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

custom tailoring cognitive performance enhancements for goal specific tasks

augmenting NREM cortical consolidation

ocular dominance plasticity has been shown to be weakened by administering NMDA antagonists during sleep. if this idea can be translated to other cortical areas, and potentially work in reverse, the question is: has anyone attempted NMDA agonist action right before sleep after a hard days learning? more for visuospatial and motor tasks as opposed to explicit memory consolidation, but it would be interesting to know if learning is augmented.

recent idea is synaptic homeostasis hypothesis

the idea that wakefullness increases en bloc all NT potentiation, and that during sleep this potentiation is brought back down (or balanced) and possibly certain memory traces are pruned and retained during this diurnal event.

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