Case 1 71 y/o woman with HTN and asthma • February 2015 Just another neuropathy? • Suddenly noted she couldn’t write with her right What not to miss. hand. No pain. Could not feel pain if she cut herself • One week later, left foot started to flop and worsened over several days Ann Poncelet, MD Professor of Neurology • Several days later, left hand became weak and William G. Irwin Endowed Chair numb 2/15/2018 • Several days later, right foot gave out Case 1 Case 1 • March 2015 • April 2015 • Admitted to OSH with diagnosis of Guillian Barre • Progressed to being wheelchair bound Syndrome • NCS by local neurologist showed no motor or • CSF with negative gram stain and culture, WBC sensory responses in legs. Arms not tested. 0, RBC 9. Glucose 61. TP 38. HSV prc neg. West nile IgG and IgM neg. • May 2015 • Treated with IVIg but no response to treatment. • Treated again with IVIg but no improvement • Discharged to home • Neuropathy stopped progressing at this point 1
Case 1 Review of Systems • April 2015 and July 2015 Gen: (+) 40lb weight loss (-) fevers, chills, malaise, diaphoresis • Asthma attacks treated with prednisone taper. HEENT: (+) oral ulcers (-) congestion, hearing changes,nose bleeds, Improved breathing but no effect on neuropathy vertigo, hoarseness, changes in vision, photphopbia/diplopia • August 2015 Resp: (+) cough/wheeze, scant yellow phlegm. (-) hemoptysis/dyspnea • Right index finger tip became discolored and CV: (-) chest pain, palpitations, orthopnea progressively darker GI: (-) n/v/d/c • Admitted to OSH for further evaluation GU: (-) hematuria/dysuria • Had multiple tests, consults with oncology, MSK: (-) arthralgias, myalgias, back/neck pain gastroenterology without a clear diagnosis and Neuro: (+) weakness/numbness of bilateral hands and feet. (-) recommendation to see a vascular specialist seizure, syncope, headaches • Left AMA to have appointment in neuromuscular Heme: (-) bruising/bleeding clinic at UCSF Oropharynx exam Skin Exam 2
Case 1-Neurologic Exam Skin Exam (cont) • Mental status and cranial nerve: exam normal • Motor examination: decreased bulk in the intrinsic muscles of the hands, APB, lower legs with diffusely decreased bulk due to weight loss. There is decreased tone in the fingers and ankles. DTR: absent at the ankles, right knee and 2+ elsewhere • Muscle Deltoid Biceps Tricep IS SS WE FCR Case 1-Neurologic Exam Right 5 5 5 5 5 5 5 Left 5 5 5 5 5 5 5 • Gait: Unable to walk FCU Pron Sup EDC EIP FPL Right 5 5 5 5 4 4 • Coordination: slow finger tap, unable to do foot Left 5 4 4 4 4 4+ tap, finger-nose and heel-knee-shin. FDP 1/2 FDP 3/4 ADM FDI APB BR • Sensory examination: absent vibration to right Right 5 5 3 3 0 5 Left 5 4 1 1 0 4 PIP joint and left wrist and in legs, absent IP Glut Hip Hip abd Quad Ham position sense in bilateral DIP joint and absent to Max add knees, absent pinprick to the wrists, right knee Right 5 5 5 5 3 5 Left 5 5 5 5 5 5 and mid left calf. Dorsi Plantar Ever Inv Toe Toe flex flex flex ext Right 0 0 0 0 0 0 Left 0 0 0 0 0 0 3
Differential Diagnosis Mononeuritis Multiplex Neuro-anatomic Localization Ischemic neuropathies Drug-induced neuropathies* Neuropathies related to neoplastic disease Peripheral nerve vasculitis Montelukast and other leukotriene receptor Direct infiltration of nerves by tumor antagonists Livedoid vasculopathy Interferon alpha Paraneoplastic (vasculitic and non vasculitic) Sickle cell anemia TNF-alpha inhibitors Multifocal peripheral nerve tumors Cholesterol emboli syndrome Antibiotics (PCN, sulfonamides, minocycline) Neurofibromatosis 2 Atrial myxoma Drugs of abuse (amphetamines, cocaine, heroin) Lymphoid granulomatosis Thrombophilic states Other (see article) Intravascular large B cell lymphoma Inflammatory/immune mediated neuropathies Genetic neuropathies Neoplastic meningitis Sarcoidosis* HNPP Primary AL amyloidosis Mononeuritis Multiplex (MM) Multifocal CIDP Hereditary neuralgic amyotrophy Intraneural hemorrhage Multifocal motor neuropathy CMT (many) ITP Multifocal variants of GBS Porphyria Acute leukemia Neuralgic amyotrophy Familial amyloid polyneuropathy Hemophilia Neuropathy with eosinophilic disorders* Tangier disease Degenerative disorders Neuropathy with GI disorders (Crohn, UC, Celiac Krabbe disease Motor neuron disease with sensory sprue)* involvement Graft vs host disease Mitochondrial disorders Sensory perineuritis* Mechanical neuropathies Infectious/toxic neuropathies* Multiple peripheral nerve