Possible introduction of Huntington's chorea into Pacific Islands by New England whalemen.

Huntington's disease (HD) has been reported in two isolated South Pacific islands, New Britain in Papua New Guinea, and Malaita in the Solomon Islands. The earliest presumptive choreics in these foci were born in the latter part of the 19th century, prior to the colonial period. It is known that these islands were visited by certain New England whalers in the precolonial era, and inspection of the crew lists of these whalers shows many men with surnames common to families in New England known to have produced HD individuals. In some instances, the name, age, and origin of whalemen correspond with those of known choreic individuals. It is suggested that New England whalemen carrying the trait for HD introduced the disease to these islands.

Late-childhood-onset spinal muscular atrophy in three Melanesian families in Papua New guinea.

Late-childhood-onset spinal muscular atrophy (SMA) with rapid progression from proximal to distal muscle groups and leading to severe incapacity or death in adolescence or early adult life, was studied in two Melanesian families in Papua New Guinea. Probable SMA with cardiomyopathy was observed in a 12-year-old boy in a related family. The pattern of inheritance in the study group did not conform to any of the conventional Mendelian modes and it was conjectured that the disease was expressed in heterozygote carriers of the SMA gene only when an allelomorphic activator gene was present.

Oculopharyngeal and distal myopathy: a case study from Papua New Guinea.

Distal limb myopathy with onset at 30 years, followed by the development of progressive ptosis, external ophthalmoplegia, and pharyngeal myopathy was observed in a 37-year-old Melanesian man from the Gulf Province of Papua New Guinea. Ptosis and external ophthalmoplegia without apparent distal muscle involvement or dysphagia with onset at 35 and 25 years of age, respectively, were noted in the patient's 44- and 27-year-old sisters. Autosomal recessive inheritance appears to be likely in this family. This myopathic syndrome has not previously been reported in Melanesians.

Huntington disease in black Zimbabwean families living near the Mozambique border.

Huntington disease (HD) was identified in a black (Bantu) family living in the Manicaland region of Zimbabwe near the border with Mozambique. The pedigree included 11 affected individuals in 4 generations. There were 2 other probable HD patients from 2 unrelated black families in the same region. The prevalence rate of HD in this region of Zimbabwe was estimated to be 0.5-1 per 100,000. HD could have been introduced by Portuguese colonists from Mozambique, or by other European visitors, possibly before 1875. DNA studies may ultimately indicate if HD was introduced to this community, or if it arose by mutation. HD was previously reported in Zimbabwean blacks in 1978, when 4 cases of juvenile HD were described in a Bantu family with no apparent history of the disease.

Seckel syndrome in a Yemeni family in Saudi Arabia.

We studied a 28-year-old Yemeni woman with typical Seckel syndrome. Two deceased sibs, a twin sister and a brother, had also been affected. The father of these individuals was married to his cousin's daughter: the pedigree suggests autosomal recessive inheritance. Classical Seckel syndrome has not been reported previously in Arabs, and this appears to be the first report from the Middle East.

Q Fever in human and livestock populations in Oman.

In 2000, Q fever was documented for the first time in the Sultanate of Oman in two patients, one with chronic pericarditis and the other with acute pneumonia. In 2001, a study of a randomly selected group of 102 adult patients from different provinces in northern Oman, presenting to the University Hospital in Muscat with unrelated conditions (e.g., diabetes mellitus, ischemic heart disease), revealed that 10 (9.8%) were seropositive for previous Coxiella burnetii infection. Examination of sera from a randomly selected group of 54 healthy goats from eight different herds from three different provinces of Oman, obtained by the Veterinary Research Center in Muscat, revealed that 28 (52%) had been infected, and 5 sheep, each from one of four herds, were seropositive for C. burnetii. We suspect that Q fever is widely prevalent in human populations in Oman, and that infection is widespread in goat, and probably sheep and other livestock populations, throughout the country.

Severe staphylococcal pneumonia complicating pyomyositis.

