Ruptured de novo aneurysm induced by ethyl 2-cyanoacrylate: case report

OBJECTIVE AND IMPORTANCE: We report a rare case of a ruptured de novo aneurysm induced by ethyl 2-cyanoacrylate. CLINICAL PRESENTATION: A 44-year-old woman had undergone microvascular decompression for a right-sided facial spasm. The preoperative vertebral angiogram did not show any aneurysmal dilation. The right anteroinferior cerebellar artery, which was compressing the exit zone of the facial nerve, was detached and fixed to the dura mater with ethyl 2-cyanoacrylate. Nine years later, the patient suffered a subarachnoid hemorrhage caused by the rupture of a newly developed aneurysm of the right anteroinferior cerebellar artery. INTERVENTION: The aneurysm was clipped 2 days after onset of the subarachnoid hemorrhage. It consisted of two bulges in the arterial wall on the proximal side of the meatal loop. One bulge was stuck to the dura mater of the pyramis by ethyl 2-cyanoacrylate, which had been used in the microvascular decompression 9 years previously. CONCLUSION: This is the first reported clinical case of a de novo aneurysm induced by a cyanoacrylate adhesive. Ethyl 2-cyanoacrylate can damage the arterial wall and induce a de novo aneurysm.     

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.     

Fenestrated oculomotor nerve caused by internal carotid-posterior communicating artery aneurysm: case report

OBJECTIVE AND IMPORTANCE: The fenestrated oculomotor nerve associated with the internal carotid-posterior communicating artery aneurysm is very rare. CLINICAL PRESENTATION: A 48-year-old woman had a history of subarachnoid hemorrhage caused by a ruptured right middle cerebral artery aneurysm, which was wrapped with good postoperative course. Twenty years later, the patient suffered frontal headache with a mild oculomotor nerve paresis in the right side. Follow-up neuroimaging studies demonstrated a de novo right internal carotid-posterior communicating artery aneurysm. INTERVENTION: The aneurysm was exposed and clipped via a right pterional route. The fenestrated oculomotor nerve associated with the aneurysm was confirmed at surgery. CONCLUSION: We speculated that the fenestration was most likely caused, by the growth of the aneurysm.     

Ehlers-Danlos syndrome type I. Ultrastructural study

Ehlers-Danlos syndrome comprises a very heterogeneous group of collagen diseases characterised in clinical terms by fragility and cutaneous hyperextensability, ligamentous hyperlaxity, ecchymosis, scarring, visceral and neurological manifestations. Having been described in detail by Ehlers in 1899 and Danlos in 1908, it was subsequently classified into various clinical types. At present at least 11 forms are recognised on the basis of their clinical characteristics, methods of transmission and biochemical defect; the first four types of the syndrome account for approximately 95% of cases. Almost all forms are transmitted with a dominant autosomic character. Specific genetic mutations have been ascertained whereas the biochemical defect has been identified in numerous types. Ehlers-Danlos type 1 syndrome is the most frequent and most severe form. The biochemical anomaly underlying the altered deposition of collagen fibre is still unknown and this is responsible for the "storiform" appearance of collagen fibre on ultrastructural examination. The authors have described a typical case of "Ehlers-Danlos type 1 syndrome" in which the diagnosis was confirmed by comparing clinical data and the results of ultrastructural tests which revealed the characteristic "pattern" of collagen fibres.     

Repeat angiography and magnetic resonance imaging (MRI) of dissecting aneurysms of the intracranial vertebral artery. Report of four cases

We here present 4 cases with dissecting aneurysm (DA) of the intracranial vertebral artery, who were followed up by repeat cerebral angiography and MRI. The patients consisted of 2 males and 2 females, and the mean age was 43 years. Two cases were associated with polyarteritis nodosa (PN) and hypertension, respectively. Three of the cases developed subarachnoid haemorrhage (SAH), while the other one suffered from lateral medullary syndrome. In cerebral angiography, "pearl and string" signs were revealed in all cases, while a "double lumen" indicating a true diagnostic sign of DA was demonstrated in only one case. Repeat angiography showed that a bleb formation with a bulging of the aneurysmal sac was seen in 2 cases, and an irregularity of the wall in one case. On the other hand in one case, the ectatic part shrank, while the stenotic part was restored. In magnetic resonance imaging (MRI), a hyperintensity mass on T 1-weighted image (T 1-WI) adjacent to flow void suggesting either an intramural haematoma or a linear shape hyperintensity on T 1-WI were demonstrated in 3 cases. In the follow up MRI done in 2 cases, a serial change in the intensity from iso-intensity to hyperintensity on T 1-WI was observed in one case suggesting intramural haemorrhage, while an enlargement of the ectasic flow void was seen in the other case. Three of 4 cases were operated on by trapping of the aneurysms. One, who had systemic vascular diseases due to PN, and repeat angiography showed a regression of the aneurysm, was conservatively treated.(ABSTRACT TRUNCATED AT 250 WORDS)     

