Answer of July 2007

Clinical History:

A 58-year old lady presented with on and off right upper and lower limb weakness and numbness for one month. Physical examination showed increased jerks and spastic tetraplegia.

Diagnosis:

Neurofibromatosis

Discussion:

Cervical spine x-ray showed cervical lordosis was reversed with scoliosis. Gross atlanto-axial subluxation was seen with distance between posterior surface of anterior arch of C1 and anterior surface of dens approached 2cm. Prevertebral soft tissue was increased. 
MRI spine showed cervical kyphoscoliosis. There was atlanto-axial subluxation with preodontoid distance measuring up to 1cm. Thecal sac was spacious. Dural ectasia causing posterior scalloping was seen, more obviously over C6/7, T1 vertebral bodies. Multiple lateral meningoceles with adjacent bone remodeling were noted. Numerous skin nodules were noted at the posterior neck region.

Overall pictures are consistent with neurofibromatosis.