Palliative care is offered to ameliorate symptoms, but there are no medications that can slow, stop, or improve the relentless progression of the disease. No medications for DLB are approved by the US Food and Drug Administration (FDA) as of 2020, although donepezil is licensed in Japan and the Philippines for the treatment of DLB. As of 2020, there has been little study on the best management for non-motor symptoms such as sleep disorders and autonomic dysfunction; most information on management of autonomic dysfunction in DLB is based on studies of people with Parkinson's disease. Management can be challenging because of the need to balance treatment of different symptoms: cognitive dysfunction, neuropsychiatric features, impairments related to the motor system, and other nonmotor symptoms. Individuals with DLB have widely different symptoms that fluctuate over time, and treating one symptom can worsen another; suboptimal care can result from a lack of coordination among the physicians treating different symptoms. A multidisciplinary approach—going beyond early and accurate diagnosis to include educating and supporting the caregivers—is favored. Medication Pharmacological management of DLB is complex because of adverse effects of medications and the wide range of symptoms to be treated (cognitive, motor, neuropsychiatric, autonomic, and sleep). Anticholinergic and dopaminergic agents can have adverse effects or result in psychosis in individuals with DLB, and a medication that addresses one feature might worsen another. For example, acetylcholinesterase inhibitors (AChEIs) for cognitive symptoms can lead to complications in dysautonomia features; treatment of movement symptoms with dopamine agonists may worsen neuropsychiatric symptoms; and treatment of hallucinations and psychosis with antipsychotics may worsen other symptoms or lead to a potentially fatal reaction. Extreme caution is required in the use of antipsychotic medication in people with DLB because of their sensitivity to these agents. Severe and life-threatening reactions occur in almost half of people with DLB, and can be fatal after a single dose. Antipsychotics with D2 dopamine receptor-blocking properties are used only with great caution. According to Boot (2013), "electing not to use neuroleptics is often the best course of action". People with Lewy body dementias who take neuroleptics are at risk for neuroleptic malignant syndrome, a life-threatening illness. There is no evidence to support the use of antipsychotics to treat the Lewy body dementias, and they carry the additional risk of stroke when used in the elderly with dementia. Medications (including tricyclic antidepressants and treatments for urinary incontinence) with anticholinergic properties that cross the blood-brain barrier can cause memory loss. The antihistamine medication diphenhydramine (Benadryl), sleep medications like zolpidem, and benzodiazepines may worsen confusion or neuropsychiatric symptoms. Some general anesthetics may cause confusion or delirium upon waking in persons with Lewy body dementias, and may result in permanent decline. Cognitive symptoms There is strong evidence for the use of AChEIs to treat cognitive problems; these medications include rivastigmine and donepezil. Both are first-line treatments in the UK. Even when the AChEIs do not lead to improvement in cognitive symptoms, people taking them may have less deterioration overall, although there may be adverse gastrointestinal effects. The use of these medications has demonstrated a reduced burden on caregivers, and improvement in activities of daily living for the individual with DLB. The AChEIs are initiated carefully as they may aggravate autonomic dysfunction or sleep behaviors. There is less evidence for the efficacy of memantine in DLB, but it may be used alone or with an AChEI because of its low side effect profile. Anticholinergic drugs are avoided because they worsen cognitive symptoms. To improve daytime alertness, there is mixed evidence for the use of stimulants such as methylphenidate and dextroamphetamine; although worsening of neuropsychiatric symptoms is not common, they can increase the risk of psychosis. Modafinil and armodafinil may be effective for daytime sleepiness. Motor symptoms Motor symptoms in DLB appear to respond somewhat less to medications used to treat Parkinson's disease, like levodopa, and these medications can increase neuropsychiatric symptoms. Almost one out of every three individuals with DLB develops psychotic symptoms from levodopa. If such medications are needed for motor symptoms, cautious introduction with slow increases to the lowest possible dose may help avoid psychosis. The anticonvulsant zonisamide has been approved in Japan since 2009 for treating Parkinson's disease and since 2018 to treat<unk>  in DLB. There is high certainty according to the GRADE certainty rating approach that it is effective for treating motor symptoms in DLB. Neuropsychiatric symptoms Neuropsychiatric symptoms of DLB (aggression, anxiety, apathy, delusions, depression and hallucinations) do not always require treatment. The first line of defense in decreasing visual hallucinations is to reduce the use of dopaminergic drugs, which can worsen hallucinations. If new