International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions.

The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017-2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.

Epilepsy surgery in Africa: state of the art and challenges.

OBJECTIVE: Epilepsy is an important public health problem representing 0.6% of the global burden of disease that particularly impacts people living in the lowest income countries where epilepsy incidence may be 10 fold more than in the developed world. The battery of treatments designed to counteract the clinical manifestations of this disease are various and range from a wide spectrum of antiseizure medicationand specific diets, to surgical techniques for resection of the epileptogenic focus. The aim of our study was to describe the State of the art of Epilepsy Surgery (ES) in Africa and examine ways to deal with the high surgical treatment gap. METHODOLOGY: In an observational study, we prospectively disseminated questionnaires via email or directly administered to main epileptologists and neurologists involved in epilepsy care, in key African countries. We also conducted a literature search using PubMed, Google scholar on ES in all the African countries. RESULTS: We received responses from the majority of African countries, which allowed us to identify 3 levels of care for ES in African countries, a first level that uses ES with invasive presurgical evaluation, a second level that uses ES but without invasive presurgical evaluation, and a third level that does not use ES, and we summarized these results on a map. DISCUSSION: This paper studied the availability of ES as a treatment modality in several African countries. We aimed to establish optimal pathways for initiating ES with noninvasive Electroencephalography and readily available investigations. This could be achieved through collaboration with epilepsy programs in developed countries directly or by using telemedicine.

Atypical presentations of acute disseminated encephalomyelitis (ADEM) in HIV infection.

Acute disseminated encephalomyelitis is a monophasic demyelinating disorder of the central nervous system associated with various viral infections including HIV infection. We present the findings of seven HIV-infected patients with mild to moderate immunosuppression presenting with atypical features. Four patients had a multiphasic course; three patients had tumefactive lesions, and two patients had corpus callosum lesions. Two patients with the multiphasic course also had tumefactive lesions. Their clinical and radiological findings are presented. Despite the few cases, we propose that the dysimmune process lying between marked immunosuppression (CD4 < 200 cells/µL) and normal CD4 counts (CD4 > 500 cells/µL) might be responsible for these atypical presentations.

The burden of suspected strokes in uMgungundlovu - Can biomarkers aid prognostication?

Background: The burden of stroke is increasing worldwide. The hierarchical healthcare referral system in South Africa (SA) poses unique challenges to clinicians when caring for people with suspected strokes (PsS). To improve health outcomes, novel strategies are required to provide adequate care, including prognostication, in SA. Aim: To determine the subjective burden of and challenges posed by suspected stroke cases and the potential usefulness of biomarkers in prognostication. Setting: This study was conducted in the uMgungundlovu Health District (UHD), KwaZulu-Natal, SA. Methods: An online questionnaire was distributed to doctors within the UHD. Demographic data and answers to a series of 5-point-Likert-type statements were collected. Results: Seventy-seven responses were analysed. A third of doctors worked in primary healthcare facilities (PHCare) and saw ≥ 2.15 suspected strokes-per-doctor-per-week, compared to ≥ 1.38 seen by doctors working in higher levels of healthcare. Neuroimaging was relied upon by > 85% of doctors, with nearly half of PHCare doctors having to refer patients to facilities 5 km - 20 km away, with resultant delays. Knowledge about prognostic biomarkers in strokes was poor, yet most doctors believed that a biomarker would assist in the prognostication process and they would use it routinely. Conclusion: Doctors in this study faced a significant burden of strokes and rely on neuroimaging to guide their management; however, many challenges exist in obtaining such imaging, especially in the PHCare setting. The need for prognostic biomarkers was clear. Contribution: This research lays the platform for further studies to investigate prognostic biomarkers in stroke in our clinical setting.

Circulating Biomarkers in Long-Term Stroke Prognosis: A Scoping Review Focusing on the South African Setting.

Cerebrovascular disease, including both ischaemic and haemorrhagic strokes, remains one of the highest causes of global morbidity and mortality. Developing nations, such as South Africa (SA), are affected disproportionately. Early identification of stroke patients at risk of poor clinical prognosis may result in improved outcomes. In addition to conventional neuroimaging, the role of predictive biomarkers has been shown to be important. Little data exist on their applicability within SA. This scoping review aimed to evaluate the currently available data pertaining to blood biomarkers that aid in the long-term prognostication of patients following stroke and its potential application in the South African setting. This scoping review followed a 6-stage process to identify and critically review currently available literature pertaining to prognostic biomarkers in stroke. An initial 1191 articles were identified and, following rigorous review, 41 articles were included for the purposes of the scoping review. A number of potential biomarkers were identified and grouped according to the function or origin of the marker. Although most biomarkers showed great prognostic potential, the cost and availability will likely limit their application within SA. The burden of stroke is increasing worldwide and appears to be affecting developing countries disproportionately. Access to neuroradiological services is not readily available in all settings and the addition of biomarkers to assist in the long-term prognostication of patients following a stroke can be of great clinical value. The cost and availability of many of the reviewed biomarkers will likely hinder their use in the South African setting.

Myasthenic Crisis Precipitated by Raoultella Planticola in an Immunocompromised Host.

We present a case of a 39-year-old male patient who was previously diagnosed with myasthenia gravis. He presented in a myasthenic crisis secondary to a lower respiratory tract infection, with the implicated organism being Raoultella planticola. He was referred to the intensive care unit (ICU) and required ventilatory support due to respiratory insufficiency. Early broad-spectrum antibiotics for a suspected bacterial infection were provided in combination with management specific to the myasthenic crisis. The patient made a full recovery and has displayed a good clinical response. This case report explores his presentation and aims to provide further literature on the incidence and description of R. planticola.

The "Hot Cross Bun Sign" in Spinocerebellar Ataxia Types 2 and 7-Case Reports and Review of Literature.

Background: The "hot cross bun" sign is a cruciform hyperintensity is seen on T2 weighted imaging within the pons. The sign is considered to be pathognomic for Multiple system atrophy type C. The clinical and radiological features of Multiple system atrophy type C overlap with the autosomal dominant inherited ataxias. We present a case series of 3 African patients with genetically proven Spinocerebellar Ataxia presenting with the Hot cross bun sign and a scoping review of similar studies. Cases: We described the phenotypic and radiological presentation of genetically confirmed SCA-2 in two, and SCA-7 in one patient, with the "hot cross bun" sign. Literature Review: We performed a scoping review on the Hot Cross Bun Sign.A total of 66 articles were retrieved. We describe the diverse aetiologies of the sign and associated phenotypic and radiological features. We review the Spinocerebellar Ataxias described with a Hot cross bun sign and make comparisons to Multiple System Atrophy Type C [Ref. 1,2]. Conclusions: To our knowledge this is the first description of an African cohort presenting with the Hot Cross Bun Sign. We expand the differential diagnosis of the Hot Cross Bun Sign.