Unusual case of a brachial plexus disorder
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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


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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