11/17/2019
6th Annual Scientific Meeting of Thai Neuromuscular and Electrodiagnostic Medicine Society
Challenging EDX and Ultrasound Cases
David C Preston, MD Bashar Katirji, MD Professor of Neurology Professor of Neurology
Neurological Institute University Hospitals – Cleveland Medical Center Cleveland, Ohio
History – Case 1
• 44 year-old-lady woman with relapsing remitting Multiple Sclerosis presented with acute right wrist drop. Went to sleep the night before without any problem. Awoke with the inability to extend her wrist or fingers with numbness over the dorsal hand. • Nothing unusual about her sleep. Slept in her own bed. Did not drink alcohol or take any sedating medications. • Prior history of diabetes and presumed diabetic neuropathy affecting her feet.
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Exam- Case 1
Motor Deltoid 5/5 Triceps 5-/5 Brachioradialis 2/5 Wrist extension 1/5 Finger extension 1/5 All other muscles normal Reflexes RT LF BR 0 0 Biceps 2 2 Triceps 3 2 Sensory: Decreased over the lateral dorsum of the right hand, in the distribution of the superficial radial nerve.
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Motor NCS
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Sensory NCS
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EMG
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Case 1 Diagnosis:
Obvious radial neuropathy at the spiral groove
“Must have sleep on it funny”
“Probably will get better”
“A second year medical student could have figured this out with an EMG”
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What?
You ordered a neuromuscular ultrasound!
What a waste of money and resources!
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Case 1 (Real) Diagnosis:
Radial neuropathy secondary to compression by a large ganglion cyst arising from the elbow joint that was compressing the deep and superficial branches of the radial nerve and the branch to the brachioradialis.
“Didn’t sleep on it funny”
“It is not going to get better without surgical intervention”
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Case 2
• A 56 year old female, recently moved to USA. • Consultation for a longstanding peripheral neuropathy. • Symptoms began 18 years prior to presentation. • First symptom--right leg weakness and numbness with gait difficulty. • 10 years later- developed right hand weakness. • Her left leg also became very weak leading to falls. • 6 months prior, developed left hand weakness (dependent hand). • Main fear was if the disease would continue to progress.
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Neurological Examination • Mental Status and Cranial nerve : Normal • Motor Exam: Severe diffuse atrophy Muscle strength (MRC out of 5)
Right Left
Shoulder abduction 44 Elbow flexion and extension 44 Finger flexion 00 Finger extension 30 Finger abduction 11 Hip flexion and extension 4+ 4+ Knee flexion/ extension 44 Ankle dorsiflexion 00 Ankle plantarflexion 33 Eversion 13 Inversion 13
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Neurological Examination
• Sensory exam: • Decreased sensation to pinprick to the mid-forearm in the upper limbs and above the knee in the lower limbs. • Vibration sense was absent in the ankles but intact at the knees. • Reflexes: absent all over. • Gait: showed bilateral steppage gait.
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Based on her history and examination we know that she has a chronic polyneuropathy. We do not know if it is primarily demyelinating? Or axonal?
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EMG
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EMG wave
Ulnar Wr-ADM
Below Elbow-Ulnr Wr
Above Elbow-Ulnr Wr
Ulnar nerve CMAP 0.2mV/D 5ms/D
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EMG
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What is the next step?
• Retrieving her prior EMGs. • MRI of the brachial plexus and lumber spine. • Proximal NCS ( Erb’s, roots stim). • Nerve biopsy. • Lumbar puncture. • Neuromuscular ultrasound.
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Next Step in Work up
• MRIs are costly, time consuming and not practical when multiple nerves need to be studied.
• Proximal NCS technically difficult and some patients cannot tolerate a full study.
• Neuromuscular ultrasound (NMU) is a noninvasive, painless, inexpensive, and radiation-free approach for evaluating multiple peripheral nerves in one study.
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EMG
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Neuromuscular ultrasound
NMU of the trunks in a normal subject NMU of our patient
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Upper Trunk
CSA 32.7mm2 NL<8
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Middle Trunk
CSA 265.4 mm2 NL <8
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Lower Trunk
CSA 22.4 mm2 NL<8
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Median Nerve at The Arm
CSA 83.2 mm2 NL<10
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Median Nerve at the Forearm
CSA 38.9 mm2 NL<10
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Interpretation of the Neuromuscular Ultrasound
• Study was markedly abnormal.
• Severe nerve enlargement at non entrapment sites.
• Severe nerve enlargement at multiple locations.
• Normal median and ulnar nerves at wrist
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CIDP
• Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder of the peripheral nervous system and results in sensory and motor impartment.
• The diagnosis is based on combination of clinical picture and electrodiagnostic findings consistent with demyelination.
• Sometimes, these findings are absent on EMG.
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Neuromuscular Ultrasound in CIDP
• 2009, Zaidman and colleagues studied 36 patient with CIDP. And 36 with axonal neuropathy as part of larger neuropathy study.
• 31/36 CIDP patients had enlarged nerves.
• 7/36 axonal neuropathy patients had enlarged nerves.
• Conclusion: CSA is larger in demyelinating neuropathy.
