Supplementary figures and legends

Figure e-1 Skin lesions and deformity of left hand

Bilateral congenital campylodactyly and erythematous patches of skin (A & B) with a

suture at the site of skin biopsy (A). There was wasting of the intrinsic hand muscles

with new flexion deformity of the first, second and third fingers

Figure e-2 Peripheral nerve sonography

An ultrasound image of a mildly enlarged right median nerve at the wrist (A) of a

patient with carpal tunnel syndrome is presented for comparison. Image B

demonstrates the markedly enlarged left median nerve seen in cross-section (B) and

longitudinally (C) with thickened perineurium, hypoechoic nerve and rarefication and

enlargement of nerve fascicles. The non-lepromatous nerve measures 2.4 x 7.2mm

with a circumference of 16.4mm and has a cross-sectional area of 13mm2 while the

lepromatous nerve is 14.4 x 8.3mm with a circumference of 36.4mm and a cross-

sectional area of 94mm2

Figure e-3 Skin histology

Hyperkeratosis and mild hyperplasia were noted on skin biopsy (A) with well-defined,

non-necrotising granulaomas seen in the dermis (B and C)

Table e-1 Serial nerve conduction study results. NR, no response; ND, not done; AHB abductor hallucis brevis; EDB extensor digitorum brevis

Nerve 17 June 2013 16 Sept 2013

Stimulate Record Amplitude Velocity Amplitude VelocityR L R / L R L R / L

Ulnar sensory Wrist 9 NR 54 / NR 8Median sensory Wrist 18 NR 50 / NR 14 NRRadial sensory Wrist 19 5 55 / 40 26 7Sural Ankle 26 24 42 ND 24Supf peroneal Ankle 15 5 ND 9Medial plantar Ankle 15 3 ND 7Median motor Thenar Wrist 12 11.2 64 / 40 11.4 11.9 Elbow 12 5.5 / 54 11.8 9.5 58 / 42 Axilla ND 5.0Ulnar motor Hypothenar Wrist 9.2 NR Below elbow 8.6 55 / NR Above elbow 8.9 50Axilla 7.7Tibial motor AHBAnkle 14.9 5.5 ND 7Peroneal motor EDB Ankle 6.3 8.1 Fibula head 8.5

Table e-2 Cross-section area of selected left upper limb peripheral nerves as

measured by sonography at presentation and at 3 months after initiation of

treatment. NA not available

Affected peripheral nerve Cross-sectional area (mm2)

At presentation At 3 months / 12 months

Left median at the wrist 94 38 / 24

Left ulnar above the elbow 89 46 / 60

Left superficial radial sensory nerve over distal radius at forearm

10 2 / NA