Case 1 71 y/o woman with HTN and asthma February 2015 Just another - - PowerPoint PPT Presentation

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2/15/2018

Ann Poncelet, MD Professor of Neurology William G. Irwin Endowed Chair

Just another neuropathy? What not to miss.

• February 2015
• Suddenly noted she couldn’t write with her right
• hand. No pain. Could not feel pain if she cut

herself

• One week later, left foot started to flop and

worsened over several days

• Several days later, left hand became weak and

numb

• Several days later, right foot gave out

Case 1

71 y/o woman with HTN and asthma

• March 2015
• Admitted to OSH with diagnosis of Guillian Barre

Syndrome

• CSF with negative gram stain and culture, WBC

0, RBC 9. Glucose 61. TP 38. HSV prc neg. West nile IgG and IgM neg.

• Treated with IVIg but no response to treatment.
• Discharged to home

Case 1

• April 2015
• Progressed to being wheelchair bound
• NCS by local neurologist showed no motor or

sensory responses in legs. Arms not tested.

• May 2015
• Treated again with IVIg but no improvement
• Neuropathy stopped progressing at this point

Case 1

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• April 2015 and July 2015
• Asthma attacks treated with prednisone taper.

Improved breathing but no effect on neuropathy

• August 2015
• Right index finger tip became discolored and

progressively darker

• Admitted to OSH for further evaluation
• Had multiple tests, consults with oncology,

gastroenterology without a clear diagnosis and recommendation to see a vascular specialist

• Left AMA to have appointment in neuromuscular

clinic at UCSF

Case 1

Review of Systems

Gen: (+) 40lb weight loss (-) fevers, chills, malaise, diaphoresis HEENT: (+) oral ulcers (-) congestion, hearing changes,nose bleeds, vertigo, hoarseness, changes in vision, photphopbia/diplopia Resp: (+) cough/wheeze, scant yellow phlegm. (-) hemoptysis/dyspnea CV: (-) chest pain, palpitations, orthopnea GI: (-) n/v/d/c GU: (-) hematuria/dysuria MSK: (-) arthralgias, myalgias, back/neck pain Neuro: (+) weakness/numbness of bilateral hands and feet. (-) seizure, syncope, headaches Heme: (-) bruising/bleeding

Oropharynx exam Skin Exam

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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

Case 1-Neurologic Exam

Muscle Deltoid Biceps Tricep IS SS WE FCR Right 5 5 5 5 5 5 5 Left 5 5 5 5 5 5 5 FCU Pron Sup EDC EIP FPL Right 5 5 5 5 4 4 Left 5 4 4 4 4 4+ FDP 1/2 FDP 3/4 ADM FDI APB BR Right 5 5 3 3 5 Left 5 4 1 1 4 IP Glut Max Hip add Hip abd Quad Ham Right 5 5 5 5 3 5 Left 5 5 5 5 5 5 Dorsi flex Plantar flex Ever Inv Toe ext Toe flex Right Left

• Gait: Unable to walk
• Coordination: slow finger tap, unable to do foot

tap, finger-nose and heel-knee-shin.

• Sensory examination: absent vibration to right

PIP joint and left wrist and in legs, absent position sense in bilateral DIP joint and absent to knees, absent pinprick to the wrists, right knee and mid left calf.

Case 1-Neurologic Exam

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Mononeuritis Multiplex (MM)

Neuro-anatomic Localization

Current Opinion 2012;25:573-585.

Differential Diagnosis Mononeuritis Multiplex

Collins, Curr Opin 2012 Ischemic neuropathies Drug-induced neuropathies* Neuropathies related to neoplastic disease Peripheral nerve vasculitis Montelukast and other leukotriene receptor antagonists Direct infiltration of nerves by tumor 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 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 sprue)* Krabbe disease Motor neuron disease with sensory 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) Collins Curr Opin 2012

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Primary systemic vasculitis 4) Infection 1) Predominantly small vessal vasculitis HBV Microscopic polyangiitis (MPA) HCV Eosinophilic granulomatous polyangiitis (EGPA or Churg Strauss Syndrome) HIV Granulomatosis with polyangiitis (Wegener’s) CMV Essential mixed cryoglobulinemia (non-HCV) Leprosy Henoch-Schonlein purpura (HSP) Lyme disease 1) Predominantly medium vessel vasculitis HTLV-1 Polyarteritis nodosa (PAN) 5) Drugs 1) Predominantly large vessel vasculitis 6) Malignancy 7) Inflammatory bowel disease Secondary systemic vasculitis 8) Hypocomplementemic urticarial vasculitis syndrome 1) Connective tissue diseases Rheumatoid arthritis Nonsystemic/localized vasculitis SLE 1) Nonsystemic vasculitis neuropathy Primary Sjogren’s syndrome 1) Non-diabetic radiculoplexus neuropathy Systemic sclerosis 1) Diabetic radiculoplexus neuropathy Dermatomyositis 1) Localized cutaneous/neuropathic vasculitis Mixed connective tissue disease Cutaneous PAN 1) Sarcoidosis Others 1) Behcet’s disease

