Biomedical subjects
J Joubert
Publications and source records attributed to J Joubert.
Flunarizine--effective add-on therapy in reading epilepsy.
Since it was first described in 1956, reading epilepsy, an uncommon disorder, has engendered great interest among neurologists, the reason being that it is probably one of the most complex of the sensory-evoked reflex epilepsies and one in which the mechanism of seizure production is as yet unresolved. Unfortunately, it often defies the standard medications useful in the treatment of other forms of epilepsy. A patient with typical reading epilepsy, preceded by jaw-jerking, is described in this report. An additional feature of this case is the presence of a stutter from early childhood. Therapy with phenytoin, carbamazepine and sodium valproate failed to provide satisfactory control. Flunarizine, as add-on therapy to sodium valproate, produced an excellent therapeutic response.
The MEDUNSA Stroke Data Bank. An analysis of 304 patients seen between 1986 and 1987.
Stroke is a major cause of mortality and morbidity in black Africans. The MEDUNSA Stroke Data Bank (MSDB) was established in 1984. Its goal is to gather data on all aspects of cerebrovascular disease in South African blacks, and it is supported by modern investigative techniques such as computed tomography (utilised in 82.2% of cases). The majority of the patients (71.2%) had cerebral infarction. Certain features, such as the importance of hypertension and increasing age as risk factors, were found to be similar to Western stroke populations; also, probable or definite cardiac source for cerebral embolism was present in 46% of the patients. In contrast, however, other features, such as transient ischaemic attacks, peripheral vascular disease, coronary artery disease and lacunar strokes, occurred far less frequently than is reported in Western patients. It is hoped that the MSDB will lead to a better understanding of cerebrovascular disease in blacks and possibly to in-depth comparative studies of the ubiquitous problem of atherosclerosis.
Human T-lymphotropic virus type I-associated myelopathy. A case report.
Tropical spastic paraparesis (TSP) has been reported from various parts of the world for several decades. Recently reports have emerged from Japan and also countries in tropical zones associating endemic TSP with abnormally high titres of antibodies to human T-lymphotropic virus type I (HTLV-I). Data indicate that this lymphotropic retrovirus is neuropathogenic, either by direct invasion or via immunopathological mechanisms. A South African man is described who met the diagnostic criteria of TSP. Both serum and cerebrospinal fluid were antibody-positive for HTLV-I. It is possible that HTLV-I infection may be implicated in the 'myelopathies of undetermined cause' that form a substantial subgroup of spinal cord disease occurring in black South Africans.
Neurocysticercosis in 578 black epileptic patients.
In a study of 578 epileptic patients at the Ga-Rankuwa Hospital, 281 had abnormal computed tomography (CT) of the brain (48.6%), and in 162 (average age 32 years) there was evidence of cysticercosis (57.6%). In 68 of the infected patients (41.9%) active cysts were seen on CT. Neurocysticercosis, either active or inactive, was thus implicated in 28% of this group of epileptic patients, which emphasises the importance of this disease in the causation of epilepsy in the black population of South Africa.
Genetic linkage between Huntington disease and the D4S10 locus in South African families: further evidence against non-allelic heterogeneity.
A study of genetic linkage between Huntington disease (HD) and the D4S10 locus (G8) has been undertaken in 10 South African (SA) families originating from the black, white and mixed acestry population groups. Allele frequencies at the D4S10 locus have been established in the non-Caucasoid population groups. There are significant differences in the allele frequencies at the D4S10 locus between the various SA populations. Clearly, information about population-specific frequencies for all polymorphisms is essential prior to the implementation of predictive testing in different population groups. Linkage has been demonstrated within this mixed group of HD families in SA using the HindIII, EcoRI and MspI polymorphisms, detected by G8. A maximum lod score of 8.14 at a recombination fraction of 0.00 (confidence limit 0-0.058) has been calculated using a combined haplotype of the HindIII and MspI polymorphisms. Taking into account the diverse ethnic backgrounds of the different SA population groups in this investigation, the data obtained from the study provide further evidence that there is probably only a single HD locus.
Hemicrania continua in a black patient--the importance of the non-continuous stage.
