Occult bowel cancer presenting as Morvan syndrome.

A man in his mid-60s presented with a 3-month history of progressive muscle twitching, agitation, cognitive impairment, insomnia, hyperhidrosis and lower limb pain. He had fasciculations, myokymia, myoclonus, exaggerated startle response and significant postural hypotension. Electrophysiological studies showed evidence of peripheral nerve hyperexcitability with neuromyotonia. Contactin-associated protein-like 2 antibodies (CASPR2) were strongly positive. A diagnosis of Morvan syndrome was made. CT of the chest, abdomen and pelvis was undertaken to identify any occult malignancy, and a large bowel carcinoma in situ was identified and resected. His central nervous system and autonomic symptoms significantly improved following surgery, but neuromyotonia persisted, and this was treated with intravenous immunoglobulins and steroids. Early detection of bowel cancer in this patient enabled curative treatment.

Tapia's syndrome in a patient with COVID-19.

A 42-year-old man developed bilateral Tapia's syndrome (recurrent laryngeal and hypoglossal nerves paralysis) following prolonged ventilation for COVID-19 pneumonia. Examination showed global tongue atrophy and bilateral asymmetric vocal cord palsy. He improved rapidly without specific treatment, suggesting that neuropraxia was the likely mechanism of injury. Tapia's syndrome has been reported disproportionately more often in association with COVID-19, possibly from injury to hypoglossal and vagal nerves during ventilation in the prone position.

Update on management of epilepsy in women for the non-neurologist.

Epilepsy is a common neurological disorder, prevalent in about 1% of the population. Almost half of the patients with epilepsy are women. Epilepsy and antiepileptic drugs can affect each aspect of the female human life cycle which includes menstrual cycle, contraception, fertility, conception, pregnancy and menopause. The interplay of the female hormonal state and epilepsy is complex and has to be taken in to consideration when managing their epilepsy. This review focuses on the management of women with epilepsy related to their role in reproduction.

British Neurotoxin Network recommendations for managing cervical dystonia in patients with a poor response to botulinum toxin.

Botulinum toxin (BoNT) injections are an effective treatment for cervical dystonia. Approximately 20% of patients eventually stop BoNT treatment, mostly because of treatment failure. These recommendations review the different therapeutic interventions for optimising the treatment in secondary poor responder patients. Immunoresistance has become less common over the years, but the diagnosis has to be addressed with a frontalis test or an Extensor Digitorum Brevis test. In case of immunoresistance to BoNT-A, we discuss the place the different therapeutic options (BoNT-A holidays, BoNT-B injections, alternative BoNT-A injections, deep brain stimulation). When poor responders are not immunoresistant, they benefit from reviewing (1) injections technique with electromyography or ultrasound guidance, (2) muscles selection and (3) dose of BoNT. In addition, in both scenarios, a holistic approach including drug treatment, retraining and psychological support is valuable in the management of these complex and severe cervical dystonia.

ANCA negative eosinophilic granulomatosis with polyangiitis: sometimes it really IS vasculitis.

A 21-year-old woman, with a background of asthma, presented to medical admissions ward, with diarrhoea and vomiting; the clinical picture during her admission evolved to include acute shortness of breath, seizures, unsteadiness, low mood and apathy. Investigations revealed pericardial, pleural effusions, myocardial infiltration and vasculitic lesions in the brain. Although serological tests for autoantibodies were negative, an eventual diagnosis of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, was performed based on the clinical picture. The multiorgan involvement meant that the initial diagnosis and effective management required multidisciplinary input from cardiology, neurology, rheumatology, psychiatry, immunology and occupational and physiotherapy.

Neuroborreliosis: the Guillain-Barré mimicker.

A 34-year-old woman presented to the medical admissions unit with progressive ascending weakness of her limbs and areflexia. Diagnosis of Guillain-Barré syndrome was suspected and she was started on intravenous immunoglobulins. Owing to a poor initial response, further exploratory history revealed travel to the New Forest and a possible tick bite; subsequent investigations confirmed positive serology for antibodies against Borrelia. The patient's weakness improved with intravenous ceftriaxone for neuroborreliosis, a manifestation of Lyme disease. With inpatient neurorehabilitation, she made good recovery and was able to mobilise with a stick from being completely bed bound 6 weeks after completion of her antibiotics.

Proximal myopathy: diagnostic approach and initial management.

Proximal myopathy presents as symmetrical weakness of proximal upper and/or lower limbs. There is a broad range of underlying causes including drugs, alcohol, thyroid disease, osteomalacia, idiopathic inflammatory myopathies (IIM), hereditary myopathies, malignancy, infections and sarcoidosis. Clinical assessment should aim to distinguish proximal myopathy from other conditions that can present similarly, identify patients who need prompt attention, like those with cardiac, respiratory or pharyngeal muscle involvement, and determine underlying cause of myopathy. Initial evaluation should include simple tests, like creatine kinase, thyroid function and (25)OH vitamin D levels, but further evaluation including neurophysiological studies, muscle imaging and muscle biopsy should be considered for patients in whom no toxic, metabolic or endocrine cause is found, and in those with clinical features suggestive of inflammatory or hereditary myopathy. Additionally, screening for malignancy and testing for anti-Jo1 antibody is indicated for selected patients with IIM. Management depends on underlying cause, and includes measures, such as removal of offending agent, correction of endocrine or metabolic problem, corticosteroids and immunosuppressive therapy for IIM, and physical therapy, rehabilitation and genetic counselling for muscular dystrophies.

