A 15-year-old boy with central nervous system vasculopathy presenting with dysarthria-clumsy hand syndrome

Dysarthria-clumsy hand stroke syndrome has been described frequently in adults but not in children. We report a 15-year-old right-handed boy with sudden onset of dysarthria, dysphagia, right facial weakness, and mild right-hand clumsiness. Computed tomographic scan and magnetic resonance imaging demonstrated infarction in the genu and posterior limb of the left internal capsule. Magnetic resonance angiography and conventional angiography demonstrated stenosis of the supraclinoid portion of the left internal carotid artery and the origin of the left ophthalmic artery. Lacunar infarction in an older adult is not the only mechanism leading to dysarthria-clumsy hand syndrome.     

Cardio-facio-cutaneous syndrome and moyamoya syndrome

We reported a patient with cardio-facio-cutaneous (CFC) syndrome associated with moyamoya syndrome. The patient was referred at 6 years 5 months with left hemiplegia and right-sided eye deviation. He had an apparently short stature, macrocephaly, left ptosis and atopic skin, and was odd looking. He exhibited an incomplete right bundle branch block on electrocardiogram and an atrial septal defect on ultrasound cardiography. He was diagnosed as having CFC syndrome. Head magnetic resonance imaging showed a flow void in the bilateral basal ganglia, but did not show any ischemic changes. Magnetic resonance angiography showed bilateral stenosis with an internal carotid artery at the Willis artery ring level and bilateral moyamoya. Contrast angiography demonstrated occlusion of both middle cerebral arteries. Cerebrovascular anomalies have not previously been reported in CFC syndrome. This is the first case of CFC syndrome associated with moyamoya syndrome.     

A case presenting with Raeder's syndrome-like symptoms due to vertebral artery aneurysm]

A 50-year-old man complained of headache around his left orbit, left frontal pain and paresthesia associated with left incomplete Horner syndrome. MRI demonstrated a mass at the level of medulla oblongata. Left vertebral angiogram revealed an aneurysm of left vertebral artery. Following the removal of the aneurysm, these Raeder's syndrome-like symptoms improved. Therefore, they were probably caused by a compression of the spinal tract of the trigeminal nerve and the central sympathetic tract by the aneurysm. This is the first report of Reader's syndrome-like symptoms caused by vertebral artery aneurysm, thus indicating that MRI and cerebral angiogram are necessary for differential diagnosis of this syndrome.     

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.     

Magnetic resonance findings in a case of Gradenigo syndrome: widespread inflammation involving the paranasal sinuses and middle ear

Gradenigo syndrome is a rare condition consisting of otitis media, trigeminal neuralgia and abducens palsy. We report here a 6-year-old girl with this syndrome. Cranial magnetic resonance imaging (MRI) demonstrated inflammatory lesions in the left petrous apex and cavernous sinus, as well as stenosis of the left carotid artery. She was conservatively treated with antibiotics, which successfully improved her clinical and MRI findings. Surgical treatment including mastoidectomy was avoided. This case illustrates the usefulness of MRI in the diagnosis and management of Gradenigo syndrome.     

Atypical Wallenberg's syndrome due to spontaneous vertebral arterial dissection: case report]

