Objective: This study aimed to evaluate the changes in causes of death among residents of a facility for individuals with severe motor and intellectual disabilities (SMID) over a 62-year period. Methods: The causes and age of death were retrospectively examined for 216 deaths at our center from May 1961 to December 2022. The causes of death were classified in disease groups and compared by the hospitalization period of every decade. Results: The number of deaths among residents in the past 62 years was 216. The number of the death and the median age were 50 cases and five years old in 1960s, 31 cases and ten years old in 1970s, 38 cases and 13.5 years old in 1980s, 35 cases and 17 years old in 1990s, 26 cases and 22 years old in 2000s, 31 cases and 49 years old in 2010s, five cases and 50 years old in 2020s, respectively. The cumulative causes of death were respiratory diseases in 114 cases (51%), cardiovascular diseases in 31 cases (14%), gastrointestinal diseases in 12 cases (5.6%), urological diseases in five cases (2.3%), central nervous system diseases in eight cases (3.7%), infectious diseases in 15 cases (6.9%), malignant tumors in 20 cases (9.3%), and others in 11 cases (5.1%). In the 1960s, respiratory diseases accounted for 64%, followed by cardiovascular diseases (14%), infectious diseases (6%), and malignant tumors (4%). In the 2010s, respiratory diseases accounted for 23%, followed by cardiovascular diseases (19%), malignant tumors (16%), and infectious diseases (13%). Between the 1960s and 2010s, deaths by respiratory diseases decreased and deaths by malignant tumors increased. Conclusions: Early detection of malignant tumors should be in aging of individuals of residents in facilities for SMID.

　　A 7 month-old boy was admitted to hospital with repetitive tonic posturing of the four limbs suspected with epileptic seizures. The patient was originally diagnosed with chondrodysplasia punctata (CDP) branchytelephalangic type (BCDP) based on a peculiar facial appearance and calcific stippling around the hyoid, vertebrae and tarsal bones at 2 months. From 3 months, the patient had repetitive movements with both upper limbs in flexion at both elbow joints and both lower limbs in extension in a tonic posturing for 20 seconds. The patient could not turn over at the initial visit at 7 months, and increased muscle tone and spastic paralysis in the extremities were observed. Deep tendon reflexes were enhanced, and pathological reflexes were positive. An ictal electroencepharogram showed no abnormal epileptic discharges consistent with tonic posturing, diagnosed as non-epileptic movements. A cervical MRI showed spinal canal stenosis, subluxation of the annular axial vertebra, and cervical spinal cord compression at the first to second cervical vertebrae level. A cervical X-ray showed that the first cervical vertebra was anteriorly deviated and further anteriorly deviated in an anteversion position. First cervical vertebra laminectomy and craniocervical posterior fusion were performed at 1 year and 2 months, and bilateral peri arthroplasty and bilateral knee flexor tendon lengthening were performed at 4 years and 10 months. At 8 years old, he had spastic quadriplegia but could sit up and walk with support. The upper limbs could be used for writing and oral intake with assistive devices. Early detection of cervical spinal canal stenosis associated with BCDP detected at an early can improve the neurological sequelae by reducing cervical spinal cord compression. When cervical lesions are suspected, a cervical MRI should be performed, and surgical treatment should be actively considered, even at younger ages.