Nodal-paranodal antibodies in HIV-immune mediated radiculo-neuropathies: Clinical phenotypes and relevance.

BACKGROUND: The frequency of nodal-paranodal antibodies in HIV-infected patients with chronic immune-mediated radiculo-neuropathies (IMRN) has not been previously described. METHODS: HIV-infected patients who met the inclusion criteria for chronic IMRN were screened for immunoglobulin G (IgG) antibodies directed against nodal (neurofascin (NF)186) and paranodal (NF155, contactin-1 (CNTN1) and contactin-associated protein(Caspr1)) cell adhesion molecules, using a live, cell-based assay. To explore potential pathogenicity, binding of human IgG to myelinated co-cultures was assessed by incubation with patients' sera positive for nodal or paranodal antibodies. Normal human serum was added as a source of complement to assess for complement activation as a mechanism for myelin injury. RESULTS: Twenty-four HIV-infected patients with IMRN were included in the study, 15 with chronic inflammatory demyelinating polyneuropathy (CIDP), 4 with ventral root radiculopathies (VRR), and 5 with dorsal root ganglionopathies (DRG). Five patients with CIDP had combined central and peripheral demyelination (CCPD). Three patients (12.7%) tested positive for neurofascin IgG1 antibodies in the following categories: 1 patient with VRR was NF186 positive, and 2 patients were NF155 positive with DRG and mixed sensory-motor demyelinating neuropathy with optic neuritis, respectively. CONCLUSION: The frequency of nodal-paranodal antibodies is similar among IMRN regardless of HIV status. Interpretation of the results in the context of HIV is challenging as there is uncertainty regarding pathogenicity of the antibodies, especially at low titres. Larger prospective immune studies are required to delineate pathogenicity in the context of HIV, and to establish a panel of antibodies to predict for a particular clinical phenotype.

Molecular deletion patterns in Duchenne and Becker muscular dystrophy patients from KwaZulu Natal.

There exists much phenotypic heterogeneity in Duchenne muscular dystrophy and its allelic variant, Becker muscular dystrophy. The molecular findings on 53 patients with Duchenne and 15 patients with Becker type muscular dystrophy in KwaZulu Natal, South Africa are reported. Multiplex PCR was performed using primers targeting 18 hot-spot exons throughout the dystrophin gene. Analysis of the multiplex PCR data revealed that 39/68 (57.0%) patients included in the study showed a deletion (33 DMD and 6 BMD patients). Twenty-five patients were Black, 4 were White and 10 were Indian. Using the Chamberlain and Beggs multiplex PCR assays, the region of the genome most frequently affected by a deletion includes exons 47-51. The distal region of the dystrophin gene was most frequently affected by the deletion in both Black and Indian patients. There were too few White patients for conclusions to be drawn concerning the most frequently affected part of the gene. Although the numbers are insufficient to determine whether ethnic differences are present, the Chamberlain and Beggs multiplex PCR assays detect deletions with the same frequency in South African DMD/BMD patients as that reported in the literature.

Ischemic stroke in young HIV-positive patients in Kwazulu-Natal, South Africa.

Cerebrovascular disease occurs in HIV-positive individuals, but no relationship between the two has been established. The authors reviewed a cohort of patients aged 15 to 44 years to evaluate stroke in HIV-positive and negative subjects. Patients who were HIV-positive with no other identifiable etiology were compared to age- and race-matched HIV-negative patients. HIV-positive and HIV-negative groups did not differ in angiographic, cardiac, or serologic tests. A positive HIV test does not provide causal information or diagnosis.

Problems in the optimal management of myasthenia gravis patients--a prospective clinical survey at Kalafong Hospital.

OBJECTIVES: This study forms part of a clinical survey of problems in the optimal management of patients with inherited neuromuscular diseases seen at Kalafong Hospital in Pretoria. Our objectives were to determine the problems associated with providing patients with optimal management until true remission (cure), and to apply the findings to ongoing improvement of optimal management. This is the first report of the series. METHODS: Twenty-six patients were studied prospectively from 1986 to 1998. Early sternal-splitting thymectomy on class II-V patients as well as anticholinesterases, corticosteroids, azathioprine, plasma exchange, intensive care and various combinations of these constituted part of the optimal management. An assessment of the total monthly income and distance from hospital was done for each patient. RESULTS: Five of the 15 thymectomised patients (33.3%) were lost to follow-up after reaching remission. Of the remaining 10 patients, 6 (40%) are in true remission and the remaining 4 (26.7%) are in pharmacological remission. Four of the 11 patients (36%) treated non-surgically were lost to follow-up. Of the remaining patients, 1 (9.1%) is in true remission and the remaining 6 (54.5%) are in pharmacological remission. The average monthly income of patients lost to follow-up in the thymectomised group was lower than that of patients who continued follow-up, and their homes were further away from hospital. In the non-surgical group the average monthly income of patients lost to follow-up was higher than that of patients who continued follow-up and their homes were nearer to the hospital. CONCLUSION: Early thymectomy (the aggressive approach) resulted in 40% cures, 26.7% pharmacological remissions, no mortality, minimal morbidity, and early discharge. Loss to follow-up was one of the biggest problems in providing optimal management for these patients. We modified optimal management in response to our patients' concerns without sacrificing excellence, and found that poverty and poor access to tertiary hospitals were possible contributory factors to loss to follow-up. Suggestions are made with regard to tackling the problems. Myasthenia gravis (MG) is a disorder of neuromuscular function resulting from an immunologically based premature destruction of acetylcholine receptors.