Skin Neuro Asthma

EGPA PAN Cryoglobulinemic Vasculitis SLE Sjogren’s Syndrome GPA/MPA Levamisole Vasopressors Emboli Hep B RA Leprosy Lyme HIV Sensory neuritis MMN Heart Failure Sarcoid Allergic BronchoPulm Aspergillosis Hypereosinophillic pneumonitis Sneddon’s Syndrome Mulifocal CIDP Diabetes

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OSH Labs

4/1 5/13 7/1 8/4 9/4 9/8 WBC 15.8 25.9 27.1 18.4 29.6 20.3 Eos 11.3 18.6 19.8 12.3 25.5 10.7 Hb 11.3 11.7 9.3 7.2 7.7 9.0 Hct 36 37/2 30.9 25.6 25.6 29.2 Plt 367 493 459 459 406 372 ESR 97 77

• 108
• 4/1

5/13 7/1 8/4 9/4 9/8 Na 141 141 139 138 135 134 K 3.3 2.8 3 4.5 3.7 3.3 BUN 5 9 18 21 17 11 Cr 0.24 0.29 0.85 0.65 0.68 0.78 Ca 8.8 10.1 9.7 9.4 9.9 8.8 Alb 2.2

• 2.1
• 2.0
• CRP

61.4 61.4

• 92.9
• Urine Studies
• UA 9/4: protein 30, mod blood, 38

WBC, 4 RBC, 8 squams, 6 transitional epi

• Serologies
• ANCA 9/5: MPO 391.8, PR3 <1
• ANA 9/5: negative
• Prev (-) Ro/La/RNP/Sm, dsDNA, SCL70,

anti-TPO, RF, gastric parietal cell ab

• Imaging
• MRI Brain 3/27: small basal ganglia
• infarcts. Severe R maxillary and

ethmoid sinusitis

• MRI C/T spine 3/27: no acute
• ECHO 9/6: No valve abnl, nl LVEF
• Pathology
• Small bowel 9/5 – with focal non-

specific inflammation

• Colon 9/6: focal granulation tissue

and acute on chronic inflammation c/w ulcer. No neoplasm.

• BMBx: PENDING
• Motor NCS
• Peroneal, tibial and median CMAPs absent
• Left ulnar CMAP absent

Case 1—EMG/NCS

Ulnar.R to Abductor digiti minimi (manus).R Wrist 5.7 0.3 0.6 2.8 31.8 Abductor digiti minimi (manus)-Wrist 5.7 80 Radial.R to Extensor indicis proprius.R Forearm 3.3 0.8 6.7 11.8 31.5 Extensor indicis proprius-Forearm 3.3 80 Lateral brachium 6.2 0.8 6.9 31.8 Forearm-Lateral brachium 2.9 120 41 Radial.L to Extensor indicis proprius.L Forearm 3.2 1.7 13.6 12.0 30.9 Extensor indicis proprius-Forearm 3.2 80 Lateral brachium 5.0 1.7 14.2 12.3 31.0 Forearm-Lateral brachium 1.8 85 47

• Sensory NCS
• Sural, superficial peroneal, median, ulnar and radial

SNAPs absent

Case 1—EMG/NCS

Spontaneous Volitional MUAPs Max Vol Act Muscle Fib/PSW Fasc Dur Amp Poly Recruit Interference Max Freq Comment Tibialis anterior.L 2+ None no muaps Gastrocnemius (Medial head).L 2+ None no muaps Vastus medialis.L None None 9-14 0.6-1.6 None Normal Mild 30 Hz Biceps femoris (short head).L None None 12-16 1.0-2.0 Few Normal Mild 30 Hz Deltoid.L None None 12-16 1.0-2.0 Few Normal Mild 30 Hz Biceps brachii.L None None 12-16 1.0-2.0 Many Normal Mod Dec 20 Hz pain limited 1st dorsal interosseous.L 3+ None no muaps Extensor indicis proprius.L 1+ None 12-16 1.0-2.0 Many Normal Mild 30 Hz

• Recent adult-onset, steroid dependent asthma,

severe eosinophilia, positive MPO, renal involvement (more than doubling of creatinine since 3/2015, proteinuria, ?hematuria), and polyneuropathy is highly concerning (and nearly pathognomonic) for eosinophillic granulomatosis with polyangiitis (formerly known as Churg-Strauss Syndrome).

