Abstract
Vestibular migraine is an underdiagnosed but increasingly recognized neurological condition that causes episodic vertigo associated with other features of migraine. It is now thought to be ...the most common cause of spontaneous (non-positional) episodic vertigo, affecting up to 1% of the population. A meta-analysis of preventative treatments for vestibular migraine was published in 2021, but the authors were unable to establish a preferred treatment strategy due to low quality of evidence and heterogeneity of study design and outcome reporting. Therefore, there remains a clinical need for pragmatic management guidelines specific to vestibular migraine using the available evidence. Here, we provide a practical review utilizing a systematic qualitative assessment of the evidence for abortive and preventative interventions in adults. The overall evidence base for vestibular migraine treatment is of low quality. Nevertheless, we provide practical treatment recommendations based on the available evidence and our experience to help guide clinicians treating patients with vestibular migraine. We also discuss how future clinical trials could be designed to improve the quality of evidence in this condition.
POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth factor (VEGF). Dueto its rarity, ...there are no internationally agreed standards of care, with verylimited data to guide management in the relapse setting. Agents used in myelomaare rational choices and have been employed. Daratumumab has been reported intwo case studies with lenalidomide‐dexamethasone, one in the upfront and one inthe relapsed setting. We are the first to report here three cases ofdaratumumab‐bortezomib‐dexamethasone (DVd) use in relapsed POEMS postautologous stem cell transplant with good VEGF and clinical responses. Our casesadd to the literature on efficacy of daratumumab and are the first to report onits safe use with bortezomib in relapsed POEMS. It should be considered as aclinical option, in patients not responding to conventional first linetherapies.
Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial ...comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 12–16 months, by which time patients can be severely disabled. There are no established consensus guidelines. We provide clinicians a comprehensive blueprint for managing POEMS from diagnostic suspicion through the work‐up, selection of therapy, follow‐up, and treatment of relapse based on published evidence and our large single‐center experience. A multidisciplinary approach is essential including expert hematologists, neurologists, histopathologists, radiologists, and neurophysiologists. The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. Response assessment is centered on clinical, neuropathy, hematological, vascular endothelial growth factor, and radiological criteria. Future clinical trials are welcomed in this setting where evidence is limited.
Bilateral vestibular failure (BVF) is an uncommon condition with numerous etiologies. It causes chronic oscillopsia and imbalance and is usually irreversible. We report 2 cases of BVF due to unusual ...causes, both of which improved with treatment of the underlying condition.
The first patient was a 39-year-old female who developed profound BVF due to neurosarcoidosis. She was started on steroids and azathioprine and her vestibular function gradually improved, with essentially normal function 4.5 years after starting treatment. The second patient was a 54-year-old female who developed BVF in the context of glucagonoma. After treatment with octreotide, her vestibular function improved to almost normal, and she thus met the criteria for a probable paraneoplastic syndrome.
While BVF is usually permanent, this report demonstrates that some cases are likely to be reversible with treatment of the underlying cause. It is therefore imperative for clinicians to ensure that patients with BVF are thoroughly investigated.
ObjectiveBecause clozapine and risperidone have been shown to reduce neuroinflammation in humans and mice, the Clozapine and Risperidone in Progressive Multiple Sclerosis (CRISP) trial was conducted ...to determine whether clozapine and risperidone are suitable for progressive multiple sclerosis (pMS).MethodsThe CRISP trial (ACTRN12616000178448) was a blinded, randomised, placebo-controlled trial with three parallel arms (n=12/arm). Participants with pMS were randomised to clozapine (100–150 mg/day), risperidone (2.0–3.5 mg/day) or placebo for 6 months. The primary outcome measures were safety (adverse events (AEs)/serious adverse events (SAE)) and acceptability (Treatment Satisfaction Questionnaire for Medication-9).ResultsAn interim analysis (n=9) revealed significant differences in the time-on-trial between treatment groups and placebo (p=0.030 and 0.025, clozapine and risperidone, respectively) with all participants receiving clozapine being withdrawn during the titration period (mean dose=35±15 mg/day). Participants receiving clozapine or risperidone reported a significantly higher rate of AEs than placebo (p=0.00001) but not SAEs. Specifically, low doses of clozapine appeared to cause an acute and dose-related intoxicant effect in patients with pMS who had fairly severe chronic spastic ataxic gait and worsening over all mobility, which resolved on drug cessation.InterpretationThe CRISP trial results suggest that patients with pMS may experience increased sensitivity to clozapine and risperidone and indicate that the dose and/or titration schedule developed for schizophrenia may not be suitable for pMS. While these findings do not negate the potential of these drugs to reduce multiple sclerosis-associated neuroinflammation, they highlight the need for further research to understand the pharmacodynamic profile and effect of clozapine and risperidone in patients with pMS.Trial registration numberACTRN12616000178448.
Aim: To identify factors that predict admission to a rehabilitation hospital for patients over 65 years of age.
Methods: This study reviewed the destinations of all patients over 65 years admitted ...to a Department of General and Vascular Surgery over 1 year to analyse factors associated with subsequent admission to a rehabilitation hospital. Data recorded included demographic characteristics, type of admission, length of stay at the primary hospital, operation, speciality type, previous admission to rehabilitation hospital, diagnoses and procedures.
Results: Of the 2632 patients examined, 8.7% were subsequently admitted to a rehabilitation hospital. Multivariate analysis showed that previous admission to the rehabilitation hospital, increasing age, number of diagnoses and admission under vascular service were all independently associated with admission for rehabilitation.
Conclusions: Factors associated with increased risk of requiring transfer to a rehabilitation hospital can be identified. This allows early recognition of at‐risk patients.
COVID‐19 in a UK neurology hospital Khoo, Anthony; Smyth, Duncan; Vivekanandam, Vinojini
Internal medicine journal,
August 2020, Volume:
50, Issue:
8
Journal Article
•Auto-immune encephalitis can complicate COVID-19.•Consider autoimmune antibody panels in atypical post-COVID encephalitis.•MRI and CSF studies may be normal in anti-LGI1 mediated disease.
Reliable and responsive tools for monitoring disease activity and treatment outcomes in patients with neuropathies are lacking. With the emergence of ultrasensitive blood bioassays, proteins released ...with nerve damage are potentially useful response biomarkers for many neurological disorders, including polyneuropathies. In this review, we provide an overview of the existing literature focusing on potential applications in polyneuropathy clinical care and trials. Whilst several promising candidates have been identified, no studies have investigated if any of these proteins can serve as response biomarkers of longitudinal disease activity, except for neurofilament light (NfL). For NfL, limited evidence exists supporting a role as a response biomarker in Guillain-Barré syndrome, vasculitic neuropathy, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Most evidence exists for NfL as a response biomarker in hereditary transthyretin-related amyloidosis (hATTR). At the present time, the role of NfL is therefore limited to a supporting clinical tool or exploratory endpoint in trials. Future developments will need to focus on the discovery of additional biomarkers for anatomically specific and other forms of nerve damage using high-throughput technologies and highly sensitive analytical platforms in adequality powered studies of appropriate design. For NfL, a better understanding of cut-off values, the relation to clinical symptoms and long-term disability as well as dynamics in serum on and off treatment is needed to further expand and proceed towards implementation.
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