Neurology
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Case Reports
Intrathecal baclofen for stiff-person syndrome: life-threatening intermittent catheter leakage.
Intrathecal baclofen (ITB) is used for unresponsiveness to other treatment for patients with stiff-person syndrome (SPS). The authors report a patient with SPS who developed acute and life-threatening baclofen withdrawal symptoms. Open surgery disclosed a small position-dependent leak in the catheter connector. This catheter failure was not detected by standard noninvasive checking methods.
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Patients with hemispatial neglect restricted to near (within reaching distance) or to far space (beyond reaching distance) have been described. This constitutes a double-dissociation considered by current neurocognitive thinking as compelling evidence for separate networks. ⋯ If the organization of represented space was similar to that of perceived space, it should contain a far/near dissociation as well. This paper describes a patient with pure representational neglect restricted to far space.
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Mutations in various genes of the neuromuscular junction may cause congenital myasthenic syndromes (CMS). Most mutations identified to date affect the epsilon-subunit gene of the acetylcholine receptor (AChR), leading to end-plate AChR deficiency. Recently, three different mutations in the RAPSN gene have been identified in four CMS patients with AChR deficiency. ⋯ The RAPSN mutation N88K is a frequent cause of rapsyn-related CMS in European patients. In general, patients (RAPSN N88K) were characterized by mild to moderate myasthenic symptoms with favorable response to anticholinesterase treatment. However, severity and onset of symptoms may vary to a great extent.
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Comment Letter Randomized Controlled Trial Clinical Trial
Randomized clinical trial of magnesium, diazepam, or both after out-of-hospital cardiac arrest.