The effect of a preparation from the horns of the saiga antelope on the goal-directed drinking behavior of rats

We analyzed 79 consecutive patients with aneurysms and found a patient who lacked type III collagen. Collagen was extracted from the skin, and the lack of type III collagen was determined by means of sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Only 1 of the 79 patients was found to lack type III collagen. This patient was a 55-year-old man who had sustained an abdominal aortic aneurysm and aortic dissection. He did not show any of the typical clinical symptoms of Ehlers-Danlos syndrome type IV, such as hyperextensible skin and joints. He had none of the usual risk factors nor any clear family history of the syndrome. Furthermore, his collagen fibrils demonstrated a homogeneous appearance. This case may represent a new form of type III collagen deficiency.     

Acute mast cell leukemia disclosed by vasomotor flushing

INTRODUCTION: Acute mast cell leukemia is a rare and severe disease. We report herein a case associated with a flush syndrome. CASE REPORT: A 44-year-old man, presented with a flush of face and trunk. Bone marrow was infiltrated with immature mast cells. In spite of chemotherapy and bone marrow transplantation the patient deceased. DISCUSSION: Pheochromocytoma, carcinoid tumor, and mastocytosis are associated with a flush syndrome. In our patient the diagnosis was an acute mast cell leukemia. Acute mast cell leukemia can follow systemic mastocytosis or occur de novo. This disease is of poor prognosis. No treatment is available.     

Prenatal diagnosis of de novo distal 5q duplication associated with hygroma colli, fetal oedema and complex cardiopathy

We report the first prenatal diagnosis of de novo distal 5q duplication after the echographic findings of hygroma colli and complex cardiopathy in a female fetus of 16 weeks' gestation. Cytogenetic studies on amniocytes showed a de novo inverted distal 5q duplication: karyotype: 46,XX, inv dup(5) (pter-->q3.53::q3.53-q3.33::q3.53-->qter). Based on the findings in the literature, a review of the malformative syndrome associated with partial distal 5q trisomy is given.     

Timing of surgery in patients with aneurysmal subarachnoid haemorrhage: rebleeding is still the major cause of poor outcome in neurosurgical units that aim at early surgery

OBJECTIVE: To investigate prospectively the proportion of patients actually operated on early in units that aim at surgery in the acute phase of aneurysmal subarachnoid haemorrhage (SAH) and what is the main current determinant of poor outcome. METHODS: A prospective analysis of all SAH patients admitted during a one year period at three neurosurgical units that aim at early surgery. The following clinical details were recorded: age, sex, date of SAH, date of admission to the neurosurgical centre, whether a patient was referred by a regional hospital or a general practitioner, Glasgow coma scale and grade of SAH (World Federation of Neurological Surgeons (WFNS) score) on admission at the neurosurgical unit, results of CT and CSF examination, the presence of an aneurysm on angiography, details of treatment with nimodipine or antifibrinolytic agents, and the date of surgery to clip the aneurysm. At follow up at three months, the patients' clinical outcome was determined with the Glasgow outcome scale and in cases of poor outcome the cause for this was recorded. RESULTS: The proportion of patients that was operated on early--that is, within three days after SAH--was 55%. Thirty seven of all 102 admitted patients had a poor outcome. Rebleeding and the initial bleeding were the main causes of this in 35% and 32% respectively of all patients with poor outcome. CONCLUSIONS: In neurosurgical units with what has been termed "modern management" including early surgery, about half of the patients are operated on early. Rebleeding is still the major cause of poor outcome.     

Etiology of immune stromal (interstitial) keratitis

BACKGROUND: Subarachnoid hemorrhage (SAH) with a spinal origin is a rare clinical entity. The most common source of SAH is an arteriovenous malformation. Only six cases of spinal hemangioblastomas causing SAH have been reported. CASE DESCRIPTION: This 48-year-old man had experienced severe headache 12 years earlier, which was diagnosed as SAH by computed tomography (CT) scan and lumbar puncture. However, cerebral angiography could not detect either aneurysm or arteriovenous malformation. Thereafter, he spent an uneventful life until he was incidentally diagnosed with a cervical tumor on magnetic resonance imaging (MRI) study for Meniere's disease. The tumor was situated at the cervicomedullary junction, and fed by the left vertebral artery. Histologic findings of the tumor showed features typical of hemangioblastoma. CONCLUSIONS: We describe a case of cervical spinal extramedullary hemangioblastoma with a past history of SAH. In our case, the SAH of 12 years ago perhaps occurred from the cervicomedullary junction hemangioblastoma. Since the etiology of SAH could not be detected at the first angiography, not only follow-up reangiography, but also MRI of the spinal cord and spinal angiography could have played an important role.     

