plexopathies: complex & perplexingvideotaping or taking pictures of the slides associated with...
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2019
Plexopathies: Complex & PerplexingRyan D. Jacobson MDAssistant Professor of NeurologyRush University Medical Center, Chicago IL
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2019
Financial Disclosure• I have no relevant disclosures
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2019
WarningVideotaping or taking pictures of the slides associated with this presentation is prohibited. The information on the slides is copyrighted and cannot be used without permission and author attribution.
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Agenda
• Goal: to provide a case-based discussion of common and uncommon issues in the neuromuscular evaluation of brachial plexopathies with focus on key clinical pearls
• The approach will be more clinical and decision-making based rather than anatomic or electrodiagnostic.
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Agenda
• Brachial Plexus Anatomy & Trauma
• Brachial Neuritis / Parsonage-Turner Syndrome– Pathophysiology & Anatomic Specifics– Imaging– Prognosis– Rare Causes & Associations
• Malignancy: Infiltration vs. Radiation
• We will not delve into MR vs. ultrasound evaluation of the plexus much.
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• Part 1: Brachial Plexus Anatomy & Trauma
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Case 1: Caught in the Crossfire• 27-year-old man presents for evaluation of right hand pain and
weakness• 4 months prior, he was caught in the crossfire at a gas station, and
shot in the right chest. Bullet exited right armpit.• Immediately thereafter, the right arm was “frozen.”
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Case 1, continued
• Over the subsequent months, his right arm strength has improved markedly.
• However, he continues to experience severe weakness of the right hand.
• He has numbness over the right 4th and 5th digits, and medial hand.
• Referred for EMG to evaluate localization of lesion, for prognostication
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Case 1, examination
• On focused examination, there is wasting of the right hand intrinsics and the anterior forearm.
• There is weakness of the deep finger flexors, interossei, abductor pollicis brevis, flexor pollicis longus, and wrist flexors.
• Proximally, the arm is strong, and pronation, wrist extension, and finger extension seem strong.
• Pinprick is diminished over the 5th digit and medial 4th digit.
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Where would you localize this patient’s symptoms?
• C8 nerve root?
• Lower trunk of the brachial plexus?
• Medial cord of the brachial plexus?
• Lateral cord of the brachial plexus?
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42 patients seen following World War 2 with penetrating brachial plexus injuries.
13 upper trunk injuries – all recovered antigravity strength
21 injuries of posterior cord – majority recovered antigravity strength
25 injuries of C8/ T1 / medial cord. – any recovery rare
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• Part 2: Brachial Neuritis / Parsonage-Turner Syndrome
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Inflammatory: Neuralgic Amyotrophy, Brachial Plexitis, Parsonage Turner Syndrome
• Tend to have unilateral pain, followed by weakness and wasting
• Tends to affect upper trunk, tends to be very patchy• Special predilection for long thoracic nerve, suprascapular
nerve, sometimes distal involvement (AIN)• Pathology microvasculitic• Prognosis for continued recovery good• Steroids: may help pain, little evidence for help in motor
recovery• MRI may be abnormal but mostly a clinical diagnosis
What’s in the literature?
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Annual incidence of 1.64 per 100,000 population
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Precipitating Causes
• Early case series:– Spillane 1946: 26 of 46 cases were already hospitalized at the time of
presentation– Parsonage and Turner: some precipitating factor or illness in 98 of 136.
66 were already hospitalized
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Prognosis and Natural History
• Early reports from Parsonage and Turner (1957)
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Treatment of Parsonage-Turner?
• Tsairis, 1972: 10 patients given oral prednisone, 5 given IM ACTH, 15 with cortisone injections into the shoulder. Some pain relief.
• Van Alfen 2006: 41 of 246 patients treated with corticosteroids (oral). 25% found it helpful, median time to decrease in pain was 5 days compared to 20 days.
• Van Eijk 2009: 50 patients treated with oral prednisone for a month and compared to historical controls. See next slide.
27
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28
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Case 2: Inpatient with Shoulder Pain
• 69-year-old man with multiple myeloma presented for evaluation of right shoulder pain and weakness
• History of multiple myeloma 9 months prior when he presented with back pain and compression fracture. X-rays revealed numerous lucencies.
• SPEP/IFIX abnormal, and bone marrow biopsy consistent with myeloma
• Treated with Bortezomib, Lenalidumide, Dexamethasone
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Case 2, continued
• Admitted 3/2017 for autologous stem cell transplant with melphalan conditioning “PBSCT”
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Case 2, continued• 3/13, 3/14/17: Melphalan infusion• 3/15/17: PBSCT• 3/20/17: developed severe right shoulder
pain and received morphine• 3/21/17: notes difficulty in lifting arm above
his head• 3/24/17: neurology consultation, unable to
abduct shoulder• 3/27/17: discharged from hospital
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EMG, 4/7/2016
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Case 2, continued
• 4/18/17: follows up in neurology clinic.
