show the proximal part of the right vertebral artery. A right vertebral angiogram revealed right vertebral artery occlusion at the level of C 1. He underwent anticoagulation and wore a cervical collar. He was discharged with hypalgia of left leg. A 53-year-old man was admitted to our hospital after an automobile accident. A CT scan revealed a subarachnoid hemorrhage and an intraventricular hemorrhage. A cervical CT scan revealed fractures of the C 5 facet joint and C 6 vertebral body. A MR angiogram did not show the proximal part of the left vertebral artery. A subsequent left vertebral angiogram revealed left vertebral artery occlusion at the level of C 6. He underwent anticoagulation and wore a cervical collar. In addition, he underwent coil embolization of the left vertebral artery. He was discharged with no neurological deficits. It is said that traumatic vertebral artery injuries cause cerebral infarction with time lags. The therapeutic point is to prevent propagation of the thrombus and distal embolism; therefore wearing a collar, anticoagulation, and endovascular interventional therapy is recommended.     

Compression neuropathy of cranial nerves in the course of Takayasu arteritis

Takayasu arteritis is a rare vasculitis of the aorta and its branches. Neurological manifestation usually results from central nervous system ischaemia. We report a case presenting with unilateral paresis of the cranial nerves (V, IX and XII nerve) caused by a vascular conflict due to Takayasu arteritis. A 38-year-old male was admitted to the hospital complaining of dysarthria, dysphagia, numbness of the right side of the tongue and a headache localized behind the right eye. The symptoms had sudden onset. Neurological examination revealed isolated trigeminal, glossopharyngeal and hypoglossal nerve dysfunction on the right side without other neurological symptoms. Magnetic resonance angiography showed internal carotid artery dissection and prominent thickening of walls of both vertebral arteries as well as the left renal artery with narrowing of lumen. Compression of glossopharyngeal and hypoglossal nerves and the trigeminal ganglion was a result of a markedly dilated intracranial segment of the right carotid artery. The clinical and radiological findings were consistent with the diagnosis of Takayasu arteritis.     

The correlation between vertebral artery asymmetry and pontine infarction--an MR angiography study]

The purpose of this study is to evaluate the correlation between variation of the vertebral artery (VA) and the incidence of pontine infarction. A total of 206 patients were examined using magnetic resonance imaging (MRI) and 3-dimension time-of-flight MR angiography (MRA) of the brain. Of these, 54 patients had pontine infarctions (23 symptomatic and 31 asymptomatic), and the majority of them were located in the pontine base. The sites of dominant lesion in the pons were right in 18 cases, left in 8 cases, and bilateral in 28 cases. The number of patients with VA asymmetry (the ratio of internal diameters 1:2 or more) were 89 (43.2%). Of these, 67 patients had small diametric VA of right side, and 22 of left side. Among the 117 patients with normal VA pattern, 19 (16.2%) had infarction, while among the 89 patients with VA asymmetry, 35 (39.3%) had infarction. The patients with small diametric VA of right side significantly had infarctions in the same side of the pons. The results of this study suggest that VA asymmetry is considered to be one of the risk factors of pontine infarction and that MRA can be useful in the examination of the cerebral artery as a valuable and non-invasive screening method.     

"Fou rire prodromique" heralding a brainstem stroke.

A patient manifested pathological laughter heralding the onset of brainstem stroke leading to a "locked-in" state. The pathological laughter did not recur. MRI revealed a bilateral ventral pontine infarct. The clinico-anatomical correlations of this rare phenomenon of fou rire prodromique are discussed.     

颈总动脉闭塞血管内开通治疗

病例摘要 患者,男,76岁,因“右侧无力2月”人院。患者于2012年9月8日无明显诱因出现右侧肢体麻木、无力,至当地医院就诊,磁共振成像（magnetic resonance imaging,MRI）提示“脑内多发腔梗,左侧顶叶新鲜梗塞”,予以抗血小板聚集,降脂,控制血压等对症支持治疗,病情平稳后,为进一步诊疗人我院,入院查体：     

Infarcts presenting with a combination of medial medullary and posterior inferior cerebellar artery syndromes

Cerebellar and medial medullary infarctions are well-known vertebrobasilar stroke syndromes. However, their development in a patient with distal vertebral artery occlusion has not been previously reported. A 49-year-old man with longstanding hypertension suddenly developed vertigo, right-sided Horner syndrome, and left-sided weakness. An MRI of the brain showed acute infarcts in the right inferior cerebellum (posterior inferior cerebellar artery territory) and the right upper medial medulla (direct penetrating branches of vertebral artery). Magnetic resonance angiogram showed occlusion of the distal vertebral artery on the right side. Atherothrombotic occlusion of the distal vertebral artery may cause this unusual combination of vertebrobasilar stroke.     

