Infants with Sandhoff disease typically appear normal until 3–6 months of age. As the disease progresses, they present with symptoms such as loss of motor skills, exaggerated startle response to loud noise, seizures, visual loss, and paralysis. We encountered a rare case of a 22-month-old girl with Sandhoff disease characterized by progressive motor weakness and dysphagia, who initially showed signs of aspiration at 20 months of age. The major problems related to dysphagia were oromotor dysfunction and abnormal feeding posture. Within 3 months of identification of difficulty in swallowing, the patient showed a significant decrease in food intake, with rapid deterioration of nutritional status. We report our case with a review of the literature.
To investigate the effect of Comprehensive Hand Repetitive Intensive Strength Training (CHRIST) on upper limb function in children with cerebral palsy using motion analysis.
The subjects in this study included 19 children (10 males, 9 females, mean age=8.8 years) with cerebral palsy. The experimental group (n=10) received CHRIST and general rehabilitation therapy. The control group (n=9) received a home program as well as general rehabilitation therapy. Both groups received 30 sessions of CHRIST or home program training for 60 minutes per session 3 times a week during the 10-week period. The reaching movements were captured by a motion analysis system. Kinematic variables including movement time (MT), mean velocity (MV), normalized jerk score (NJS), mean angular velocity (MAV) and normalized jerk score of the shoulder, elbow and wrist joint with comfortable and fast speed were analyzed between groups and the pre-post training group.
After pre- and post-training experimental group, MT, MV, NJS, MAV of shoulder, elbow, wrist and NJS of elbow and wrist improved significantlyin reaching movement of both comfortable and fast speed (p<0.05). However, After pre- and post-training control group, MV improved significantlyin reaching movement of only comfortable speed (p<0.05). Between two groups, MT and MAV of the elbow at comfortable speed and NJS of the elbow at fast speed were statisticallysignificant (p<0.05).
CHRIST proved to be an effective intervention for improving upper limb extremity function of reaching movement in children with cerebral palsy.
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