Clinical significance of micromegakaryocytes in de novo AML

Bone marrow specimens obtained from 54 patients with de novo AML and 7 patients with AML evolving from MDS were retrospectively examined for the presence of micromegakaryocytes defined as cells of less than 30 microns in diameter with one or two nuclei. At least 25 megakaryocytes were counted in each patient. Micromegakaryocytes were found in 17 cases (31%), M1:1/11, M2:5/18, M3:0/4, M4:5/12, M5:1/4, M6:4/4, M7:1/1. The median age of the patients was higher in de novo AML with micromegakaryocytes (57 years) than in de novo AML without micromegakaryocytes (41 years) (p = 0.014). Chromosomal analysis revealed that deletion of 5 or 5q-, 7 or 7q- was recognized only in the group of de novo AML with micromegakaryocytes and that t(15;17), t(8:21) and inv (16) were not recognized in this group. Micromegakaryocytes were identified in each bone marrow specimen obtained from 9 of 10 patients with de novo AML with trilineage myelodysplasia. The complete remission rate was significantly lower in de novo AML with micromegakaryocytes (33%) than in de novo AML without micromegakaryocytes (86%) (p = 0.001). The duration of survival of the patients with de novo AML with micromegakaryocytes was shown to be shorter than that of the patients with de novo AML without micromegakaryocytes (p = 0.017). Micromegakaryocytes were recognized in all of 7 patients with AML evolving from MDS. The presence of micromegakaryocytes in bone marrow of the patients with AML indicates a subset of AML with poor prognosis that may be closely associated with myelodysplastic syndrome.     

Ehlers-Danlos syndrome

We have described a patient with type 4 Ehlers-Danlos syndrome who died of a ruptured pulmonary artery. Of the six subtypes of Ehlers-Danlos syndrome, which can usually be clinically differentiated, only types 1 and 4 appear to be associated with a substantial risk of arterial rupture.     

Risk of recurrent subarachnoid hemorrhage after complete obliteration of cerebral aneurysms

BACKGROUND and PURPOSE: The neck clipping of cerebral aneurysms is a well-established treatment for subarachnoid hemorrhage (SAH) caused by aneurysmal rupture. However, it is still unclear how great a risk of recurrence patients with a successfully treated aneurysm carry over a long-term period. METHODS: Of 425 patients with SAH surgically treated in Aizu Chuou Hospital from 1976 to 1994, 220 cases meeting the following criteria were studied: (1) all aneurysms detected by 3- or 4-vessel cerebral angiography were clipped, (2) complete obliteration of aneurysm(s) was confirmed by postoperative angiography, and (3) the patient survived >3 years. All patients were traced until January 1998 for recurrent SAH or death. The mean follow-up period was 9.9 (range, 3 to 21) years. RESULTS: Six patients (2.7%) had recurrent SAH, each with an interval ranging from 3 to 17 years (mean, 11 years) since the original treatment. In addition, 2 patients were found to have regrowth of the originally operated aneurysms. The cumulative recurrence rate of SAH, calculated using the Kaplan-Meier method, was 2.2% at 10 years and 9. 0% at 20 years after the original treatment. CONCLUSIONS: The recurrence rate was considerably higher than the previously reported risk of SAH in the normal population, and the rate increased with time. These data indicate that patients with ruptured cerebral aneurysms still carry higher risks for SAH in a long-term period, even after complete obliteration of the aneurysm, and that periodic examination to detect recurrent aneurysms may be indicated for such patients.     

Rupture of a previously documented small asymptomatic intracranial aneurysm in a patient with autosomal dominant polycystic kidney disease. Case report

Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.     

Capillaries with fenestrations around regenerating muscle fibers in the soleus muscle of the dystrophic (dy) mouse

A 65-year-old woman developed nephrotic syndrome 7 years after receiving a cadaveric renal allograft. Renal biopsy and clinical laboratory evaluation revealed the underlying disease process to be AL amyloidosis. To our knowledge, this is the first reported case of de novo AL amyloid occurring in a renal allograft.     