• Recommended immediate treatment with

glucocorticoids due to life threatening organ involvement

• Recommended skin, kidney biopsies and review of

prior bone marrow biopsy to confirm

Rheumatology Consultation

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• Skin biopsy of left third finger
• HYPOINFLAMMATORY SMALL VESSEL

VASCULITIS WITH ASSOCIATED SMALL VESSEL THROMBOSIS

• Kidney biopsy:
• Focal crescentic glomerulonephritis, pauci-

immune;

• Interstitial fibrosis and tubular atrophy, moderate
• Bone marrow biopsy:
• Normocellular marrow with trilineage

hematopoiesis and increased eosinophils

Case 1-Inpatient Evaluation

Final Diagnosis: Eosinophilic Granulomatosis with Polyangiitis

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• Pulse IV steroids followed by very slow taper of oral
• prednisone. Off prednisone since 11/2017
• Cyclophosphamide 500 mg IV monthly x 6 doses
• Maintenance on oral azathioprine
• Close monitoring by rheumatology and neurology
• Slow but incomplete improvement of neurologic
• function. Continues to improve as of last visit

11/2017!

Clinical Course

• Gait: able to walk with assistance with steppage gait.

Can walk a little ways without the walker while wearing her braces.

• Sensory examination: In the hands, vibration is

decreased in the fingertips. Vibration is decreased to the left ankle and distal right toe or base of the ankle. Normal position sense in the fingers and to the base of the toes. Normal pinprick in the upper and lower extremities

Clinical Course Clinical Course

Muscle Deltoid Biceps Tricep IS SS WE FCR Right 5 5 5 5 5 5 5 Left 5 5 5 5 5 5 5 FCU Pron Sup EDC EIP FPL Right 5 5 5 5 5 (4) 5 (4) Left 5 5 (4) 5 (4) 5 (4) 5 (4) 5 (4+) FDP 1/2 FDP 3/4 ADM FDI APB BR Right 5 5 3+ (3) 3+ (3) 1 (0) 5 Left 5 5 (4) 2 (1) 2 (1) 5 (4) IP Glut Max Hip add Hip abd Quad Ham Right 5 5 5 5 5 5 Left 5 5 5 5 5 5 Dorsi flex Plantar flex Ever Inv Toe ext Toe flex Right 2+ (0) 3 (0) 3 (0) 1 (0) Left 2 (0) 4- (0) 3+ (0) 2 (0)

383 French patients dx between 1957-2009 91% of patients had asthma at the time of diagnosis

• Present for mean 9.3 ± 10.8 years
• 3.4% patients developed asthma within 6 mos of EGPA dx
• Remaining 8.9% had ENT sxs or developed asthma within 6 mos of

EGPA dx Most common manifestations at dx:

• Weight loss – 49.3%
• Mononeuritis multiplex - 46.0%
• Nonerosive sinusitis/polyposis – 41.8%
• Skin lesions – 39.7%
• Lung infiltrates – 38.6%

EGPA Manifestations

Arth Rheum 2013, 65(1):270-281.

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Treatment of EGPA

• RTC of GC + CYC (6 or 12 pulses)
• Included at least 1 poor prognostic feature
• Glucocorticoids were 15 mg/kg x 3 days, then 1 mg/kg then

tapering

• CYC pulses 0.6 g/m2 x 6 or 12 months

Outcome 6 pulse CYC N=23 12 pulse CYC N=25 p Clinical Remission 21 (91.3) 21 (84) NS Failure 2 (8.7) 4 (16) NS Patients with relapse 18(78.2) 13(52) 0.07 Major relapse 10(55.5 ) 8(61.5) NS Minor relapse 14(77.7) 6(46.1) 0.02 Severe adverse event 11(47.8) 13(52) NS Deaths 2(8.7) 2(8) NS Arth Rheum 2007, 57(4):686-693.

• British case series
• 41 patients from 2003-2014 (initial, refractory, relapsed)
• 6 months: 34% in remission, 49% had partial response
• 12 months: 49% in remission, 39% with partial response
• 12 month remission: ANCA (+) 80%, ANCA (-) 38%
• Steroid requirement: 53% patients < 10 mg/day at 12 months, 6%
• n no steroids
• German case series
• 9 patients with refractory EGPA
• 1 patient with complete remission, 8 partial remissions
• CYC pulses were continued for three months after rituximab and

then transitioned to an oral DMARD

Rituximab in EGPA

Ann Rheum Dis 2014, 0:1-6. Arth Res Therapy 2013, 15:R133.

