Analysis of HIV-related mortality data in a tertiary South African neurology unit, 2006 - 2012

BACKGROUND. South Africa (SA) has a high prevalence of HIV infection with almost 11% of the population aged >2 years living with HIV. At the Steve Biko Academic Hospital, Pretoria, the Neurology Department has seen a steady increase in HIV-related neurology patients. OBJECTIVE. To evaluate the mortality data of this unit as it relates to HIV infection. METHODS. The study was a retrospective analysis of records. Patient mortality statistics for 2006, 2008, 2010 and 2012 were analysed regarding cause of death, sex, age and HIV status. RESULTS. During 2006, 85 patients died: 33% were HIV-positive, 13% were HIV-negative and 54% had not tested for HIV. By 2010, these figures were 50%, 22% and 28%, respectively, changing little in 2012 (48%, 28% and 24%, respectively). Causes of death in the HIV-positive group were meningitis in 58% - with tuberculous meningitis the most common aetiology - followed by strokes (14%), space-occupying lesions (8%) and status epilepticus (7%). Among HIV-positive patients aged 20 - 30 years, a larger proportion of young women died than men. In the combined untested and HIV-negative group, strokes accounted for the vast majority of deaths. CONCLUSION. Neurological complications of HIV remain common in SA and contribute significantly to the overall mortality in our tertiary neurology unit, with TB posing a serious threat. A strong corps of clinical neurologists with training in infective neurology is needed urgently in the coming years to care for this growing number of patients.http://www.sajhivmed.org.zaam2013ay201

Guest editorial : Autoimmune encephalitis

Since the identification of anti-N-methyl-D-aspartate (NMDA) receptor antibodies about 15 years ago, many patients with rapidly progressing psychiatric symptoms, abnormal movements, seizures or unexplained coma, have been diagnosed with autoimmune encephalitis (AE). The symptom onset is often unspecific and might mimic psychiatric disease, but the later course is frequently characterized by severe disease, often requiring intensive care. Clinical and immunological criteria are helpful in identifying the patients, but no biomarkers exist to guide the clinician in therapy or predict outcome. While persons of all ages can be affected by AE, some types of AE affect more children and young adults and are more prevalent in women. This review will focus on encephalitides associated with neuronal cell-surface or synaptic antibodies, which can result in characteristic syndromes, and are often recognizable on clinical grounds. AE subtypes associated with antibodies against extracellular epitopes can occur with or without tumours. Because the antibodies bind and alter the function of the antigen, the effects are often reversible if immunotherapy is initiated, and the prognosis is favourable in most instances. The first part of this series will introduce the topic, provide an overview of current neuronal surface antibodies and how they present, describe the most common subtype, anti-NMDA receptor encephalitis, and discuss the difficulties in recognizing patients with underlying AE amongst patients with new onset psychiatric disorders.https://samajournals.co.za/index.php/samj/indexhj2024NeurologyNon

Autoimmune encephalitis : epidemiology, pathophysiology and clinical spectrum (Part 1)

Since the identification of anti-N-methyl-D-aspartate (NMDA) receptor antibodies about 15 years ago, many patients with rapidly progressing psychiatric symptoms, abnormal movements, seizures or unexplained coma have been diagnosed with autoimmune encephalitis (AE). The symptom onset is often unspecific, and might mimic psychiatric disease, but the later course is frequently characterised by severe disease, often requiring intensive care. Clinical and immunological criteria are helpful in identifying the patients, but no biomarkers exist to guide the clinician in therapy or predict outcome. While persons of all ages can be affected by AE, some types of AE affect more children and young adults and are more prevalent in women. This review focuses on encephalitides associated with neuronal cell-surface or synaptic antibodies, which can result in characteristic syndromes, and are often recognisable on clinical grounds. AE subtypes associated with antibodies against extracellular epitopes can occur with or without tumours. Because the antibodies bind and alter the function of the antigen, the effects are often reversible if immunotherapy is initiated, and the prognosis is favourable in most instances. The first part of this series introduces the topic, provides an overview of currently known neuronal surface antibodies and how they present, describes the most common subtype anti-NMDA receptor encephalitis, and discusses the difficulties in recognising patients with underlying AE among patients with new-onset psychiatric disorders.https://samajournals.co.za/index.php/samj/indexam2024NeurologySDG-03:Good heatlh and well-bein

Basedow paraplegia: A possible misnomer

Thyrotoxic myopathy frequently occurs in clinical practice; however, the association of hyperthyroidism with a flaccid, areflexic paraplegia, so-called Basedow paraplegia, appears to represent a controversial and doubtful entity. An 18-year-old female with undiagnosed and untreated Graves’ disease presented with acute onset of global weakness predominantly in the lower limbs, but also affecting the upper limbs. The weakness was accompanied by hypotonia and areflexia. Clinically, the patient had a goitre and signs of thyroid ocular disease. Laboratory testing confirmed the presence of hyperthyroidism, and thyroid-stimulating hormone receptor antibodies were positive. The cerebrospinal fluid protein level was raised. The electroneuronographic and needle examinations were compatible with a clear denervation process, such as acute motor axonal neuropathy, a variant of Guillain-Barré syndrome. Intravenous immunoglobulin therapy, carbimazole and propranolol were administered. The occurrence of hyperthyroidism with a flaccid, areflexic paraplegia appears to represent more of a fortuitous than a causative association. It is important to consider and treat other causes, such as acute idiopathic polyneuritis.http://www.samj.org.zaam201

