First familial Becker muscular dystrophy in Tanzania: Clinical and genetic features.

In African neurological practice, muscle disorders are either underdiagnosed or underrepresented. This may in part be due to the large burden of other more common neurological disorders. In this report we describe the first Tanzanian patient with genetically confirmed Becker muscular dystrophy. His phenotype and genotype were compatible with elsewhere in the world. Remarkably, this patient reported his progressive weakness of the legs with difficulty in walking only after a fall. We demonstrate that muscular dystrophies occur in sub-Saharan Africa. Neurologists must however be aware that patients are likely to delay seeking medical care for muscle disorders.

Diabetes is not caused by cassava toxicity. A study in a Tanzanian community.

OBJECTIVE: To test the hypothesis that consumption of cassava with liberation of cyanide causes diabetes in malnourished individuals. RESEARCH DESIGN AND METHODS: Glucose tolerance was assessed in two rural communities in Tanzania; in one (Nyambori), the main source of calories was cassava; and in the other (Uswaa), cassava was rarely eaten. Undernutrition was prevalent in both communities. The people of Nyambori were known to have high dietary cyanide exposure for many years from consumption of insufficiently processed cassava. Of the 1435 people in Nyambori greater than or equal to 15 yr old, 1067 (74%) were surveyed, and 1429 of 1472 (97%) eligible subjects in Uswaa were surveyed. All had 75-g oral glucose tolerance tests and measurement of BMI. Plasma and urine thiocyanate and blood cyanide also were measured in some subjects. RESULTS: Mean +/- SD plasma and urine thiocyanate levels in Nyambori were 296 +/- 190 and 497 +/- 457 microM (n = 204), respectively, compared with 30 +/- 37 and 9 +/- 13 microM, respectively, in Uswaa (n = 92) (P less than 0.001 for all differences). The mean blood cyanide level in Nyambori was elevated (1.4 [range 0.1-30.2] microM; n = 91). The prevalence of diabetes in the cassava village (Nyambori) was 0.5% compared with 0.9% in Uswaa (NS). The prevalence of IGT was similar in the two villages in the 15- to 34- and the 34- to 54-yr-old age-groups; but in those greater than or equal to 55 yr old, IGT was higher in Nyambori (17.4 vs 7.2%, P = 0.029). Mean fasting and 2-h blood glucose levels were slightly higher in Nyambori village after adjusting for age, sex, and BMI (4.5 vs. 4.2 and 5.0 vs. 4.4 mM, respectively). CONCLUSIONS: High dietary cyanide exposure was not found to have had a significant effect on the prevalence of diabetes in an undernourished population in Tanzania. Cassava consumption is thus highly unlikely to be a major etiological factor in so-called MRDM, at least in East Africa.

Neurological disorders in AIDS and HIV disease in the northern zone of Tanzania.

This study presents the main clinical findings on 200 AIDS patients at Kilimanjaro Christian Medical Centre in the northern zone of Tanzania, with detailed neurological findings on 135 out of 200 cases and 53 controls. Results show that 21 out of 200 (10.5%) had an obvious focal neurological disorder, including cranial nerve palsies, hemiparesis and paraparesis. Ninety-seven out of 135 (72%) had less obviously detectable neurological disorders, versus 36% of controls (P less than 0.005). Most frequent were AIDS dementia complex (54%), retinopathy (23%), areflexia (21%), pyramidal tract signs (19%) and tremor and incoordination (19%). Frontal lobe release signs (FLRS) were found in 103 out of 135 (76%) patients, versus 36% of controls (P less than 0.005). Advanced and terminal AIDS cases were more likely to have neurological disorders than early AIDS patients. A further study on 87 non-AIDS patients with acute unexplained neurological disorders showed 10 out of 87 to be HIV seropositive. Three case studies are presented. This study suggests that neurological disorders are among the main clinical features of AIDS and HIV disease in Africa.

[AIDS in the Third World. Africa suffers most]. / AIDS i den tredje verden. Afrika er hardest rammet.

AIDS is now world-wide, and the HIV infection is spreading rapidly via the heterosexual route. Among the Third World countries those in sub-Saharan Africa are the hardest hit, it is estimated that one in 40 of the adult population is already infected. Half of the victims are women, who will give birth to a large number of infected children. The clinical picture of full-blown AIDS in an African patient is not very different from elsewhere. However, other AIDS-related conditions seem to be influenced by a variety of endogenic pathogens which might explain the development of the typical wasting syndrome, i.e. "Slim" disease. Tuberculosis is the most typical opportunistic infection in Africa, and adds another dimension to the misfortune. The outlook is gloomy in the light of the potential for widespread disruption of normal social and economic activities.

