Giant axonal neuropathy: central abnormalities demonstrated by evoked potentials

Previous studies of giant axonal neuropathy have reported clinical and pathological findings that indicate involvement of the central nervous system. We studied 3 boys with giant axonal neuropathy, who were 14 to 16 years of age, using auditory, visual, and somatosensory evoked potentials. Absence of waveforms and prolongation of peak and interwave latencies were found. Abnormalities were noted in all modalities. The auditory brainstem evoked response in particular indicated a significant increase in brainstem conduction time. These studies add clinical neurophysiological confirmation of the central nervous system involvement in this disorder and may also provide a means of quantitative evaluation of its progression.     

The simultaneous determination of cerebrospinal fluid and plasma adenosine deaminase activity as a diagnostic aid in tuberculous meningitis

The simultaneous determination of cerebrospinal fluid (CSF) and plasma adenosine deaminase (ADA) activity was evaluated as a diagnostic aid in tuberculous meningitis (TBM). CSF and plasma ADA activity were determined in four groups of patients: (i) a 'no meningitis' group of 174 children investigated for possible meningitis, but found to be uninfected; (ii) an aseptic meningitis group of 40 children; (iii) a bacterial meningitis group of 31 children; and (iv) a TBM group of 27 patients (24 children and 3 adults). CSF ADA alone was determined in a further 23 children with aseptic meningitis, 19 with bacterial meningitis and 13 children and 7 adults with TBM. Both the CSF/plasma ADA ratio and the absolute CSF ADA activity were raised in TBM (mean values 0,24 and 12,61 U/I respectively) and bacterial meningitis (mean values 0,59 and 15,43 U/I respectively), but not in the aseptic meningitis group (mean values 0,06 and 2,00 U/I) or the 'no meningitis' group (mean values 0,04 and 1,51 U/I). Both values will distinguish TBM from aseptic meningitis, but do not appear to hold any marked advantages over conventional CSF criteria in the diagnosis of TBM.     

Traumatic infarction of the spinal cord in children

Infarction of the spinal cord in childhood is rarely due to trauma. During a 15-year period (1971 to 1985), eight children were admitted to The Hospital for Sick Children, Toronto, with a diagnosis of traumatic infarction of the spinal cord. All of these patients had delayed onset of neurological signs varying between 2 hours and 4 days after their initial trauma. No bone abnormalities were seen on plain spine x-ray films. Myelography was carried out in seven of these children and found to be normal in all seven. Six patients who were paraplegic at the time of admission remained permanently paraplegic, but two with incomplete cord signs did show some improvement.     

An ultrastructural study of Elschnig's pearls in the pseudophakic eye

In two pseudophakic human eyes, obtained post mortem, Elschnig's pearls were visible biomicroscopically. One eye contained a medallion lens and the other an iridocapsular lens (implanted for 53 months and 39 months, respectively). The medallion lens was fixed to the iris but was not attached to the Soemmerring's ring. Elschnig's pearls and star-shaped cells were found on the posterior capsule in the pupillary space. One loop of the iridocapsular lens was encased in the Soemmerring's ring whereas the other was located between the iris and the lens remnants. The Elschnig's pearls were on the anterior side of the ring; only a few were in the pupillary space. Two other pseudophakic eyes with clear posterior capsules also contained small numbers of Elschnig's pearls on or just near the peripheral lens remnants.     

Iatrogenic choroidal neovascularization in sickle cell retinopathy

Fifteen patients (16 eyes) with proliferative sickle retinopathy treated with argon or xenon arc feeder vessel photocoagulation had chorioretinal (CRN) or choriovitreal (CVN) neovascularization develop. These patients were followed from 2 1/2 to 11 1/2 years with a mean follow-up of 6 years and 8 months. Clinically important late complications of the choroidal neovascularization included vitreous hemorrhage (in three of eight patients with CVN). However, in only two of these three eyes was there any drop in vision related to residual vitreous hemorrhage, and this was limited to loss of only one line of Snellen visual acuity. Therefore, treatment is not usually recommended if CRN or CVN develops after intense photocoagulation. Vitreous fluorophotometry was performed on these patients to examine the breakdown of the blood-retinal barrier. This gave a quantitative measure of fluorescein leakage not obtained with fluorescein angiography. Midvitreous measurements, which are more representative of these peripherally located proliferative lesions than are pre-retinal measurements, suggest that vitreous fluorophotometry may be helpful in differentiating the higher leakage of CVN from the CRN.     

Role of membrane N-linked oligosaccharides in host cell interaction with invasive forms of Trypanosoma cruzi

The removal of N-linked oligosaccharides by peptide-N4-[N-acetyl-beta-glucoseaminyl]asparagine amidase (previously known as aspartoglycosylamine amidohydrolase and abbreviated N-glycanase) from the surface of blood or insect-transmissible forms of Trypanosoma cruzi markedly increased the capacity of these organisms to associate with (i.e., bind and penetrate) either mouse peritoneal macrophages or rat heart myoblasts. This effect was evidenced by a significant elevation in both the percentage of infected host cells and the average number of parasites per 100 cells. Conversely, N-glycanase treatment of either host cell markedly reduced both parameters to levels significantly below those obtained with cells mock treated with medium alone. The N-glycanase effect on the parasites was inhibited by heat inactivation of the enzyme or by the presence of fetuin, an N-glycanase substrate. The enhanced capacity of N-glycanase-treated T. cruzi to engage the host cells started to subside 2 h after the treatment, indicating the reversibility of the effect. The decreased reactivity of N-glycanase-treated macrophages or myoblasts with T. cruzi suggests that N-linked oligosaccharides on these host cells are involved in the initial phase of the cell infection process. Instead, because T. cruzi interacted more effectively with host cells after treatment with N-glycanase, parasite surface N-linked oligosaccharides would seem to interfere with the association.