A boy with infantile-onset fibromuscular dysplasia showing recurrent cerebrovascular attacks

We report here a boy with infantile-onset fibromuscular dysplasia (FMD). At 8 months of age, he had the first cerebrovascular attack presenting with left facial palsy, followed by recurrent attacks of alternating hemiparesis. Involvement of systemic arteries was manifested by variable somatic symptoms: retarded growth of the right arm due to stenosis of the brachial artery, constipation and recurrent abdominal pain due to intestinal ischemia, and renovascular hypertension. Cerebral angiography disclosed stenosis of the bilateral internal carotid arteries and right vertebral artery, with development of moyamoya disease like collateral vessels. Systemic arteriography revealed stenosis of the right brachial and left femoral arteries, which was either tubular or mimicking a string of beads. This case was characterized by the early onset and involvement of many cerebral and systemic arteries resulting in severe and variable symptoms.     

Isolated intracranial fibromuscular dysplasia of the posterior cerebral artery

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease that most commonly affects the renal and internal carotid arteries, but intracranial FMD is very rare. We report a patient with FMD involving the posterior cerebral arteries (PCAs). A 24 year old female was presented with a 4 day history of right homonymous hemianopsia with throbbing headache in the left temporo-occipital area. The brain magnetic resonance imaging (MRI) revealed an acute ischemic stroke in the left PCA territory, while the Time of Flight (TOF) magnetic resonance angiogram (MRA) showed segmental luminal irregularities in the left proximal PCA. The conventional angiogram revealed the “string of beads” appearance, a characteristic that is pathognomonic for FMD. The patient’s inhospital clinical course was stable, while there was no recurrence of stroke. This is the second report of FMD of the PCA. Notwithstanding incredibly rare incidences of isolated intracranial FMD and nonspecific findings of MRA, such a pathophysiology should be considered as the cause for a stroke in young patients, especially those with no cardiovascular risk factor.     

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.     

A case of ischemic oculopathy followed by border zone infarction

We reported a 72-year-old male with ischemic oculopathy due to ophthalmic artery stenosis followed by ipsilateral border zone infarction due to internal carotid artery stenosis. The patient had history of hypertension and diabetes mellitus. He had severe headache and visual disturbance of the right eye. He was diagnosed right neovascular glaucoma and left diabetic retinopathy (simple type), and received diuretics, beta-blockade and other anti-hypertensive drugs. One month later, he noticed left mild hemiparesis in a morning, and he experienced progression of left hemiparesis over a week. He was admitted to our hospital on the 11th day. He showed left complete hemiplegia, left sensory disturbance, anosognosia and left unilateral spatial neglect. His right eye was diagnosed neovascular glaucoma but left eye was normal. The 5th days CT showed low density area in the right terminal zone and bilateral periventricular lucency. At the same area, the 46th days MRI showed high intensity area in the T2-weighted image and low intensity area in the T1-weighted image. Cerebral angiography performed on the 33rd day, disclosed severe kinking at the cervical segment and 50% stenosis at the intracavernous segment in the right internal carotid artery, and 90% stenosis and post-stenotic dilatation of the right ophthalmic artery. Left internal carotid artery had each 60% stenosis at the cervical segment and the intracavernous segment. Left ophthalmic artery had severe stenosis from its beginning to distal part. This infarction was considered berder zone infarction by it's localization (terminal zone) and internal carotid artery stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

An Uncommon Variant of Fibromuscular Dysplasia

Fibromuscular dysplasia is a rare cause of stroke affecting mostly young females. It is characterized by the typical “string of beads” sign located mostly bilaterally in the midcervical portion of the carotid or vertebral arteries. We present the uncommon case of borderzone hemispheric infarction in a man with isolated unilateral fibromuscular dysplasia affecting continuously the distal extracranial and proximal intracranial portion of the left internal carotid artery leading to distal hypoperfusion and ischemia.     

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.     

