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Hydrosyringomyelia, case 1

 

Chiari malformation and hydromyelia
Hydrocephalus and syringomyelia were diagnosed 8 years before in this 39-year-old female. At that time, she underwent ventriculo-peritoneal shunting. Four years later she complained of progressive paresis and loss of sensation in both arms, as well as gait impairment due to leg weakness. She underwent surgery at the cranio-cervical junction, specifically posterior fossa decompresion.

Neurological follow-up
Gait improved, with a corresponding decrease in upper extremity paresis. The pyramidal syndrome disappeared. However, the sensory deficits remained, causing a severe handicap. Several follow-up MRI showed no evolution of the hydromyelia over three years.

MRI
Images 1 - 2: Sagittal T1WI show the abnormal position of the tonsils and the extensive central syrinx at the cervical level.
Image 3: Sagittal T1WI. The hydromyelia involves the thoracic spinal cord as well.

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Fig. 1

Hydrosyringomyelia, case 1, Fig. 1
Hydrosyringomyelia, case 1, Fig. 2
Hydrosyringomyelia, case 1, Fig. 3