Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease, characterized by mass forming inflammatory lesions which respond well to steroid therapy. Pancreas is the most common site of involvement, and other organ involvements are also common. However, there are only a few reports about central nervous system involvement. We report a case of IgG4-related sclerosing disease which involves spinal cord causing paraplegia. A middle-aged female presented with sudden lower limb weakness. Magnetic resonance imaging showed a soft tissue mass which was diffusely compressing spinal cord along the C7 to T5 levels. Intravenous steroid pulse therapy and emergent operation was performed. The immunopathologic findings revealed IgG4-related sclerosing pachymeningitis postoperatively. There was no evidence of other organ involvement. Her neurologic deficit remained unchanged after two months of comprehensive rehabilitation therapy.
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To investigate the factors which contribute to the improvements of the gross motor function in children with spastic cerebral palsy after physical therapy.
The subjects were 45 children with spastic cerebral palsy with no previous botulinum toxin injection or operation history within 6 months. They consisted of 24 males (53.3%) and 21 females (46.7%), and the age of the subjects ranged from 2 to 6 years, with the mean age being 41±18 months. The gross motor function was evaluated by Gross Motor Function Measure (GMFM)-88 at the time of admission and discharge, and then, the subtractions were correlated with associated factors.
The GMFM-88 was increased by 7.17±3.10 through 52±16 days of physical therapy. The more days of admission, the more improvements of GMFM-88 were attained. The children with initial GMFM-88 values in the middle range showed more improvements in GMFM-88 (p<0.05). The children without dysphagia and children with less spasticity of lower extremities also showed more improvements in GMFM-88 (p<0.05).
We can predict the improvements of the gross motor function after physical therapy according to the days of admission, initial GMFM-88, dysphagia, and spasticity of lower extremities. Further controlled studies including larger group are necessary.
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