Nodding disease is a neurological disorder of children and adolescents that is endemic in South Sudan, Tanzania, and northern Uganda. The disease is characterized by constant nodding seizures at mealtimes and gradual physical and mental deterioration. Typically, nodding disease leads to death within a few years.
What is nodding disease?
Nodding disease is a disease that occurs only in East Africa. It has been observed in Tanzania and South Sudan since the early sixties. In this case, it is characterized by nodding seizures during eating or when cold, and by gradual mental retardation. A satisfactory explanation for its development could not be given until today. Above all, the neurotoxicologist Peter Spencer investigated the disease more closely. He was able to identify typical symptoms. At present, however, only conjectures can be made as to the cause. Peter Spencer described nodding disease as a slowly progressing fatal functional disorder. He suggested an average lifespan of individuals suffering from this disease of between three and four years. However, there are also cases where this disease has existed for over ten years. Even cures have been reported. The nodding seizures are also often associated with classic epileptic seizures. Brain wave studies have shown that the nodding seizures are accompanied by abnormal brain wave patterns similar to those seen in epilepsy. The disease currently occurs only in South Sudan in riverine settlements, with a prevalence of 2.3 to 6.7 percent. By 2008, the disease had also spread to some areas of northern Uganda.
Causes
To date, only speculation can be made regarding the cause of nodding disease. To date, it is not clear what causes the disease in the first place and why it occurs only in a limited territory. However, it is suspected that it is an infectious disease or an autoimmune disease triggered by an infection. Another conjecture focuses on chronic poisoning from environmental toxins that contaminated the area during the civil war. However, there is a strong hint regarding a connection with the nematode Onchocerca volvulus. It is already known that this worm is spread by the blackfly and is the causative agent of river blindness. The nematode was detected in almost all patients affected by nodding disease. However, it is also strange that there are no cases of nodding disease in other areas where this nematode is spread. Therefore, the assumption for further co-factors for the development of this disease is obvious. These could be chemicals that have not been detected so far. There is also the possibility that the nematode in this area is a carrier of specific microorganisms or parasites, which could be true triggers of nodding disease. Autoimmune disease in response to infection is also thought to be a possibility.
Symptoms, complaints, and signs
Nodding disease, as mentioned earlier, is a slowly progressive neurological disorder whose main symptom is constant nodding movements of the head. The affected child stops growing and mental development stops. Over time, even mental retardation takes place. The nodding seizures are triggered when eating or even just looking at traditional food or when cold. If unfamiliar foods such as chocolate are served, nodding seizures do not occur. Nodding seizures also stop after the meal is over. During an attack, between 10 to 20 nodding movements of the head may occur. In very severe seizures, collapse may even occur. This often leads to further damage. It is not uncommon for the children to fall down and injure themselves considerably. It has already happened that affected persons have fallen into open fireplaces or onto pointed objects. During the seizure, the children also become disoriented and often get lost. The prognosis of the disease is very poor. According to previous experience, it is not curable and even progresses. After several years, nodding disease is usually fatal. There are different statements about the duration of the disease. According to some observations, nodding disease should lead to death within three to four years on average. On the other hand, there have also been reports of people suffering from this disease for more than ten years.However, there are also statements that a few young people have also recovered.
Diagnosis and course of the disease
Nodding disease is diagnosed mainly on the basis of typical symptoms. Brain wave measurements have demonstrated norm deviant brain wave patterns during nodding seizures. MRI scans may reveal severe brain mass wasting. The hippocampus and glial cells also show severe damage. So far, however, the studies have not provided any clues as to the true triggers of the disease.
Complications
As a variant of early childhood epilepsy, nodding syndrome almost always leads to complications. Unfortunately, the associated nodding seizures do not remain the only symptom. However, this disease symptom alone can cause affected children to fall during a seizure. They sustain injuries, some of them severe. During the attacks, the affected persons are no longer in control of their limbs. Since the disease occurs primarily in African countries such as Uganda, the children often fall into open fireplaces or touch sharp objects during the fall. In addition, such children often get lost. Without any protection, the disoriented children easily become victims of wild animals. In addition, nodding disease is a disease that is usually fatal. It is progressive and a severe neurological disease. The biggest problem is the rarity and the narrow regional area in which nodding disease occurs. There is no medical care available there. Modern diagnostics are equally scarce. But even when these things exist, nodding disease is not yet curable. Why a number of sufferers experience complications and mental retardation but not death is unclear. It may be a reflection of a parasitic or infectious trigger that is common in these regions.
