Table 2

Clinical manifestations of neurofibromatous neuropathy in eight individuals with neurofibromatosis 1

Patient no.	First neuropathic symptom	Weakness	Sensory impairment	Reflexes	Diffuse nerve thickening	Disability Rankin Scale
*Ankle and toe weakness due to left sciatic plexiform neurofibroma.
1	Numbness andtingling in handsand achingin feet	None	Impairment of light touch and pain up toelbows and to right knee and left mid-calf. Impaired vibration at right hallux andleft ankle	Reduced	Present	1³⁰
2	Pain andweaknessin feet	Distally predominant weaknessand wasting ofupper and lower limbs	Severe impairment of all modalities up toelbows and inguinal ligament	Absent	Present	4
3	Pes cavus	Weakness of toe flexion	Impaired vibration sensation on toes	Absent anklereflexes	Present	1
4	No symptoms	None	Mild impairment of pain on fingers and feetand light touch on toes	Normal	Marked(fig 2)	0
5	Numbness andtingling in handsand feet	Weakness of interossei andankle dorsiflexion	Light touch, vibration, and pinprick sensationreduced to the ankles and vibration sensation on the fingers	Reducedanklereflexes	Present	1
6	Pes cavus	Mild weakness of dorsal interossei, left ankle dorsiflexion, left toedorsiflexion, plantar flexion*	Impaired pinprick sensation in glove andstocking distribution up to themid-forearms and mid-shins	Present	Absent	1
7	Tingling andnumbness in feet	None	Impaired light touch and pain to mid-palms, the left thigh, and the right forefoot. Absentvibration in lower limbs	Present	Absent	1
8	Pes cavus, weaknessin legs	Wasted anterior compartmentsbilaterally. Bilateral weakness ofhip flexion, knee extension,ankle dorsiflexion, plantarflexion, inversion, eversion	Loss of pain sensation below both knees.Reduced vibration sense at the ankles.Reduced joint position senseat the hallux bilaterally	Absent anklejerks	Notrecorded	4