We report here a case of atypical Wallenberg's syndrome due to spontaneous vertebral artery (VA) dissection. A 52-year-old woman was admitted to our department because of a sudden onset of left orbital pain. Emergency CT scan disclosed no evidence of intracranial hemorrhage. Neurological examination at the time of the current admission, showed dysphagia, left soft palate palsy, hoarseness, left Horner syndrome, hypalgesia with thermohypesthesia on the right side of her face, however, hypalgesia with thermohypesthesia on the right side of her body. The diagnosis of atypical Wallenberg's syndrome was based on the above findings. MR images disclosed the infarcted lesion at the left lateral medulla depicted as high-intensity on T2-weighted & FLAIR images. We carried out conservative treatment with antiplatelet & hemodilution therapies and the blood pressure control. Left vertebral angiograms obtained 18 days after the onset, showed the segmental severe stenosis of the VA between the ramification of the posterior inferior cerebellar artery (PICA) and the union of the VAs. In the venous phase, retention of contrast medium in the VA and the PICA was observed. The flow rate of the parent artery was decreased. We strongly suspected that her initial symptom of left orbital pain was due to dissection of the VA itself. Three-dimensional CT angiograms obtained 30 days after the onset, demonstrated the defect of the left VA between the ramification of the left PICA and the union of the VAs. Left vertebral angiograms obtained 36 days after the onset, showed the occlusion of the VA between the ramification of the PICA and the union of the VAs. The neurological findings gradually improved and the patient was discharged. Follow up left vertebral angiograms obtained 4 months & 16 months after the onset, revealed almost no changes of left VA occlusion.     

A case of brain infarction with nephrotic syndrome]

A 47-year-old man lost his consciousness and brought to our hospital by ambulance. On admission, he had aphasia and upper right limb paresis. Diffusion weighted MR image of the brain on admission showed multiple high intensity areas in the left middle cerebral artery (MCA) territory. Brain angiography performed on the 2nd hospital day revealed the left MCA severe stenosis. We started intravenous antithrombotic therapy on the 1st day. The left carotid angiography on 12th day demonstrated that the left MCA stenosis was improved. He had medical history of hypertension, diabetes mellitus and gout. But he had only slight atherosclerosis, and had no arrhythmia and patent foramen ovale. Blood chemistry test showed marked hypoproteinemia and hyperlipidemia, and urine examination showed proteinuria. He was diagnosed as nephrotic syndrome for the first time. Nephrotic syndrome brought hypercoagulability, so we suspected that nephrotic syndrome concerned with brain infarction.     

A case of antiphospholipid antibody syndrome showing a neurological deterioration and infarct development over a month]

A 67-year-old man was admitted to our hospital because of a sudden onset of gait disturbance and behavioral abnormalities. On the admission, he had a moderate consciousness disturbance and right hemiparesis with left internal carotid artery occlusion. Eight days after the stroke, the patient further developed left hemiparesis in association with right internal carotid artery occlusion. Despite anticoagulation therapy and plasma volume loading, neurological symptoms deteriorated over a month, during which CT scan demonstrated a progressive expansion of infarct size. Laboratory tests revealed the presence of lupus anticoagulant. Antiphospholipid antibody syndrome may be associated with a progression of ischemic stroke.     

Variant of Subclavian Steal in the Setting of Ipsilateral Common Carotid Artery Occlusion: Case Report

Subclavian steal is a well-described angiographic finding and clinical syndrome that rarely results in vertebrobasilar ischemic symptoms. In classic subclavian steal, left subclavian artery (SA) stenosis occurs proximal to the left vertebral artery (VA) origin. We report a symptomatic variant of this syndrome that occurred in the setting of left common carotid artery occlusion and anomalous origin of the left VA directly from the aortic arch. The steal and symptoms resolved after stenting of the left SA stenosis.     

Treatment of subclavian steal syndrome by percutaneous transluminal angioplasty: a case report

It has been known that significant narrowing of the subclavian or innominate artery may cause cerebrovascular ischemic symptoms, especially vertebrobasilar insufficiency. This condition has been named as the subclavian steal syndrome (SSS). Recently, percutaneous transluminal angioplasty (PTA) has been developed and used in treating occlusive vascular lesions. In this report, we presented a 61-year-old man who suffered from SSS and treated by PTA. He complained of dizziness and dysesthesia on his left upper extremity which got worse on exertion. The left radial pulse was diminished and the left brachial blood pressure was lower about 50 mmHg than the right. Bruits were recognized over the left suprasubclavicular area. Arch aortogram revealed an 80% stenosis of the proximal left subclavian artery and retrograde flow of the left vertebral artery on late arterial phase. PTA was successfully performed at the same time as angiography via the right femoral artery without any complications. His clinical symptoms improved immediately following PTA. A month after PTA, left retrograde vertebral angiogram demonstrated that the dilation of the treated segment of the left subclavian artery continuously remained. Because of several advantages such as a technical easiness and a more safety, PTA will be a useful therapeutic tool for not only SSS but also other obstructive diseases of brachiocephalic arteries prior to some surgical treatments. However, it is necessary to avoid some possible complications, especially embolism into the cerebral circulation in this technique, and the careful procedure should be done under the co-operation with vascular surgeons and radiologists.     

