The Study Evaluating the Improvement of Nutritional Status and Frailty With Silkworm Pupa Powder Among Patients With Motor Neuron Disease

Inclusion criteria

• Voluntarily participate in the clinical study, fully understand and be informed about the study, and sign the Informed Consent Form (ICF); willing to follow and capable of completing all trial procedures.
• Gender is not limited, age at the time of signing ICF: ≥18 years old, ≤80 years old; if the ALS patient is at least 18 years old, then the weight must be over 40 kilograms.
• Diagnosis conforms to the confirmed and probable ALS in the ALS2020 diagnostic criteria (Gold Coast Criteria).
• Clinical, neurophysiological, or pathological examination confirms evidence of lower motor neuron involvement.
• Frailty Phenotype scale (FP) ≥1.
• Exclude other diseases.
• Agree to provide peripheral blood, fecal, and urine samples for biomarker analysis during the study period.

Exclusion criteria

• Patients with other neurological diseases similar to ALS symptoms or affecting drug efficacy evaluation, such as cervical spondylosis, lumbar disease, dementia, etc.
• Patients with other autoimmune diseases, such as Multiple Sclerosis (MS), Polymyositis, Myasthenia Gravis, Guillain-Barré Syndrome, Ankylosing Spondylitis, Rheumatoid Arthritis, Systemic Lupus Erythematosus, Vitiligo, etc.
• Severe renal insufficiency: Creatinine clearance < 30 mL/min (Cockcroft-Gault formula), or other known severe renal insufficiency diseases.
• Severe liver damage: ALT, AST > 3 times the upper limit of normal, or other known liver diseases such as acute and chronic active hepatitis, cirrhosis, etc.
• Patients with severe pulmonary function insufficiency such as Chronic Obstructive Pulmonary Disease (COPD), pulmonary fibrosis, etc.
• During the screening period, patients with acute myocardial infarction or interventional treatment within the last 6 months, heart failure patients (classified as NYHA class III-IV patients).
• Patients with other severe primary diseases of the nervous system, heart, lungs, hematopoietic system, or endocrine system, and mental illness patients.
• Those suspected or confirmed to have a history of alcohol or drug abuse.
• Expected survival ≤ 3 months.
• Pregnant or breastfeeding women, subjects of reproductive age (including male subjects who have had heterosexual intercourse and their female partners with childbearing potential) who plan to become pregnant or are unwilling to take effective contraceptive measures from the start of screening to 3 months after discontinuing medication.
• Those who are allergic to known ingredients of the trial products; or have a history of drug allergies or severe allergic diseases (anaphylactic shock, etc.).
• During the study, individuals assessed by researchers as potential drug abusers who may affect the efficacy of therapeutic drugs.
• Presence of other severe physical or mental diseases or abnormal laboratory tests that may increase the risk of participating in the study and patients deemed unsuitable for participation by the researcher.
• Patients with malignant tumors.