1/18. Arteriovenous and lymphatic malformations, linear verrucous epidermal nevus and mild overgrowth: another hamartoneoplastic syndrome?We report a 22 year old female presenting with slowly progressive paraparesis, who appeared to have many (mainly subcutaneous) hamartomas. The neurological symptoms were caused by intraspinal masses and arteriovenous malformations. In addition, she had mild overgrowth of one leg and lymph vessel malformations. This combination of symptoms resembles proteus syndrome, but is different in symptomatology and progression and may be yet another hamartoneoplastic syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/18. Extracranial vertebral artery dissection causing cervical root lesion.The authors report an unusual manifestation of extracranial vertebral artery dissection (VAD), presenting with a predominantly motor radicular manifestation. Cervical magnetic resonance imaging (MRI) revealed the intramural hematoma in the dissected vessel wall, compressing mainly the segmental motor root and, to a lesser degree, the sensory ganglion. In the digital subtraction angiography (DSA), a circumscribed narrowing of the incriminated vessel was demonstrated. color-coded Duplex imaging (CDDI) revealed complete recanalization after a few days of anticoagulation treatment. Complete neurologic recovery was seen after 3 months. Considering the MRI data, the likely pathogenetic mechanism was compression of the nerve root by the intramural hematoma. The synopsis with similar cases in the literature points to the characteristic features, i.e., the association of neck pain with radicular motor deficit and the absence of degenerative disk disease. The respective syndrome should raise the suspicion of vertebral artery dissection, especially in young individuals.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
3/18. Systemic lupus erythematosus associated with moyamoya syndrome.moyamoya disease is an uncommon clinical entity, characterized by bilateral occlusion of the internal carotid artery and the development of collateral arteries. An 18-year-old Saudi male with systemic lupus erythematosus (SLE) presented with mild right hemiparesis, followed by recurrent ischemic stroke. cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation (moyamoya vessels). There was no evidence of active SLE or other risk factors for cerebral occlusion, such as antiphospholipid antibody syndrome. Medical and surgical interventions did not influence the poor outcome of the recurrent ischemic insults.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/18. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/18. carbon disulfide vasculopathy: a small vessel disease.We present the clinical manifestations of 4 male patients with acute stroke-like symptoms and polyneuropathy after long-term exposure to carbon disulfide (CS2) in a viscose rayon plant. The ages of onset of polyneuropathy ranged from 42 to 45 years with a duration of CS2 exposure between 6 and 21 years. The ages of onset of stroke were from 42 to 48 years. The risk factors for stroke including heart disease and diabetes were denied, except for smoking in 4, hyperlipidemia in 2 and hypertension in 1. At the initial visit in 1992, only 2 patients developed sudden onset of hemiparesis suggesting a lacunar stroke before the diagnosis of CS2 intoxication. brain computed tomography (CT) scans showed low-density lesions in the basal ganglia in 2 patients, cortical atrophy in 1 and normal in 1. brain magnetic resonance image (MRI) study disclosed multiple lesions in the corona radiata and basal ganglia on T(2)-weighted images in 3 patients and cortical atrophy in 1. After the diagnosis, they left their jobs for a CS2-free environment, and improvement of the working conditions was noted. During 5 years follow-up period, another 2 patients also developed an acute episode of stroke with hemiparesis. brain CT and/or MRI follow-up studies in these 2 patients revealed new lesions in the basal ganglia and corona radiata. Intriguingly, a patient with previous stroke also developed new lesions in the bilateral thalami and brainstem. Carotid Doppler scan, transcranial Doppler scan and/or cerebral angiography did not show any prominent stenosis or occlusion in the major intracranial large arteries. We conclude that encephalopathy may occur in patients after long-term CS2 exposure, probably due to impaired cerebral perfusion. The lesions tend to occur in the basal ganglia, corona radiata and even brainstem, particularly involving the small-sized vessels. In addition, the cerebral lesions may progress even after cessation of CS2 exposure. Therefore, we suggest that CS2 exposure may be a risk factor for stroke.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
6/18. subarachnoid hemorrhage caused by dural arteriovenous fistula of the sphenobasal sinus--case report.A 59-year-old woman presented with a rare middle fossa dural arteriovenous fistula (AVF) unrelated to the cavernous sinus manifesting only as subarachnoid hemorrhage. Angiography revealed shunts between the meningeal branches of both the internal and external carotid arteries and the sphenobasal sinus. The AVF drained into the superficial middle cerebral vein (SMCV) which had a varix and an anastomosis to a superior cerebral vein. The arterial supply vessels were eliminated surgically and the sinus was excised. Bleeding did not recur and there was no venous infarction. Dural AVF of the sphenoparietal sinus is associated with pulsatile exophthalmos and dural AVF of the sphenopetrosal sinus with tinnitus, but dural AVF of the sphenobasal sinus has no obvious symptom. Simple interruption of the SMCV at the penetration of the arachnoid membrane was possible because of the absence of a draining vessel to preserve AVF patency, but the arteries were eliminated in this patient to prevent formation of another AVF.