Table 1

 Clinical and neuroimaging features in eight individuals with neurofibromatosis 1 and neurofibromatous neuropathy

Patient no.Age at last examination(years)Age of neuropathy onset (years)Additional clinical problemsPresence of multiple spinal nerve root neurofibromas on MRI
MPNST, malignant peripheral nerve sheath tumour.
15342High grade MPNST left iliac fossa removed in 1986. Norecurrence. Benign plexiform neurofibroma left abdominal wallYes (fig 1)
22717Congenital adrenal 21-hydoxylase deficiency. Cervicalintradural neurofibromas removed at age 22. Massiveinoperable pelvic neurofibromasYes
326InfancyComplex partial seizures since childhoodYes
43127Mild cognitive impairment. Brachial plexus neurofibromasYes
54847Cognitive impairmentYes
6193Left sciatic plexiform neurofibromaNo, only lumbar spine scanned
76255Bony dysplasia of spine and clavicle. Plexiform neurofibromainvolving neck, anterior chest wall, left arm. Died from chronicobstructive airways disease at age 62No
84525Benign neurofibromas removed from right L4 and L5 nerveroots at age 33. High grade MPNST left sciatic nerveremoved at age 44. Died from lung metastases at age 45Yes on myelogram; MRI not performed