Neurologic manifestations of scleroderma

Neuromuscular manifestations of systemic sclerosis - comes mainly from case reports/case series

frequency 0-66%

Cranial, entrapment, peripheral cutaneous, autonomic NEUROPATHIES

Myopathy

CNS/headache/seizure/stroke/vascular disease/radiculopathy/myelopathy

Increased risk w/antiScl-70 (anti topoisomerase) & anti U1RNP (normally in diffuse)

Pathophys

Cranial/peripheral - vascular (deterioration of vasa nervorum)

Compression 2/2 edema/fibrosis

Autoimmune -antimyelin antibodies do not correlate w/neuropathy

Cranial Neuropathy

most common is trigeminal (less so 2-9, rarely 8)

CNIII - inflammatory/restrictive ophthalmopathy

Trigeminal V2V3 - numbness, pain (burn), paresthesia - 24months to max deficit

MOTOR NOT AFFECTED

tx - steroids mixed efficacy - antidepressants/anticonvulsants

Entrapments

Median nerve at the carpal tunnel 5-25% 2/2 fibrosis/calcific deposition

ulnar, posterior tib, ilioinguinal, meralgia (lat fem cutaneous)

Peripheral polyneuropathies - common in diffuse

pathology - ^^collagen, loss of myelinated fibers, intimal thickening/hyalinosis of endo/perineurial blood vessels

Sensory - distal/symmetric

Mixed sensorimotor

Mononeuritis multiplex -2/2 inflammation of vasa nervorum, rarely infarct,

Brachial plexopathy

Cutaneous

Autonomic - GI motility, impotence, sympathetic skin response

Radiculoapthy -2/2 nerve compression by vertebral destruction/epidural calcinosis

NMJ - SSc and myasthenia can co-occur

Myopathy & Myositis

myopathy - proximal - mild increase in CK, EMG - decreased duration of motor unit potential & increased frequency of polyphasic motor units

TX - indolent usually nonprogressive

myositis - severe weakness, CK 2x nml - Dx w/muscle biopsy - TX glucocorticoids +/- cytotoxic

CNS - headache/stroke/seizure/myelopathy - sever CNS abnormalities are RARE

Headache - tx similar but do not give ergots bc vasoconstricting

Seizure - normally 2/2 compmlications (uremia/htn)

Stroke - causal relationship not yet demonstrated, stroke 2/2 athero/htn

CNS vasculitis - RARE - tx -glucocorticoids, cyclophos, cyclosporine

Myelopathy - 2/2 calcinosis