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Neuromuscular Ultrasound in CIDP
• Goedee and colleagues in 2017 published a paper with axonal neuropathy CSA cutoffs. • Study also found that enlargement of brachial plexus specifically upper trunk is highly characteristic of CIDP. • They also found that in CIDP, even if upper limb was not clinically affected, it was common to see enlargement of the median nerve and brachial plexus.
From Goedee HS et al. Diagnostic value of sonography in treatment- naive chronic inflammatory neuropathies. Neurology. 2017 88:143-151.
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Nerve size cross sectional area mm2
Normal Axonal Our neuropathy patient
Median Nerve at the Mid- forearm <10 10 R 38.9 L 36
Median Nerve at the Mid-arm <10 13 R 83.2 L 154.1
Upper trunk <8 8 R 32.7 L 50.5
Middle trunk <8 8 R 265.4 L 302.8
Lower trunk <8 8 R/L 22.4
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From Goedee HS, van der Pol WL, van Asseldonk JH, Franssen H, Notermans NC, Vrancken AJ et al. Diagnostic value of sonography in treatment-naive chronic inflammatory neuropathies. Neurology. 2017 88:143-151.
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Conclusion
• Our patient had severe multifocal nerve enlargement at non entrapment sites.
• Severe nerve enlargement at multiple locations.
• There was no diffuse enlargement of the peripheral nerves.
• She had enlargement of upper trunk and median nerve, which highly characteristic of CIDP (Lewis Sumner syndrome).
• Neuromuscular Ultrasound was the key to confirming the diagnosis.
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History – Case 3
• A 33-year-old woman developed worsening numbness over her right little finger associated with elbow pain and weakness of the hand. • She had a history of some type of ulnar nerve surgery on the elbow over 10 years ago, but did not know any details of what exactly was done. • However, she described her symptoms as similar to what she had experienced before the surgery.
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Exam- Case 3 Clawed fifth finger
Slight atrophy of the ulnar intrinsic hand muscles
Pronounced weakness of the interossei, and long finger flexors to digits 4 and 5
Subtle loss of sensation over the medial hand into the fifth digit on both the volar and dorsal sides of the hand.
“Curious” finding that all of her fingers were longer and wider on that hand.
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Case 3 Diagnosis:
Ulnar neuropathy
Non-localizable
Demyelinating in multiple segments and axonal loss
Etiology not clear. Mononeuropathy presentation of CIDP????
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Case 3 (Real) Diagnosis:
Neural fibrolipoma (fibrolipomatous hamartoma among other names)
Most commonly reported in the median nerve at the wrist
It can affect other nerves, including the ulnar nerve
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Case 3 (Real) Diagnosis:
“Benign” tumor with growth of fibrous and adipose tissues around the nerve sheath and within the nerve
Macrodactyly is present in about two-thirds of patients
Ultrasound: unmistakable appearance of an enlarged nerve (often dramatically enlarged) with hypoechoic fascicles with additional tissue between the fascicles
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Case 4: History
• 46 y/o man • 8 years of pain in the proximal dorsal forearm • No definite weakness of numbness • Sent for a NCS / EMG
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Exam
• Strength: normal • Sensation: normal • Reflexes: normal • Muscle bulk: Normal • Marked tenderness to palpation over the dorsal proximal forearm
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Summary
• Normal neurological exam
• Normal NCS
•Normal EMG
Now What?
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Radial tunnel syndrome
• Isolated pain and tenderness in the extensor forearm, thought to result from compression of the posterior interosseous nerve near its origin
• However, this is one of the more controversial and disputed nerve entrapment syndromes
• As opposed to patients with a true posterior interosseous neuropathy, typically have no objective neurologic signs on examination, and have normal EDX studies
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Radial tunnel syndrome
• Space formed posteriorly by the distal humerus and radiocapitellar joint, the brachialis muscle medially, the brachioradialis muscle anteriorly and extensor carpi radialis brevis muscle laterally
• Radial tunnel is approximately 5 cm long and runs between where the radial nerve pierces the lateral intermuscular septum to where the deep motor branch enters the proximal edge of the supinator
• Some consider the radial tunnel continues to where the posterior interosseous nerve leaves the distal border of the supinator
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Case 5: History
• 12-year-old boy collided with a friend and fell, sustaining a fracture of the left radius and ulna • Presented to a local emergency room. • No obvious neurovascular injury • Taken to the OR for closed reduction and application of a long arm splint • When the splint was removed, noted numbness of digit and 4, and clawing of digits 4 and 5.
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Case 5: Exam • Froment’s sign • Clawing of D4-5 • Weakness of ulnar intrinsic hand muscles with mild atrophy • Decreased sensation over D5 and medial D4 (volar side). Dorsal side equivocal sensory loss. • Decreased ROM of the wrist
• Tinel’s sign over the ulnar nerve at the wrist • Tinel’s sign over the ulnar nerve at the elbow
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Hand Surgery Evaluation
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Motor NCS
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Sensory NCS
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Limited EMG
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Impression
• Ulnar neuropathy at or above the wrist
• The reduced ulnar sensory amplitude with symmetric and normal dorsal ulnar amplitudes suggests a localization at the wrist, but a more proximal localization cannot be excluded.
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Now What?
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Neuromuscular US
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Wrist XR
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Wrist XR
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Summary
• US and EDX studies were complimentary
• EDX allowed information about the severity and localization
• US demonstrated exact localization and etiology
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