Presentation: a 76 year-old woman, complaining of leg pain and unsteady gai
t for 3 years, presented with a spastic paraparetic gait, severe spasticity
and touch, thermal and pain sensory loss limited to arms, lower thorax and
upper abdomen. Brain and spinal cord magnetic resonance imaging showed a l
arge loculated syrinx. Cerebellar tonsillar herniation into the foramen mag
num was also seen (Amold-Chiari malformation, type I).
Outcome: the patient had successful cervico-spinal surgical decompression w
hich resulted in marked reduction in hypertonia and weakness, normal gait a
nd normal joint movement at 6 months.
Conclusion: this unusual, late clinical presentation of a congenital diseas
e underlines the importance of a comprehensive diagnostic work-up in the el
derly patients with complex neurological signs.