Prognostic factors for adenocarcinoma of the gallbladder: an analysis of 162 cases

We experienced a case with sudden unexpected death caused by rupture of an intracranial aneurysm, which was confirmed by autopsy. Depending on this case, we reported the significance of the cerebellar tonsillar herniation on the cause of sudden death of ruptured cerebral aneurysm. A 58-year-old man was admitted to us for treatment of subarachnoid hemorrhage (SAH). The CT scanning showed diffuse SAH in the whole cistern. Cerebral angiography on admission revealed an aneurysm at the bifurcation of the left middle cerebral artery in association with bleb like configuration. The aneurysmal neck was clipped on the day of admission. The postoperative course was uneventful. In the early morning of postoperative 23rd day, he was found being expired. To clarify the cause of death, an autopsy was done, disclosing diffuse SAH in association with tonsillar herniation more marked on the left. Thus, the distortion of the spinomedullary junction due to asymmetrical herniation was considered to be responsible for unexpected sudden death in this case. Examination of the major cerebral artery disclosed a ruptured anterior communicating artery aneurysm.     

Gynecologic disorders in women with Ehlers-Danlos syndrome

OBJECTIVE: We characterized the population with Ehlers-Danlos syndrome with regard to genital prolapse, urinary incontinence, and other gynecologic disorders. METHODS: Forty-one adult women who had registered for a first-ever Ehlers-Danlos multidisciplinary clinic participated in the study. Each had a comprehensive standardized evaluation, including gynecologic history, physical examination, urodynamic testing, and physical therapy evaluation. Qualitative and quantitative data were analyzed to determine means for various gynecologic disorders of Ehlers-Danlos syndrome. RESULTS: The frequencies of incontinence complaints (59%), endometriosis (27%), dyspareunia (57%), and previous hysterectomy (44%) were higher than expected for a population with a mean age of 41 years. Incontinence could not be demonstrated objectively. Prolapse was diagnosed in 12 (29.3%). CONCLUSIONS: Careful attention should be paid to women with Ehlers-Danlos syndrome because of an association with many gynecologic complaints. Women with Ehlers-Danlos syndrome should be questioned regarding incontinence, genital prolapse, endometriosis, and dyspareunia.     

Cardiac outcome in patients with subarachnoid hemorrhage and electrocardiographic abnormalities

OBJECTIVE: Approximately 25% of patients with subarachnoid hemorrhage (SAH) have electrocardiographic (ECG) abnormalities consistent with myocardial ischemia or myocardial infarction (MI), and their cardiac prognosis remains unclear. The objective of this study was to determine the cardiac and all-cause mortality rate of a series of patients with SAH with ECG changes consistent with ischemia or MI. METHODS: Using an existing database of patients with SAH and predetermined ECG criteria for ischemia or MI, a study group of patients with abnormal ECG results within 3 days of presentation and before aneurysm surgery was identified. Database patients without abnormal ECG results served as a control group. Cardiac mortality, defined as death resulting from arrhythmia, congestive heart failure, or cardiogenic shock, was assessed by chart review. RESULTS: Of 439 patients with SAH in the database, 58 met the criteria for the study group. Forty-one of these patients were treated neurosurgically. No deaths resulting from cardiac causes occurred, and 20 patients died as a result of noncardiac causes. In a multivariable analysis, age older than 65 years and Hunt and Hess grade of at least 3 were predictive of all-cause mortality. ECG abnormalities, however, were not a statistically significant predictor. CONCLUSION: In patients with SAH and ECG readings consistent with ischemia or MI, the risk of death resulting from cardiac causes is low, with or without aneurysm surgery. The ECG abnormalities are associated with more severe neurological injury but are not independently predictive of all-cause mortality.     

An evidence-based approach to management of increased intracranial pressure

This article reviews the current understanding of genetic factors involved in the development of intracranial aneurysms. Of the large number of heritable connective tissue disorders that have been associated with intracranial aneurysms, three are discussed in detail: autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome type IV, and alpha 1-antitrypsin deficiency. The author reviews the familial occurrence of intracranial aneurysms, which is found in about one-fifth of patients with aneurysm subarachnoid hemorrhage. Guidelines are provided for the evaluation of patients with familial intracranial aneurysms and screening of their relatives.     

Klinefelter syndrome associated with 13/14 translocation abnormality 46,XXY,t(13q;14q)

In this communication, a case of Klinefelter syndrome associated with a 13/14 translocation is described. Such a rare occurrence is most probably due to the de novo arrangement of chromosomes related to the advanced ages of both parents at conception.