Abnormal proteins in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease.

We studied more than 300 cerebrospinal fluid proteins from 21 patients with Creutzfeldt-Jakob disease. We also examined cerebrospinal fluid from 100 normal controls and more than 400 patients with various neurologic disorders other than Creutzfeldt-Jakob disease. Four abnormal proteins that were identified in the patients with Creutzfeldt-Jakob disease were absent in the normal persons.

Neuropathologic verification of Creutzfeldt-Jakob disease in the exhumed American recipient of human pituitary growth hormone: epidemiologic and pathogenetic implications.

Beginning at age 12, a boy with idiopathic hypopituitarism was treated with cadaver pituitary-derived human growth hormone during the period from 1963 to 1969. Fifteen years after the last treatment, the then 32-year-old man developed Creutzfeldt-Jakob disease (CJD). The illness was atypical in showing predominantly cerebellar signs, little mental deterioration, and no abnormal movements or periodic EEG activity.

Diagnosis of Creutzfeldt-Jakob disease by Western blot identification of marker protein in human brain tissue.

We tested purified preparations of brain tissue from 39 patients with Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, or kuru, and from 32 patients with a variety of nonspongiform degenerative diseases, with the use of Western blots against an antiserum to a similarly purified fraction made from scrapie-infected hamster brain. Positive reactions occurred in 81 percent of the 31 specimens from the patients with Creutzfeldt-Jakob disease (and in all of the 7 specimens that were stored frozen for less than one year), in 3 of the 4 specimens from the patients with kuru, in 3 of the 4 specimens from the patients with Gerstmann-Sträussler-Scheinker syndrome, and in none of the specimens from the patients with other neurologic degenerative disorders, including familial or sporadic Alzheimer's disease; dementia associated with myoclonus, motor neuron disease, or parkinsonism; and acquired-immunodeficiency-syndrome encephalopathy. Immunologic testing has thus begun to provide a useful and rapid adjunct to neuropathological examinations and animal-transmission experiments for the diagnosis of the spongiform encephalopathies.

Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam: a 30-year evaluation of clinical and neuropathologic trends.

We reviewed the records of 279 Guamanian Chamorro patients with amyotrophic lateral sclerosis (ALS) and 293 patients with parkinsonism-dementia (PD), who had onset of symptoms between 1950 and 1979, to determine if there were changes in the clinical and neuropathologic features that might clarify the declining incidence rates in the past decade. There were no major temporal changes in the frequencies of physical findings or histopathologic features, but in the past three decades, an increase in age at onset was observed for both ALS and PD. There was also a shorter duration of illness in ALS and a longer duration in PD.

Observations on the clinical presentations and the neuropathological findings of amyotrophic lateral sclerosis in Australia and Guam.

Among 20 consecutive autopsies of amyotrophic lateral sclerosis (ALS) occurring in Caucasians in Western Australia (WA), 85% were males. The median age of onset was 58.9 years and the average duration of illness was 2.

Transmissible virus dementia: evaluation of a zoonotic hypothesis.

Creutzfeldt-Jakob disease (CJD) and kuru are subacute transmissible dementing encephalopathies characterized by spongiform changes in the brain. Scrapie is a similar slow viral encephalopathy which affects sheep, goats and certain other animals. Anecdotal reports suggest that Creutzfeldt-Jakob disease could be a zoonosis.

[Creutzfeldt-Jakob disease in the Paris area: ethnic origin of the patients].

Twenty six (27 p. 100) of 96 patients dying from Creutzfeldt-Jakob disease in the Paris metropolitan area between 1968 and 1982 were born in foreign countries although the proportion of foreign-born residents account for only 15 p. 100 of the population.

Collagenase activity in skin fibroblasts of patients with amyotrophic lateral sclerosis.

In the current study we have measured collagenase activity released from skin explants and fibroblasts of patients with both Guam-type and sporadic amyotrophic lateral sclerosis and controls. The rationale for such a study derives from work reported more than 20 years ago demonstrating abnormalities in skin collagen metabolism in patients with the disease. We were not able to find significant differences in collagenase activity when fibroblasts were compared relative to the total protein secreted.

Pathogenesis of experimental Hantaan virus infection in laboratory rats.

Weanling Fischer rats inoculated intramuscularly with Hantaan virus (strain 76-118) developed subclinical infections characterized by transient viremia and shedding of virus in saliva, persistence of virus in lung, pancreas, spleen and liver, and development of fluorescent and neutralizing antibodies in serum with immune complex deposition in lung. Viremia and virus shedding in saliva occurred 10 to 13 days after inoculation. Horizontal intracage transmission of infection occurred between 35 and 63 days post-inoculation, long after disappearance of virus in oropharyngeal secretions and blood.

Involvement of the central nervous system in Schistosoma mansoni and S. haematobium infection. A review.

A review of reports of confirmed cases of involvement of the CNS in Schistosoma mansoni (SM) and S. haematobium (SH) infection was undertaken. Deposition of ova in the brain has been reported in 17 studies of SM infection and 4 studies of SH infection.

