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http://neurologyminutiae.blogspot.com/2007/03/als-pearls-als-mimics.html
Thursday, March 08, 2007
ALS pearls-- ALS mimics
spondylitic myelopathy -- limb fibs should go away within six months of fixing spine
inclusion body myositis pearl check finger flexors esp FPL FDP, forearm atrophy and check for absent fascics and symmetry
spinal cord AVM
heavy metal intoxication
lymphoproliferative disorder
multifocal motor neuropathy with conduction block (anti GM1 seen in about half) rsponds to IVIG
vasculitis
motor polyradiculopathy
infectious (polio, WNV)-- EMG shows poor recruitment, then fibs in 4-6 weeks
Bulbospinal mononeuropathy (Kennedy's syndrome) X linked, presents in men with symmetrical weakness, atrophy and fasciculations of bulbar and proximal limb muscles, testicular atrophy and gynecomastia.Occurs early, diagnosed by a CAG repeat, and is associated with a normal lifespan. Consider in males with slow course. Fascics are more pronounced in this disorder, but its all LMN.
Spinal muscular atrophy (Werdnig Hoffman, Kugelberg Welander)-- kids, young and old adults
Progressive lateral sclerosis (UMN variety) is associated with a normal lifespan
Benign fasciculations
cramp fasciculation syndrome-- easily treated with membrane stabilizers
Monomelic atrophy-- focal variant
frontotemporal dementia- identical path finding seen by Neumann ALS spectrun disorder?
Machado Jospeh disease= spinocerebellar atrophy tpe 3. See prominent anterior horn disorder plus ataxia and myelopathy
polyglucosan body disease-- rare UMN and LMN findings plus sensory changes and shite matter changes on MRI, with rare glucosan bodies seen on sural nerve biopsy.
Acid maltase deficiency may mimic PM abnormal respiratory muscles.
Syrinx
true neurologic TOS thoracic outlet syndrome -- shows decreased median CMAP, normal median SNAP, decreased ulnar SNAP, decreased medial antebrachial SNAP; needle shows median > ulnar hand involvement; lower trunk median/radial innervated forearm muscles (FPL, EIP)
myofibrillar myopathy-- = Desmond's myopathy-- mimics IBM. Get quad atrophy, medial gastroc, dysphagia, atrophy of VM/VL but rectus femoris is spared and is normal
kill me or cure me