Developing and testing a South African Brief Cognitive Score in literate and illiterate people of mixed language groups

BACKGROUND: The Folstein mini mental state exam (MMSE) is the most commonly administered assessment of cognitive functioning; however, its utility in illiterate individuals is limited. In South Africa, more than eight million adults are considered functionally illiterate and cognitive evaluation using standard scales is inaccurate. Other countries have developed adapted MMSE scales for their local purposes. AIM: The first aim of this study was to develop a South African Brief Cognitive Score (SA BCS) for use in minimally literate or illiterate individuals. The second aim was to test this SA BCS against the original Folstein MMSE in patients with memory problems. SETTING: The study was conducted in Tshilamba, Tshiombo, Tshifudi, Dzimauli and Pile in Venda as well as Rethabiseng and Zithobeni in Bronkhorstspruit for the illiterate study group, and Steve Biko Academic Hospital for the literate study groups. METHODS: The SA BCS was developed considering our local requirements and substituting questions needing literacy with items that did not. Both the original Folstein MMSE and the SA BCS were administered to groups of literate and illiterate normal individuals. Thereafter, the tests were repeated in groups of literate and illiterate patients with cognitive decline. RESULTS: Firstly, 33 illiterate and 31 literate subjects were assessed. The average original Folstein score was 29.29/30 for the literate subjects, and for the SA BCS 29.80. For the illiterate subjects, the average score for the original Folstein MMSE was 21.24/30 and for the SA BCS 27.45. Kruskall-Wallis equality of population rank test confirmed a significant improvement in the scores of the SA BCS in the illiterate group when compared to the original Folstein (p = 0.0001). In the second part of the study, 20 literate and 20 illiterate patients were assessed as before. In the literate group, the average original Folstein MMSE score was 20.5, while the average score for the South African BCS was 22.5. In the illiterate group, the average Folstein MMSE was 18.9; and the average score in the South African BCS was 22.8. The Kruskal-Wallis equality of population rank test showed a significant difference (p = 0.008) between the scores of the illiterate versus literate patients when the Folstein MMSE was used to assess cognition. With the SA BCS, no significant difference was found between the groups (p = 0.79). CONCLUSION: The SA BCS appears to have potential to be a more reliable scale when assessing cognition in illiterate or minimally literate subjects when compared to the original Folstein MMSE.http://www.sajpsychiatry.orgNeurolog

Successful treatment of disabling paroxysmal nonkinesigenic dyskinesia with deep brain stimulation of the globus pallidus internus

Paroxysmal nonkinesigenic dyskinesia (PNKD) causes episodes of treatment-resistant involuntary movements. Previous case reports showed effective treatment of PNKD with deep brain stimulation (DBS). We report 2 patients in whom DBS was highly successful when other treatment modalities had failed. METHODS : Two patients aged 34 and 24 years with a longstanding history of PNKD were treated with globus pallidus internus (GPi) DBS. Motor effects were monitored and followed up postoperatively and again at 6 months after surgery. RESULTS : Both patients responded very well to GPi DBS with complete suppression of dyskinesia after surgery in 1 patient and in the second after an additional adjustment of stimulation. CONCLUSION : GPi DBS might be an effective alternative treatment modality for PNKDhttp://www.karger.com/Journal/Home/224132hb201

AIDS-related progressive multifocal leukoencephalopathy (PML) : a retrospective study from Pretoria, South Africa

INTRODUCTION AND OBJECTIVES: Progressive multifocal leukoencephalopathy (PML), caused by the John Cunningham (JC) virus, results from lytic infection of predominantly oligodendrocytes. Following the HIV pandemic, the incidence of PML has risen sharply, but has rarely been reported in Africa. An increasing number of PML cases were seen recently in a tertiary South African hospital, and this study describes their clinical and radiological features. METHODS Patients with positive cerebrospinal fluid (CSF) JC virus confirmed by real-time polymerase chain reaction (PCR) were retrospectively identified from January 2008 to June 2012. Adults seen at Neurology with PML were identified, and clinical features, laboratory findings and imaging studies were analysed. RESULTS: Of 121 specimens, 19 were positive; records of 17 patients were available (ages 27 - 64; CD4 counts 11 - 328 x106/μl); clinical manifestations included focal weakness (47%), impaired co-ordination (41%), and speech disturbances (12%), and CSF analysis showed high protein in 76%, and pleocytosis in 35%. Fifteen patients had CT brain scans, showing white matter involvement in 12; MRI studies in 13 patients showed typical PML lesions. CONCLUSION: This report is the first case series of patients with PML from a South African neurology unit, emphasising the fact that PML occurs commonly in South African patients with HIV infection.http://www.samj.org.zaam2014ay201

Cryptococcal meningitis in a tertiary hospital in Pretoria, mortality and risk factors – a retrospective cohort study