Ondine's curse in association with diabetes insipidus following transient vertebrobasilar ischemia.

Ischemic lesions of the brainstem can lead to complex neurologic deficits. Failure of the automatic control of ventilation (Ondine's curse syndrome) is a possible but rare syndrome following localized brainstem dysfunction. We report on a 49-year-old man with intermittent bradycardia, cranial nerves' dysfunctions and a slight right-sided hemiparesis. An acute brainstem ischemia was diagnosed and treated immediately with high-dose heparin. Cerebral angiography revealed a proximal occlusion of the left vertebral artery but a normal right vertebral artery and a hyperplastic right posterior inferior cerebellar artery. Cranial Computed Tomography and MRI scan demonstrated multiple ischemic lesions in the posterior circulation. During a 4-week treatment course the patient underwent six episodes of acute severe hypoxia and hypercapnia requiring orotracheal intubation twice and manual ventilation by air mask over a few minutes for four times after a tracheostomy had been performed. Twice a short-term episode of hypothalamic Diabetes insipidus was observed following hypoventilation. We conclude that both Ondine's curse syndrome and diabetes insipidus were due to transient vertebrobasilar ischemia.     

Successful intravenous thrombolysis for acute stroke in a child

We report an 8-year-old white girl with no previous medical history who developed sudden onset right hemiplegia, left gaze preference, and global aphasia. An acute left middle cerebral artery stroke syndrome was diagnosed. She was treated with intravenous recombinant tissue plasminogen activator, 2 hours after the onset of symptoms. A magnetic resonance image demonstrated an acute left middle cerebral artery stroke, and a magnetic resonance angiography showed a patent left middle cerebral artery. At discharge, she was able to speak normally and walk without support. She was also able to raise the right arm up to the level of the shoulder and showed increased motility of the hand and fingers. No treatment-related complications happened. To the best of our knowledge, this is, so far, the youngest child successfully treated with intravenous recombinant tissue plasminogen activator for acute ischemic stroke.     

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.     

A case with frequent episodes of transient ischemic attack presenting the Wallenberg syndrome before and after the onset of brain infarction]

A 52-year-old man with diabetes mellitus, hyperlipidemia and smoking habit, experienced transient ischemic attacks (TIAs) with symptoms of left orbital pain, left blepharoptosis and hoarseness lasting for five minutes on March 10, 1997. Subsequently, the same symptoms repeated once or twice daily. On March 28, he had dysphagia, numbness and disturbance of pain and temperature sensation (segmental dissociated sensory disturbance) on the right side of the body above the level of the Th10, the right upper limb and face. The deficits persisted for more than 24 hours. Angiographic studies revealed an occlusion of the left vertebral artery immediately after branching of the posterior inferior cerebellar artery. MRI demonstrated a hyperintense lesion on MRI T2 weighted image in the left lateral medulla. About three months after the completed stroke, he had six episodes of TIAs of left Horner's sign and hoarseness. To our knowledge, this is the rare case that had frequent TIAs presenting the Wallenberg syndrome before and after the onset of lateral medullary infarction. We speculate that the TIAs resulted from microembolism from the proximal end of occluted left vertebral artery and failure of the microcirculation in and around the lateral portion of the medulla oblongata.     

