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Nakalanga syndrome is a condition that was described in Uganda and various other African countries decades ago. Its features include growth retardation, physical deformities, endocrine dysfunction, mental impairment, and epilepsy, amongst others. Its cause remains obscure. Nodding syndrome is a neurological disorder with some features in common with Nakalanga syndrome, which has been described mainly in Uganda, South Sudan, and Tanzania. It has been considered an encephalopathy affecting children who, besides head nodding attacks, can also present with stunted growth, delayed puberty, and mental impairment, amongst other symptoms. Despite active research over the last years on the pathogenesis of Nodding syndrome, to date, no convincing single cause of Nodding syndrome has been reported. In this review, by means of a thorough literature search, we compare features of both disorders. We conclude that Nakalanga and Nodding syndromes are closely related and may represent the same condition. Our findings may provide new directions in research on the cause underlying this neurological disorder., Competing Interests: The authors have declared that no competing interests exist.

Objectives: Nodding syndrome (NS), a new epilepsy disorder of sub-Saharan Africa, has only recently been classified. In a study conducted in southern Tanzania in 2005, 62 patients with NS were analyzed in great detail. The present study, a follow-up investigation, was conducted to evaluate the progression of NS over time and to obtain serial electroencephalography (EEG) data., Methods: Of the 62 NS patients, 53 (85.5%), the majority of whom were currently on some form of antiepileptic treatment, could be reevaluated in 2009 with a standardized questionnaire. A subset of these patients (25/53) underwent EEG investigation., Results: In patients with "head nodding (HN) only" in 2005, 10 (43.5%) of 23 remained with the same diagnosis, whereas 5 (21.7%) of 23 had developed "HN plus" (i.e., HN and generalized tonic-clonic seizures). Six patients (26.1%) had seizures other than HN only, and two patients (8.7%) had fully recovered. In the "HN plus" group of 2005, 9 (30.0%) of 30 patients remained "HN plus," and 15 patients (50.0%) had seizures other than HN only. Four patients (13.3%) reverted to "HN only," and two patients (6.7%) stopped all seizures. In 11 (44.0%) of 25 patients, electroencephalography (EEG) showed generalized slowing. Six (54.6%) of these 11 abnormal EEG studies further showed generalized epileptiform discharges: (1) ictal electroencephalographic pattern with generalized 2.5 Hz spike and waves in two patients and (2) interictal bursts of 1.5-2 Hz spike and waves in four patients., Significance: This follow-up study confirms that HN represents an epilepsy disorder, possibly of the atypical absence type with dynamic development over time., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

The social stigma toward people with epilepsy (PWE) varies greatly between cultures. In this study, 167 people (59 PWE, 62 relatives, 46 villagers) in a rural area of northern Tanzania were interviewed at the hospital and in the community regarding their prevailing beliefs about epilepsy and attitudes toward PWE. Seventy-eight of those interviewed (46.7%) thought that epilepsy was due to supernatural causes, but 86 (51.5%) assumed that epilepsy is caused by brain disorders or is inherited. According to the interviewees, epilepsy impacts on the lives of affected people. 65.3% (n=109) thought that PWE should not attend school or go to work and 38.3% (n=64) were of the opinion that PWE had decreased chances of getting married. A minority (11.4%; n=19) thought that epilepsy was a reason not to have children. In summary, supernatural and more scientific ideas about the causes of epilepsy seem to coexist. Nevertheless, there is considerable stigma toward PWE, which needs to be interpreted within the sociocultural context of the study., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Introduction: Several reports indicate high prevalences of both onchocerciasis and epilepsy in some regions of Africa. This raises the question of whether these diseases are associated. We therefore investigated people with epilepsy and/or onchocerciasis living in an area in Tanzania endemic for Onchocerca volvulus (O. volvulus)., Methods: We collected clinical information, skin snips, and blood from 300 individuals, and cerebrospinal fluid (CSF) from 197. Participants were allocated to 4 groups consisting of people with epilepsy and onchocerciasis (n=135), those with either epilepsy (n=61) or onchocerciasis only (n=35), and healthy individuals (n=69). Samples were evaluated for microfilaria, IgG4 antibodies against O. volvulus, O. volvulus antibody index (CSF/serum), and CSF routine parameters. Polymerase chain reaction (PCR) was performed on skin snips and CSF., Results: No difference was found in microfilarial density between participants with and without epilepsy (P=0.498). The antibody index was raised in 2 participants. CSF PCR was negative in all samples tested., Discussion: Our results do not give evidence of a relationship between O. volvulus and epilepsy. Despite the fact that 2 participants had raised antibody index, the existence of cerebral onchocerciasis caused by migration of microfilariae into the CSF appears unlikely. However, to date unexplored reactions to the infestation with O. volvulus causing epilepsy cannot be excluded.

