Influence of malnutrition on developing rat peripheral nerves.

Animals with experimentally induced neuropathies frequently fail to gain weight normally and appear poorly nourished. To determine whether or not malnutrition alone contributes to peripheral nerve dysfunction in these disorders, we subjected healthy 40-day-old rats to three grades of food restriction. After 4 weeks, food-restricted rats weighed 203 +/- 5 (mean +/- SE), 152 +/- 12, and 97 +/- 5 g, respectively, whereas control rats having free access to food weighed 379 +/- 22 g.

Cerebrospinal fluid abnormalities in homosexual men with and without neuropsychiatric findings.

We have studied cerebrospinal fluid obtained from 38 homosexual or bisexual men participating in a prospective study of the neuropsychological disorders associated with human immunodeficiency virus (HIV) infection. Twenty-two subjects had neuropsychiatric findings and seropositivity, 11 asymptomatic subjects had seroconverted within 6 to 24 months, and 5 subjects were seronegative controls. Only 1 had acquired immunodeficiency syndrome-related complex, and none had the acquired immunodeficiency syndrome when initially studied.

Treatment of the neuromuscular complications of human immunodeficiency virus infection.

Neuromuscular disorders are increasingly being reported in individuals with human immunodeficiency virus (HIV) infection. The majority of these disorders resemble diseases occurring in HIV-seronegative patients and include inflammatory demyelinating polyneuropathies, multiple mononeuropathies, and polymyositis. In HIV-seronegative patients, these diseases are believed to be immune system-mediated.

A new dietary therapy for adrenoleukodystrophy: biochemical and preliminary clinical results in 36 patients.

A new dietary regimen has been administered for periods ranging from 60 days to 1 1/2 years in 34 patients with various forms of X-linked adrenoleukodystrophy (ALD), as well as in 1 patient with neonatal ALD and 1 patient with infantile Refsum's disease. The diet combines the administration of a glyceryl trioleate oil (GTO) with the dietary restriction of very-long-chain fatty acids (VLFA), particularly hexacosanoic acid (C26:0). Reductions in the levels of plasma C26:0 and other VLFA were achieved in 25 of the 36 patients.

Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection.

Nine patients with inflammatory demyelinating polyneuropathies (IDP) were found to have human T-cell lymphotropic virus type III (HTLV-III) infection. The 8 men, 6 of whom were homosexual, and 1 woman, a former intravenous drug user, presented with progressive weakness. Two had lymphadenopathy but all were otherwise asymptomatic.

Monitoring azathioprine therapy in myasthenia gravis.

Azathioprine (AZA) is used increasingly in the treatment of selected patients with myasthenia gravis (MG). The "usual" dose is 2 to 3 mg/kg/d, but guidelines do not exist to determine a specific dose for an individual patient. We reviewed our previously reported MG patients to determine what laboratory studies correlated with therapeutic efficacy.

Disorders of neuromuscular transmission in infants and children.

Disorders of neuromuscular transmission in infants and children can be divided into two groups. The first group includes conditions similar to those seen in adults: autoimmune myasthenia gravis, Lambert-Eaton myasthenic syndrome, and botulism. The second includes several disorders that are unique to this age group, such as the congenital myasthenias and infantile botulism.

Immunoreactive myelin basic protein in cerebrospinal fluid of patients with peripheral neuropathies.

The presence of myelin basic protein (MBP)-like material in cerebrospinal fluid (CSF) usually reflects breakdown of central nervous system myelin. Immunoreactive MBP levels were measured in 70 CSF specimens from 66 patients with a variety of peripheral neuropathies. Immunoreactive MBP was present in CSF in 70% (16 of 23) of patients with biopsy-proved chronic demyelinating polyneuropathies, whereas in neuropathies producing primarily axonal damage it was present in only 22% (5 of 23).

Transient conduction block following acute peripheral nerve ischemia.

We studied the effects of transient focal ischemia on the electrophysiologic function of rat sciatic nerves. Focal and generalized impairment of impulse conduction, measured by falling evoked muscle and nerve compound action potential amplitudes, occurred within 10 minutes of femoral artery occlusion. Conduction failure reached a nadir at 45-60 minutes and then improved to normal within 24 hours.

Chronic demyelinating polyneuropathy in systemic lupus erythematosus.

Mononeuritis multiplex and acute demyelinating polyneuropathy (Guillain-Barré syndrome) are well known in systemic lupus erythematosus (SLE). Chronic acquired demyelinating polyneuropathy has been reported rarely in SLE. Two young women had monophasic progressive weakness, areflexia, elevated CSF protein, and slow nerve conduction velocities as the first manifestation of SLE.

Azathioprine in the treatment of myasthenia gravis.

Twenty-four patients with myasthenia gravis were treated with azathioprine. Eighteen of the patients tolerated the drug. Six discontinued azathioprine therapy because of toxicity.

Clinical electrophysiology of infantile botulism.

Infantile botulism is a recently recognized cause of acute hypotonic paresis and respiratory failure in young infants. Electrophysiological testing has proven useful in early diagnosis in suspected cases by demonstrating abnormal neuromuscular transmission as is known to occur in botulism. Twenty-five infants with bacteriologically proven botulism were studied by uniform methods in our laboratory and characteristic electrophysiological abnormalities were found.

Neurophysiological and neuropsychological function in mercury-exposed dentists.

In a study of the relation between cumulative exposure to mercury and chronic health impairment 298 dentists had their mercury levels measured by an X-ray fluorescence technique. Electrodiagnostic and neuropsychological findings in the dentists with more than 20 micrograms/g tissue mercury levels were compared with those of a control group consisting of dentists with no detectable mercury levels. 30% of the 23 high mercury dentists had polyneuropathies.

Creutzfeldt-Jakob disease in a 20-year-old woman.

A 20-year-old woman developed ataxia, extrapyramidal movements, myoclonus, and progressive dementia. Brain biopsy disclosed status spongiosus, diagnostic of Creutzfeldt-Jakob disease; this is the youngest spontaneous case ever reported. Creutzfeldt-Jakob disease can occur in young adulthood.