Multiple cerebrovascular occlusive disease associated with neurofibromatosis

Multiple cerebrovascular occlusive disease is rarely seen in patients with neurofibromatosis. Two cases of such lesions are presented and literatures dealing with the clinical and angiographical aspects of this occlusive disease are reviewed. Case 1; A 38-year-old normotensive man had sudden onset of vomiting, left hemiparesis and disturbance of consciousness, one day before the admission. He had family history of neurofibromatosis, and examination showed café au lait spots over the body. CT scans revealed a subcortical hematoma in the right temporal lobe. Angiogram revealed multiple occlusive lesions of the cerebral arteries, including occlusions of the right internal carotid artery (ICA) at the distal end, middle (MCA) and anterior (ACA) cerebral artery at the proximal portion, and stenosis of the left ICA and ACA. Abnormal vascular networks at the base of the brain were also seen bilaterally. Decompressive craniectomy, removal of the hematoma and bilateral ventricular drainage were performed. Postoperative course was excellent. Angiogram performed five and a half years later, during which time without any surgical procedures, demonstrated no apparent angiographic differences from the previous one. Case 2; A 29-year-old woman without family history of neurofibromatosis presented with sudden onset left hemiparesis. Café au lait spots were found over the body. A CT scan revealed small infarctions in the territory of the right MCA, and angiogram demonstrated multiple occlusive lesions of the cerebral arteries, including stenosis of the bilateral ICA, the left MCA, both ACAs at the proximal portion, and the right posterior cerebral artery, and occlusions of the right MCA.(ABSTRACT TRUNCATED AT 250 WORDS)     

Traumatic aneurysm of the peripheral cerebral artery]

Two cases of traumatic aneurysm of peripheral cerebral artery were reported. Case 1. A 6-year-old girl was severely injured on her head by automobile accident. Plain skull films showed depressed fracture in the left frontal region. Left common carotid angiogram 25 days after the injury revealed small aneurysm of a cortical branch of the anterior cerebral artery. Cranioplasty and removal of the aneurysm was performed. Postoperative course of this patient was uneventful. Case 2. A 4-year-old girl fell downstaris and struck her left temporal region. On admission, she was unconscious and plain skull films showed multiple linear fractures. No aneurysm was demonstrated in the right common carotid angiogram immediately after the head trauma. Since her general condition gradually improved, she discharged 23 days after the head trauma. 63 days after the injury, she developed sudden onset of severe headache, vomiting, and status epilepticus. Right common carotid angiogram showed a large aneurysm arising from a branch of right pericallosal artery at the free edge of the falx. Parent artery of the aneurysm was clipped. Postoperatively, the patient made uneventful recovery. 60 reported cases of traumatic aneurysm of peripheral cerebral artery were reviewed and analyzed in etiology, diagnosis, clinical course, treatment and pathogenesis.     

A case of anterior cerebral artery dissection causing enlargement of infarction]

A 46-year-old woman presented with weakness in the right arm and leg. She had developed difficulty in moving the right arm and leg while exercising and had noticed headache simultaneously. On admission, she had hemiparesis of the right side. Angiography on day 1 disclosed irregularity of the left anterior cerebral artery (ACA). We started argatroban and aspirin to prevent exacerbation of ischemia. Diffusion-weighted MR image on day 2 disclosed acute infarction in the territory of the left ACA. When she became able to move her leg against gravity (MMT 3/5), we discontinued antithrobotic therapy on day 3. MRI on day 10 disclosed enlargement of the infarcted area. Angiography on day 16 disclosed stenosis and dilation corresponding to pearl and string sign involving the left ACA. Spontaneous ACA dissection was established based on clinical features and angiographic findings. Antithrombotic therapy in patients with intracranial artery dissection remains controversial because subarachnoidal hemorrhage can complicate the course due to rupture of dissecting aneurysm, for there is no external elastic lamina in intracranial arteries. However, some cases of ACA dissection have showen progression of ischemic symptoms and enlargement of the infarcted area during the acute or subacute phase.     

Cerebral infarction in a boy with subdural abscess]

A 13-year-old boy was reported with a subdural abscess in the left anterior cranial fossa and an infarction in the lesion supplied by the left middle cerebral artery. Cranial computed tomography revealed a gradual increase of a low density area with surrounding capsule formation, and later another low density area which indicated in occlusion of the left middle cerebral artery. Cerebral angiography disclosed a stenosis at the trifurcated portion of the left internal carotid artery near the abscess. These findings suggested the occurrence of infarction due to angiitis of the internal carotid artery.     

