1/80. susac syndrome: microangiopathy of the retina, cochlea and brain.BACKGROUND: susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. methods: Analysis of two male and two female cases of susac syndrome recognized in australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behcet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/80. Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis.OBJECTIVES: Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. case reports: All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T(2) weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insufficiency. DISCUSSION: Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier.- - - - - - - - - - ranking = 4keywords = vasculitis (Clic here for more details about this article) |
3/80. Predictive value of ANCA in atypical primary brain localization of Wegener's granulomatosis.Wegener's Granulomatosis is a necrotizing vasculitis generally involving upper and lower respiratory tract and kidneys. The central nervous system is involved in less than 10% of the patients during the course of the disease and primary involvement is even rarer. We present and discuss the case of a patient with primitive cerebral localization of Wegener's Granulomatosis in which the diagnosis and the beginning of correct therapy were delayed, in spite of a rising c-ANCA titer, due to a misinterpretation of a bioptic specimen. This delay caused renal damage and pulmonary cavitations which needed a long time to recover. This case report suggests that the central nervous system can be the site of a primary localization of Wegener's Granulomatosis even without any other organ involvement. The diagnosis must be made as soon as possible in order to prevent spread to other sites since the disease is usually very aggressive and severe.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
4/80. Neuro-Behcet's disease mimicking a cerebral tumor: a case report.We report a rare case of neuro-Behcet's disease (NBD) presenting as an inflammatory pseudotumor in the brain. A 52-year-old woman was evaluated for subacute dizziness and headache. Brain magnetic resonance (MR) imaging showed a right cerebellar mass, which disappeared 2 weeks later. After a year, recurrent mucocutaneous manifestations of Beh et's disease were observed. Immunosuppressant and steroid maintenance treatment were started. She experienced two more neurologic attacks and brain MR imaging revealed an enhancing mass in the right temporal lobe. The second attack showed a good response to steroid pulse therapy, but the third attack did not respond to steroid and her neurologic signs suggested an impending transtentorial hernia. The right temporal lobectomy was performed for the purpose of life-saving. The pathologic finding of the mass was a chronic inflammatory vasculitis, compatible with NBD.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
5/80. Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches.OBJECTIVE: Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches. RESULTS: The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years. CONCLUSIONS: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.- - - - - - - - - - ranking = 2keywords = vasculitis (Clic here for more details about this article) |
6/80. Malignant angioendotheliomatosis (intravascular lymphomatosis) clinically simulating primary angiitis of the central nervous system.Primary angiitis of the central nervous system (PACNS) and malignant angioendotheliomatosis (MAE) are two unrelated and lethal diseases that frequently affect the brain. Both are uncommon and both require histologic documentation of their characteristic lesions for a definitive diagnosis. Because brain biopsies are not routinely included in the diagnostic workup of non-neoplastic CNS diseases, a number of unconfirmed nonvasculitic conditions may mimic PACNS. To my knowledge, MAE simulating PACNS clinically and angiographically has not been previously reported, and 3 such cases are described herein.- - - - - - - - - - ranking = 504.83907173484keywords = angiitis (Clic here for more details about this article) |
7/80. Hashimoto's encephalopathy: postmortem findings after fatal status epilepticus.The clinical features of Hashimoto's encephalopathy have been attributed to a cerebral vasculitis, but pathologic material is rarely available. The authors describe an individual with Hashimoto's encephalopathy complicated by fatal status epilepticus. Postmortem examination demonstrated mild perivascular lymphocytic infiltration throughout the brain and leptomeninges plus diffuse gliosis of gray matter in the cortex, basal ganglia, thalami, hippocampi, and, to a lesser extent, the parenchymal white matter.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
8/80. Postpartum angiopathy with reversible posterior leukoencephalopathy.BACKGROUND: Postpartum angiopathy (PPA) is a cerebral vasoconstriction syndrome of uncertain cause that affects large and medium-sized cerebral arteries. Postpartum angiopathy is frequently complicated by ischemic stroke. The reversible posterior leukoencephalopathy syndrome (RPLS) is a distinct clinical-radiological entity characterized by transient vasogenic edema on brain imaging. The pathophysiological features of RPLS are related to small-vessel dysfunction and breakdown of the blood-brain barrier. OBJECTIVES: To report the coexistence of PPA and RPLS in 4 patients and to discuss possible interrelationships between these 2 entities. DESIGN: Four case reports and a review of the literature. RESULTS: Four women developed a clinical-radiological syndrome overlapping PPA and eclampsia shortly after an uncomplicated pregnancy. All had acute severe ("thunderclap") headaches and hypertension. Three developed seizures. All patients had reversible angiographic narrowing of large and medium-sized cerebral arteries. Serial magnetic resonance imaging showed transient nonischemic brain lesions, resembling the lesions described in patients with RPLS. The results of extensive tests for cerebral vasculitis were negative. CONCLUSION: These cases, and the literature, suggest an interrelationship between RPLS and cerebral vasoconstriction syndromes such as PPA.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
9/80. Image directed stereotactic surgery for brain stem lesions.Advances in neurological imaging may have increased the diagnostic accuracy and the detection rate of intrinsic brain stem lesions, but a histological diagnosis is still an essential requirement for rational and appropriate management. Open exploration allows biopsy and resection in cases where an exophytic component is present. The surgical inaccessibility and the resultant morbidity of these approaches, however, associated with a low diagnostic yield in cases with no visible surface abnormality, are important limiting factors. A series of 45 brain stem lesions stereotactically approached with CT or MRI guidance is presented. A transcortical frontal pre-coronal trajectory was used in all of them. Haematoma was preoperatively diagnosed in 10 cases and the procedure was for therapeutic aspiration. Of 35 cases where the diagnosis was uncertain, although intrinsic tumour was suspected, positive results were obtained in 33, while unexpected findings of granuloma, lymphoma, angioma, leucoencephalopathy, vasculitis and radiation necrosis were found in over 10% of the cases. There were no operative deaths and the morbidity was low. In no case was there a permanent neurological deterioration directly related to the procedure, although there was a transient deterioration in two patients and one patient required early reaspiration of a haematoma. Image directed stereotactic approaches to brain stem lesions can combine a high degree of accuracy (offering positive histological diagnoses) with a low operative morbidity. MRI directed biopsies can complement CT guided ones thus increasing the number of suitable cases and improving the success rate. The frontal precoronal transcortical trajectory provides safe access to the majority of the brain stem targets.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
10/80. Primary angiitis of the central nervous system: diagnostic criteria and clinical approach.Primary angiitis of the central nervous system has until recently been considered rare, usually progressive, and untreatable. In 1987, preliminary diagnostic criteria for the disorder were proposed in the hope of gaining a more accurate description of the disease. We used these criteria in reviewing all cases reported in the English language (99 cases) and those seen at The Cleveland Clinic Foundation (9 cases). We believe that the disorder is heterogeneous, and has a better prognosis than previously thought. Furthermore, a relatively benign subset of this disorder, termed "benign angiopathy of the central nervous system," can be defined on clinical grounds. We propose a revised set of diagnostic criteria and a practical approach to treatment.- - - - - - - - - - ranking = 504.83907173484keywords = angiitis (Clic here for more details about this article) |
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