Severe staphylococcal pneumonia developed secondary to pyomyositis of the buttock in an adult male Melanesian in East New Britain, Papua New Guinea. Penicillin-resistant Staphylococcus aureus was cultured from the abscess, and S. aureus, together with Gram-negative organisms, were cultured from sputum. Despite treatment with high dose cloxacillin together with supportive chemotherapy, progressive deterioration of lung function occurred and acute respiratory failure developed. As suitable ventilators were unavailable, tracheal intubation was performed, and manual assisted ventilation was carried out for 4 days. Progressive improvement in ventilation and general status resulted, and the patient recovered without sequelae.

Infectious and tropical diseases in Oman: a review.

Oman is generally hot and dry, but the Salalah region in southern Dhofar province is relatively cool and rainy during the summer monsoon, and has a distinctive pattern of infection. Important, notifiable infections in Oman include tuberculosis, brucellosis (endemic in Dhofar), acute gastroenteritis, and viral hepatitis: 4.9% of the adults are seropositive for hepatitis B surface antigen and approximately 1.2% for hepatitis C virus. Infection with human immunodeficiency virus is uncommon, and leprosy, rabies, and Crimean-Congo hemorrhagic fever are rare. Between 1990 and 1998, the incidence of malaria, (>70% due to Plasmodium falciparum) decreased from 32,700 to 882 cases. Cutaneous and visceral leishmaniasis (caused by Leishmania tropica and L. infantum, respectively) and Bancroftian filariasis occur sporadically. Intestinal parasitism ranges from 17% to 42% in different populations. A solitary focus of schistosomiasis mansoni in Dhofar has been eradicated. There are major programs for the elimination of tuberculosis, leprosy, and malaria, and to control brucellosis, leishmaniasis, sexually transmitted diseases, trachoma, acute respiratory infection in children, and diarrheal diseases. The Expanded Program on Immunization was introduced in 1981: diphtheria, neonatal tetanus, and probably poliomyelitis have been eliminated.

Creutzfeldt-Jakob disease in Oman: report of two cases.

Sporadic Creutzfeldt-Jakob disease (CJD) was diagnosed in two Oman Arab men, aged 50 and 75 years respectively, both with a history of rapidly developing dementia and myoclonic jerks. Illness developed over a period of 3 months in the first case and over six months in the second. Electroencephalography in both subjects showed periodic triphasic sharp waves characteristic of CJD. In neither case was it possible to obtain a brain biopsy or perform autopsy (autopsy is contrary to Islamic practice in the Middle East), however, electrophoresis of cerebrospinal fluid from the second patient revealed the distinctive double protein spots characteristic of CJD. This is the first report of CJD from Oman.

A clinico-pathological study of a case of kuru.

Kuru was diagnosed in a 42-year-old Melanesian male from the Eastern Highlands Province of Papua New Guinea. The clinical features indicated predominant cerebellar degeneration together with widespread cortical neuronal dysfunction and involvement of the diencephalon, hippocampus and basal ganglia. Dementia was an early and prominent feature. The duration of clinical illness was about 12 months and atypically he spent the last 7 months in hospital allowing continuous assessment. At autopsy, spongiform encephalopathy was demonstrated, and inoculation of brain tissue into 4 squirrel monkeys and 1 capuchin monkey resulted in the development of kuru. This is the longest continuous study of kuru in a hospital setting to be recorded in the adult human subject. The virus isolated from the brain of this patient has been adopted as a standard reference strain of kuru for future use and repository.

Distribution of Angiostrongylus cantonensis in Papua New Guinea.

Examination of 97 rats trapped in Port Moresby and Lae on the mainland of Papua New Guinea and in Rabaul, East New Britain Province, showed that 12.4% were infected by Angiostrongylus cantonensis. Larval An. cantonensis were found in Achatina fulica snails collected at these towns, from the Ramu Valley near Madang on the mainland and at locations in West New Britain, New Ireland and Bougainville Island. The mollusc infection rates ranged from 12.7% at Port Moresby to 32.4% at Kimbe in West New Britain (mean: 19.2%). Larvae obtained from snails from Port Moresby, Lae, the Ramu Valley and Rabaul were fed to laboratory rats and mature nematodes obtained subsequently provided confirmation that the species was An. cantonensis. Ach. fulica has not yet colonized the Highland region of Papua New Guinea and rats trapped in Goroka were free of infection. Human angiostrongyliasis has now been reported from Rabaul and studies have shown a high level of immunological responsiveness to the parasite in the sera of residents of East New Britain.

