Primary stenting of the extracranial internal carotid artery in a patient with multiple cervical dissections: technical case report

OBJECTIVE AND IMPORTANCE: Spontaneous dissection of the extracranial internal carotid artery (ICA) and vertebral artery (VA) is a well-documented cause of stroke in young, previously healthy patients. The majority of patients with spontaneous dissection are successfully treated with antiplatelet or anticoagulation therapy, but a significant proportion of patients progress to suffer devastating morbidity and mortality. Surgical intervention has primarily consisted of proximal ligation, extracranial-intracranial bypass, or endarterectomy. Generally, these procedures are technically demanding and yield disappointing clinical results. CLINICAL PRESENTATION/INTERVENTION: A 36-year-old man without a significant medical history initially presented with a several-day history of episodic right upper extremity weakness and numbness and visual obscurations. Cerebral angiography revealed bilateral ICA long segment narrowing (95%), distal left VA high-grade (95%) stenosis compatible with dissections, and right VA proximal occlusion. While therapeutically anticoagulated on heparin, the patient continued to experience crescendo episodes of right upper extremity paresis and paresthesias as well as aphasia. The patient underwent primary stenting of the left ICA, using a series of six overlapping stents (three Gianturco-Roubin coronary stents and three Palmaz-Schatz coronary stents). The patient remained symptom-free without neurological complications, and subsequent angiography performed at the 9-month follow-up examination confirmed continued patency of the stented left ICA as well as recanalization of the right ICA and VA. CONCLUSION: Neurovascular stents offer a minimally invasive and potentially efficacious treatment for the prevention of cerebral ischemia in patients with spontaneous extracranial dissection who remain symptomatic despite therapeutic anticoagulation.     

Bilateral vertebral artery dissection in a patient with afibrinogenemia

BACKGROUND: Afibrinogenemia, a rare coagulation disorder, has not been associated with vertebral artery dissections. CASE DESCRIPTION: A 28-year-old woman with afibrinogenemia developed spontaneous neck pain followed by a right medullary infarction, and MR angiography showed extensive bilateral vertebral artery dissection. She was treated with fibrinogen replacement and anticoagulants and showed a favorable evolution, with only mild residual right upper arm incoordination. CONCLUSIONS: In this patient spontaneous bilateral vertebral artery dissection complicated afibrinogenemia. Since anticoagulant therapy is usually indicated for arterial dissection, this association created a therapeutic problem. This patient received anticoagulants with fibrinogen replacement, which resulted in a favorable evolution.     

Development of 11 beta-hydroxysteroid dehydrogenase expression in the rat cochlea

Spontaneous coronary dissection is rare and the diagnosis is usually post-mortem. Less than 60 cases have been diagnosed at coronary angiography. The authors report, to the best of their knowledge, the first case of multiple spontaneous coronary artery dissections in a type IV Ehlers-Danlos syndrome in a young woman admitted to hospital for acute myocardial infarction. She had a previous history of regressive complete tetraplegia due to dissection of the basilar artery and episodes of dizziness related to a dissecting aneurysm of the left vertebral artery. The diagnosis of type IV Ehlers-Danlos syndrome was established after skin biopsy had shown typical histological changes. The patient died several months later after an acute abdominal syndrome probably related to dissection of the aorta. An autopsy was refused by her family. The authors believe this to be the first case of spontaneous coronary dissection related to a type IV Ehlers-Danlos syndrome.     

Use of polymerase chain reaction in clinical diagnostic laboratories in viral hepatitis B and C]

alpha 1-Antitrypsin deficiency has been associated with a variety of vascular disorders including arterial aneurysms, spontaneous extracranial arterial dissections, and arterial fibromuscular dysplasia. We determined the distribution of alpha 1-antitrypsin phenotypes in patients with intracranial arterial dissections, a rare cause of subarachnoid hemorrhage. The study population consisted of 4 consecutive patients with subarachnoid hemorrhage due to spontaneous intracranial arterial dissections. The vertebral artery was involved in 3 patients and the posterior inferior cerebellar artery in 1 patient. Three of these 4 patients were found to have a heterozygous alpha 1-antitrypsin deficiency (PiMZ or PiMS phenotypes). These data support previous studies suggesting that patients with alpha 1-antitrypsin deficiency may be at an increased risk of developing spontaneous arterial dissections.     

Cerebellar infarction with hydrocephalus caused by spontaneous extracranial vertebral artery dissection--case report

A 38-year-old male suffered sudden onset of rotational vertigo without headache. Consciousness disturbance developed on the 3rd day after the onset. Computed tomography showed cerebellar infarction with obstructive hydrocephalus. External ventricular drainage was performed. Angiography showed bilateral extracranial vertebral artery dissection. Antiplatelet therapy was given. Repeat angiography showed improvement of the dissection. His neurological deficits completely resolved. Vertebral artery dissections may cause both lateral medullary or cerebellar infarction and hydrocephalus due to the cerebellar infarction manifesting as various symptoms so careful evaluation and treatment are required.     