We have previously described a seizure disorder characterized by head nodding (HN). In a prospective study in southern Tanzania, we evaluated 62 patients with HN. Here, we report the patients' clinical characteristics and those of their seizures, which indicate high seizure frequency, unsatisfactory seizure control, a high burden of cognitive impairment and disease-associated barriers to education.

Most people with epilepsy (PWE) live in developing countries with limited access to health care facilities. In sub-Saharan Africa with approximately 12 million PWE, 90% do not receive adequate medical treatment. In this context, traditional medicine, being easily accessible, plays an important role. However, in sub- Saharan Africa, studies on the attitude of people (both affected and not affected by epilepsy) towards traditional medicine for treatment of epilepsy are scarce. In this study, 167 people (59 PWE, 62 relatives, 46 villagers) were interviewed at the hospital and in the community with a semi-structured validated questionnaire regarding the prevailing attitude towards traditional medicine for treatment of epilepsy in a rural area of northern Tanzania. Various traditional healing methods (THM) could be ascertained, i.e. traditional herbal medicine, spiritual healing, scarifications and spitting. 44.3% (n=74/167) of the interviewed people were convinced that epilepsy could be treated successfully with THM. Interestingly, 34.1% (n=57/167) thought that Christian prayers could cure the cause and/or treat symptoms of epilepsy. Significantly more PWE and their relatives were in favour of THM compared to villagers not knowing about epilepsy or not being immediately affected by epilepsy (χ(2)-test, p=0.004). Further factors influencing people's attitudes towards THM were gender, tribe, religion and urbanity of people's dwellings. Our study demonstrates that not only THM but also prayers in the Christian sense seem to play an important role in people's beliefs regarding successful treatment of epilepsy. Factors influencing this belief system have been identified and are discussed.

Purpose: In the 1960s in Tanzania, L. Jilek-Aall observed a seizure disorder characterized by head nodding (HN). Decades later, "nodding disease," reminiscent of what was seen in Tanzania, was reported from Sudan. To date this seizure disorder has not been classified and possible causes still remain obscure., Methods: In a prospective study in southern Tanzania, we evaluated 62 patients with HN. Selected patients underwent blood (n = 51) and cerebrospinal fluid (CSF) (n = 48) analyses. Others were chosen for MRI (n = 12) and EEG (n = 10)., Results: Seizure type was classified as "head nodding only" and "head nodding plus," the latter being combined with other types of seizure (n =34). During HN, consciousness was impaired in 11 patients (17.7%) and supportive signs of epileptic seizures were described by 15 (24.2%) patients. Precipitating factors were confirmed by 11 (17.7%) patients. Fifty-six (90.3%) patients had at least one relative with epilepsy. EEG confirmed interictal epileptic activity in two patients and unspecific changes in four patients. MRI showed hippocampus pathologies (n = 5) and gliotic changes (n = 5). Skin polymerase chain reaction (PCR) positivity for Onchocerca volvulus was significantly associated with lesions on MRI. However, PCR of the CSF was negative in all cases., Conclusions: We present a comprehensive clinical description of the "HN syndrome," possibly a new epilepsy disorder in sub-Saharan Africa. MRI lesions and their association with positive skin PCR for O. volvulus despite negative PCR of the CSF is intriguing and deserves attention. Furthermore, the high prevalence of hippocampus sclerosis and familial clustering of epilepsy may point toward other potential pathogenetic mechanisms.