Angiostrongylus cantonensis in East New Britain, Papua New Guinea.

The first case of angiostrongyliasis (eosinophilic meningitis) to be diagnosed in Papua New Guinea in 1977, led to a survey of adult Melanesian residents of East New Britain province where the case occurred, to assess the level of immunological responsiveness to Angiostrongylus cantonensis. In 104 subjects tested using an indirect fluorescent antibody test, the serum antibody titres to A. cantonensis equalled or exceeded 1:16 in 50.7% and ranged from 1:32 to 1:64 in 17.3% of subjects. No individual tested showed evidence of active or recent angiostrongyliasis but in a patient suspected to have ocular angiostrongyliasis, the antibody titre of A. cantonensis was 1:128 in the single serum specimen obtained. In a programme to determine the distribution of this parasite in the natural hosts, 19 rats were trapped; one had adult A. cantonensis in its pulmonary arteries. Examination of 200 Achatina fulica snails revealed Angiostrongylus larvae in 62 (31%) and when these larvae were fed to laboratory rats, adult A. cantonensis were subsequently recovered from their pulmonary arteries.

Chronic pulmonary cryptococcosis in a Rattus rattus from Rabaul, Papua New Guinea.

A wild Rattus rattus trapped in Rabaul, East New Britain Province, Papua New Guinea, was noted to have chronic, cystic pulmonary cryptococcosis. R. rattus is common in settlements in Papua New Guinea whereas pigeons, which have been suspected as a possible source of human cryptococcosis, rarely nest in villages in this country. If R. rattus was susceptible to chronic pulmonary cryptococcosis, because of its habit of nesting in roofs, it could serve as a possible vector of human cryptococcosis in Papua New Guinea and perhaps in other countries as well.

Crimean-Congo haemorrhagic fever virus infection in the western province of Saudi Arabia.

In 1990, an outbreak of suspected viral haemorrhagic fever involving 7 individuals occurred in Mecca in the Western Province of Saudi Arabia. Congo-Crimean haemorrhagic fever (CCHF), not previously known to be present in Saudi Arabia, was incriminated. A study of the epidemiology of this virus was therefore carried out in Mecca, and in nearby Jeddah and Taif in 1991-1993; 13 species of ixodid ticks (5 Hyalomma spp., 5 Rhipicephalus spp., 2 Amblyomma spp., 1 Boophilus sp.) were collected from livestock (camels, cattle, sheep, goats), and of these 10 were capable of transmitting CCHF. Camels had the highest rate of tick infestation (97%), and H. dromedarii was the commonest tick (70%). Attempts to isolate virus from pools of H. dromedarii and H. anatolicum anatolicum were unsuccessful. The source of infection in 3 confirmed cases of CCHF was contact with fresh mutton and, in a suspected case, slaughtering sheep. An investigation in Mecca, which included a serological survey of abattoir workers, identified 40 human cases of confirmed or suspected CCHF between 1989 and 1990, with 12 fatalities. Significant risk factors included exposure to animal blood or tissue in abattoirs, but not tick bites. It is suspected that the CCHF virus may have been introduced to Saudi Arabia by infected ticks on imported sheep arriving at Jeddah seaport, and that it is now endemic in the Western Province.

A probable case of ocular angiostrongyliasis in New Britain, Papua New Guinea.

Unilateral iritis, keratitis and episcleritis presented in a 45-year-old female Melanesian from New Britain, Papua New Guinea. A motile nematode was seen in the anterior vitreous but was not recovered. Inflammation resolved with topical corticosteroid and antibiotic treatment, but visual impairment persisted. She failed to return for repeat serology after discharge, and measurement of serum antibody levels to Angiostrongylus cantonensis in the single specimen obtained, using the direct and indirect immunofluorescent tests, showed a titre of 1:128. Ocular angiostrongyliasis is briefly reviewed and the differential diagnosis of intra-ocular nematode infestation is presented.

Ross River virus arthritis in Papua New Guinea.

