[Eaton-Lambert syndrome. Long-term treatment with corticoids].
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Biomedical subjects
Publications and source records attributed to I Illa.
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A series of 23 patients with thalamic hemorrhage with computed tomography confirmation is reported. Nine of these died, all had hematomas larger than 3.3 cm. The value of the syndrome of downward and convergent ocular deviation is stressed, and its possible mechanisms are analyzed. The characteristics and mechanisms of the pupillary abnormalities are reported, as well as the speech abnormalities observed in patients with lesions of the dominant hemisphere. Prognostic conclusions are drawn.
INTRODUCTION: In the last 15 years a number of autoantibodies against antigens of the peripheral nervous system have been associated, in some cases, to specific clinical features. DEVELOPMENT: Antibodies to MAG or gangliosides have been described in neuropathies associated to monoclonal gammopathy or inflammatory polyneuropathies, such as Guillain-Barré syndrome or multifocal motor neuropathy. A lot of research is devoted to the characterization of known antibodies to glycolipids and the discovery of new ones. Furthermore, experimental models both with animals and in vitro preparations are performed in order to unravel the possible immunopathological role of these antibodies. CONCLUSIONS: It is very important to define the clinical features of these patients precisely in order to establish consistent associations between: presence of antibodies to specific antigens of the peripheral nervous system and clinical syndromes. A deeper knowledge of the antigens and antibodies involved in these neuropathies may be very helpful in the follow-up of these patients and also for future therapies.
BACKGROUND: We report our experience in the study of antiganglioside antibodies and define their clinical value establishing associations between clinical syndromes and immunological findings. METHODS: We analysed 275 sera: Guillain-Barré syndrome (GBS) (78), Miller-Fisher syndrome (MFS) (37), chronic inflammatory demyelinating polyneuroapthy (CIDP) (17), multifocal motor neuropathy (NMM) (42), chronic axonal mixed polyneuropathy (PNP) (54), amyotrophic lateral sclerosis (ALS) (28) and lower motor neuron disease (LMND) (17). We have studied the presence of IgG and IgM antibodies to 9 gangliosides using ELISA and TLC. RESULTS: We have detected anti-GQ1b antibodies in 36/37 (97,3%) of patients with MFS, being undetectable after 4 weeks in 83%. A 34 % (26/78) of patients with GBS were positive for several antiganglioside specificities being GalGalNAc the most frequent (54%). Two out of three sera positive for GD1a corresponded to axonal Guillain-Barré. IgM class anti-GM1 antibodies were positive in 10/12 patients with MMN, while only a 3-9% of patients with ALS, CIDP, PNP and LMND presented antiganglioside antibodies. CONCLUSIONS: Analysis of anti-GQ1b antibodies confirms the diagnosis of MFS, excluding other acute brainstem pathologies and, in this study, detection of anti-GD1a antibodies indicates axonal damage in GBS and suggest a worse prognosis. IgM anti-GM1 antibodies are only found in MMN. These findings confirm a disease specific correlation between specific neuropathies and antiganglioside antibodies clinically useful.
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Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis which affects middle calibre arteries and which has a tendency to form aneurysms. It may appear as one of a great variety of clinical forms depending on the vascular territory afflicted. Almost any organ may be involved although the lung is usually unaffected. Neurological manifestations are very frequent in this disease (global incidence being around 80%), especially at the peripheral nervous system level. Multiple mononeuritis is the most usual manifestation classically associated with this type of vasculitis, but it may also appear as distal sensorimotor polyneuropathy, this being its most infrequent form of presentation. The presence of nerve conduction blocks has also been recently described, usually associated with demyelinating processes. However peripheral nerve affectation in vasculitis has its origins in small ischaemic infarcts in the sensorimotor conduction fibres. It may well be that segmentary demyelination and axonal degeneration reflect different levels of ischaemic damage.
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