Serotypic classification of hantaviruses by indirect immunofluorescent antibody and plaque reduction neutralization tests.

Antisera prepared against 16 strains of hantaviruses isolated from patients with hemorrhagic fever with renal syndrome (HFRS) or from rodents captured in HFRS-endemic and nonendemic regions were titrated against Hantaan virus strain 76-118, Puumala virus strain Sotkamo, and Prospect Hill virus strain Prospect Hill-I by using the indirect immunofluorescent antibody and plaque reduction neutralization tests. Isolates fell into one of four distinct groups or serotypes. Serotype 1 included Apodemus-derived strains, serotype 2 included Rattus-derived strains, serotype 3 included Clethrionomys-derived strains, and serotype 4 included Microtus-derived strains.

Creutzfeldt-Jakob disease: possible medical risk factors.

To explore possible risk factors in the past medical history of patients with Creutzfeldt-Jakob disease (CJD), we conducted a case-control study among 26 cases and 40 matched controls. Statistically significant odds ratios were obtained for intraocular pressure testing; injury to or surgery on the head, face or neck; and trauma to other parts of the body. The odds ratios were nearly significant for head trauma and procedures requiring sutures.

Population distribution of the human vitamin D binding protein: anthropological considerations.

The polymorphism of the serum vitamin D binding protein (DBP) in humans is based on the existence of three common alleles, Gc1F, Gc1S, and Gc2, and 84 rare alleles. The geographical distribution of Gc1F, Gc1S, and Gc2 alleles shows north to south clines, together with a balanced equilibrium between the Gc1F or Gc1S allele frequency and the Gc2 frequency. The distribution of the FST values shows high variability within a geographical area.

A case-control study of Creutzfeldt-Jakob disease. Dietary risk factors.

The mode of natural transmission of Creutzfeldt-Jakob disease remains unknown. In a case-control study conducted in 1981-1983 to evaluate possible dietary and other sources of the disease, 26 cases were ascertained in the mid-Atlantic region of the United States, 23 of which were obtained from accumulated records of the Laboratory of Central Nervous System Studies of the National Institutes of Health. Controls included 18 family members and 22 hospital-matched individuals (total sample size, 66).

Definition of three serotypes of Hantaviruses by a double sandwich ELISA with biotin-avidin amplification system.

A total of seven different isolates of Hantaviruses from five mammalian species and six geographical areas were examined by a double sandwich ELISA with biotin-avidin amplification system. Three serotypes were recognized by comparing antigen titres to homologous and heterologous antibody and blocking antibody titres to homologous and heterologous antigens. Serotype 1 included four strains: Hantaan virus isolated from Apodemus agrarius in South Korea, Hupei-I isolated from acute-phase serum from a patient with epidemic haemorrhagic fever (EHF) in China, SR-11 isolated from a laboratory rat associated with an EHF outbreak in Sapporo, Japan, and Tchoupitoulas isolated from a wharf rat (Rattus norvegicus) trapped in New Orleans, La.

Experimental infection with Puumala virus, the etiologic agent of nephropathia epidemica, in bank voles (Clethrionomys glareolus).

Subclinical chronic infections characterized by transient viremia, prolonged virus shedding in oropharyngeal secretions and feces, and virus persistence in tissues (particularly lung) developed in laboratory-bred weanling bank voles (Clethrionomys glareolus) inoculated intramuscularly with Puumala virus (strain Hällnäs), the etiologic agent of nephropathia epidemica. Viral antigen, as evidence by granular fluorescence, was detected in the lungs, liver, spleen, pancreas, salivary glands, and small intestine. Infectious virus was found in the lungs from 14 to 270 days postinoculation, and feces and urine collected 35 to 130 days postinoculation were regularly and sporadically infectious, respectively.

The 200- and 150-kDa neurofilament proteins react with IgG autoantibodies from chimpanzees with kuru or Creutzfeldt-Jakob disease; a 62-kDa neurofilament-associated protein reacts with sera from sheep with natural scrapie.

Sera from 46 chimpanzees with spongiform encephalopathy (18 kuru, 28 Creutzfeldt-Jakob disease) and sera from 12 sheep with natural scrapie were tested for reactivity with immunoblots of neurofilament preparations obtained from mouse brain. The sera from the chimpanzees reacted mainly with the 200- and 150-kDa proteins of the neurofilament triplet and less frequently with the 70-kDa component of the triplet and with a 62-kDa neurofilament-associated protein. In contrast, the sera of sheep with natural scrapie reacted exclusively against the 62-kDa protein.

High incidence of Creutzfeldt-Jakob disease in North African immigrants to France.

During the 15-year period 1968-1982, 328 French residents died of Creutzfeldt-Jakob disease (CJD); 273 had been born in France (annual mortality rate of 0.38 per million inhabitants). Of the 55 foreign-born cases, 12 came from Tunisia and 11 from Algeria (mortality rates of 4.