Histological patterns of atherosclerotic plaques in unstable angina patients vary according to clinical presentation

Aneurysms of the cervical vertebral artery (VA) are uncommon; they are often caused by trauma or spontaneous dissection. A fusiform aneurysm without evidence of atherosclerosis or dissection has not been reported previously. A 46-year-old man presented with a pontine infarct. Imaging revealed a fusiform aneurysm of the left VA at the C5-6 level, with occlusion of the basilar artery. Associated minor anomalies included fusion of the vertebral bodies of C5 and C6, cervical rib and platybasia. The left VA arose directly from the aortic arch and entered the transverse foramen at the C4 level. Hyperextension and left lateral flexion of the neck caused kinking of the VA proximal to the aneurysm. Turbulent flow in the aneurysm lumen was noted on angiography.     

The traA gene of the Enterococcus faecalis conjugative plasmid pPD1 encodes a negative regulator for the pheromone response

Cranial nerve palsies are rare complications of internal carotid artery (ICA) dissections. The aim of this study is to evaluate the incidence of cranial nerve palsies in consecutive patients with ICA dissection and to describe clinical and radiological characteristics and their evolution over time. This study was conducted in 52 consecutive patients with dissection of the ICA. We have analyzed clinical data of patients with cranial nerve palsy as complication of ICA dissection. We defined ICA dissection as angiographic evidence of a string sign, double lumen, or internal flaps or visualization on magnetic resonance imaging (MRI) or computed tomographic scans of an enlarged arterial wall due to the hematoma. Of 52 consecutive patients with ICA dissection 7 had cranial nerve palsies: 2 had an involvement of the Vth cranial nerve and 5 had lower cranial nerve palsies. Five patients totally recovered while 2 did not after a 2 to 10-month period. The frequency of cranial nerve palsies associated with ICA dissection is higher in our study than in those of the literature. Many patients presenting with cranial nerve palsies due to ICA dissection without any ischemic event are probably not referred to stroke units. Angiography is less sensitive than cervical MRI to detect such patients. Cranial nerve palsies could either be due to compression by the enlarged ICA wall or an ischemia of the nerve.     

Daily dosimetric quality control of the MM50 Racetrack Microtron using an electronic portal imaging device

Dissection of cerebral arteries as a cause of stroke is rarely recognized in children. Two patients with stroke due to extracranial carotid artery dissection are reported. A 7-year-old girl with a 2-week history of right arm chorea had a left basal ganglia infarct and is receiving haloperidol for persistent chorea. The second patient, a 15-year-old boy, developed aphasia and right hemiparesis a day before admission during a football game without obvious trauma. He had a large left middle cerebral artery infarct and died of cerebral edema and herniation. We believe that strokes due to arterial dissection are more common than currently recognized, partly because of a lack of history of trauma, and suggest that cerebral artery dissection be considered as an etiology of childhood strokes. Greater awareness of arterial dissection as a cause of stroke and availability of noninvasive techniques like magnetic resonance angiography should result in a more accurate diagnosis and improved prognosis in these patients.     

Insulin-like growth factor binding proteins from adult-hamster pancreatic islets: influence of glucose concentration

The recognized incidence of extra- and intracranial carotid artery dissection has increased concomitantly with the progress and development of diagnostic methods. However, management for this condition is still controversial. We report in the present study the management and long-term follow-up results of 15 patients with carotid artery dissection. Mean age of the patients at onset was 47.8 years old, and male/female ratio was 12:3. Two patients were considered to be traumatic dissections and the other 13 patients were spontaneous dissections. Dissection occurred in 10 patients at the extracranial carotid artery, in 4 at the intracranial carotid artery and in 1 at the middle cerebral artery. Nine of 15 patients demonstrated hemiparesis and 5 complained of headache or facial pain. However, it was not possible to identify a characteristic symptom of dissection. Final diagnosis of dissection was made by cerebral angiography in all patients. Serial angiography was carried out in 10 of those, and 5 of the 10 patients showed some improvements of dissection in the cerebral angiogram. Treatment for those patients was selected according to the neurological and angiographical changes. Five patients were managed conservatively and 10 patients underwent surgical revascularization. During the follow-up period (mean 77.6 months), none of them showed any symptoms of reattack, and all but one, who died of heart failure 193 months after revascularization surgery, have lived independently. Although diagnosis of dissection was difficult because of the lack of characteristic symptom, serial angiography was a useful method for diagnosis and adequate management has led to a good clinical outcome.     

