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Unusual Case of a Brachial Plexus Disorder. Eddie Patton Jr. M.D, Cecile Phan M.D., Y. Harati M.D. Baylor College of Medicine Neuromuscular Diseases. History.

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unusual case of a brachial plexus disorder

Unusual Case of a Brachial Plexus Disorder

Eddie Patton Jr. M.D, Cecile Phan M.D., Y. Harati M.D.

Baylor College of Medicine

Neuromuscular Diseases

history
History
  • This is the case of a 44 y/o right handed man who suffered from a sudacuteonset of a slowly progressive right arm weakness and atrophy, particularly of the biceps, beginning 4 months before his presentation to clinic.
  • Three weeks before presentation he developed right leg pain and weakness
  • No history of back pain or trauma
  • No history of bowel or bladder dysfunction
history1
History
  • PMH: Noncontributory
  • PSH: Sinus surgery
  • SH: denies tobacco, ETOH, or illicit drugs, animal groomer who lives at home with his wife
  • FH: No history of muscle or nerve disorders
  • ROS: Positive for bi-frontal headaches beginning 2 wks before presentation and decreased sleep
history2
History
  • Physical exam pertinent positives
    • Severe atrophy of right biceps
    • 4/5 strength in right suprascapular, deltoid, brachioradialis, triceps, hand intrinsics, illiopsoas, quadriceps, hamstring, tibialis anterior, extensor hallicus longus
    • 3/5 strength in right wrist extensors
    • 1/5 strength in right biceps
    • Reflexes +1 right brachioradialis and biceps, +3 bilateral patellar and ankle
    • Babinski absent bilaterally
    • Sensation mildly decreased to LT and PP right lateral leg
emg summary
EMG Summary
  • Neurogenic signs in 3 proximal muscles of the right upper extremity (Biceps C5-6, Infraspinatus C5-6, Triceps C6-8) and one distal muscle (Flexor Carpi Radialis C6-7).
  • +1 low amplitude reinnervation units in Biceps
  • Neurogenic signs in 1 distal muscle (Tibialis Anterior)
  • Comments: “Findings of patchy denervation of the right brachial plexus, predominantly in the C5 and C7 distribution with a non-conductible right musculocutaneous nerve. Mild involvement of the right L4/5 and S1 muscles”
differential diagnosis
Differential Diagnosis ?
  • HNPP
  • Focal variant of CIDP
  • Vascular (ischemic steal syndrome, thoracic outlet syndrome, subclavian or axillary aneurysm)
  • Radiation induced plexopathy
  • Traction or mechanical injury
  • Neuralgia Amyotrophy ( Parsonage-Turner Syndrome)
  • Neoplasm
    • Primary (Schwannoma or nerve sheath tumor)
    • Secondary ( Pancoast tumor)
further work up
Further work-up
  • MRI brachial plexus
radiology
Radiology

“Abnormal thickening of the right brachial plexus probably at the level of the superior trunk with enlargement also of the right C5-C6 nerve roots”

further work up1
Further Work-up?
  • Focal biopsy of right brachial plexus mass
slide13
Semi Thin: onion-bulbs

Neuro-filament: axial view of axon staining positive for NF within onion-bulb

differential diagnosis1
Differential Diagnosis ?
  • Focal nerve enlargement
    • Schwannoma
    • Neurofibroma
    • Solitary circumscribed neuroma
    • Perineuronoma
    • Dermal nerve sheath myxoma
    • Hybrid benign peripheral nerve sheath tumor
    • Focal CIDP
ema and s 100 protein stains
EMA and S-100 protein stains
  • “ EMA confirms the formation of concentric rings of positively staining spindle cells consistent with perineurial cells. Although S100 is positive in axons, it is a dominant component of nonlesional nerve”
  • “Subsequent review of electron microscopy shows both Schwann cells and cells with discontinuous basal lamina and occasional pinocytosis surrounding centrally placed axons”
perineurioma
Perineurioma
  • 1978- Lazarus and Trombetta coined term after case of a 45 y/o man with a calf tumor
  • Clinically presents as progressive loss of motor function
    • Sensory deficit and pain are uncommon
  • True tumor consists of whorls and fascicles of spindle cells with ultrastructure of perineurial cells
    • Incomplete basal lamina
    • Poorly formed tight junctions
    • Pinocytotic vesicles
  • Gold Standard- + EMA stain and – S-100
    • Neoplastic perineurial cells express immunoreactive epithelial membrane antigen (EMA)
    • Schwann cells immunoreactive to S-100 protein
treatment of nerve sheath tumors
Treatment of Nerve Sheath Tumors

Observation

Surgical removal

Controversial

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