• Examination: atrophy of right infraspinatus. Full strength other than:– R Deltoid 2/5 strength– R Infraspinatus 3/5 strength– No obvious scapular winging
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Diagnosis?
Brachial Neuritis
Why?
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• Several autoimmune complications, including neuromuscular complications, possible after allogeneic SCT
• Case 1: autologous SCT, melphalan. Bilateral upper extremity weakness and areflexia 14 days later.
• Case 2: autologous SCT, melphalan. Bilateral upper extremity weakness and wrist drop “within 14 days.”
• Case 3: autologous SCT, melphalan. Unilateral hand pain and C5/C6 myotome weakness 14 days later.
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Case 3: Swim in the Ganges gone wrong
• 29-year-old man is referred for evaluation of left shoulder pain and weakness
• Recently admitted for acute liver failure• Traveling in India from mid-December through
mid-March 2017• In mid-March, he went swimming in the Ganges
River with his roommate to celebrate the end of the trip
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Case 3, continued
• Late March 2017: developed fever, chills, nausea, and abdominal pain. Developed unusual “greasy” bowel movements
• 4/8/2017: transferred to academic center
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Case 3, continued
• 4/9/2017: begins to note unusual, vague, dull left shoulder pain
• 4/10/17: noted to have numbness, paresthesias over left L deltoid muscle. PT notes difficulty with shoulder abduction and external rotation. X-ray normal.
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Case 3, continued
• 4/21/2017: continues to note severe L shoulder pain and mild shoulder weakness.
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5/8/17• Neurology visit: pain has improved to 4/10 severity, but still with severe
left shoulder pain
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Hepatitis E
• ssRNA virus• Spread by fecal-oral route, blood. Often spread through drinking
water.• Most common cause of acute infectious hepatitis in the world.• Only 2-5% of infected patients are symptomatic.• Endemic in Asia, Africa
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• About 30-40 cases in the literature• None in the US as far as I can tell • 26 of the 30 with bilateral involvement
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Case 3, update
• Prescribed 50 mg prednisone
• Physical therapy
• Weakness gradually improved
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Case 4: Complex Patient with a Floppy Thumb
• 49 year old man presents for evaluation of left thumb weakness• History of lupus nephritis, renal transplant• About 5 weeks ago, fell about 8 feet and bruised his ribs, spent
two days in bed• While in bed, noted worsening left shoulder, elbow, and hand pain
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Case 4, continued
• While recovering, noted that his left thumb was weak and could not be bent correctly.
• Also developed unusually severe pain over the pad of the left thumb.
• Over time, the pain has gradually improved but severe thumb flexion weakness persists.
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Case 4, examination
General: old fistula site in left volar mid forearm
Neurologic ExamMotor: full strength other than no movement of left thumb flexion. Thumb opposition, abduction strong. Pronation strong, finger flexion strong.Sensory: pinprick sensation normal throughout
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Predilection for the AIN
• First seen by Parsonage & Turner, further defined by Kiloh and Nevin (1952)
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• Reviewed 16 cases of AIN palsy• 4 traumatic, 12 spontaneous
ANTERIOR INTEROSSEOUS NERVE PALSYA review of 16 cases
M. K. SOOD and F. D. BURKE
From the Pulvertaft Hand Centre, Derbyshire Royal Infirmary, Derby, UK
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• Part 3: Malignancy, Infiltration vs. Radiation
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Case 5
• 63 year old woman presents for evaluation of right arm weakness and numbness• 2014: diagnosed with R breast cancer, treated with surgery, radiation, chemotherapy• 2016-2017: presented with recurrent disease affecting R chest skin, R chest wall. Treated
with chemotherapy• 2017: underwent right carpal tunnel release
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Case 5
• She now presents to the neurology clinic for worsening R arm symptoms.
• Despite CTS surgery, she has worsening numbness of the lateral hand and lateral forearm
• Also notices mild weakness of the R arm
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Case 5
• Exam:4-/5 weakness in R bicepsTrace weakness R deltoid, triceps, infraspinatus.Hyporeflexic throughout.Diminished pinprick sensation over lateral hand and forearm.
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MR Brachial Plexus
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Neoplastic Plexopathy
• Most common in lung/breast cancer• More likely to affect the lower trunk• More likely to be painful• EMG: dysfunction in distribution of injury• Imaging: may show enhancement, nodularity, presence of tumor
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Radiation Plexopathy
• Most common following treatment of breast cancer• Tends to develop insidiously over months – mean of 10 months after radiation
completion• More likely to affect upper trunk• Less likely to be painful• EMG: dysfunction in distribution of injury, fasciculations and myokymia• Imaging: may show hyperintensity, enhancement of plexus
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• THANK YOU
• Please see the printed handout for references.