When should you see a doctor?
Parents who notice signs of mental retardation or the typical nodding seizures in their child should have it checked out by a doctor immediately. Prolonged symptoms must be examined and treated by a specialist in order to avert permanent damage. If circulatory collapse occurs, the emergency physician must be called. The affected child must then be treated in a hospital. If the described signs of illness occur during or after a stay in one of the risk areas, a doctor must be consulted immediately. At the latest after returning home, the traveler should undergo a comprehensive medical check-up and, if necessary, receive treatment. Nodding disease must be treated by a neurologist or other internist. Since the disease is usually associated with long-term damage, therapeutic treatment is also advisable. Since nodding disease is a progressive condition, close medical monitoring is also necessary. Otherwise, it can lead to further health problems, which further limit the quality of life of the sufferer.
Treatment and therapy
Because the causes are completely unclear, there are also no satisfactory treatment methods to date. So-called anticonvulsants are used. Anticonvulsants are drugs used to treat epileptic seizures. However, there is still no documentation on the extent to which the disease is influenced by these drugs. Antimalarials are also used. Again, no results have been published.
Outlook and prognosis
The prognosis of nodding disease is unfavorable. Despite all medical advances and efforts, the disease has so far been fatal for the affected person within a few years of life. One difficulty is that the disease has so far occurred exclusively in East Africa. Another challenge is that up to now no sufficient clarification of the cause could take place. Therefore, there are numerous questions that remain unanswered according to the current state of affairs and thus worsen or even prevent adequate medical care. The patients suffer from motor disorders as well as a reduced mental capacity. Uncontrolled seizures occur, which without the fastest possible medical care result in the immediate demise of the patient. Due to the existing symptoms, the general risk of injury is increased in those affected. Sudden accidents may occur, which show a life-threatening development.Open fire or sharp objects often become a health hazard. The patients are mostly disoriented and are therefore often defenseless in their home country at the mercy of the wilderness. They cannot classify natural dangers and react accordingly. Since the disease is considered incurable, relatives are often overwhelmed or show a rejecting behavior towards the patient for religious reasons. This further worsens the general situation and leads to more severe health conditions.
Prevention
Nothing can be said so far about prevention from nodding disease, because the real causes are not known. There are assumptions that the poor hygienic conditions still promote the outbreak of the disease. Certainly, protection against the nematode Onchocerca volvulus infestation plays an important role in the control of nodding disease.
Aftercare
Nodding disease is a poorly understood condition that has yet to be treated causally. Follow-up care focuses primarily on providing medical surveillance for a disease that has been cured. Regular check-ups ensure that the medication is optimally adjusted and that any complications are clarified quickly. In addition, any adverse events are clarified during follow-up care. Parents of affected children must inform the doctor about any falls or injuries, for example. If necessary, it may be useful to prescribe a sedative. Aftercare also includes remedying possible triggers. For this purpose, parents should consult regularly with the responsible medical professional. Follow-up care is provided by the physician who diagnosed and treated the condition. Sometimes other specialists have to be consulted, as the disease is extremely rare and the knowledge of a general practitioner is usually not sufficient. As part of the follow-up care, the symptoms are reduced with medication and behavioral therapy. It is also important to educate the affected children about their disease. Comprehensive education ensures that children suffering from nodding disease recognize seizures early and take the necessary preventive measures on their own in adolescence and adulthood.
Here’s what you can do yourself
Nodding disease is a usually fatal condition. Those affected can support the therapy in the first place by complying with the doctor’s instructions. Above all, strict personal hygiene and a balanced diet are important factors in the self-therapy of nodding disease. In addition, a diary should be kept in which the patient notes any accompanying symptoms and any side effects or interactions caused by the prescribed medication. Discussions with other patients, as well as with friends and family members, are often helpful. Especially in the case of a severe course of the disease, talking helps the patient to accept the disease. The relatives can support the affected person and often also contribute to recovery through a change in lifestyle. For example, a hygienic household helps to ensure that the infection at least does not spread further. In the case of a serious illness, a place in a palliative ward or hospice should be organized at an early stage. The relatives of the affected person should speak with the responsible physician for this purpose. Since the chances of recovery are relatively poor, therapeutic support may also be advisable, which supports the affected person and the relatives during the illness and also helps with organizational tasks.