Dissection of the carotid artery and vertebral artery--diagnosis and therapy

Carotid and vertebral artery dissections typically occur in young adults after major trauma, although they can arise spontaneously or after trivial injury. Many patients with carotid dissections have minor symptoms such as a subject bruit or Horner's syndrome. Cephalic pain is also frequent and often inaugural in carotid dissection. However, extracranial dissection is a well recognised cause of ischaemic stroke. The diagnosis of dissection was based on angiographic findings. Noninvasive imaging also allows prompt and reliable diagnosis. Our goal was to demonstrate the spectrum of neuroradiologic (CT, MR and angiographic) findings in craniocervical arterial dissection and compare the diagnostic utility of CT, MR, MR angiography. Clinical data imaging studies, and outcome were reviewed and compared with the results in four patients with carotid artery dissection.     

Diagnosis of a "hereditary pancreatitis" by the detection of a mutation in the cationic trypsinogen gene

HISTORY AND CLINICAL FINDINGS: A 71-year-old woman was admitted with the suspected diagnosis of pancreatic carcinoma. As a child she had had repeated attacks of abdominal pain of undetermined cause. When aged 48 years she had developed diabetes mellitus. Her now 42-year-old daughter had from the age of 9 years suffered from repeated attacks of acute pancreatitis that had finally led to chronic pancreatitis. The patient's 15-year-old grandchild was having recurrent bouts of abdominal pain. INVESTIGATIONS: Imaging procedures revealed calcifications in the pancreas and an infiltrating space-occupying lesion, about 3 cm in diameter, in the head of the pancreas with lymph node and liver metastases. Cytological analysis of material aspirated from the space-occupying mass showed typical findings of ductal pancreatic carcinoma. FURTHER TESTS, TREATMENT AND COURSE: At first the patient's course was not typical for a genetically-determined disease, but the family history raised the suspicion of hereditary pancreatitis. A genetic test (Afl-III-RFLP test) demonstrated the mutation Arg 117 His in the cationic trypsinogen gene in all diseased or symptomatic family members. The patient died of the complications of the pancreatic cancer. CONCLUSION: Genetic tests are valuable in the diagnosis of hereditary pancreatitis, because the increased cancer risk can be met by frequent examinations in affected family members.     

Spontaneous dissection of extra-intracranial intern carotid

Dissection of internal carotid artery is an unusual cause of stroke. It generally affects the extracranial portion of the vessel, rarely the intra-cranial portion and exceptionally both sections simultaneously. We present two cases of spontaneous dissection with extra and intra-cranial involvement. Two females, 46 and 36 years old, presented as stroke of the right internal carotid (ICA) associated with headaches and ipsilateral Horner's syndrome. An echo-Doppler was done on the first patient, which turned to be normal, and carotid angiography was done to both patients. The first patient showed a filiform stenosis of the right ICA that ran from the origin to the carotid siphon. The second patient showed a longitudinal stenosis of the right ICA 2 cm from the origin, which ended in an obstruction of the terminal branches. The control angiographs at five and six months respectively, showed partial re-channelling or complete re-channelling. The first case was treated with anti-aggregants and the second with anticoagulants. There were no new episodes in either cases. Dissection of the ICA usually only affects the extracranial portion of the artery, stopping in the petrous portion. We do not know why dissection also affected the intra-cranial section of the artery in these two cases.     

Chronic atrophic acrodermatitis and circulating anticoagulant

The most common initial symptom of aortic dissection is chest pain. Other initial symptoms include pain in the neck, throat, abdomen and lower back, syncope, paresis, and dyspnoea. Headache as the initial symptom of aortic dissection has not been described previously. A 61-year-old woman with a history of migraine and arterial hypertension developed continuous bifrontal headache. Two hours later, right-sided thoracic pain and a diastolic murmur were suggestive of aortic dissection that was confirmed by echocardiography and subsequent surgery. The dissection commenced in the ascending aorta and involved all cervical arteries until the base of the skull. Headache as the initial manifestation of aortic dissection was assumed due to either vessel distension or pericarotid plexus ischemia. Aortic dissection has to be considered as a rare differential diagnosis of frontal headache, especially in patients who develop aortic regurgitation or chest pain for the first time.     

Acute aortic dissection and ventricular septal defect repair

A 37-year-old male with long-standing heart murmur and ventricular septal defect developed acute chest pain and was diagnosed with an aortic dissection and aortic insufficiency. The ventricular septal defect and aortic dissection were repaired successfully as a combined procedure. At late follow-up (three years), he continued to enjoy an excellent result.     