neuropsychiatric symptoms appear, the use of medications (such as anticholinergics, tricyclic antidepressants, benzodiazepines and opioids) that might be contributing to these symptoms is reviewed. Among the AChEIs, donepezil and rivastigmine can help reduce neuropsychiatric symptoms and improve the frequency and severity of hallucinations in the less severe stages of DLB. For treating psychosis and agitation in DLB, there is low evidence for memantine, olanzapine and aripiprazole, and very low evidence for the efficacy of quetiapine. Although clozapine has been shown effective in Parkinson's disease, there is very low evidence for its use to treat visual hallucinations in DLB, and its use requires regular blood monitoring. Apathy may be treated with AChEIs, and they may also reduce hallucinations, delusions, anxiety and agitation. Most medications to treat anxiety and depression have not been adequately investigated for DLB. Antidepressants may affect sleep and worsen RBD. Mirtazapine and SSRIs can be used to treat depression, depending on how well they are tolerated, and guided by general advice for the use of antidepressants in dementia. Antidepressants with anticholinergic properties may worsen hallucinations and delusions. People with Capgras syndrome may not tolerate AChEIs. Sleep disorders The first steps in managing sleep disorders are to evaluate the use of medications that impact sleep and provide education about sleep hygiene. Sleep medications are carefully evaluated for each individual as they carry increased risk of falls, increased daytime sleepiness, and worsening cognition. Injurious dream enactment behaviors are a treatment priority. Frequency and severity of RBD may be lessened by treating sleep apnea, if it is present. RBD may be treated with melatonin or clonazepam. Melatonin may be more helpful in preventing injuries, and it offers a safer alternative, because clonazepam can produce deteriorating cognition, and worsen sleep apnea. Memantine is useful for some people. Modafinil may be used for hypersomnia, but no trials support its use in DLB. Antidepressants (SSRIs, SNRIs, tricyclics, and MAOIs), AChEIs, beta blockers, caffeine, and tramadol may worsen RBD. Autonomic symptoms Decreasing the dosage of dopaminergic or atypical antipsychotic drugs may be needed with orthostatic hypotension, and high blood pressure drugs can sometimes be stopped. When non-pharmacological treatments for orthostatic hypotension have been exhausted, fludrocortisone, droxidopa, or midodrine are options, but these drugs have not been specifically studied for DLB as of 2020. Delayed gastric emptying can be worsened by dopaminergic medications, and constipation can be worsened by opiates and anticholinergic medications. Muscarinic antagonists used for urinary symptoms might worsen cognitive impairment in people with Lewy body dementias. Other There is no high-quality evidence for non-pharmacological management of DLB, but some interventions have been shown effective for addressing similar symptoms that occur in other dementias. For example, organized activities, music therapy, physical activity and occupational therapy may help with psychosis or agitation, while exercise and gait training can help with motor symptoms. Cognitive behavioral therapy can be tried for depression or hallucinations, although there is no evidence for its use in DLB. Cues can be used to help with memory retrieval. For autonomic dysfunction, several non-medication strategies may be helpful. Dietary changes include avoiding meals high in fat and sugary foods, eating smaller and more frequent meals, after-meal walks, and increasing fluids or dietary fiber to treat constipation. Stool softeners and exercise also help with constipation. Excess sweating can be helped by avoiding alcohol and spicy foods, and using cotton bedding and loose fitting clothing. Physical exercise in a sitting or recumbent position, and exercise in a pool, can help maintain conditioning. Compression stockings and elevating the head of the bed may also help, and increasing fluid intake or table salt can be tried to reduce orthostatic hypotension. To lessen the risk of fractures in individuals at risk for falls, bone mineral density screening and testing of vitamin D levels are used, and caregivers are educated on the importance of preventing falls. Physiotherapy has been shown helpful for Parkinson's disease dementia, but as of 2020, there is no evidence to support physical therapy in people with DLB. Caregiving Demands placed on caregivers are higher than in AD because of the neuropsychiatric symptoms associated with DLB. Contributing factors to the caregiver burden in DLB are emotional fluctuations, apathy, psychosis, aggression, agitation, and night-time behaviors such as parasomnias, that lead to a loss of independence earlier than in AD. Caregivers may experience depression and exhaustion, and they may need support from other people. Other family members who are not
medical cause of Parkinsonism
medical cause of Dementia
symptom of Pantothenate kinase-associated neurodegeneration
complications of Myotonic dystrophy
symptom of Amyotrophic lateral sclerosis
symptom of Spinocerebellar ataxia type 1
medical cause of Polyneuropathy
medical cause of Dystonia