Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with cervical diastematomyelia are reported here. A nineteen year-old-man (patient 1) admitted to our hospital because of muscular weakness of right upper limb. He noted muscular atrophy of right upper limb at 16 years old, and then paresthesia was gradually aggravated in the ulnar side of the right hand. Physical examination showed muscular atrophy of right upper limb and hypesthesia in the right eight cervical and first thoracic dermatomes. The deep tendon reflexes were decreased in the right upper limb and were increased in the lower limb without pathological reflexes. In electromyographic examination, neurogenic motor units were observed in the upper right limb, dominantly in 1st interosseous muscle (between the fourth cervical and the first thoracic dermatome). Metrizamide computed tomographic (CT) myelography revealed sagittal splitting of the spinal cord from the third to the sixth cervical vertebra, producing two asymmetrical hemicords. A osseous or fibrous septum were not seen. The right hemicord was smaller than the left one. Patient 2 was a twenty-four-year-old woman. She visited our hospital because of muscular weakness of the right upper limb. In physical examination, there were the muscular atrophy of right hand and hypesthesia in the right eighth and first thoracic dermatomes. The deep tendon reflexes were decreased in the right upper limb and were increased in the right lower limb without pathological reflexes. The EMG studies revealed the neurogenic NMU in the right upper limb (between the fourth cervical and the first thoracic dermatome). Magnetic resonance imaging showed marked narrowing of the dural sac in flexion of the neck.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Two patients with cervical diastematomyelia]. 275 66

To discuss, through a review of the literature, clinical signification and antenatal and postnatal management of diastematomyelia. We present two cases highlighting the clinical presentation and discuss management options. The first case was diagnosed in a 32-year-old primagravida female physician. The conssanguinous couple had a history of primary hypofertility. Antenatal magnetic resonance imaging revealed the presence of a sagittal spinal spur separating two asymmetrical hemi cords. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Ultrasonography performed at 22 weeks gestation provided the diagnosis in the second case. The mother was a 30-year-old primagravida anesthesist who had been addressed for suspected spina bifida. The consanguinous couple had a history of male hypoferility. The ultrasound scan revealed an echogenic spinal spur and integrity of the skin. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Diastematomyelia is a rare malformation which can be diagnosed antenatally. Careful ultrasonography can distinguish diastematomyelia from myelomeningocele. Antenatal assessment is essential to identify forms with good prognosis because the neonatal outcome of isolated diastematomyelia is generally good, even if surgical repair is required.
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PMID:[Diastematomyelia: antenatal diagnosis with successful outcome, two cases]. 1313 Feb 51