A 52-year-old black female for 19 years had severe intermittent unilateral headaches that demonstrated the "clustering" phenomenon. She was initially diagnosed as having episodic cluster headache. Response to lithium carbonate, ergotamine and courses of corticosteroids was, however, only partial. In December 1989 the headache pattern changed and she developed severe unilateral hemicranial headache that was continuous and non-remitting. This responded immediately and persistently to oral indomethacin. A diagnosis of hemicrania continua (HC) was made. The initial intermittent headache syndrome appears to have been the pre-continuous stage of hemicrania continua, and not episodic cluster headache as previously supposed. The pre-continuous phase of hemicrania continua may thus masquerade as episodic cluster headache by reason of its intermittency and "clustering". In this case, the intermittent stage was protracted. This stage may, conceivably, even be a permanent one. To our knowledge, this is the first report of hemicrania continua in a black African.
Cysticercal meningitis--a pernicious form of neurocysticercosis which responds poorly to praziquantel.
Chronic cysticercal meningitis was diagnosed in 6 of 260 cases of neurocysticercosis. Clinical features usually associated with meningitis, such as fever, cranial nerve palsies and nuchal rigidity, were absent and the diagnosis was reached because of persistently abnormal cerebrospinal fluid (CSF) associated with active neurocysticercosis on computed tomography. Other causes of chronic meningitis were excluded. Hydrocephalus, either obstructive or communicating, was present in all 6 patients. The results of medical treatment with praziquantel were poor and there was no improvement either clinically or in the CSF. Severe clinical sequelae, such as dementia, blindness and gait ataxia, were common despite protracted medical treatment and ventriculoperitoneal shunting.
Schistosomiasis of the spinal cord--underdiagnosed in South Africa?
Schistosomiasis is endemic in many areas of South Africa, particularly the northern and eastern Transvaal. At Ga-Rankuwa Hospital in the northern Transvaal, 30 km north-west of Pretoria, spinal cord disease is common. In a substantial proportion of these cases no obvious cause for the condition can be found. In an attempt to draw attention to schistosomiasis as a possibly underdiagnosed cause of spinal cord disease, 3 cases of schistosomiasis of the spinal cord seen over a period of 3 years are reported. These 3 patients had histological evidence of spinal schistosomiasis. Because myelography of the spinal cord is often non-contributory in schistosomal involvement, it is suggested that patients with 'myelopathy of unknown origin' who come from an endemic area be given a therapeutic trial of praziquantel, especially if the serological findings are positive for schistosomiasis and the lower cord is involved.
Use of indium-111-labelled platelets in black stroke patients. A pilot study.
Human blood platelets labelled with indium-111 oxine have been shown to accumulate on damaged vascular surfaces and abnormal platelet deposition has been demonstrated in the carotid arteries of white stroke patients. Gamma scintigraphy of the carotid and cerebral arteries of 5 black stroke patients and 5 age- and sex-matched controls using 111In-labelled platelets showed no abnormal accumulation indicative of carotid artery disease.
Are clinical differences between black and white stroke patients caused by variations in the atherosclerotic involvement of the arterial tree?
The differences in site and degree of atherosclerotic involvement of various vascular beds and their clinical significance are emphasised in a study of 304 black stroke patients. Detailed clinical examinations, computed tomography (CT), gated blood pool studies, echocardiography and ECG were performed and autopsy studies carried out. CT of the brain showed that non-haemorrhage, i.e. ischaemic lesions, accounted for 71.2% of strokes, a similar figure to that found in white stroke patients. However, carotid bruits (0.62%) and peripheral vascular disease (0.9%) followed by transient ischaemic attacks (1.9%) were found to be uncommon. Similarly, ischaemic heart disease (6.9%) appeared to be less common than the incidence in reported white stroke patients. In 30 patients who came to autopsy, the maximum degree of atherosclerotic stenosis of the extracranial carotid arteries was 21.7% of the lumen diameter. The differences in the site and degree of atherosclerosis in blacks not only give rise to differences in the clinical features of stroke patients but may have an important bearing on their investigation, management and prognosis.
Rapid and complete resolution of giant cysticercal cysts after administration of praziquantel. A report of 4 cases.
