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/ìl); clinical manifestations included focal weakness (47%), impaired co-ordination (41%), and speech disturbances (12%), and CSF analysis showedhigh 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

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

Severe neuropathy due to inhalant abuse in adolescents from Pretoria

Inhalation of volatile agents, or solvent abuse, is a dangerous pastime practised by many young adolescents in various parts of the world. Benzine, a distillate of petroleum, is a cheap and readily available solvent that is often inhaled or “sniffed” to produce a short-lived feeling of euphoria or disorientation. The aim of this report is to describe four adolescents with severe polyneuropathies secondary to chronic benzine inhalation who were seen at the Steve Biko Academic Hospital in Pretoria’s Neurology Department. Methods and patients: Four adolescent boys aged 15–18 years presented to the Department of Neurology from 2011 to 2013 with progressive weakness and muscle atrophy. Results: On examination all patients showed signs of a severe motor and sensory neuropathy. Two were wheelchair bound at the time of presentation and an initial diagnosis of Guillain-Barré syndrome was considered. Cerebrospinal fluid analysis was normal and electromyography showed severe mixed motor and sensory mainly axonal polyneuropathies in all patients. All investigations for causes of neuropathies were normal, but all patients eventually admitted that they had been abusing benzine by inhaling it for a period of at least six months. The inhalation occurred as a group activity, involving many children. Conclusion: Inhalant abuse appears to be a common practice amongst adolescents from Pretoria. It can lead to a catastrophic polyneuropathy, which should be considered in the differential diagnosis of a young patient presenting with a Guillain-Barré syndrome-type of clinical picture. Awareness amongst schools and drug programmes should be raised to prevent this tragic and highly disabling condition.http://medpharm.tandfonline.com/loi/ojfp20am201

Lifethreatening Listeria meningitis: Need for revision of South African acute bacterial meningitis treatment guidelines

Background. The recent listeriosis outbreak in South Africa (SA) received widespread attention in the media. More than 1 000 laboratory-confirmed cases of listeriosis occurred during an 18-month period, with a case fatality rate of 28%. Acute bacterial meningitis due to listeriosis was extremely rare at Steve Biko Academic Hospital in Pretoria until 2017/18, when we saw two very sick adults with this condition during the listeriosis outbreak.Objectives. To describe the presentation, treatment and outcome of these patients to raise awareness of this potentially fatal but treatable infection that does not respond to empirical third-generation cephalosporins.Case reports. Case 1: A 60-year-old man collapsed at home after being discharged from hospital for treatment of Listeria meningitis. On readmission he had neck stiffness and a depressed level of consciousness with right-sided hemiparesis. A computed tomography (CT) scan of the brain showed possible subarachnoid haemorrhage, but on CT angio- and venograms, extensive thrombosis of the superior sagittal, right transverse and bilateral sigmoid sinuses extending into the right internal jugular vein was noted. Patient 2: A 55-year-old HIV-positive hypertensive man on highly active antiretroviral therapy and antihypertensives visited the emergency department complaining of a new-onset headache. He was discharged on pain medication, but was readmitted the next day with a depressed level of consciousness, neck stiffness, low-grade fever and generalised tonic-clonic convulsions. A lumbar puncture revealed active cerebrospinal fluid that was culture-positive for L. monocytogenes. The patients received ampicillin and gentamicin for 3 weeks; the cerebral venous thrombosis was treated with unfractionated heparin. In both cases, the course of the disease was complicated. The first patient remained confused and suffered from psychotic episodes for 5 weeks. He was finally discharged after 6 weeks in hospital and continued to improve to the extent that he was able to return to work. The second patient needed intubation and ventilation and was treated in the intensive care unit. He improved over the next week and was finally discharged home with no residual neurological sequelae.Conclusions. Our two cases demonstrate that the listeriosis outbreak should change the way we view bacterial meningitis in SA: according to the National Institute for Communicable Diseases, empirical treatment for meningitis should include ampicillin and gentamicin in all adult patients with features of meningitis. There may be a need for an updated meningitis treatment guideline in SA

Severe porphyric neuropathy - importance of screening for porphyria in Guillain-Barré syndrome

The hepatic porphyrias are a group of rare metabolic disorders, each of which is associated with a specific enzymatic alteration in the haem biosynthesis pathway. In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered. The development of acute neurovisceral attacks is related to environmental factors, including medications, hormones and diet. A possible manifestation of a severe attack is rapidly progressing quadriparesis, which may mimic Guillain-Barré syndrome. We present four such cases, highlighting that acute porphyria should be considered in the differential diagnosis of Guillain-Barré syndrome. Three patients presented to Steve Biko Academic Hospital, Pretoria, SA, with progressive quadriparesis, and one to a private hospital with acute abdominal pain followed by rapidly progressive quadriparesis. Two patients had started antiretroviral therapy before the development of symptoms, and one had started antituberculosis therapy. All patients had marked weakness with depressed reflexes, and showed varying degrees of confusion. An initial diagnosis of Guillain-Barré syndrome led to administration of intravenous immunoglobulins in two patients. On testing for porphyria, it was found that two patients had AIP and two VP. Electrophysiological investigations revealed severe mainly motor axonal neuropathy in all. Two patients deteriorated to the point of requiring mechanical ventilation, and one of them died due to complications of critical illness. Haemin was administered to three patients, but the process of obtaining this medication was slow, which delayed the recommended early administration. The surviving patients showed minimal recovery and remained severely disabled. Porphyric neuropathy should always be considered as a differential diagnosis in a patient with an acute neuropathy, especially in SA. Absence of abdominal pain does not exclude the possibility of porphyria, and attacks may be precipitated by antiretroviral and antituberculosis medication. The outcome of our patients was not favourable; specifically, obtaining haemin was a challenge in the state hospital setting

Severe porphyric neuropathy – importance of screening for porphyria in Guillain-Barré syndrome

The hepatic porphyrias are a group of rare metabolic disorders, each of which is associated with a specific enzymatic alteration in the haem biosynthesis pathway. In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered. The development of acute neurovisceral attacks is related to environmental factors, including medications, hormones and diet. A possible manifestation of a severe attack is rapidly progressing quadriparesis, which may mimic Guillain- Barré syndrome. We present four such cases, highlighting that acute porphyria should be considered in the differential diagnosis of Guillain- Barré syndrome. Three patients presented to Steve Biko Academic Hospital, Pretoria, SA, with progressive quadriparesis, and one to a private hospital with acute abdominal pain followed by rapidly progressive quadriparesis. Two patients had started antiretroviral therapy before the development of symptoms, and one had started antituberculosis therapy. All patients had marked weakness with depressed reflexes, and showed varying degrees of confusion. An initial diagnosis of Guillain-Barré syndrome led to administration of intravenous immunoglobulins in two patients. On testing for porphyria, it was found that two patients had AIP and two VP. Electrophysiological investigations revealed severe mainly motor axonal neuropathy in all. Two patients deteriorated to the point of requiring mechanical ventilation, and one of them died due to complications of critical illness. Haemin was administered to three patients, but the process of obtaining this medication was slow, which delayed the recommended early administration. The surviving patients showed minimal recovery and remained severely disabled. Porphyric neuropathy should always be considered as a differential diagnosis in a patient with an acute neuropathy, especially in SA. Absence of abdominal pain does not exclude the possibility of porphyria, and attacks may be precipitated by antiretroviral and antituberculosis medication. The outcome of our patients was not favourable; specifically, obtaining haemin was a challenge in the state hospital setting.http://www.samj.org.zaam2016Chemical PathologyInternal MedicineNeurolog