Prevalence, clinical features and treatment outcomes of patients with myasthenia gravis positive for antibodies to muscle-specific kinase in Thailand

A small but variable subgroup of patients with myasthenia gravis (MG) who have antibodies to muscle-specific kinase (MuSKAb-MG) can present with distinct phenotypes and are often treatment-resistant. The prevalence, clinical phenotypes and outcomes of treatment of patients with MuSKAb-MG in Thailand were determined. Eight (16.3%) of the 49 patients with generalized MG who were negative for acetylcholine receptor antibodies (AChRAb) were positive for muscle-specific kinase antibodies. Most patients had predominant oculobulbar features and respiratory failure occurred in three. At follow up, three out of the seven patients who underwent thymectomy were in complete stable remission and four had improved and were on reduced immunosuppression medication, suggesting a possible benefit of thymectomy. © 2012 Elsevier Ltd. All rights reserved

Good clinical practice points on the use of acetylcholinesterase inhibitors in myasthenia gravis: Recommendations from the Special Interest Group in Myasthenia Gravis in Asia

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Good clinical practice points on the use of acetylcholinesterase inhibitors in myasthenia gravis: recommendation from a South-East Asian regional special interest group on myasthenia gravis

Conference Theme: Innovation in Neurolog

Verbal adynamia in parkinsonian syndromes: behavioral correlates and neuroanatomical substrate

Verbal adynamia (impaired language generation, as during conversation) has not been assessed systematically in parkinsonian disorders. We addressed this in patients with Parkinson's dementia, progressive supranuclear palsy and corticobasal degeneration. All disease groups showed impaired verbal fluency and sentence generation versus healthy age-matched controls, after adjusting for general linguistic and executive factors. Dopaminergic stimulation in the Parkinson's group selectively improved verbal generation versus other cognitive functions. Voxel-based morphometry identified left inferior frontal and posterior superior temporal cortical correlates of verbal generation performance. Verbal adynamia warrants further evaluation as an index of language network dysfunction and dopaminergic state in parkinsonian disorders

PID associated with fertility regulating agents. Task Force on Intrauterine Devices, Special Programme of Research, Development and Research Training in Human Reproduction, World Health Organization.

A WHO sponsored matched case-control study to examine the association between acute pelvic inflammatory disease (PID) and contraceptive use was conducted in 8 developing country and 4 developed country centres. 608 cases of acute febrile non-postpartum PID were individually matched with 1216 controls; 437 of the cases came from centres in developing countries and 171 from developed country centres. Among parous women the relative risks of a first episode of PID associated with current IUD use were 2.3 in developing and 4.1 in developed country centres. Nulliparous women in developed countries had a relative risk of 11.5, but the risks could not be estimated for nulliparous developing country women because there were insufficient IUD users. The high risk among nulliparous women was in part due to bias arising from probable differences in sexual activity in unmarried cases and controls. The risks for parous women aged 15-24 were 9.1 in developed and 2.9 in developing country centres. The risks of PID associated with current IUD use were much higher in women with a past history of PID. An increased PID risk was also found with past IUD use. Use of oral contraceptives or conventional contraceptives was associated with a reduced risk of first episode PID in parous but not in nulliparous women. Irrespective of contraceptive use, a past history of PID increased the risk of recurrent infection, and abortion was associated with an increased risk of first episode and recurrent PID. In conclusion, except for women under 25, the present study showed similar risks of PID related to contraceptive use, past infection or abortion in parous women from developed and developing country centres

Guillain–Barré and Miller Fisher syndromes—new diagnostic classification

Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several clinical subtypes with different neurological features and presentations. Although the typical clinical features of GBS and MFS are well recognized, current classification systems do not comprehensively describe the full spectrum of either syndrome. In this Perspectives article, GBS and MFS are classified on the basis of current understanding of the common pathophysiological profiles of each disease phenotype. GBS is subclassified into classic and localized forms (for example, pharyngeal-cervical-brachial weakness and bifacial weakness with paraesthesias), and MFS is divided into incomplete (for example, acute ophthalmoparesis, acute ataxic neuropathy) and CNS subtypes (Bickerstaff brainstem encephalitis). Diagnostic criteria based on clinical characteristics are suggested for each condition. We believe this approach to be more inclusive than existing systems, and argue that it could facilitate early clinical diagnosis and initiation of appropriate immunotherapy