PIP: AIDS is now worldwide, and the HIV infection is spreading rapidly via the heterosexual route. Among Third World countries, those in sub-Saharan Africa are hardest hit, and it is estimated that 1 in 40 adults is already infected. 1/2 of these victims are women, who will give birth to a large number of infected children. The clinical picture of full-blown AIDs in an African patient is not very different from other locations. However, other AIDS-related conditions seem to be influenced by a variety of endogenic pathogens which might explain the development of the typical wasting syndrome; i.e., "Slim disease". Tuberculosis is the most typical opportunistic infection in Africa, and adds another dimension to the misfortune. The outlook is gloomy in light of the potential for widespread disruption of normal social and economic activities. (author's modified)

Konzo, an epidemic upper motor neuron disease studied in Tanzania.

An epidemic of spastic paraparesis was studied in a drought-affected rural area of Tarime district in northern Tanzania. The uniform clinical findings in 39 cases, aged 4-46 yrs, indicated abrupt symmetric isolated and permanent but not progressive damage to the upper motor neurons. Due to the failure of other food crops, the diet at onset consisted almost exclusively of bitter cassava roots, a drought-tolerant starchy root crop widely cultivated in Africa. The drought increased the natural occurrence of cyanogenic glucosides in the cassava roots, and the processing procedure normally applied in order to remove cyanide before consumption was shortened because of food shortage. The resulting high dietary cyanide exposure was confirmed by very high serum levels of thiocyanate, the detoxification product of cyanide. Tests for HTLV-1 antibodies were negative and no other findings supported an infectious aetiology. The clinical findings and the associations with cassava toxicity are almost identical to those reported from outbreaks of spastic paraparesis in Mozambique and in Zaire, where this disease was first reported under the name 'konzo'. We thus conclude that konzo constitutes a distinct upper motor neuron disease entity, probably caused by a toxic effect from insufficiently processed cassava under adverse dietary circumstances.

The snout and palmomental reflexes in HIV disease in Tanzania.

Following earlier observations on the snout (SR) and palmomental(PMR) reflexes in AIDS in Tanzania, a series of 1127 adults, 649 HIV-positive and 478 HIV-negative, from 4 groups at different risk of HIV infection were examined neurologically between 1987 and 1992. The prevalence of SR and PMR was calculated according to HIV status, HIV stage, demographic factors and neurologic findings. In the total series of HIV positives the prevalence ranged from SR 39.3% and PMR 22.6% in asymptomatic HIV disease to SR 87% and PMR 69% in terminal AIDS. In HIV negatives the prevalence of SR was 19.2% and PMR 15.3%, and increased with age. There was no relationship with age in the HIV positives and no gender differences. SR and PMR were also associated with neuropathy, myelopathy and AIDS dementia complex independent of HIV stage. This study shows a strong association between SR and PMR and HIV disease in Africa. The prevalence increased with HIV stage and related neurological disorders.

Guillain-Barré syndrome in northern Tanzania: a comparison of epidemiological and clinical findings with western Norway.

To study Guillain-Barré syndrome (GBS), the clinical files of GBS patients, 59 in northern Tanzania (1984-1992) and 56 in western Norway (1980-1992), were retrospectively reviewed and compared. The average annual incidence rate for GBS in the Kilimanjaro region was 0.83/100,000 population as compared to 1.2/100,000 reported in western Norway (25). GBS patients in the Tanzanian series were younger, had less antecedent infection and were a longer time interval from onset to admission and in hospital. On examination arm and sensory involvement were less common and urinary sphincteric involvement more common in the Tanzanian series. The overall mortality rate was higher in the Tanzanian series, 15.3% (9/59) versus 1.8% (1/56). HIV infection was found in 30.5% (11/36) of GBS patients in the Tanzanian series as compared to 3.4% (161/4687) in corresponding blood donors. There was no evidence of HIV infection in the Norwegian series. The HIV-seropositive GBS patients in comparison with HIV seronegatives had a shorter duration of onset, greater neurological involvement and a higher mortality rate, 45.5% (5/11) versus 16% (4/21). This study shows that apart from minor clinical differences and an increased mortality rate in the Tanzanian series GBS was similar in both series. GBS in the Tanzanian series was strongly associated with HIV infection, and seropositives by comparison with seronegatives appeared to have more severe disease.

Konzo: a distinct disease entity with selective upper motor neuron damage.

Two Tanzanian patients with konzo were severely disabled by a non-progressive spastic paraparesis, since the sudden onset during an epidemic six years earlier. At the time of onset they had a high dietary intake of cyanide from exclusive consumption of insufficiently processed bitter cassava roots. MRI of brain and spinal cord were normal but motor evoked potentials on magnetic brain stimulation were absent, even in the only slightly affected upper limbs. Other neurophysiological investigations were largely normal but the more affected patient had central visual field defects. Konzo is a distinct disease entity with selective type upper motor neuron damage.