Four patients with giant cysts, one cyst with a diameter of 55 mm, were treated with a 30-day course of praziquantel (Biltricide; Bayer-Miles) 50 mg/kg/d. One patient had corticosteroids administered simultaneously. All 4 patients had anticonvulsant cover. In all cases there was rapid and complete resolution of the cysts. In 2 patients this occurred within 30 days. The computed tomographic features of giant cysticercal cysts are: thin walled cystic lesions containing clear fluid, localised thickening of the wall (probably representing the scolex), absence of pericyst oedema, and the presence of associated smaller and more typical cysticercal larval forms. Giant cysticercal cysts resolve remarkably rapidly on medical therapy. A therapeutic trial of praziquantel can, in suspected giant cysticercal cysts, obviate the need for exploratory craniotomy.
Treatment of neurocysticercosis.
The clinical and laboratory data of 88 black patients with computed tomographic (CT) evidence of active neurocysticercosis were analysed. The CT appearance of neurocysticercosis was distinctive in the majority. Seizures, chronic headache and neuropsychiatric changes were the most common clinical presentations. Hydrocephalus was diagnosed in 17 patients (9 obstructive and 8 communicating) and cerebral infarction with focal neurological deficit occurred in 4 cases. All patients were treated with praziquantel (Biltricide: Bayer Miles). There was complete cyst clearance after two courses in 14 patients and in 16 cases the mean percentage reduction in cyst number was 85.88% and in cyst size 80.84%. In 1 patient there was no response to treatment. Although praziquantel was effective in parenchymal neurocysticercosis, it was ineffectual in the treatment of 5 patients with cysticercal meningitis. There were 2 deaths in the series.
The cardiovascular status of the black stroke patient.
The cardiac status of 102 consecutive black stroke patients entered to the Medunsa Stroke Databank was determined. Cardiological examination, echocardiography and a gated blood pool scan revealed structural and/or functional cardiac abnormalities in 73.6% of patients. Rheumatic heart disease was diagnosed in 15.6%, mitral valve prolapse in 5.8% and mitral annulus calcification in 4.9% of cases. 'Possible' cardiac sources of cerebral embolism were detected in 22.5% and 'definite' sources in 23.5% of patients. Hypertensive heart disease was diagnosed in 35.2% and cardiomyopathy in 13.7% of the study population. Ischaemic heart disease was present in 6.86%. Ultrasonography revealed ventricular bands in 29.4% of patients. The high incidence of structural cardiac abnormalities detected by non-invasive means is in keeping with recent studies in white stroke patients.
Chorea and psychiatric changes in organophosphate poisoning. A report of 2 further cases.
The acute muscarinic and nicotinic side-effects of organophosphate poisoning are well known. Less commonly encountered are neurological symptoms such as chorea and psychiatric disturbances such as psychoses and depression. Two patients with organophosphate poisoning are described, both exhibiting marked choreiform dyskinesias and one experiencing severe depression and emotional lability. Both responded well to the appropriate treatment. Because of the widespread use of organophosphate insecticides in agriculture, the neurological and psychiatric effects of chronic low-dose exposure to organophosphates in farmers and their employees deserves attention.
Cluster headache in black patients. A report of 7 cases.
Cluster headache is an uncommon form of headache syndrome. It is characterised by paroxysms of severe unilateral head pain typically involving the orbit. There are often associated autonomic changes on the affected side such as lacrimation, nasal congestion and Horner's syndrome. Apart from episodic cluster headache, various subtypes such as chronic cluster headache, cluster headache variant and chronic paroxysmal hemicrania have been identified. There have been few reports of the incidence and clinical features of cluster headache in blacks; 7 black patients with various types of cluster headache are described.
Huntington disease in South African blacks. A report of 8 cases.
Huntington chorea is rare in ethnically pure blacks; 8 South African black patients, 6 adults and 2 children, from 4 families are described. The symptoms do not appear to differ materially from Huntington chorea in other races. Increased awareness of the disease in this population group is warranted.
Chronic paroxysmal hemicrania in a South African black. A case report.
A 35-year-old black man with a 4-year continuous history of multiple paroxysms of unilateral temporal and ocular pain is reported. Multiple (that is, 15-20) attacks occurred in 24 h. There were no symptom-free periods. Mild exophthalmos, marked sweating, nasal congestion, and lacrimation were present on the affected side. There was no response to ergot preparations, steroids, or lithium but dramatic and sustained relief with indomethacin.