Spontaneous carotid dissection presenting lower cranial nerve palsies

Cranial nerve palsy in internal carotid artery (ICA) dissection occurs in 3--12% of all patients, but in 3% of these a syndrome of hemicranias and ipsilateral cranial nerve palsy is the sole manifestation of ICA dissection, and in 0.5% of cases there is only cranial nerve palsy without headache. We present two cases of lower cranial nerve palsy. The first patient, a 49-year-old woman, developed left eleventh and twelfth cranial nerve palsies and ipsilateral neck pain. The angio-RM showed an ICA dissection with stenosis of 50%, beginning about 2 cm before the carotid channel. The patient was treated with oral anticoagulant therapy and gradually improved, until complete clinical recovery. The second patient, a 38-year-old woman, presented right hemiparesis and neck pain. The left ICA dissection, beginning 2 cm distal to the bulb, was shown by ultrasound scanning of the carotid and confirmed by MR angiogram and angiography with lumen stenosis of 90%. Following hospitalisation, 20 days from the onset of symptoms, paresis of the left trapezius and sternocleidomastoideus muscles became evident. The patient was treated with oral anticoagulant therapy and only a slight right arm paresis was present at 10 months follow-up. Cranial nerve palsy is not rare in ICA dissection, and the lower cranial nerve palsies in various combinations constitute the main syndrome, but in most cases these are present with the motor or sensory deficit due to cerebral ischemia, along with headache or Horner's syndrome. In the diagnosis of the first case, there was further difficulty because the cranial nerve palsy was isolated without hemiparesis, and the second case presented a rare association of hemiparesis and palsy of the eleventh cranial nerve alone. Compression or stretching of the nerve by the expanded artery may explain the palsies, but an alternative cause is also possible, namely the interruption of the nutrient vessels supplying the nerve, which in our patients is more likely.     

Vertebral arterial dissection associated with Klippel-Feil syndrome in a child

BACKGROUND: Vertebral artery dissection resulting in stroke is rare in children. We report here on a 7-year-old boy with Klippel-Feil abnormality, who presented with a pontine infarction after a supervised swimming session. METHODS: Evaluation after a second acute neurological event included a formal cerebral angiogram, which revealed a complete upper basilar artery occlusion and right vertebral arterial dissection. Cervical spine radiographs demonstrated an associated fusion of the C2 and C3 vertebrae. Anticoagulation therapy was initiated, and the neurological deficits associated with the pontine infarction resolved. Anticoagulation was discontinued after 6 months of therapy, with no recurrence of symptoms. CONCLUSION: Vertebral artery dissection may rarely be associated with Klippel-Feil abnormality in children.     

Cerebral embolism in a patient with polycythemia rubra vera

Polycythemia rubra vera (PRV) is a rare haematological disorder that has a high risk of stroke, although the pathophysiological origin of the cerebral ischaemia in this disease is not well known. We report a case of a stroke patient with PRV in whom bilateral embolic signals were detected by transcranial Doppler (TCD). Cerebral computed tomography showed a cortical middle cerebral artery infarction, echocardiography was normal, duplex-scan showed moderate left carotid stenosis and digital angiography disclosed right siphon stenosis. TCD examinations in the acute phase repeatedly showed a great number of bilateral microembolic signals (MESs). Four months later magnetic resonance angiography showed no flow signal in the right siphon and a severe stenosis of the proximal right MCA. The detection of bilateral MESs in the absence of cardiac sources of embolism observed in this patient suggests that ischaemic cerebral events in PRV may have an embolic origin favoured by a prothrombotic state.     

Magnetic resonance angiography in 12 patients with Wallenberg's syndrome]

Magnetic resonance angiography (MRA) was performed in 12 patients with Wallenberg's syndrome (WS) in chronic stage to investigate the lesion of the vertebral artery (VA). The laterality of infarction in the medulla oblongata was confirmed with MRI: the right in four patients and the left in eight. In this study, time-of-flight MRA was evaluated. Coronal and OM sections in MRA were examined, because the former is useful to observe asymmetry and curve of the cervical VA, and the latter is useful to observe asymmetry and stenosis of the intracranial VA. In nine out of 12 patients, the ipsilateral VA was rather hypoplastic compared to the contralateral VA, and the flow of the hypoplastic VA stopped before and after entering the intracranial region. The contralateral VA of the hypoplastic cases showed curve in the neck in five patients and stenosis in the intracranial portion in one patient. A comparative study was conducted between vertebral angiogram (VAG) and MRA in four patients. Both VAG and MRA were useful in detecting hypoplasia and stoppage of the flow. It was considered that VA hypoplasia is frequently responsible for WS, and that MRA is useful in screening the vascular lesion as a non-invasive procedure to reveal changes of the bilateral VA.     

A case of transient blindness due to severe stenosis of the internal carotid artery with persistent primitive hypoglossal artery (PPHA)]

We report a patient having transient blindness due to severe stenosis of the internal carotid artery (ICA) with persistent primitive hypoglossal artery (PPHA). This 73 year-old man was admitted because of the transient visual impairment. At first, he had bilateral blindness for a several minutes and after that the right amaurosis continued for an hour. MRI showed an old lacunar infarction of the right caudate nucleus. Carotid duplex ultrasonography and conventional angiography demonstrated severe stenosis of the origin of the right ICA, and PPHA was arising from the right ICA at the level of 2nd cervical spine. The left ICA was normal. Because of the aplasia of the right vertebral artery and hypoplasia of the left vertebral artery, almost all blood flow of the basilar artery was supplied from the right ICA via PPHA. We considered that transient ischemia of both the bilateral posterior cerebral arteries and the right ocular artery occurred due to stenosis of the right ICA which branching PPHA. When ischemic neurological symptoms of multiple vascular territories occurr at the same time, we often think that ischemic mechanism was cardiogenic embolism. But we should recognize that stenosis of the ICA with PPHA cause the complex neurological deficits.     

Bilateral faciobrachial paresis as a consequence of symmetrical capsular infarcts.

A 32-year old man presented with a bilateral faciobrachial paresis, pyramidal signs in the upper limbs and dysarthria. Computer tomographic (CT-)scans showed bilateral cortical zones of contrast enhancement and strikingly symmetrical capsular hypodensities. Angiography revealed a stenosis of the left internal carotid artery and an occlusion of the right internal carotid artery. Essential thrombocythemia was diagnosed as the underlying disorder. Since there are no indications of pontine lesions, we assume that the signs and symptoms in this patient could mainly be attributed to the bilateral capsular lesions, that resemble lacunar infarcts.