Costs of adult functional neurological disorders at a tertiary hospital in central South Africa.

Background: Functional neurological disorders (FND) lead to increased care requirements and costs, negatively impacting healthcare budgets. Healthcare expenditure in FND has escalated beyond other neurologic disorders during the past decade. Objectives: To assess inpatient costs in adults admitted to the neurology ward at Universitas Academic Hospital (UAH) in central South Africa. Methods: A retrospective observational study with a comparative component was conducted on patients admitted during 2018 and 2019. All FND cases (n = 29) and a systematic sample of other neurological disorders were included in the comparison group (n = 29). Data were obtained from the Meditech billing system and clinical records. Results: FND patients accounted for 5.5% of 530 admissions in the neurology ward during the study period. No significant differences regarding daily median cost, age categories, gender or medical comorbidity were observed between FND and the comparison group. However, the length of stay was significantly shorter for the FND patients (median of four versus eight days), translating to approximately half the total costs of patients admitted for other neurological disorders. Conclusion: The daily median cost was similar for FND and other neurology-related admissions. The lower overall inpatient costs for FND patients were only related to significantly shorter durations of stay, which may reflect new diagnostic approaches resulting from changes in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) diagnostic criteria. The prevalence of FND was similar to those reported in previous studies conducted at neurology clinics. Contribution: The study contributes towards better understanding the prevalence and cost of FND in local neurology inpatient care settings.

Restless legs syndrome in chronic renal failure patients on dialysis.

Background: Restless legs syndrome (RLS) occurs in patients with chronic renal failure (CRF). Objectives: To determine the prevalence and morbidity of RLS in CRF patients on dialysis. Methods: This cross-sectional questionnaire-based study included 100 dialysis patients (50 on haemodialysis [HD]; 50 on peritoneal dialysis [PD]). A focused lower limb examination was done. Patients were classified with RLS when reporting uncomfortable feelings in their legs that improved with movement and worsened when resting and at night. Results: Gender distribution was equal. The median age was 43 (19-67) years. Six patients (HD and PD n=3 each) had international criteria-confirmed RLS. Twenty-four patients reported symptoms suggestive of RLS. Fourteen and 16 patients with RLS symptoms were on HD and PD, respectively. Sleep disturbances occurred in 43.3% (n=13) of patients with RLS symptoms, compared to 20.0% (n=20) of the large cohort. Sleep disturbances, peripheral sensory loss, chronic disease-related anaemia, increased urea and decreased albumin levels were more common among patients with RLS symptoms. Conclusion: RLS symptoms occurred in 30.0% of the entire cohort, although only 6.0% met the international criteria. The type of dialysis had no impact on the incidence of symptoms. Identifying RLS in patients with CRF on dialysis will allow for early intervention.

Does antiretroviral therapy alter the course of Parkinson's disease in people living with HIV?

South Africa has the world's largest antiretroviral programme which has resulted in an increase in life expectancy in persons living with HIV. Parkinson's disease (PD) is an age-related neurodegenerative disorder. No data has been published in this setting with regards to the interaction between PD and people infected with HIV. This was a retrospective study which matched two HIV non-infected PD patients to one HIV-infected patient with PD. Patients with secondary causes of Parkinsonism were excluded. Demographic, clinical and laboratory data were extracted from the charts. Hoehn and Yahr scale was used to assess PD severity. Twenty PD patients were recruited from 1 January 2008 to 31 October 2020 and were diagnosed with HIV for a median of 72 months. The median age at onset of PD was 52 years. All patients were on antiretroviral therapy. There were no statistically significant differences in the levodopa equivalent daily dose, clinical phenotype, impulse control disorders (ICDs) and frequency of a positive family history between the two groups. HIV-infected patients had a higher frequency of dopamine dysregulation syndrome. At the end of follow-up, 3 (15%) PLH-PD had moderate to severe PD compared to 16 (40%) of PD controls. The OR of having moderate to severe PD in HIV non-infected PD patients was 4. Persons living with HIV and Parkinson's disease present with PD symptoms at a younger age, progress slower to a severe stage and respond well to dopaminergic replacement therapy.

Are interpersonal communication skills adequately taught at postgraduate specialist level in South Africa? The neurology experience.