In 1975 it was reported that antibodies to Ross River virus (RRV) were present in the sera of many population groups in Papua New Guinea. We describe here 3 cases of polyarthritis that occurred in Port Moresby, the capital of Papua New Guinea, during 1980-81 and in which the diagnosis of RRV infection was confirmed by serological tests, and 3 other cases in which serological tests suggested RRV infection but were not diagnostic. A possible case of fatal RRV encephalitis is also reported.

Levels of serum cholesterol, triglyceride, HDL-cholesterol, apoproteins A-I and B, and plasma glucose, and prevalence of diastolic hypertension and cigarette smoking in Papua New Guinea highlanders.

Coronary heart disease (CHD) is rare in Papua New Guinea (PNG) highlanders. Fifty-two men and 69 women randomly selected from three rural communities and a low socioeconomic urban community in the Eastern Highlands Province were assessed for hyperlipidemia, diabetes mellitus, diastolic hypertension and cigarette smoking. There was no significant difference between the findings in the rural and urban groups. The mean fasting levels of serum cholesterol, HDL cholesterol and apoproteins A-I and B were significantly lower (p less than 0.001) than those of rural Australians in a comparative study but the serum triglyceride levels were significantly higher in men less than 30 yr and women less than 40 yr of age. There was no significant difference in the serum cholesterol levels in men and women, and the levels of serum cholesterol and triglyceride did not rise with age. The mean fasting levels of plasma glucose were generally lower in PNG subjects and only two (1.7%) had diabetes mellitus. The proportions of highlanders who had diastolic hypertension or who smoked cigarettes were similar to those of Australian populations generally. The low incidence of CHD in PNG highlanders is probably related to the low serum cholesterol and apoprotein B levels, in turn probably related to their basically vegetarian diet and physically active life-style.

Friedreich's ataxia, with retained lower limb tendon reflexes, in a Saudi Arabian family.

Friedreich's ataxia (FA) was studied in a large inbred Arab family living near Jeddah, Saudi Arabia, in which DNA linkage studies localised the disease gene to 9q13-q21.1. Five siblings (aged 19-35 years), and their 27 year old cousin, had the typical features of FA, however in two patients, tendon reflexes were retained and were indeed brisk in the lower limbs, 13 and 19 years respectively after onset of symptoms: retention of lower limb tendon reflexes is exceptional in FA. Another 6 deceased individuals from two related families are presumed to have had FA.

Cavernous sinus thrombophlebitis in Zimbabwe.

Cavernous sinus thrombophlebitis (CST) was diagnosed in 19 black African patients who presented to two large, general hospitals in Harare, Zimbabwe, over an eight-year period. Diagnosis was based on clinical criteria. The mean age of patients was 22.5 years (range 8 months-70 years). Only three patients (15.5 pc), all of whom were promptly diagnosed and commenced on a regimen including intravenous, high-dose cloxacillin, recovered completely. In ten cases (52.6 pc), initial treatment was penicillin and chloramphenicol. Four of the 19 patients who had serious sequelae including residual blindness, complete ophthalmoplegia or unilateral proptosis, and six patients (31.6 pc) died. The higher than usual mortality rate in this series can be attributed to various factors including late presentation, delay in diagnosis and delay before initiation of effective antibiotic treatment. Whenever CST is suspected, antibiotic treatment should be administered without delay pending further evaluation, and the initial regimen should include high-dose antistaphylococcal antibiotics.

Do-it-yourself information notes for AIDS counselling in developing countries.

PIP: Provision of a health information booklet is an important component of AIDS prevention counseling. This measure is essential because people may not fully understand verbal explanations or need time to think about the details. In developing countries, however, implementation of this strategy may be limited by the expense involved in printing booklets, difficulties maintaining adequate supplies to meet the demand, the presence of multiple languages in a given area, and high rates of illiteracy. Recommended, in such a context, is the preparation of basic explanatory information and graphic illustrations on a single sheet of paper that can be duplicated as necessary or translated into local languages. Local customs or religious beliefs, as well as community risk factors (e.g., scarification by traditional healers), should be considered in writing the text. An appendix presents a sample information sheet that was prepared in Swahili, Arabic, and English for Omani patients attending the infectious disease clinic of the Sultan Qaboos University Hospital.^ieng