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/18. Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy.BACKGROUND: The main hereditary vascular conditions involving both retinal and cerebral vessels include cerebroretinal vasculopathy, HERNS (hereditary endotheliopathy with retinopathy, nephropathy, and stroke), and hereditary vascular retinopathy; all are linked to the same locus on chromosome 3p21. Hereditary retinal arteriolar tortuosity is a distinct, autosomal dominant condition characterized by retinal arteriolar tortuosity and recurrent retinal hemorrhages. This condition is known to affect only retinal vessels. methods: Clinical and brain MRI investigations of eight members of a three-generation family and extensive biological and systemic vascular investigations within one affected family member were conducted. RESULTS: Six of eight family members were clinically symptomatic; disorders included infantile hemiparesis (2), migraine with aura (3), and retinal hemorrhage (1). Five individuals had retinal arteriolar tortuosities. A diffuse leukoencephalopathy in association with dilated perivascular spaces was observed in six individuals. Two family members had silent, deep cerebral infarcts as demonstrated on MRI. genetic linkage analysis strongly suggests that this disorder is not linked to the 3p21 hereditary vascular retinopathy/cerebroretinal vasculopathy/HERNS locus. CONCLUSIONS: The authors describe a novel hereditary autosomal dominant condition affecting both retinal and cerebral vessels and characterized by infantile hemiparesis, migraine with aura, retinal hemorrhage, retinal arterial tortuosity, and leukoencephalopathy with dilatation of perivascular spaces and microbleeds on brain MRI. Investigation of additional families should help to map the gene and to better categorize the spectrum of hereditary cerebroretinal small vessel diseases.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
8/18. Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome.INTRODUCTION. Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder. It is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery disease and cerebrovascular disease. CASE REPORT. A 10-year-old boy with HGPS was admitted to our department because of progressive deterioration after a mild head injury. The CT scans revealed epidural hematoma in posterior fossa and another one in the temporal region on the left side. On admission the child was given an estimated score of 10 on the GCS. Neurological examination revealed right hemiparesis. The boy was operated on, and both hematomas were evacuated. In a few days the neurological symptoms disappeared, and he was discharged from the hospital with only residual, minimal right hemiparesis. CONCLUSION. Intracranial pathology was certainly caused by the head trauma, but was more severe than would have been expected had the trauma been the sole cause. We suggest that progressive atherosclerosis of intracranial vessels was responsible for formation of the hematomas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/18. central nervous system involvement in a child with polyarteritis nodosa and severe atopic dermatitis.polyarteritis nodosa is a diffuse vasculitis of small- and medium-sized muscular arteries that can involve the vessels of one or several organ systems. We report an 11-year-old boy with severe atopic dermatitis further complicated with erythroderma and cerebellar symptoms. Laboratory studies showed negative antinuclear antibodies and antineutrophil cytoplasmic antibodies; increased serum complement, IgG cryoglobulins 96.8 mg/dL (0-80) and serum IgG 2,160 mg/dL (613-1,295), and positive alpha-cardiolipin test. The IgE value was high. skin prick tests and RAST were positive for dust mites and casein. The viral profile was negative. Imaging studies disclosed tortuousity in the vessels and ischemic infarction in the cerebellum and thalamus. A diagnosis of polyarteritis nodosa with central nervous system involvement was made. The patient improved with immunosuppressive therapy and thalidomide. Currently, the patient is controlled and in a rehabilitation program. Other cases of polyarteritis nodosa associated with atopic dermatitis have not been reported.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/18. basilar artery stenosis mimicking the lacunar syndrome of pure motor hemiparesis.BACKGROUND: Stereotyped, repeated transient ischemic attacks manifesting as pure motor hemiparesis are most often attributed to ischemia of the internal capsule or ventral pons resulting from in situ disease of the small penetrating arteries. CASE DESCRIPTION: We report a 61-year-old man presenting with recurrent episodes of left-sided weakness consistent with the lacunar syndrome of pure motor hemiparesis. Subsequent neuroimaging revealed infarction of the right ventral pons and a critical basilar artery stenosis as the inciting lesion. Despite maximal antithrombotic therapy, he continued to have repeated symptoms. angioplasty and stenting were attempted but both failed due to plaque recoil and technical difficulties. After the procedure, the patient had no further ischemic episodes and remained symptom-free at two months. CONCLUSIONS: This case illustrates the imprecise and discordant relationship between the mode of presentation of a stroke syndrome and its presumed pathophysiology. The lacunar syndrome of pure motor hemiparesis should be recognized by clinicians as a mode of stroke presentation due not only to small vessel disease, but also to large artery atherosclerotic disease such as basilar artery stenosis. Prompt institution of treatment can lead to a good clinical outcome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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