PRACTICAL NEUROLOGY最新文献

Sweet's syndrome is an autoinflammatory disease characterised by systemic symptoms and a cutaneous neutrophilic dermatosis. Neurological involvement is rare but important to recognise. Patients may have headache, confusion, seizures, and focal neurological deficits; MR brain scanning may show widespread T2-hyperintense lesions, with a CSF pleocytosis. Clinicians should suspect neurological Sweet's syndrome in patients with central nervous system dysfunction, who have unexplained fever or systemic inflammation, and a pustular neutrophilic dermatosis. The condition responds well to corticosteroids, which can prevent long-term neurological sequalae.

Somatosensory evoked potentials (SSEPs) are electrical potentials that measure various parts of the ascending somatosensory pathways. They are elicited through stimulating mixed nerves, with subsequent orthodromic stimulation of sensory nerves. Despite advances in imaging, SSEPs complement both the clinical examination and peripheral neurophysiological studies when assessing the functional integrity of the sensory pathways, being especially helpful when imaging is inconclusive. We describe the technique of performing SSEPs and the anatomical substrates of their waveforms, and we highlight important clinical applications both in central and peripheral nervous system disorders.

Immune checkpoint inhibition unleashes the power of the immune system against tumour cells. Immune checkpoint inhibitors (ICIs) block the inhibitory effects of cytotoxic T-lymphocyte associated protein 4, programmed death protein 1, programmed death ligand 1 and lymphocyte activation gene 3 molecules on T-cells, and so enhance physiological cytotoxic effects. ICIs can significantly improve survival from cancers, including those previously associated with poor treatment response, such as metastatic melanoma. However, on-target off-tumour effects of ICIs result in immune-related adverse events. These toxicities are common and require new multidisciplinary expertise to manage. ICI neurotoxicity is relatively rare but ominous due to its severity, heterogenous manifestations and potential for long-term disability. Neurotoxic syndromes are novel and often present precipitously. Here, we describe ICI mechanisms of action, their impact on cancer outcomes and their frequency of immune-related adverse events. We focus particularly on neurotoxicity. We discuss the current appreciation of neurotoxic syndromes, management strategies and outcomes based on clinical expertise and consensus, multi-specialty guidance. The use of immunotherapy is expanding exponentially across multiple cancer types and so too will our approach to these cases.

Neuromuscular junction (NMJ) disorders represent a heterogenous group of acquired and congenital disorders that present in variable and distinctive ways. The diagnosis is typically reached through a combination of clinical, serological, pharmacological and electrophysiological evaluation. While the diagnosis can be fairly straightforward in some cases, the overlap with other neurological disorders can make diagnosis challenging, particularly in pure ocular presentations and in seronegative patients. The over-reliance on serological tests and electrophysiological evaluation in isolation can lead to misdiagnosis. In this article, we provide an overview of the NMJ disorders, discuss red flags for the key differential diagnoses (mimics) and report the atypical ways in which NMJ disorders may present (chameleons).