injuries or burns Leprosy Multiple entrapment neuropathies Lyme disease Wartenberg’s migrant sensory neuritis (mechanical cases) Viral (HVC, HBV, HIV, HTLV-1, VZV, CMV, parvovirus B19, EBV, west nile virus, measles) Other (see article) Current Opinion 2012;25:573-585. Collins, Curr Opin 2012 Primary systemic vasculitis 4) Infection 1) Predominantly small vessal vasculitis HBV Microscopic polyangiitis (MPA) HCV Eosinophilic granulomatous polyangiitis (EGPA or Churg Strauss HIV Cryoglobulinemic Syndrome) Granulomatosis with polyangiitis (Wegener’s) CMV Vasculitis Sjogren’s Essential mixed cryoglobulinemia (non-HCV) Leprosy Skin Syndrome Emboli Henoch-Schonlein purpura (HSP) Lyme disease 1) Predominantly medium vessel vasculitis HTLV-1 Polyarteritis nodosa (PAN) 5) Drugs Vasopressors Levamisole 1) Predominantly large vessel vasculitis 6) Malignancy Sneddon’s 7) Inflammatory bowel disease Syndrome 8) Hypocomplementemic urticarial vasculitis syndrome Secondary systemic vasculitis RA SLE 1) Connective tissue diseases Hep B Rheumatoid arthritis Nonsystemic/localized vasculitis Allergic GPA/MPA SLE 1) Nonsystemic vasculitis neuropathy HIV BronchoPulm EGPA Leprosy Primary Sjogren’s syndrome 1) Non-diabetic radiculoplexus neuropathy PAN Aspergillosis Lyme Systemic sclerosis 1) Diabetic radiculoplexus neuropathy Asthma Neuro Dermatomyositis 1) Localized cutaneous/neuropathic vasculitis Sarcoid Mixed connective tissue disease Cutaneous PAN 1) Sarcoidosis Others Heart Failure Diabetes Sensory neuritis 1) Behcet’s disease Hypereosinophillic MMN Mulifocal CIDP pneumonitis Collins Curr Opin 2012 15 4
OSH Labs Case 1—EMG/NCS Urine Studies UA 9/4: protein 30, mod blood, 38 - 4/1 5/13 7/1 8/4 9/4 9/8 WBC, 4 RBC, 8 squams, 6 transitional epi WBC 15.8 25.9 27.1 18.4 29.6 20.3 • Motor NCS Serologies Eos 11.3 18.6 19.8 12.3 25.5 10.7 • Peroneal, tibial and median CMAPs absent ANCA 9/5: MPO 391.8, PR3 <1 - Hb 11.3 11.7 9.3 7.2 7.7 9.0 ANA 9/5: negative - • Left ulnar CMAP absent Prev (-) Ro/La/RNP/Sm, dsDNA, SCL70, Hct 36 37/2 30.9 25.6 25.6 29.2 - anti-TPO, RF, gastric parietal cell ab Plt 367 493 459 459 406 372 Ulnar.R to Abductor digiti minimi (manus).R Imaging Wrist 5.7 0.3 0.6 2.8 31.8 Abductor digiti minimi (manus)-Wrist 5.7 80 ESR 97 77 --- --- 108 --- MRI Brain 3/27: small basal ganglia - infarcts. Severe R maxillary and ethmoid sinusitis 4/1 5/13 7/1 8/4 9/4 9/8 Radial.R to Extensor indicis proprius.R MRI C/T spine 3/27: no acute Forearm 3.3 0.8 6.7 11.8 31.5 Extensor indicis proprius-Forearm 3.3 80 - Na 141 141 139 138 135 134 ECHO 9/6: No valve abnl, nl LVEF - K 3.3 2.8 3 4.5 3.7 3.3 Lateral brachium 6.2 0.8 6.9 31.8 Forearm-Lateral brachium 2.9 120 41 Pathology BUN 5 9 18 21 17 11 Small bowel 9/5 – with focal non- Radial.L to Extensor indicis proprius.L - Cr 0.24 0.29 0.85 0.65 0.68 0.78 specific inflammation Forearm 3.2 1.7 13.6 12.0 30.9 Extensor indicis proprius-Forearm 3.2 80 Colon 9/6: focal granulation tissue - Ca 8.8 10.1 9.7 9.4 9.9 8.8 Lateral brachium 5.0 1.7 14.2 12.3 31.0 Forearm-Lateral brachium 1.8 85 47 and acute on chronic inflammation Alb 2.2 --- 2.1 --- 2.0 --- c/w ulcer. No neoplasm. BMBx: PENDING CRP 61.4 61.4 --- --- 92.9 --- - Case 1—EMG/NCS Rheumatology Consultation • Sensory NCS • Recent adult-onset, steroid dependent asthma, severe eosinophilia, positive MPO, renal involvement • Sural, superficial peroneal, median, ulnar and radial (more than doubling of creatinine since 3/2015, SNAPs absent proteinuria, ?hematuria), and polyneuropathy is Spontaneous Volitional MUAPs Max Vol Act highly concerning (and nearly pathognomonic) for Muscle Fib/PSW Fasc Dur Amp Poly Recruit Interference Max Comment eosinophillic granulomatosis with polyangiitis Freq Tibialis anterior.L 2+ None no muaps (formerly known as Churg-Strauss Syndrome). Gastrocnemius (Medial 2+ None no muaps head).L Vastus medialis.L None None 9-14 0.6-1.6 None Normal Mild 30 Hz • Recommended immediate treatment with Biceps femoris (short None None 12-16 1.0-2.0 Few Normal Mild 30 Hz glucocorticoids due to life threatening organ head).L Deltoid.L None None 12-16 1.0-2.0 Few Normal Mild 30 Hz involvement Biceps brachii.L None None 12-16 1.0-2.0 Many Normal Mod Dec 20 Hz pain limited • Recommended skin, kidney biopsies and review of 1st dorsal interosseous.L 3+ None no muaps prior bone marrow biopsy to confirm Extensor indicis proprius.L 1+ None 12-16 1.0-2.0 Many Normal Mild 30 Hz 5
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