measures that should be taken for lenas before diagnosis. however, prevention of secondary complications can be taken once diagnosis is confirmed. prevention of secondary complications social problems such as unemployment, divorce, financial troubles, and alcoholism as well as suicidal tendencies are often associated as this disease worsens over time. some of these social consequences may be avoided if family members are informed early about the nature of this disorder as well as if it is diagnosed early enough. as depression is a major symptom associated, suicidal tendencies may occur. antidepressant medications can be prescribed for this depression for the attempt to help with depression but they have not shown long-term benefit to date. agents and circumstances to avoid the following should be avoided not to prevent the disease, but rather to prevent this disease from progressing faster and causing symptoms to worsen: use of first-generation neuroleptics: these increase seizure risk and risk of additional parkinsonian signs. treatment agents for multiple sclerosis: these have no benefit and have major side effects. treatment no specific therapy for lenas is currently known or proven to cure this disease, but management of it should be immediately initiated following diagnosis. while more research should be taken, some suggest that hematopoietic stem cell transplantation may show a therapeutic role for this disease. management management is important in support of this disease and includes attention to general care and nutrition requirements and other possible drug therapies that may help or slow the progression of the disease. l-dopa or other dopaminergic therapies have not yet been beneficial in individuals with this disease but was noted that it may be worth trying as it does not show negative effects. antipsychotics are in general not recommended due to extrapyramidal side effects. however, they may be used in aggressive individuals. anti-seizure medications should be initiated in individuals who are having seizure activity along with having this disease as it is reported to be beneficial. surveillance periodic clinical evaluation and surveillance to monitor the progression of the disease is appropriate to determine if changes need to be made: changes in mobility, communication, and behavior, which could indicate the need to alter care and support systems (for example, moving to a nursing facility or getting personal care, access to wheelchair or walker, etc). onset of seizure activity may cue in the need for anti-seizure therapy contractures, which could indicate the need to change medical management and physical therapy more severe behavioral changes that appeared to have worsened. this could include inappropriate emotions and actions, problems following directions, memory loss, and incontinence all which indicate curtailing of independence difficulties in swallowing or weight loss, which trigger physicians consideration for gastrostomy need for physical therapy to minimize contractures and maintain locomotion prognosis concept of a lumbar puncture being performed because of how rare this genetic condition is an exact prognosis is still not known and varies. some data has suggested that the median age of onset is 45 years old, but patients with onset as young as 18 years old has also been described. further, it is believed that the median life expectancy is 6 years but is also extremely variable as it has also been reported that some patients survived up to 29 years after the onset of symptoms. while gaining a definitive prognosis for this disease studies suggest a few different techniques that may or may not benefit in getting a better prognosis for the individual patient: lumbar puncture to measure neurofilament light protein (nfl) in the cerebrospinal fluid (csf) to follow the progression of the disease. an increase nfl level on csf examinations may suggest faster disease course as well as a worse prognosis. longitudinal mri studies annually can potentially also help with prognosis. this was found from another study which found that, as throughout the disease course, the more rapid the confluence of patchy or focal t2-weighted hyperintensities and the progression of cortical atrophy suggests the poorer the prognosis appears to be. epidemiology epidemiological studies for rare diseases are difficult to have exact and known values that are still difficult to find. however, most studies show the mean age of onset was 43 years old (range 18–78 years old), with a mean death at 53 years (range 23–84 years) and the mean disease duration was found to be 6.8 years (range 1–29 years). history lenas was first reported in multiple members of a large swedish pedigree in 1984. in this family, 17 out of 71 subjects from 4 generations were affected with this disease. it was found that the age onset of this family varied from 8 to 60 years of age. the age of death in this family was 39 to 89 years with a time between onset and death varied from 3 months to over 30 years. some patients in this family rapidly developed severe dementia and died a few months after this onset whereas others had a more prolonged progression of the disease. this family was also to have reported sporadic patients as well. prevalence to date, the prevalence of this disease is unclear. literature has few publications to date of this due to the rarity of the disease. however, a study done in 2011 found information dating back to 1970 that identified 51 individual cases fulfilling criteria to be identified. today, there could be more confirmed cases but these data have not increased significantly. research the need for future research in this disease is necessary for varied reasons. first, this disease is still so rare and it is to this date very difficult to diagnose. further, the signs and symptoms of this disease are often confused with other diseases that are more well known and misdiagnosis often occurs. this disease can commonly be mistaken for multiple sclerosis (ms) or another form of a more common leukoencephalopathy or neurodegenerative diseases as well. however, some current research has been done primarily on case studies in which scientists are attempting to find new mutations or new ways to diagnose. there are a few studies however that pose to lean in the direction of therapeutical's using hematopoietic stem cell transplantation therapy.hematopoietic stem cells can develop into a vast variety of different cells in our body themselves (self-differentiation). hematopoietic stem cell therapy bone marrow is the soft spongy area in some larger bones of the body that produces many cells which make up red blood cells, white blood cells and platelets. these cells are developed from a type of cell found in bone marrow, termed hematopoietic stem cells. the body is able to direct these stem cells to develop in the blood at any given moment and this is a rapid process. most of the stem cells remain in the marrow until they are mature which then they are released for specific functions in the body such as carrying oxygen, providing infection protection, and helping blood clotting. stem cells found in circulating blood are able to be extracted for stem cell therapy use and research. a study done using hematopoietic stem cell therapy (hsct) showed clinical benefit but suggested further exploration must be done. findings using hsct was beneficial in recessive disorders and saw that it may similarly enhance csf1r signaling after partial loss seen in lenas. in the subjects who had lenas were introduced to hsct and the finding of further progressed cells was minimal 15 years after the therapy was finished. the most important finding in the subject was that they retained a high level of communication and survived beyond 15 years after onset of symptoms. this is very rare for lenas as it has been reported to be averaged at 6.8 years for surviving after the onset. this finding provides hope for future research direction and suggests that there may be great benefit to slow the progression of lenas using hsct.