PRACTICAL NEUROLOGY最新文献

Progressive multifocal leukoencephalopathy (PML) is an infectious disease of the central nervous system that is typically severe and classically occurs in the setting of overt immunocompromise. It is caused by the John Cunningham virus (JCV), a ubiquitous polyomavirus that only rarely leads to neurological complications. Prompt diagnosis of PML is essential for patient management, but can be complicated by disease development in individuals who are relatively immunocompetent, the lack of highly specific clinical symptoms, unfamiliarity with recently described neuroimaging signs and the imperfect sensitivity of assays used to detect JCV in cerebrospinal fluid (CSF). We review the supportive clinical features, neuroimaging findings and confirmatory testing for PML and propose an algorithm to facilitate accurate diagnosis in clinical practice.

A 60-year-old woman presented with a 38-month history of progressive tremor and gait impairment. Examination was consistent with a myeloradiculopathy, cerebrospinal fluid (CSF) analysis was inflammatory and imaging showed meningeal and white matter disease. Revisiting the history identified long periods spent gardening in the months before symptom onset with significant tick exposure. Lyme testing was strikingly positive in serum and CSF: a diagnosis of late Lyme neuroborreliosis was confirmed. There was clinical and radiological response to appropriate antimicrobial treatment with ongoing improvement at follow-up. This case highlights the importance of thorough history taking, a stepwise approach to investigating a complex neurological syndrome and the invaluable input of multiple specialties required to manage such cases.

There are many possible causes for acute encephalitis, and systemic causes can be easily overlooked. We report a woman with initial status epilepticus, marked working memory impairment and ataxia, which developed 1 week following the ChAdOx1 SARS-CoV-2 (Astra-Zeneca) vaccination. Although lumbar puncture detected a paraprotein, it was not until she developed the hallmark cutaneous features of scleromyxoedema several months later that we recognised this as the dermato-neuro syndrome. Given the temporal association, the SARS-CoV-2 vaccination was a likely trigger, and the decision whether to give subsequent vaccinations during the heat of the COVID-19 pandemic added a layer of complexity to decision making. We review the literature regarding dermato-neuro syndrome in the setting of SARS-CoV-2 vaccination and explore its unique features.

A man in his 30s from South Asia presented with progressive neck pain, occipital headache and right-sided tongue deviation (occipital condyle syndrome). Initial imaging identified a destructive skull base lesion involving the right clivus, occipital condyle and C1 vertebra with compression of the hypoglossal nerve, raising concerns for malignancy. Concurrent necrotic mediastinal lymphadenopathy prompted endobronchial ultrasound-guided biopsy, which confirmed necrotising granulomatous lymphadenitis with Mycobacterium tuberculosis complex DNA. Quadruple antituberculous therapy and adjunctive corticosteroids gave significant clinical and radiological improvement at 6 months. This case highlights the importance of considering tuberculosis in the differential diagnosis of destructive skull base lesions even in non-endemic regions and in patients with previously negative TB screening.