Case report: sequential bilateral carotid dissection leading to Raeder's syndrome

A 39-year-old man complained of unilateral headache, diplopia and marked anterior neck oppression "like a necklace". MRA was used to study this case of subsequent bilateral internal artery dissection (ICAD) which initially appeared as Raeder's syndrome. MRA proved to be a non-invasive alternative for studying stenosis, occlusions and dissections of the intracranial and extracranial vessels.     

Beyond self-interest. Professional advocacy and the integration of theory, research, and practice

Autosomal dominant inheritance of thoracic aortic aneurysms and dissections occurs in subjects with Marfan syndrome, which results from mutations in the FBN1 gene on chromosome 15. A second chromosomal locus on 3p24-25 has been identified for a Marfan-like condition with thoracic aortic aneurysms. We describe here 6 families with multiple members with thoracic aortic aneurysms and dissections in the absence of the ocular and skeletal complications of Marfan syndrome. Medical records and autopsy reports on affected subjects in families with multiple members with thoracic aortic aneurysms and dissections were reviewed. Subjects in these families at risk for developing aortic disease underwent echocardiography to evaluate the aorta. The pattern of inheritance of thoracic aortic aneurysms and dissections was autosomal dominant in these families. Most affected subjects presented with aortic root dilatation or acute type I dissection, but the age of onset of disease was variable and there was decreased penetrance of the disorder. In 2 of the families, the syndrome was not linked to FBN1 or 3p24-25. Familial thoracic aortic aneurysm and dissection is an autosomal dominant condition with marked variability in the age of onset of aortic disease and decreased penetrance, making identification of affected subjects difficult. This condition is not due to mutations in the FBN1 gene or the unidentified gene on 3p24-25.     

A surgical case of acute three-channeled aortic dissection in Marfan syndrome

We surgically treated a 35-year-old male with acute 3-channeled aortic dissection in Marfan syndrome. He had acute type A aortic dissection, and underwent Bentall's type operation, simultaneous graft replacement of the ascending aorta and total aortic arch. Pain recurred 5 years and 9 months after the first operation. CT scan showed two adjacent false lumens in the descending aorta. The morphology of the first and second dissections was Stanford type A + B. The second dissection was acute. In the second false lumen, a re-entry formation was observed in the abdominal aorta. Because severe pain was persistent, we immediately replaced the descending aorta using a femoro-femoral partial cardiopulmonary bypass. The patient was doing well and was discharged. When pain recurs in a Marfan patient with an aortic dissection, a 3-channeled aortic dissection should be suspected, and we recommend emergency surgery.     

Collateralization of an occluded internal carotid artery via a vas vasorum

BACKGROUND: Reopening of an occluded internal carotid artery (ICA) is often seen in dissections but only rarely occurs in atherothrombotic occlusion of the internal carotid artery. CASE DESCRIPTION: A 60-year-old man suffered a minor stroke with dysphasia in March 1995. Color-coded duplex ultrasonography of his neck arteries revealed a left ICA occlusion. He was placed on a regimen of aspirin and followed up clinically and with ultrasonography. At follow-up 18 months later, the patient was asymptomatic. On duplex ultrasonography his left occluded ICA was found to be reopened, with a residual, proximal, high-grade stenosis. However, intra-arterial digital subtraction angiography demonstrated a persistent ICA occlusion and a vas vasorum originating from the carotid bulb and draining into the ICA distal to the occlusion. CONCLUSIONS: The rare collateralization of an occluded ICA by vasa vasorum seems to take several months. It can be a pitfall in the ultrasound diagnosis of carotid artery occlusive disease.     

Common phobias

Mrs. Brown, a 65-year-old woman, was terrified of being alone at night and suffered from nyctophobia. Her husband had been dead for several years, and her 40-year-old son lived in another state with his family. She often called him in the middle of the night in a state of panic, and he felt helpless about her situation. She lived in a senior housing community that never had any problems with crime. Nonetheless, she lived in a continual stage of anxiety.     

Deletion mapping by FISH with BACs in patients with partial monosomy 22q13

We describe three siblings with hyperparathyroidism due to multiple parathyroid adenomas without evidence of other endocrinological abnormalities. A 22-year-old woman had two parathyroid adenomas complicated by multiple ossifying jaw fibromas. Her sister, aged 29, also suffered from primary hyperparathyroidism associated with two parathyroid adenomas one of which was also suspected to be a carcinoma. These two female patients had unusual multiple small uterine polyps, which were diagnosed as adenomyomatous polyps. Their brother, aged 17, had two parathyroid adenomas complicated by urolithiasis. These three patients are characterized by young adult-onset familial isolated hyperparathyroidism due to multiple adenomas with various complications including ossifying jaw fibroma and uterine adenomyomatous polyps. These clinical features are different from those of familial hyperparathyroidism associated with multiple endocrine neoplasia.