[A septated syringomyelia with a dramatic clinical course in a young patient with MELAS; a case report]

This is a report of a young girl who showed a recurrence of acute worsening and remission of neurological manifestations, with consistent MRI demonstration of transaxial septated syrinxes in the cervical and the lumbar spinal cord in addition to a tight filum terminale. This 8 year-old girl had developed normally since her birth until August 1989 when she developed a gait disturbance. This worsened acutely on January 1, 1990, with the additional manifestation of a urinary bladder disturbance. General examination failed to show any abnormality or scoliosis. Neurologic examination revealed a monoparesis of the right lower extremity with muscle atrophy and pyramidal tract sign. Fecal constipation and urinary retention were noted. The MRI T1 weighted sagittal image demonstrated an incontinuous low intensity signal in the C1-C7 as well as in the T12-L2 without swelling of the cord. The axial image clearly demonstrated the septations in the syrinx which looked like eye glasses. No definite Gd enhancement was demonstrated. Chiari malformation was not associated, but the tethered cord was well identified. With the administration of steroid, she showed a marked improvement of neurological manifestations. She was able to urinate without difficulty and also walk by herself. For one month thereafter she remained well with minor neurological deficits until she developed a worsening of the gait disturbance with a newly manifested weakness of the left upper extremity. Sensory impairment was also demonstrated below L3. In contrast to the worsening of the clinical symptoms, no definite change in the abnormalities found by MRI was noted.(ABSTRACT TRUNCATED AT 250 WORDS)