A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, Gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the Kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.
From an extensive serial magnetic resonance imaging (MRI) study in multiple sclerosis (MS) we have identified 4 cases in which disruption of the blood-brain barrier, as detected by gadolinium-DTPA enhancement, preceded other MRI abnormalities and in 1 case clinical evidence of the new lesion. This supports the view that a defect in the blood-brain barrier, and therefore inflammation, is an early and possibly crucial event in the pathogenesis of the new lesion in MS. These cases showed a marked discrepancy between MRI abnormality and symptoms. The mechanisms contributing to this disparity are discussed, and it is concluded that far from being surprising it is to be expected.
Objective-To evaluate the efficiency of mitoxantrone in multiple sclerosis. Methods-Forty two patients with confirmed multiple sclerosis, selected as having a very active disease on clinical and MRI criteria were randomised to receive either mitoxantrone (20 mg intravenously (IV) monthly) and methylprednisolone (1 g iv monthly) or methylprednisolone alone over six months. In the steroid alone group five patients dropped out due to severe exacerbation. Results-Blinded analysis of MRI data showed significantly more patients with no new enhancing lesions in the mitoxantrone group compared with the steroid alone group, (90% v 31%, P < 0.001). In
SUMMARY Thirty-one patients with ectasia of intracranial arteries were studied. The carotid system was more frequently involved than the vertebrobasilar. The condition is relatively rare and usually occurs with other vascular disease. Ectasia presents with a variety of neurological symptoms, amongst which visual disorders are common. The overall mortality was 52 %, but the prognosis was appreciably worse for the vertebrobasilar group, probably because of a higher incidence of compression of surrounding neural structures. Defects in the internal elastic lamina, with or without associated atheroma, are often found in the affected arteries.Ectasia, or pathological enlargement of the cerebral arteries is a rare, though well recognised finding at cerebral angiography. Several series of vertebrobasilar ectasia,1-4 and one of carotid ectasia5 have previously been published, but there has not been a comprehensive account of the symptomatology and the natural history of this condition. We have therefore, reviewed the experience of ectasia of cerebral arteries in the National Hospitals over the past 22 years.
Material and methodsOf the approximately 40 000 carotid and 10 000 vertebral angiograms performed at the National Hospital, Queen Square between 1959 and 1980, films of those reported as showing dilatation and tortuosity of vessels were reviewed. Measurements were made of the diameter (corrected for magnification) of the major vessels as demonstrated by cerebral angiography. In addition, the course of the ectatic basilar and vertebral arteries was noted, as were the relationships of the internal carotid arteries and the termination of the basilar arteries in the sella and suprasellar regions. Thirty-two normal carotid and 18 normal vertebral angiograms of an age-matched group of patients were also examined. Ectasia was diagnosed when the diameter of the vessels was greater than normal along all or part of their course, and when they were also abnormally tortuous. Whereas the former was determined by measurement, the latter feature was
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