AIM : This retrospective cohort study analyzes the impact of possible risk factors on the survival chance of patients with cryptococcal meningitis. These factors include the patient's socio-economic background, age, gender, presenting symptoms, comorbidities, laboratory findings and, in particular, non-adherence versus adherence to therapy. METHODS : Data were collected from all adult patients admitted to Kalafong Hospital with laboratory confirmed cryptococcal meningitis over a period of 24 months. We analyzed the data by the presentation of descriptive summary statistics, logistic regression was used to assess factors which showed association between outcome of measure and factor. Furthermore, multivariable logistic regression analysis using all the factors that showed significant association in the cross tabulation was applied to determine which factors had an impact on the patients' mortality risk. RESULTS : A total of 87 patients were identified. All except one were HIV-positive, of which 55.2% were antiretroviral therapy naïve. A history of previous tuberculosis was given by 25 patients (28.7%) and 49 (56.3%) were on tuberculosis treatment at admission or started during their hospital stay. In-hospital mortality was 31%. Statistical analysis showed that antiretroviral therapy naïve patients had 9.9 (CI 95% 1.2-81.2, p < 0.0032) times greater odds of dying compared to those on antiretroviral therapy, with 17 from 48 patients (35.4%) dying compared with 1 out of 21 patients (4.8%) on treatment. Defaulters had 14.7 (CI 95% 1.6-131.6, p < 0.016) times greater odds of dying, with 9 from 18 patients dying (50%), compared to the non-defaulters. In addition, patients who presented with nausea and vomiting had a 6.3 (95% CI 1.7-23.1, p < 0.005) times greater odds of dying (18/47, 38.3%); this remained significant when adjusted for antiretroviral therapy naïve patients and defaulters. CONCLUSION : Cryptococcal meningitis is still a common opportunistic infection in people living with HIV/AIDS resulting in hospitalization and a high mortality. Defaulting antiretroviral therapy and presentation with nausea and vomiting were associated with a significantly increased mortality risk.http://www.uk.sagepub.com/journals/Journal202185hb2017Neurolog

Comparison of HTLV-associated myelopathy (HAM) in HIV-positive and HIV-negative patients at a tertiary South African hospital

Background. HTLV-1 associated myelopathy (HAM), or tropical spastic paraparesis, is caused by a retrovirus, the human T-cell lymphotropic virus (HTLV). Although patients with HAM and HIV infection have been described, to our knowledge no direct comparison has been made between patients who are HIV positive and suffering from HAM (HHAM) v. those who are HIV negative and suffering from HAM. Aim. We aimed to compare clinical and radiological findings in HIV-positive and -negative patients with HAM. Methods. Adult patients who presented to the Neurology Unit at the Steve Biko Academic Hospital from May 2005 to June 2012 with a progressive myelopathy and HTLV seropositivity were retrospectively identified and their clinical and radiological data were collected and reviewed. Results. 21 patients with HAM were identified, of whom 9 were HIV-positive and 11 HIV-negative. One patient, whose HIV status had not been established, was not included in the study. Although the trend did not reach statistical significance, co-infected patients tended to present at an earlier age (HHAM 6/9 (66%

Late-onset efavirenz toxicity : a descriptive study from Pretoria, South Africa

DATA AVAILABILITY : The data that support the findings of this study are available from the corresponding author, J.H., upon reasonable request.BACKGROUND : The neuropsychiatric side effects of efavirenz occur mainly early during treatment and are usually mild. A lesser-known and serious complication is late-onset efavirenz toxicity causing ataxia and encephalopathy. Data regarding this condition are limited. OBJECTIVES : We describe the clinical picture of late-onset efavirenz toxicity, investigate co-morbidities and report outcomes. METHOD : This descriptive study of all patients with late-onset efavirenz toxicity was conducted over three years at Kalafong Provincial Tertiary Hospital, Pretoria, South Africa. RESULTS : Forty consecutive patients were identified. Mean age was 42.1 years, three patients (7.5%) were male and the mean efavirenz level was 49.0 μg/mL (standard deviation [s.d.]: 24.8). Cerebellar ataxia (82.5%) and encephalopathy (47.5%) were the most common presenting features (40.0% had both); four patients presented with psychosis. Presence of encephalopathy and/or cerebellar ataxia was associated with higher efavirenz levels compared with psychosis (52.1 μg/mL, s.d.: 24.1 vs 25.0 μg/mL, s.d.: 17.1). In most patients, symptoms resolved, but four patients (10.0%) died, and one patient remained ataxic. CONCLUSION : Late-onset efavirenz toxicity typically presented with ataxia and encephalopathy, but psychosis can be the presenting feature. The outcome after withdrawal was good, but the mortality of 10.0% is concerning. Recent changes in guidelines favour dolutegravir, but many patients remain on efavirenz, and awareness of the condition is vital. WHAT THIS STUDY ADDS : This large, single-centre study contributes to the limited data of HIV-positive patients with late-onset efavirenz toxicity and emphasises its ongoing relevance in clinical practice.http://www.sajhivmed.org.zaam2024Internal MedicineNeurologySDG-03:Good heatlh and well-bein