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Τετάρτη 27 Ιουνίου 2018

Craniovertebral junction cord compression due to neurofibroma

Description

A 26-year-old man with premorbid cutaneous neurofibromatosis presented with history of insidious onset, slowly progressive, bilaterally asymmetrical quadriparesis along with incontinence of urine since 4 weeks. Simultaneous to the onset of motor symptoms, he also complained of paraesthesias in all four limbs. On examination, multiple cutaneous neurofibromas were noticed all over his trunk, abdomen and all the limbs (figure 1). The upper and lower limbs were spastic. Motor power as per Medical Research Council grading was 3/5 in all four limbs. The deep tendon reflexes were brisk. The plantar response on both sides was extensor. The sensory level was clinically around C3/C4 dermatome.

Figure 1

Multiple cutaneous neurofibromas of varying sizes on the lower chest and abdomen (red arrows).

In view of the premorbid neurofibromata, a possibility of compressive cervical myelopathy was thought of. MRI of cervical spine T2-weighted sagittal view...



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