Nakalanga Syndrome: Clinical Characteristics, Potential Causes, and Its Relationship with Recently Described Nodding Syndrome.

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.

Belief systems of epilepsy and attitudes toward people living with epilepsy in a rural community of northern Tanzania.

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.

Clinical characteristics of people with head nodding in southern Tanzania.

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.

The role of Onchocerca volvulus in the development of epilepsy in a rural area of Tanzania.

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.

Attitudes towards African traditional medicine and Christian spiritual healing regarding treatment of epilepsy in a rural community of northern Tanzania.

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.

The head nodding syndrome--clinical classification and possible causes.

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.