Multiway array decomposition analysis of EEGs in Alzheimer's disease.

Methods for the extraction of features from physiological datasets are growing needs as clinical investigations of Alzheimer's disease (AD) in large and heterogeneous population increase. General tools allowing diagnostic regardless of recording sites, such as different hospitals, are essential and if combined to inexpensive non-invasive methods could critically improve mass screening of subjects with AD. In this study, we applied two state of the art multiway array decomposition (MAD) methods to extract unique features from electroencephalograms (EEGs) of AD patients obtained from multiple sites. In comparison to MAD, spectral-spatial average filter (SSFs) of control and AD subjects were used as well as a common blind source separation method, algorithm for multiple unknown signal extraction (AMUSE), and singular value decomposition (SVD) coupled to tensor unfolding. We trained a feed-forward multilayer perceptron (MLP) to validate and optimize AD classification from two independent databases. Using a third EEG dataset, we demonstrated that features extracted from MAD outperformed features obtained from SSFs AMUSE in terms of root mean squared error (RMSE) and reaching up to 100% of accuracy in test condition. We propose that MAD maybe a useful tool to extract features for AD diagnosis offering great generalization across multi-site databases and opening doors to the discovery of new characterization of the disease.

Characterization of EEGs in Alzheimer's disease using information theoretic methods.

The number of people that now go on to develop Alzheimer's disease (AD) and other types of dementia is rapidly rising. For maximum benefits from new treatments, the disease should be diagnosed as early as possible, but this is difficult with current clinical criteria. Potentially, the EEG can serve as an objective, first line of decision support tool to improve diagnosis. It is non-invasive, widely available, low-cost and could be carried out rapidly in the high-risk age group that will develop AD. Changes in the EEG due to the dementing process could be quantified as an index or marker. In this paper, we investigate two information theoretic methods (Tsallis entropy and universal compression algorithm) as a way to generate potentially robust markers from the EEG. The hypothesis is that the information theoretic makers for AD are significantly different to those of normal subjects. An attraction of the information theoretic approach is that, unlike most existing methods, there may be a natural link between the underlying ideas of information theoretic methods, the physiology of AD and its impact on brain functions. Data compression has not been investigated as a means of generating EEG markers before and is attractive because it does not require a priori knowledge of the source model. In this paper, we focus on the LZW algorithm because of its sound theoretical foundation. We used the LZW algorithm and Tsallis model to compute the markers (compression ratios and normalized entropies, respectively) from two EEG datasets.

Development and assessment of methods for detecting dementia using the human electroencephalogram.

This paper makes an outline case for the need for a low-cost, easy to administer method for detecting dementia within the growing at risk population. It proposes two methods for electroencephalogram (EEG) analysis for detecting dementia that could fulfil such a need. The paper describes a fractal dimension-based method for analyzing the EEG waveforms of subjects with dementia and reports on an assessment which demonstrates that an appropriate fractal dimension measure could achieve 67% sensitivity to probable Alzheimer's disease (as suggested by clinical psychometric testing and EEG findings) with a specificity of 99.9%. An alternative method based on the probability density function of the zero-crossing intervals is shown to achieve 78% sensitivity to probable Alzheimer's disease and an estimated sensitivity to probable Vascular (or mixed) dementia of 35% (as suggested by clinical psychometric testing and EEG findings) with a specificity of 99.9%. This compares well with other studies, reported by the American Academy of Neurology, which typically provide a sensitivity of 81% and specificity of 70%. The EEG recordings used to assess these methods included artefacts and had no a priori selection of elements "suitable for analysis." This approach gives a good prediction of the usefulness of the methods, as they would be used in practice. A total of 39 patients (30 probable Alzheimer's Disease, six Vascular Dementia and three mixed dementia) and 42 healthy volunteers were involved in the study. However, although results from the preliminary evaluation of the methods are promising, there is a need for a more extensive study to validate the methods using EEGs from a larger and more varied patient cohorts with neuroimaging results, to exclude other causes and cognitive scores to correlate results with severity of cognitive status.

The role of ipsilateral premotor cortex in hand movement after stroke.

Movement of an affected hand after stroke is associated with increased activation of ipsilateral motor cortical areas, suggesting that these motor areas in the undamaged hemisphere may adaptively compensate for damaged or disconnected regions. However, this adaptive compensation has not yet been demonstrated directly. Here we used transcranial magnetic stimulation (TMS) to interfere transiently with processing in the ipsilateral primary motor or dorsal premotor cortex (PMd) during finger movements. TMS had a greater effect on patients than controls in a manner that depended on the site, hemisphere, and time of stimulation. In patients with right hemiparesis (but not in healthy controls), TMS applied to PMd early (100 ms) after the cue to move slowed simple reaction-time finger movements by 12% compared with controls. The relative slowing of movements with ipsilateral PMd stimulation in patients correlated with the degree of motor impairment, suggesting that functional recruitment of ipsilateral motor areas was greatest in the more impaired patients. We also used functional magnetic resonance imaging to monitor brain activity in these subjects as they performed the same movements. Slowing of reaction time after premotor cortex TMS in the patients correlated inversely with the relative hemispheric lateralization of functional magnetic resonance imaging activation in PMd. This inverse correlation suggests that the increased activation in ipsilateral cortical motor areas during movements of a paretic hand, shown in this and previous functional imaging studies, represents a functionally relevant, adaptive response to the associated brain injury.