Evolving atherosclerosis at carotid and intracranial arteries in Japanese patients with ischemic heart disease: a 5-year longitudinal study with MR angiography

Progression of atherosclerosis at extracranial carotid and intracranial arteries in patients with ischemic heart disease (IHD) is not well defined. We carried out a 5-year longitudinal study with magnetic resonance angiography (MRA) of patients with IHD to assess the incidence of progression of atherosclerosis at extracranial carotid and intracranial arteries and to determine predictors of the progression. We previously performed carotid and intracranial MRA on 67 patients who had received selective coronary angiography for the clinical diagnosis of IHD. Of these 67 subjects, 41 patients gave informed consent to undergo MRA reexaminations to evaluate changes of extra- and intracranial arteries over a 5-year period. The degree of stenosis was divided into five grades depending on the narrowness of the arteries, i.e. normal, mild, moderate, severe and occluded. The average of follow-up period with MRA examination was 58.8 months. The progression of atherosclerosis, as defined as an increase of one grade of the stenosis rating, including both the exacerbation of pre-existing stenosis and the appearance of new stenotic lesions, were found in five patients (12.2%) for the cervical carotid artery and in only one patient (2.4%) for the intracranial artery. A multiple logistic regression analysis revealed that baseline carotid artery stenosis ( P  = 0.008), age ( P  = 0.047), and coronary events during the follow-up period ( P  = 0.048) were significant and independent predictors of progression of carotid atherosclerosis. In conclusion, our findings suggest that follow-up evaluation of the carotid artery is indicated for patients with IHD in whom carotid artery stenosis was detected on an initial examination. Further study is needed with larger numbers of patients to confirm these findings.     

Intracranial fibromuscular dysplasia in a six-year-old child: a rare cause of childhood stroke

Intracranial fibromuscular dysplasia is a nonatheromatous angiopathy that most commonly affects adult women and is rarely recognized in children. Symptoms include stroke and headache, although the vasculopathy may be asymptomatic. Diagnosis is based on angiographic appearance, commonly described as a "string of beads." The etiology of intracranial fibromuscular dysplasia is not known, although possible causes include genetic predisposition, trauma, and underlying connective tissue disease. Treatment of intracranial fibromuscular dysplasia is largely supportive once symptoms become manifest. We report a 6-year-old girl who presented to our center for further evaluation of a large left middle cerebral artery distribution infarction. The patient was previously healthy, without known risk factors for stroke. Initial symptoms consisted of a dense global aphasia and a right hemiparesis. On arrival, the patient's aphasia had improved but she continued to have significant deficits in both receptive and expressive language as well as residual right hemiparesis. Magnetic resonance imaging and conventional angiographic studies demonstrated characteristic beading of the distal portion of the left internal carotid artery, as well as the proximal middle cerebral artery. Laboratory evaluation, echocardiogram, and renal ultrasound were normal. The renal vasculature did not demonstrate evidence of intracranial fibromuscular dysplasia. In conclusion, intracranial fibromuscular dysplasia should be considered in the differential diagnosis of childhood stroke. When recognized, other sites of vascular involvement should be sought, and consideration of underlying disorders is important, as connective tissue disorders have been associated with a propensity to develop this vascular abnormality. Careful follow-up is warranted, due to possible progression of disease.     

Childhood subarachnoid hemorrhage associated with fibromuscular dysplasia

A rare case of childhood subarachnoid hemorrhage (SAH) is reported. A 5-year-old boy presented with SAH. A cerebral angiogram showed obstruction of the first segment of the left anterior cerebral artery (A1). The A1 was surgically trapped to prevent rebleeding. Proliferation of myofibroblasts was revealed in the intima of the A1. Cerebral infarction appeared in the territory of the left middle cerebral artery (MCA) on day 2 after onset of SAH, and the patient suffered a second SAH on day 26. The second angiogram showed stenosis and a saccular aneurysm in the left MCA. A second surgery, neck clipping of the aneurysm, was needed. The multiple arterial lesions and histological findings indicated that the SAHs and the infarction were caused by the dissecting aneurysm affected with fibromuscular dysplasia (FMD). Intracranial FMD is mainly detected in children and causes cerebral ischemia and bleeding. Surgical treatment of childhood SAH should be discretely chosen with due consideration for FMD.     

A case of subarachnoid hemorrhage verified as cerebral vasospasm by using three-dimensional CT angiography (3 D-CTA): reference to comparison with conventional angiography]

The authors present a case of aneurysmal subarachnoid hemorrhage that were verified as cerebral vasospasm by using both three-dimensional CT angioraphy (3 D-CTA) and conventional angiography. A 45-year-old man was referred to our department 4th day after sudden onset of a severe headache. On admission, emergency 3 D-CTA showed the cerebral vasospasm involving M 1 segment. Conventional angiography performed at the same day of the left internal carotid artery confirmed the cerebral vasospasm of the same vessel as 3 D-CTA, and furthermore demonstrated the left middle cerebral artery (MCA) and anterior cerebral artery (ACA) genu aneurysms. The former was seen as a ruptured aneurysm from brain CT findings (Fisher group 3). On the 10th day after the onset, 3 D-CTA demonstrated the remaining severe cerebral vasospasm of the supraclinoid portion of left ICA and M 1 segment. Findings at the conventional angiography subsequently performed were concordant with those of 3 D-CTA. The patient was successfully treated with delayed surgical clipping for both aneurysms without the symptoms related to the cerebral vasospasm and discharged without neurological abnormality. We consider that 3 D-CTA shows promise as a minimally invasive method of evaluating the cerebral vasospasm and would take the place of the conventional angiography.     

