Medical scientists are asked to make various judgments when new diseases emerge, such as COVID–19, but even for these experts, their judgments are often incorrect. Experts always attempt to convince the public using a term “science–based evidence” but, in many cases, without real solid evidence. There are massive of unexplained diseases and pathogenicity in neurology. In order to discover and elucidate new diseases and their pathophysiological conditions, it is important to carefully examine and investigate each patient, followed by a persistent tracing sincerely and deeply. Medicine is still developing, and to make the neurology more practical and useful for the world, I would like to expect flexibility, innovation, and conscience from doctors with neurological expertise.
Nutrition therapy is extremely important for neurological diseases in order to improve patients' prognosis and quality of life. In the recent years, nutritional therapy has been recommended in the treatment guidelines for several disorders, including stroke and amyotrophic lateral sclerosis. Nutritional disorders in patients with neurological diseases vary depending on the background disease and pathological conditions. Furthermore, because of the wide range of lesions (including those in the central nerves, peripheral nerves, and muscles), several disorders such as dysphagia, respiratory disorders, motor paralysis, muscle rigidity, involuntary movements, and ataxia often coexist in patients. Moreover, patients may also have other complications such as constipation, diarrhea, pressure ulcers, and aspiration pneumonia. In order to provide appropriate nutritional therapy, each patient must be provided tailor–made interventions and support ; sharing of knowledge among multidisciplinary experts would aid the development of such interventions.
Older adults are at a high risk of developing malnutrition and sarcopenia because of various factors, including diseases, aging, unfavorable living conditions, hormonal changes, and inadequate dietary intake. It is important to screen for malnutrition and sarcopenia using appropriate diagnostic criteria. Malnutrition in older adults should be assessed using the Global Leadership Initiative on Malnutrition criteria rather than serum albumin or prealbumin levels. Meanwhile, the Asian Working Group for Sarcopenia 2019 criteria are preferred for the diagnosis of sarcopenia. These criteria may be useful in identifying the etiology of malnutrition and sarcopenia.
Individualized nutritional therapy can be effective in preventing poor outcomes. Providing effective nutritional therapy requires understanding the effects of energy, protein, and vitamins on malnutrition and sarcopenia. Therefore, it is important to clarify the etiology of malnutrition and sarcopenia when developing nutritional therapy for older adults. Moreover, attention to and individualization of nutritional therapy (energy, protein, and vitamin intake) and exercise intervention may improve these conditions.
In the nutrition therapy for stroke patient, it is important to carry out appropriate the nutritional management with understanding of the pathophysiology of each stroke types, cerebral infarction, cerebral hemorrhage, and subarachnoid hemorrhage. In addition, the nutrition management according to the severity and stage in each patient should be performed.
In the acute stage of stroke, shorten the fasting period and start the enteral nutrition as soon as possible. In cases without dysphagia, oral intake should be started promptly, and in cases with severe dysphagia or with comatose, the nutritional management by tube feeding should be performed. In severely stroke cases, a hyperglycemia condition might be likely to occur, because the catabolism may be accelerated by the invasive stress. The energy dose and the nutritional composition must be set in consideration of glycemic variability.
On the other hand, the energy consumption would be increasing during recovery stage. The appropriate nutrition therapy should be practiced for the elderly patient coexisting with frailty or sarcopenia, who make up the majority of the hospitalization on the recovery rehabilitation floor.
The basic concept of nutrition therapy in stroke is the early initiation of enteral nutrition managed by an appropriate protocol. These practices may help improve outcomes in stroke patients.
Weight loss is frequently observed in early–stage amyotrophic lateral sclerosis (ALS) and is considered an independent predictor of survival. Weight loss observed in ALS is associated with multifactorial etiology, including muscle wasting and dysphagia ; however, recent studies have implicated disease–specific hypermetabolism in weight loss and disease progression in ALS. The pathophysiology of hypermetabolism as a contributor to weight loss in ALS remains unclear ; however, hypothalamic involvement is considered an early extra–motor manifestation of ALS. TDP–43 protein aggregates detected in the hypothalamic subnuclei may be associated with weight loss or abnormalities of eating behavior in patients with ALS. Weight loss from diagnosis up to tracheostomy also predicts functional prognosis during the long–term period with ventilator. In fact, patients with ALS who survive with prolonged mechanical ventilatory support often develop significant brain and brainstem atrophy, including atrophy of the limbic motor system and the hypothalamus. Nutritional intervention to maintain body weight may be a useful disease–modifying therapeutic approach, and recent studies have reported that slowing of weight reduction rate after diagnosis may be associated with better survival and that a high–calorie diet improves survival in patients with rapidly progressive disease. Nutritional education regarding a high–calorie diet, weight control, and early gastric tube placement are important after diagnosis. Researchers from the USA, Europe, and Japan have established formulas to estimate the recommended daily energy intake. Although a lipid shift in energy metabolism might occur in the brain and muscles in patients with ALS, the effectiveness of high–fat diets requires further investigation.
The amount of weight loss in patients with Parkinson disease (PD) documented in the literature varies from 52 to 65%. As reduced energy input, hyposmia, cognitive impairment, depression, gastrointestinal dysfunction, and dysphagia are thought to be causally related to weight loss. On the other hand, weight loss and malnutrition are also the result of a negative energy balance, which means that energy expenditure exceeds intake caused by tremor, rigidity, and dyskinesia. To predict total energy expenditure, a calculation method to multiply basal energy expenditure by 1.1–1.3, or PD coefficient which is appropriate to the degree of rigidity and tremor has been proposed. For nutrition therapy to replenish energy in PD patients, functional food including medium–chain triglyceride supplements may be helpful.
Nutritional impairment in multiple system atrophy (MSA) can be divided into early and advanced stages. In the early stage, nutritional impairment due to dysphagia can develop. In the advanced stage, body fat could accumulate after the introduction of a ventilator or the placement of a gastrostomy. Nutritional impairment may be progressive in MSA patients, even if body weight is maintained. The serum albumin level is a useful indicator of nutritional status. Leptin resistance due to autonomic neuropathy may be related to the accumulation of body fat. In the early stages, sufficient nutrition should be administered to prevent the progression of nutritional disorders. In the advanced stage, the amount of administered energy is reduced to prevent fat accumulation. Further research is needed to establish the evidence for the nutritional management of MSA.
症例は39歳女性．7日間の発熱持続後に発症した意識障害を伴う全身性の強直間代性痙攣を主訴に救急搬送となった．頭蓋内疾患や代謝性障害は否定的で，複数の抗痙攣薬や鎮静薬を投与するも発作の抑制に難渋し，new–onset refractory status epilepticus（NORSE）に合致する臨床像であった．早期からの免疫療法に加え，人工呼吸器管理下でのthiamylal coma therapyを行った．発作の軽減に応じてthiamylal sodiumを漸減し，鎮静剤投与および人工呼吸器からの離脱に成功したが，離脱後から両手指と両足趾にテタニー様症状が出現した．