BACKGROUND: Interpersonal communication skills by clinicians with patients, carers, fellow health professionals and legal professionals carry many unique challenges in practice. Whilst undergraduate training in communication helps with generic information receiving and information giving, uncomfortable and demanding speciality-specific issues in the various medical specialities are not covered during under- and postgraduate training. METHODS: The aim of this study was to determine the self-perceived competence of neurology registrars and neurologists in interpersonal communication and the need for such assessment in college exit exams. We undertook a quantitative, descriptive, cross-sectional survey by using self-administered printed questionnaires and the EvaSYS online system. Neurology registrars in training from the seven training centres in South Africa and neurologists based at the training centres and in the private sector were recruited. RESULTS: We received a 62.9% response rate. One hundred and twenty-nine participants were recruited comprising 42 neurology registrars and 87 neurologists. Registrars were more commonly female, more likely to be multilingual and less likely to use translators. Undergraduate training in communication was considered insufficient, 42.9% and 39.1% for registrars and specialists respectively, and was also considered not relevant to address speciality-specific issues encountered in practice. Most training received has been by observation of others and on-the-job training. Both groups felt strongly that postgraduate training in interpersonal communication was important (registrars 95.2%, specialists 91.9%), especially when dealing with issues of death and dying, disclosing medical errors and dealing with the legal profession. CONCLUSION: Postgraduate training of interpersonal communication as required of neurology registrars and neurologists was considered insufficient. Most training has been by observation of others or experiential by trial and error. Assessment of interpersonal communication at board exit exams will drive postgraduate training and importantly will embrace the AfriMEDS framework developed to produce the holistic doctor in South Africa.

Fungal Burden and Raised Intracranial Pressure Are Independently Associated With Visual Loss in Human Immunodeficiency Virus-Associated Cryptococcal Meningitis.

Among 472 patients with human immunodeficiency virus-associated cryptococcal meningitis, 16% had severe visual loss at presentation, and 46% of these were 4-week survivors and remained severely impaired. Baseline cerebrospinal fluid opening pressure ≥40 cmH2O (adjusted odds ratio [aOR], 2.56; 95% confidence interval [CI], 1.36-4.83; P = .02) and fungal burden >6.0 log10 colonies/mL (aOR, 3.01; 95% CI, 1.58-5.7; P = .003) were independently associated with severe visual loss.

HIV-associated cavernous sinus disease.

INTRODUCTION: The underlying diagnosis of cavernous sinus disease is difficult to confirm in HIV-coinfected patients owing to the lack of histological confirmation. In this retrospective case series, we highlight the challenges in confirming the diagnosis and managing these patients. RESULTS: The clinical, laboratory and radiological data of 23 HIV-infected patients with cavernous sinus disease were analysed. The mean age of patients was 38 years. The mean CD4+ count was 390 cells/µL. Clinically, patients presented with unilateral disease (65%), headache (48%), diplopia (30%) and blurred vision (30%). Third (65%) and sixth (57%) nerve palsies in isolation and combination (39%) were most common. Isolated fourth nerve palsy did not occur. Tuberculosis (17%) was the most commonly identified disorder followed by high-grade B-cell lymphoma (13%), meningioma (13%), metastatic carcinoma (13%) and neurosyphilis (7%). In 22% of the patients, there was no confirmatory evidence for a diagnosis. The patients were either treated empirically for tuberculosis or improved spontaneously when antiretroviral therapy was started. Cerebrospinal fluid was helpful in 4/13 (31%) of patients where it was not contraindicated. Only 3/23 (13%) of the patients had a biopsy of the cavernous sinus mass. The outcomes varied, and follow-up was lacking in the majority of patients. CONCLUSION: In HIV-infected patients, histological confirmation of cavernous sinus pathology is not readily available for various reasons. In resource-limited settings, one should first actively search for extracranial evidence of tuberculosis, lymphoma, syphilis and primary malignancy and manage appropriately. Only if such evidence is lacking should a referral for biopsy be considered.

Prevalence and outcomes of central venous catheter-related bacteraemia in HIV-infected versus non-HIV-infected patients undergoing haemodialysis treatment for end-stage kidney disease.

