Myotonic dystrophy/Cervical Spondy Myopathy/TOS Flashcards
Describe myotonic dystrophy.
chronic, slowly progressing inherited multi-system disease; sign- failure of spontaneous letting go of the hands following strong handshakes due to myotonia (delayed relaxation of mms post contraction) which accompanies mm weakness
What does myotonic dystrophy cause?
wasting of mms (mm dystrophy), cataracts, heart condition defects and myotonia
What is type I myotonic dystrophy/Steinert disease?
disease most often found in facial mms, SCM, distal FA mm, hand intrinsics and ankle DFs
Describe cervical spondylitic myelopathy.
degenerative changes of Cx spine can cause compression of spinal cord. most common spinal cord disorder in people>55
What are sxs of cervical spondylitic myelopathy?
stiff neck, unilateral or bilateral deep aching neck,arm and shoulder pain, unsteady gait *Hallmark sign- weakness or stiffness of legs, may be assymetric. Hypereflexia, Babinski and Hoffmans EMG not useful; just exclude peripheral neuropathy
What is Lhermitte’s sign?
flex neck and send electric shock down center of back
Describe Neurogenic TOS
compression of brachial plexus pass thru ant/middle scalene pain worse with overhead activity, doing hair, carrying heavy objects Loss of sensation MBC and MABC nerves, intrinsic (esp median) hand wasting N/T medial arm and FA and hand, unilateral and progressive inability to use hand, atrophy median* and ulnar mms EMG- normal median SNAP, AB ulnar SNAP and MABC median and ulnar NCS normal in arm and FA decreased recruitment, fibs APB median CMAP and MABC most affected
Describe AIN.
zero sensory loss, pain in FA, +Ok sign EMG- +FDP 1,2, FPL and PQ AIN is pure motor nerve; branch off median nerve
Describe Pronater Teres Syndrome.
TTP over prox median nerve; worse with resisted pronation of FA AIN mms weak and sensory changes in D1-D3 Sensory palm affected; branch given off in FA 8cm proximal to distal wrist crease D1-D4 SNAPs decreased, decreased CMAP- APB EMG + all mms DISTAL to P.T
What condition does this picture depict?
Neurogenic TOS; picture depicts weakness and atrophy of C8-T1 mms. The T1 root more affected as such the hyperthenar ‘median’ mms are more affected than ulnar mms.
What do the waveforms on this picture depict?
Thoracic outlet syndrome
Compare and contrast myotonic dystrophy I and II
Both Type I and type II are autosomal dominant inherited muscle disorders which include bifacial wekaness, ptosis, progressive weakness, myotonia, and involvement of several organ systems outside of skeletal muscle. In both patients experience stiffness what improves with repeated contractions.
Type I involves more distal muscles which demonstrate myopathic EMG features and myotonia
Type II involves more prpoximal muscles which demonstrate myopathic EMG features and myotonia
myotonic dystrophy vs myotonia congenita
myotonia congenita is distinguished from dystrophic muscle disorders by lack of weakness in most patients and by absence of extramuscular abnormalities. muscle cooling may lead to increased duration of myotonic bursts in myotonia congenita while it has no effect on MD type I and has unkown effect of MD type II
How does exercise impact paramyotonia differently than myotonia?