Epilepsy when manifested as grand mal seizure provokes strong and ambivalent feelings in those witnessing it. Terms such as morbus sacer, denoting both a sacred and demoniac condition, or folk names indicating divine punishment, have expressed these feelings in European societies from antiquity to the Middle Ages and beyond. An atmosphere of fear, shame and mysticism surrounds epilepsy even in our days in many non-Western and also in Western cultures. In the course of work and studies in Tanzania, where I organized the Mahenge Clinic for Epilepsy in 1960, and in other parts of Africa, I found that epilepsy is conceived of as an "African'' affliction, a manifestation of supernatural forces that makes it difficult to reach epilepsy sufferers with modern medical treatment. Epilepsy is traditionally looked on as caused by ancestral spirits or attributed to possession by evil spirits. It is also thought to be due to witchcraft, and "poisoning," and often taken to be contagious. Epilepsy may, under Christian missionary teaching, have come to be considered as due to demoniac possession or divine punishment for sins, in accordance with biblical examples. In many parts of Africa, syncretic amalgamation of indigenous traditions with Judeo-Christian doctrines influenced popular attitudes toward epilepsy. We demonstrated that persistent efforts at health education in the context of organized treatment of epilepsy can result in a change of popular notions about epilepsy and consequently lead to significant improvement in the quality of life of epilepsy sufferers.

As documented by many authors, the social position of epileptics in many small scale societies of Africa is marginal at best, and is often characterized by rejection, discrimination, even ostracism. Such negative and noxious attitudes toward persons suffering from epilepsy are rooted in traditional beliefs about causes and nature of convulsive disorders and these have parallels in European history. This article focuses on the psychosociocultural aspects and indigenous concepts of epilepsy, on popular attitudes towards, and social status of, sufferers from epilepsy in a Tanzanian tribal population. The authors present a comparative analysis of focus group discussions conducted with epileptics and with matched controls in two isolated communities. In one community (Mahenge) a clinic for epilepsy has been operating for over 36 years, with a public education component during the last four years, whereas in the other community (Ruaha) epileptics have only been sporadically treated in a small mission dispensary and people have had little opportunity to learn about the nature and modern treatment of convulsive disorders. The responses obtained in focus group discussions reflect the significant change in notions about the illness, in the attitude toward and in the social status of epileptics in Mahenge, while the people of Ruaha still regard epilepsy as a typical "African" affliction fraught with supernatural danger and not effectively treatable by modern medicine.

Kifafa is the Swahili name for an epileptic seizure disorder, first reported in the early 1960s, that is prevalent in the Wapogoro tribe of the Mahenge region of Tanzania in eastern Africa. A 1990 epidemiological survey of seizure disorders in this region reported a prevalence in the range of 19/1,000-36/1,000, with a mean age at onset of 11.6 years; 80% of those affected had onset prior to 20 years of age. A team of investigators returned to Tanzania in 1992 and collected data on > 1,600 relatives of 26 probands in 20 kifafa families. We have undertaken a genetic analysis of these data in order to detect the presence of familial clustering and whether such aggregation could be attributed to genetic factors. Of the 127 affected individuals in these pedigrees, 23 are first-degree relatives (parent, full sibling, or offspring) of the 26 probands; 20 are second-degree relatives (half-sibling, grandparent, uncle, or aunt). When corrected for age, the risk to first-degree relatives is .15; the risk to second-degree relatives is .063. These risks are significantly higher than would be expected if there were no familial clustering. Segregation analysis, using PAP (rev.4.0), was undertaken to clarify the mode of inheritance. Among the Mendelian single-locus models, an additive model was favored over either a dominant, recessive, or codominant model. The single-locus model could be rejected when compared with the mixed Mendelian model (inclusion of a polygenic background), although the major-gene component tends to be recessive.(ABSTRACT TRUNCATED AT 250 WORDS)

