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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.

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Presentation on theme: "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."— Presentation transcript:

1 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

2 History This is the case of a 44 y/o right handed man who suffered from a sudacute onset 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

3 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

4 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

5 Exam

6 EMG/NCS Left Motor NervesF-waveDistal Latency Proximal Latency Distal AmpProximal AMP MCV Long Thoracic Musculocutaneous Common Peroneal Tibial608.8 (<6.6) Right Motor NervesF-WaveDistal Latency Proximal Latency Distal Amplitude Proximal Amplitude MCV Median Ulnar Long Thoracic (>2.0) Axillary Musculocutaneous NC Common Perponeal Tibial (>42)

7 EMG/NCS Left Sensory NervesDistal Latency Proximal Latency Distal AmpProximal Amp MCV Dorsal Suralabs Right Sensory Nerves Median II Ulnar V Lateral antebrachial3.0 (<2.6)10.0 Sural4.8(<4.0) Dorsal SuralAbs

8 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

9 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)

10 Further work-up MRI brachial plexus

11 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

12 Further Work-up? Focal biopsy of right brachial plexus mass

13 Semi Thin: onion-bulbs Neuro-filament: axial view of axon staining positive for NF within onion-bulb

14 Differential Diagnosis ? Focal nerve enlargement – Schwannoma – Neurofibroma – Solitary circumscribed neuroma – Perineuronoma – Dermal nerve sheath myxoma – Hybrid benign peripheral nerve sheath tumor – Focal CIDP

15 Epithelial Membrane Antigen stain

16 S100

17 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

18 Perineurioma 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

19 Treatment of Nerve Sheath Tumors -Observation -Surgical removal -Controversial


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