De novo formation of an aneurysm in a case of unusual intracranial fibromuscular dysplasia

Intracranial fibromuscular dysplasia (FMD) is a vascular disease of unknown origin occurring predominantly in young women. The internal carotid artery is most often involved, but other cerebral arteries may also be affected. We report the case of a young woman presenting with an unusual angiographic appearance of intracranial FMD of the internal carotid artery (ICA) that could not be categorized into any type of the Osborn–Anderson classification. During follow up the patient presented with an intracerebral and subarachnoid hemorrhage. Repeated angiography revealed multiple aneurysms in the pathologic segment of the vessel. The patient underwent surgical treatment with clipping of the aneurysms, wrapping of the pathologic segment of the ICA and biopsy of the superficial temporal artery. Histopathological sections revealed FMD of the intimal type. ₁-antitrypsin blood levels were normal. Cases of intracranial FMD previously reported in the literature are reviewed and various aspects of this rare disease are discussed.     

Serial magnetic resonance images in patient with the middle cerebral artery dissection]

A 30-year-old man suffered from transient left hemiparesis and was admitted under the clinical diagnosis of transient ischemic attack. Cerebral angiogram on admission disclosed severe stenosis at the horizontal portion of the right middle cerebral artery. Magnetic resonance images (MRI) on axial view showed a linear enhanced lesion along the stenosis. Images on sagittal view disclosed a crescent-like lesion surrounding a flow void of the middle cerebral artery. After administration of aspirin, he had never suffered from hemiparesis. The enhanced lesion of the middle cerebral artery gradually disappeared on MRI. Although the middle cerebral artery showed temporary improvement of the stenotic lesion, it finally occluded 9 months after the onset. A diagnosis of spontaneous dissection of the right middle cerebral artery was made. The process of the arterial hemodynamics, such as a shrinking of the pseudo-lumen and a flow improvement in the true lumen, was well documented on serial MRI with Gd-DTPA and serial MRA.     

Intracranial occlusion of the internal carotid artery after minor closed head injury]

Thrombosis of the extracranial portion of the internal carotid artery as a result of nonpenetrating head and neck injury is not uncommon. However, intracranial occlusion of the internal carotid artery after minor head and neck injury without skull fracture is rare. We report a case of 14-year-old male who suffered a minor head injury during an athletic meeting of his school and developed a right hemiparesis and a lethargy state resulting from thrombosis of the supraclinoid portion of the left internal carotid artery. On admission, skull films and a CT scan revealed no abnormality. One hour later, he fully recovered. One day later, no definite lesions were detected on T1-weighted and T2-weighted image of MRI, but an abnormal high signal lesion in the left frontal lobe was detected on diffusion-weighted image of MRI. On additional MR angiography, intracranial occlusion of the internal carotid artery due to dissection was demonstrated.     

A case report of hypoplasia of the root portion and the intermediate portion of the left internal carotid artery associated with an unusual primitive collateral circulation between the left internal carotid artery and the left external carotid artery

The internal carotid artery is one of the most stable arteries and its absence is very rare. We reported a case of hypoplasia of the root portion and the intermediate portion of the left internal carotid artery associated with an usual primitive collateral circulation between the left internal carotid artery and the left external carotid artery. A 57-year-old male developed right hemiparesis of sudden onset 3 days prior to admission. On admission, right hemiparesis and right minimal facial palsy was observed. The left direct carotid angiogram revealed that the root portion and the intermediate portion of the left internal carotid artery were hypoplastic. There was an unusual primitive collateral circulation between the left internal carotid artery and the left external carotid artery. From an embryological point of view, normally, the internal carotid artery is derived from the third aortic arch and the dorsal aorta when the embryo is attained the 3-mm stage. The root portion of the internal carotid artery is formed from the third aortic arch. The dorsal aorta between the third and the first aortic arch form the intermediate portion. The distal part of the internal carotid artery originates from the dorsal end of the first aortic arch. The common carotid artery begins to form in the 12-mm to 14-mm embryo, following involution of portion of the ventral aortic root between the third and the fourth aortic arch. The external carotid artery arises from the aortic sac and migrate up to the third aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)