Congenital myotonic dystrophy is the most severe form of inherited myotonic dystrophy, presenting with generalized muscle weakness, breathing problems, and feeding difficulties. We report a patient with congenital myotonic dystrophy who began rehabilitation early in the neonatal intensive care unit. The mother had myotonic dystrophy and experienced no complications during pregnancy. The patient was born at a gestational age of 38 weeks and 1 day, weighing 2712 g, with an Apgar score of 1/4, and was diagnosed with congenital myotonic dystrophy. Marked generalized hypotonia and respiratory compromise were observed from birth, and the patient was orally intubated and managed with a ventilator. Rehabilitation to promote sensory-motor development was started 13 days after birth. Initially, generalized muscle hypotonia and prominent limitation of joint range of motion were observed. Therefore, joint range of motion exercises, positioning, and rehabilitation of the sensory-motor system were conducted first, while monitoring circulatory and respiratory status. After weaning from the ventilator, feeding evaluation was started on the 123rd day of life. In collaboration with physicians and nurses, feeding volume was increased stepwise to safely introduce oral feeding. The infant gradually became capable of oral intake and was discharged from the hospital on the 160th day of life. In this report, we demonstrated that rehabilitation intervention for infants with congenital myotonic dystrophy from the time of admission to the neonatal intensive care unit contributed to improved motor development, initiation of oral feeding, and smooth discharge.
Introduction:Intracranial hemorrhage after severe coronavirus disease 2019 (COVID-19) is associated with increased mortality and unfavorable patient outcomes.
Case:A man in his 60s with independent activities of daily living (ADL) was diagnosed with COVID-19, and placed on a ventilator on Day (D)-3 and VV-ECMO on D-5. On D-23, an emergency craniotomy was performed for a left acute subdural hematoma. The patient was weaned from VV-ECMO on D-27. On D-33, sitting was initiated. On D-36, the patient was weaned from the ventilator and began exercise therapy. The Glasgow coma score (GCS) was E2V1TM4. Basic movement as assessed by the Functional Status Score for ICU (FSS-ICU) and Barthel Index (BI), was 3 and 0 points, respectively. On D-40, wheelchair use commenced. He began standing with a long leg orthosis on D-50, and began walking on D-53.
On D-67, the patient transferred to a convalescent hospital. His GCS was E4V4M6. Generalized cognitive decline and motor paralysis were noted. The right upper limb, hand, and lower limb were assessed as Brunnstrom recovery stage IV, V, and V, respectively. The patien's grip strength was 11.9 kg [right] and 18.3 kg [left]. His knee extensor strength was 0.13 kgf/kg [right] and 0.19 kgf/kg [left]. The FSS-ICU, walking speed, and BI were 21 points, 0.17 m/sec, and 40 points, respectively. The patient was discharged on D-240.
Discussion:The Early Mobilization and Rehabilitation Expert Consensus reports that early mobilization and active exercise can improve ADL at discharge. Our patient was weaned from VV-ECMO as soon as possible and practiced standing and walking with a long leg orthosis, resulting in a better outcome.