Prenatal multicystic encephalomalacia due to anomaly of the aortic arch

Multicystic encephalomalacia (ME) usually results from severe hypoxic-ischemic brain damage occurring during the late third trimester of gestation and birth. We report on a case of congenital ME due to a congenital anomaly of the aortic origin of brachiocephalic vessels resulting in subclavian steal syndrome. A 5-day-old term neonate presented with microcephaly and overlapping cranial sutures. Both arms were developed normally. Magnetic resonance imaging of the brain showed extensive bilateral supratentorial ME. Color duplex sonography of the aortic arch and the intracranial and extracranial vessels revealed a stenosis at the origin of the left common carotid artery and atresia of the origin of the left subclavian artery resulting in left-sided subclavian steal syndrome and retrograde perfusion of the basilar artery. Total cerebral blood flow volume was reduced to 22 mL/min. Severely reduced cerebral blood flow volume resulted from left carotid artery stenosis and atresia of the origin of the left subclavian artery with consecutive subclavian steal. Infratentorial brain structures and the left arm remained intact, but supratentorial brain structures were severely affected with ME.     

Amnesic syndrome caused by infarction in the area of the left anterior choroidal artery

A case of infarction in the left anterior choroidal artery territory presenting as a regressive amnestic syndrome, associated with the usual syndrome, is reported. This amnesia had the same characteristics as that found in the left posterior cerebral artery territory infarction. The anterior choroidal artery supplies the medial part of the temporal lobe, the amygdaloid nucleus, part of the hippocampus, uncal gyrus, and the mamillary body. Therefore, an amnestic syndrome can occur together with an infarction in the AChA territory. To our knowledge this is the first report of that occurrence.     

Agenesis of left internal carotid artery associated with megadolichobasilar anomaly and olivopontocerebellar atrophy

A case of agenesis of the left internal carotid artery associated with megadolichobasilar anomaly (MDBA) and olivopontocerebellar atrophy (OPCA) was reported. A 73-year-old female had a three-year history of slowly progressing gait disturbance. On admission neurological examination revealed bilateral cerebellar ataxia and dysarthria with increased muscle stretch reflexes and extensor planter response in the left. CT and MRI showed marked atrophy of the brain stem and cerebellum with brain stem rotation and deviation to the right. Total absence of the left internal carotid artery was demonstrated angiographically. The left anterior and middle cerebral arteries were fed by the tortuous megadolichobasilar artery through the enlarged posterior communicating artery. The left ophthalmic artery was fed by the left middle meningeal artery. By the high resolution CT at the skull base, the lack of the carotid canal was demonstrated on the left side. The interesting clinical features of a case of agenesis of the left internal carotid artery were discussed. This case showed a clinical and radiological signs and symptoms of OPCA though any influences by MDBA could not be excluded.     

Cranial Arteriopathy in Familial Vogt-KoyanagiHarada Syndrome

A 59-year-old woman with Vogt-KoyanagiHarada (VKH) syndrome presented with transient ischemic attacks. Magnetic resonance angiography showed multiple areas of stenosis affecting the intracranial portions of both internal carotid arteries. Conventional angiography confirmed these abnormalities and also demonstrated tapering stenosis of the extracran1al segment of the left internal carotid artery. This patient and her similarly affected daughter represent the first reported association between cranial arteriopathy and intergenerational passage of VKH syndrome.     

Anterior Spinal Artery Syndrome in a Heroin Addict

This first known case of heroin injected into the vertebral artery resulted in an acute spinal artery syndrome with quadriplegia, bilateral loss of pain, and temperature sensation to the C3-C4 dermatome junction and sparing of vibratory sense, proprioception, and mentation. The passage of the heroin bolus to the spinal cord rather than the brainstem was due to occlusion of the distal portion of the right vertebral artery when the head was extended and turned to the left at the time of injection. The spinal cord infarction was demonstrated by magnetic resonance imaging. A hypersensitivity reaction to heroin following a period of abstinence may explain the acute onset of symptoms following injection.     

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.