BACKGROUND: Central venous catheter (CVC) haemodialysis (HD) to implement renal replacement therapy is the preferred choice in the urgent setting. Unfortunately, CVC placement is associated with multiple complications including nosocomial bloodstream infections. There is a paucity of data on the prevalence and pattern of pathogenic organisms in haemodialysed HIV-infected versus non-HIV-infected patients with end-stage kidney disease. METHOD AND RESULTS: We undertook a retrospective study of 228 patients who were dialysed using a CVC at a tertiary referral hospital in KwaZulu-Natal, South Africa. Seventy-eight patients (34.2%) complicated with bacteraemia and sepsis requiring antibiotics. Removal of the catheter was necessary in 58 patients (74.0%). The most common organisms isolated were Staphylococcus aureus (30.8%), Staphylococcus epidermidis (24.4%) and Klebsiella pneumoniae (15.4%). There was no statistically significant difference between HIV-infected and non-infected patients with regards to infection rate, time interval from insertion of CVC to infection and final outcome. However, HIV-infected patients took longer to recover; 54.3% of non-infected patients versus 10.3% HIV-infected patients had their sepsis controlled within one week. Acidosis, hypotension, line malfunction and line discharge were infrequent signs of sepsis. Fever, rigors and raised white cell count occurred in over 80.0% of patients. CONCLUSION: The infection rate in CVC HD is not more frequent in HIV-infected patients, provided that CD4+ count is ≥ 200 cells/µL and the patient is virologically suppressed. Outcomes following intravenous antibiotic and removal of the CVC are similar in HIV-infected and non-infected patients but response to treatment is slower in HIV-infected patients. A high index of suspicion is needed in detecting CVC-related bacteraemia.

Intracanalicular Optic Nerve Swelling and Signal Change in Fulminant Untreated Idiopathic Intracranial Hypertension.

The role of the optic canal in the pathogenesis of papilloedema has been under scrutiny recently. Whether a larger canal precedes more severe papilloedema or is the result of bone remodelling from chronically raised pressure across a pressure gradient is not clear. The authors present the magnetic resonance imaging findings of a 29-year-old female with fulminant and untreated idiopathic intracranial hypertension. Imaging showed focal expansion and intrinsic signal changes of the intracanalicular optic nerve. The authors discuss the possibility of either fluid accumulation within the optic nerves from a water hammer effect across blocked optic canals resulting from the steep pressure gradient or opticomalacia (optic nerve softening) from chronic ischaemia.

HIV-associated neurocognitive disorder in a KwaZulu-Natal HIV clinic: A prospective study.

INTRODUCTION: HIV-associated neurocognitive disorder (HAND) is a consequence of HIV infection of the central nervous system. The prevalence ranges between 15% and 60% in different settings. OBJECTIVES: This prospective study determined the prevalence of HAND at a peri-urban HIV clinic in KwaZulu-Natal. Factors associated with HAND were examined, alternate neurocognitive tools were tested against the international HIV dementia scale (IHDS) score and an association between HAND and non-adherence to antiretroviral therapy (ART) was explored. METHODS: Between May 2014 and May 2015, 146 ART-naïve outpatients were assessed for HAND. IHDS score ≤ 10 established a diagnosis of HAND. Functional capacity was assessed using Eastern Cooperative Oncology Group (ECOG) score. Chi-squared test was used to identify risk factors for HAND. The get-up-and-go test (GUGT) and Center for Epidemiological Studies Depression scale - revised (CESD-r) were tested against the IHDS. HIV viral load done six months after initiating ART was used as a surrogate marker for adherence to ART. RESULTS: The prevalence of HAND was 53%. In total, 99.9% of patients with HAND had no functional impairment. Age > 50 years old was associated with HAND (p = 0.003). There was no correlation between the GUGT, CESD-r and the IHDS score. HAND was not associated with non-adherence (p = 0.06). CONCLUSIONS: While the prevalence of HAND is high, it is not associated with functional impairment which suggests that asymptomatic neurocognitive impairment is prevalent. Age > 50 years old is a risk factor for HAND. The GUGT and CESD-r are not useful diagnostic tools for HAND. The relationship between HAND and non-adherence should be further explored.

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.

Visual loss in HIV-associated cryptococcal meningitis: A case series and review of the mechanisms involved.

Permanent visual loss is a devastating yet preventable complication of cryptococcal meningitis. Early and aggressive management of cerebrospinal fluid pressure in conjunction with antifungal therapy is required. Historically, the mechanisms of visual loss in cryptococcal meningitis have included optic neuritis and papilloedema. Hence, the basis of visual loss therapy has been steroid therapy and intracranial pressure lowering without clear guidelines. With the use of high-resolution magnetic resonance imaging of the optic nerve, an additional mechanism has emerged, namely an optic nerve sheath compartment syndrome (ONSCS) caused by severely elevated intracranial pressure and fungal loading in the peri-optic space. An improved understanding of these mechanisms and recognition of the important role played by raised intracranial pressure allows for more targeted treatment measures and better outcomes. In the present case series of 90 HIV co-infected patients with cryptococcal meningitis, we present the clinical and electrophysiological manifestations of Cryptococcus-induced visual loss and review the mechanisms involved.

Bilateral Tonic Pupils as the Initial Manifestation of Sjögren's Syndrome.

Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and idiopathic, but can be caused by orbital disorders and autonomic neuropathies. There are only a few case reports of tonic pupils in patients with Sjögren's syndrome, caused by an autoimmune ciliary ganglionitis. The authors report on two cases with bilateral tonic pupils as the initial manifestation of primary Sjögren's syndrome. Both patients presented with blurred vision, bilateral tonic pupils, and sicca symptoms. The findings suggest that Sjögren's syndrome should be considered in patients presenting with bilateral Adie's tonic pupils.

Pseudotumor Cerebri and Implanon: Is Rapid Weight Gain the Trigger?

The relationship between pseudotumor cerebri and contraceptive drugs is controversial. Its association with Implanon, an implantable single-rod contraceptive containing etonogestrel (a progestogen) has not been reported but is the subject of many medico-legal cases. The authors present two case reports of patients using Implanon and who subsequently developed pseudotumor cerebri. Rapid weight gain rather than direct hormonal influence is probably the trigger. Headaches, visual obscurations, and rapid weight gain in patients using Implanon should alert one to the probable diagnosis of pseudotumor cerebri.

Visual Loss in HIV-Associated Cryptococcal Meningitis: A Case Series and Review of the Mechanisms Involved

Permanent visual loss is a devastating yet preventable complication of cryptococcal meningitis. Early and aggressive management of cerebrospinal fluid pressure in conjunction with antifungal therapy is required. Historically; the mechanisms of visual loss in cryptococcal meningitis have included optic neuritis and papilloedema. Hence; the basis of visual loss therapy has been steroid therapy and intracranial pressure lowering without clear guidelines. With the use of high-resolution magnetic resonance imaging of the optic nerve; an additional mechanism has emerged; namely an optic nerve sheath compartment syndrome (ONSCS) caused by severely elevated intracranial pressure and fungal loading in the peri-optic space. An improved understanding of these mechanisms and recognition of the important role played by raised intracranial pressure allows for more targeted treatment measures and better outcomes. In the present case series of 90 HIV co-infected patients with cryptococcal meningitis; we present the clinical and electrophysiological manifestations of Cryptococcus-induced visual loss and review the mechanisms involved

The Optic Nerve Compartment Syndrome in Cryptococcus-Induced Visual Loss.

Visual loss in cryptococcal meningitis has been postulated to be due to papilloedema and/or optic neuritis. A 28-year-old human immunodeficiency virus (HIV)-positive female presented with visual loss, swollen optic discs, and elevated intracranial pressure due to cryptococcal meningitis. Computerised tomographic cisternography and T2-weighted magnetic resonance imaging showed occlusion of the peri-optic subarachnoid space and its reopening after serial lumbar punctures. Presumably lowering of the intracranial pressure resulted in equalisation of pressure across the pressure gradient created by the fungal block. This case supports a third mechanism of visual loss in cryptococcal meningitis, namely, an optic nerve compartment syndrome, that seems more plausible as the principal mechanism.

Early clinical and subclinical visual evoked potential and Humphrey's visual field defects in cryptococcal meningitis.

Cryptococcal induced visual loss is a devastating complication in survivors of cryptococcal meningitis (CM). Early detection is paramount in prevention and treatment. Subclinical optic nerve dysfunction in CM has not hitherto been investigated by electrophysiological means. We undertook a prospective study on 90 HIV sero-positive patients with culture confirmed CM. Seventy-four patients underwent visual evoked potential (VEP) testing and 47 patients underwent Humphrey's visual field (HVF) testing. Decreased best corrected visual acuity (BCVA) was detected in 46.5% of patients. VEP was abnormal in 51/74 (68.9%) right eyes and 50/74 (67.6%) left eyes. VEP P100 latency was the main abnormality with mean latency values of 118.9 (±16.5) ms and 119.8 (±15.7) ms for the right and left eyes respectively, mildly prolonged when compared to our laboratory references of 104 (±10) ms (p<0.001). Subclinical VEP abnormality was detected in 56.5% of normal eyes and constituted mostly latency abnormality. VEP amplitude was also significantly reduced in this cohort but minimally so in the visually unimpaired. HVF was abnormal in 36/47 (76.6%) right eyes and 32/45 (71.1%) left eyes. The predominant field defect was peripheral constriction with an enlarged blind spot suggesting the greater impact by raised intracranial pressure over that of optic neuritis. Whether this was due to papilloedema or a compartment syndrome is open to further investigation. Subclinical HVF abnormalities were minimal and therefore a poor screening test for early optic nerve dysfunction. However, early optic nerve dysfunction can be detected by testing of VEP P100 latency, which may precede the onset of visual loss in CM.