Intrathecal interferon in subacute sclerosing panencephalitis.

Five patients with clinically advanced subacute sclerosing panencephalitis (SSPE) were given human leukocyte interferon (IFN) by the lumbar route, 1 million IU every other day for a total of 30 days. Intrathecal IFN produced a meningeal inflammatory reaction in all patients and was associated with transient hemiparesis in 1. It persisted in the cerebrospinal fluid at measurable levels for 48 hours after a single injection.

N-acetyl-beta-hexosaminidase B deficiency in cultured fibroblasts from a patient with progressive motor neuron disease.

A patient with a 20-year history of progressive motor neuron disease was previously found to have profoundly low levels of N-acetyl-beta-hexosaminidase (Hex) in serum and leukocytes; Hex activity in cultured skin fibroblasts was in the low normal range. By thermal inactivation and cellulose acetate electrophoresis, the residual activity appeared to be Hex A. In the present study, the residual activity in cultured skin fibroblasts was further characterized as Hex A by thermal inactivation at reduced temperatures and ion exchange chromatography; no evidence was obtained for a diffusible inhibitor of Hex activity.

Inhibition of terminal axonal sprouting by serum from patients with amyotrophic lateral sclerosis.

To investigate the pathogenesis of amyotrophic lateral sclerosis, we compared the effect of serum from patients with this disease on the regenerative sprouting of terminal axons in botulinum-treated mouse gluteus muscle with the effects of serum from controls and from patients with diabetic peripheral neuropathy. Serum from 9 of 19 patients with the sporadic form of amyotrophic lateral sclerosis and from 2 of 6 patients with the familial form caused a reduction in the proportion of sprouting terminal axons, as compared with that found in muscles treated with serum from controls or diabetic patients. Immunoglobulin from patients with amyotrophic lateral sclerosis, when tested on immunoblots, recognized a 56-kilodalton protein secreted by denervated rat diaphragm muscle; rabbit antiserum raised against this protein also suppressed terminal axonal sprouting.

Intraluminal clot of the carotid artery detected radiographically.

Nine patients had intraluminal filling defects identifiable as clot within the internal carotid artery at angiography. Thrombus was unilateral in eight, bilateral in one. Eight of the 10 clots were attached to atheromatous plaques.

Monoclonal antibody-defined immunoregulatory cells in multiple sclerosis cerebrospinal fluid.

To determine if previously reported peripheral blood suppressor cell defects are also found in the central nervous system (CNS) of patients with multiple sclerosis (MS), we studied cerebrospinal fluid (CSF) and peripheral blood lymphocytes from 40 MS patients and 15 patients with other neurological diseases. With an indirect immunofluorescence technique using the OKT series of monoclonal antibodies (OKT4, marking helper/inducer cells, OKT5 and OKT8 marking suppressor/cytotoxic cells, and OKT3 marking all peripheral T cells) we found that MS patients tested in the first 2 wk of exacerbation had invariably diminished CSF suppressor/cytotoxic cells, which was followed by an elevation of these cells in the 3rd wk of exacerbation. Repeat studies of three patients showed that perturbations of CSF suppressor/cytotoxic cells were dependent on clinical status.

Experimental allergic encephalitis; treatment with drugs which alter CNS serotonin levels.

Lennon and Carnegie have proposed that the clinical symptomatology of experimental allergic encephalitis (EAE), an acute autoimmune demyelinating disease, may be due, at least in part, to an immune response directed against CNS serotonin receptors. To test this hypothesis we treated strain 13 guinea pigs, which had been immunized with guinea pig basic protein (GPBP) in complete Freund's adjuvant (CFA), with drugs known to affect central nervous system (CNS) serotonin levels. These drugs included imipramine hydrochloride, tryptophan and carbidopa which increase CNS serotonin and reserpine which decreases it.