While working as a physician in Tanzania in the early 1960s, Dr. Louise Jilek-Aall founded an outpatient clinic for epilepsy among the Pogoro people of Mahenge mountains where epilepsy (locally termed Kifafa) had brought misery and death to an unusually high percentage of the population. With local assistance and overseas donations of phenobarbital (PB), this clinic treated approximately 200 patients for less than or equal to 10 years. The area was revisited 30 years later to trace these patients. Of the 164 patients who had started treatment, 86 (52.4%) achieved complete seizure suppression, 59 (36.0%) experienced reduction in seizure frequency, 13 (7.9%) experienced no change, and in 1 (0.6%) seizures were worse. The effect of treatment could not be assessed in 5 (3.0%) patients. After 30 years, only 36 (21.9%) of the 164 patients were known to be alive. Of the patients, 110 (67.1%) had died, and the whereabouts of 18 (11%) could not be traced. The causes of death were epilepsy related (status epilepticus, drowning, burns, dying in or after a seizure) in greater than 50% of the patients. Epilepsy-related deaths were proportionately higher after drug supply was stopped and among patients who were receiving drugs irregularly or who had only partial seizure control. Patients with epilepsy showed an increased mortality rate, which was twice that of the general rural Tanzanian population of similar age. Management of epilepsy in rural Africans should also emphasize methods to prevent epilepsy-related causes of death among patients with epilepsy.

Starting from a critical review of the concept of 'culture-bound' disorders and its development in comparative psychiatry, the authors present the changing aspects of two so-called culture-bound syndromes as paradigms of transcultural metamorphosis (koro) and intra-cultural metamorphosis (Salish Indian spirit sickness), respectively. The authors present recent data on epidemics of koro, which is supposedly bound to Chinese culture, in Thailand and India among non-Chinese populations. Neither the model of Oedipal castration anxiety nor the model of culture-specific pathogenicity, commonly adduced in psychiatric and ethnological literature, explain these phenomena. The authors' data on Salish Indian spirit sickness describes the contemporary condition as anomic depression, which is significantly different from its traditional namesake. The traditional concept was redefined by Salish ritual specialists in response to current needs imposed by social changes. The stresses involved in creating the contemporary phenomena of koro and spirit sickness are neither culture-specific nor culture-inherent, as postulated for 'culture-bound' syndromes, rather they are generated by a feeling of powerlessness caused by perceived threats to ethnic survival.

Initiation ceremonials in traditional Papua New Guinea and North American Indian cultures serve important psychohygienic functions in establishing the youth's final identity and thereby warding off the frustration, anxiety, and depression which are associated with anomie and role confusion. Inititations in Papua New Guinea are presented as a process of social learning in which group consciousness and loyalty are established through revelation of ancestral secrets, testing by ordeals, and ego-stregthening rewards. Structually patterned archetypal collective symbols gain direct access to the young person's unconscious when skillfully transmitted in the initiatory psychodrama of death and rebirth. Medical complications occuring during initiation procedures are rare accidents which have to be weighed against the psychological and social benefits for individual and group. Initiation ceremonials help the young to achieve a sense of sexual and socio-cultural identity from which feelings of emotional security and social belonging are derived.

Koro, a psychogenic anxiety syndrome interfering with genital body image and sexual functioning, has hitherto been described as occurring mainly in isolated cases of South Chinese males. The present communication reports an epidemic outbreak in November 1976 in Northeastern Thailand where within a few days at least 200 patients, most of them Thai and two-thirds males, were treated at local hospitals. Main presenting symptoms were acute anxiety, in some cases leading to fainting, (subjective) shrinking of the penis and impotency in men, shrinking and/or itching of the external genitals and frigidity in women; further complaints included initial nausea and dizziness, abdominal pains, headaches, facial numbness. All patients recovered after brief symptomatic intervention. Popular opinion and news media echoed the patients' paranoid projection of viewing the epidemic as caused by Vietnamese food and tobacco poisoning in a hideous assault against the sexual vitality and general health of the Thai people, in the context of a specific socio-cultural and politico-historical situation. It appears that an adequate interpretation of Koro and of analogous hysterical symptom formation would have to go beyond the hitherto applied psychoanalytic models by considering the specific sociodynamic factors involved in the pathogenesis of such phenomena.