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Challenging EDX and Ultrasound Cases History – Case 1 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. 2 1 11/17/2019 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. 3 Motor NCS 4 2 11/17/2019 Sensory NCS 5 EMG 6 3 11/17/2019 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” 7 8 4 11/17/2019 What? You ordered a neuromuscular ultrasound! What a waste of money and resources! 9 10 5 11/17/2019 11 12 6 11/17/2019 13 14 7 11/17/2019 15 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” 16 8 11/17/2019 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. 17 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 18 9 11/17/2019 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. 19 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? 20 10 11/17/2019 EMG 21 EMG wave Ulnar Wr-ADM Below Elbow-Ulnr Wr Above Elbow-Ulnr Wr Ulnar nerve CMAP 0.2mV/D 5ms/D 22 11 11/17/2019 EMG 23 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. 24 12 11/17/2019 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. 25 EMG 26 13 11/17/2019 Neuromuscular ultrasound NMU of the trunks in a normal subject NMU of our patient 27 Upper Trunk CSA 32.7mm2 NL<8 28 14 11/17/2019 Middle Trunk CSA 265.4 mm2 NL <8 29 Lower Trunk CSA 22.4 mm2 NL<8 30 15 11/17/2019 Median Nerve at The Arm CSA 83.2 mm2 NL<10 31 Median Nerve at the Forearm CSA 38.9 mm2 NL<10 32 16 11/17/2019 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 33 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. 34 17 11/17/2019 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. 35 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. 36 18 11/17/2019 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 37 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. 38 19 11/17/2019 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. 39 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. 40 20 11/17/2019 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. 41 42 21 11/17/2019 43 44 22 11/17/2019 Case 3 Diagnosis: Ulnar neuropathy Non-localizable Demyelinating in multiple segments and axonal loss Etiology not clear. Mononeuropathy presentation of CIDP???? 45 46 23 11/17/2019 47 48 24 11/17/2019 49 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 50 25 11/17/2019 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 51 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 26 11/17/2019 Exam • Strength: normal • Sensation: normal • Reflexes: normal • Muscle bulk: Normal • Marked tenderness to palpation over the dorsal proximal forearm 27 11/17/2019 28 11/17/2019 Summary • Normal neurological exam • Normal NCS •Normal EMG Now What? 29 11/17/2019 30 11/17/2019 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 31 11/17/2019 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 32 11/17/2019 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.
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