• Mononeuritis multiplex is an important clinical pattern

to recognize

• A skilled electromyographer can confirm a multifocal

neuropathy, whether it is axonal or demyelinating and propose a potential nerve biopsy site

• Vasculitis is potentially a neurologic and life

threatening emergency. Expedited evaluation is key

• The ROS, general exam are important to look for

systemic involvement

• The rheumatologist is your friend. Get them involved

early

Take home points

• 71 year old woman with connective tissue disorder

referred for evaluation of gait difficulty

• August 2016, woke up with pain in left arm and

numbness from left ear down entire left side of body

• December 2016 woke up with right arm numb and

whole arm weak

• Since December 2016, she has increasing

numbness of arms and left leg with inability to control the leg

• No bulbar or cognitive symptoms except left

forehead numbness

Case 2

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• Connective tissue disorder with Sjogren’s syndrome
• Multiple cranial aneurisms with paralyzed right eye
• Primary biliary sclerosis
• Migraine headache
• Denies HIV risk factors, cancer history, smoking

history, B6 ingestion, exposure to styrene, travel to places where leprosy is endemic

• Medications include aspirin, hydroxychoroquin,

lansoprazole, ursodiol

Past Medical History

• Cranial nerves
• Fixed large pupil on right with limited up and

downgaze

• Decreased light touch over left forehead, scalp,

neck, shoulder

• Motor examination: normal bulk and tone.
• DTR: absent throughout

Case 2-neurologic exam

Muscle Deltoid Biceps Tricep EDC FCU WE Right 4 5 5 4 4 5 Left 4 5 5 3 3 5 FDP 1/2 FDP 3/4 ADM FDI APB FCR Right 4 4 4 4 2 4 Left 4 4 4 4 5 4 IP Glut Max Hip add Hip abd Quad Ham Right 5 5 5 5 5 5 Left 5 4 5 5 5 5 Dorsi flex Plantar flex Ever Inv Toe ext Toe flex Right 5 5 5 5 5 5 Left 4 5 5 5 5 5

• Gait: pushes up on toes and heels. Wide base. Can't walk without
• assistance. Adventitious movements of the left leg. Unable to
• tandem. Romberg sways.
• Coordination: athetotic movements with her arms extended.

Maybe worse with the eyes closed. Slow bilateral finger taps but normal foot tap. Finger-nose shows dysdiochokinesis on the left. Unable to do heel-knee-shin on the left with adventitious movements.

• Sensory examination:
• Absent vibration to the shoulder bilaterally, absent in the left leg,

absent to the base of the right toe.

• Position sense absent to the right shoulder , left elbow, base of right

toe, left hip.

• Pinprick is slightly decreased to the right mid-foot, left forehead,

scalp, neck, arm, trunk, and leg and right arm

Case 2-Neurologic exam

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Presentation Title 37 Presentation Title 38

• Acute onset of left sided numbness and weakness

followed six months later by right arm numbness and weakness with severe large and small fiber sensory loss over the left forehead, scalp, neck trunk and limbs and right upper extremity, minimal involvement of right lower extremity with a sensory ataxia, loss of reflexes and possibly motor involvement

Clinical Summary

• Sensorimotor mononeuritis mulitplex
• Axonal
• Demyelinating
• Multifocal sensory neuropathy
• Axonal
• Demyelinating
• Sensory neuronopathy

Anatomic Localization

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• Motor NCS showed normal bilateral median, ulnar,

peroneal and tibial responses

• F-waves latencies normal except for slight

prolongation of left peroneal and tibial responses

• Sensory NCS-next slide
• EMG normal of left first dorsal interosseus, abductor

pollicis brevis, extensor digitorum communis, deltoid, biceps brachii, tibialis anterior, medial gastrocnemius, vastus medialis, tensor fascia lata and semitendinosus.

EMG/NCS

Presentation Title 42

Nerve and Stimulation Site Peak Lat ms Amp mV Temp °C Nerve Segment Onset Lat ms Dist mm CV m/s Median.R to Digit II (index finger).R Wrist NR NR 32.3 Wrist-Digit II (index finger) 140 Ulnar.R to Digit V (little finger).R Wrist NR NR 32.1 Digit V (little finger)-Wrist 110 Radial.R to Forearm.R Anatomical snuff box NR NR 32.2 Anatomical snuff box-Forearm 100 Sural.R to Ankle.R Lower leg 3.6 10 33.0 Ankle-Lower leg 2.9 140 48 Superficial peroneal.R to Dorsum of foot.R Ankle NR NR 31.9 Dorsum of foot-Ankle 140 Median.L to Digit II (index finger).L Wrist NR NR 31.3 Wrist-Digit II (index finger) 140 Ulnar.L to Digit V (little finger).L Wrist NR NR 30.8 Digit V (little finger)-Wrist 110 Radial.L to Forearm.L Anatomical snuff box 2.5 3 31.2 Anatomical snuff box-Forearm 2.0 100 50 Superficial peroneal.L to Dorsum of foot.L Ankle NR NR 26.7 Dorsum of foot-Ankle 140 Sural.L to Ankle.L Lower leg NR NR 26.6 Ankle-Lower leg 140

• Sensory ataxia
• Pseudoathetosis
• Generalized, multifocal, asymmetric impaired

sensation

• Large and small fiber sensory modalities
• Motor spared but have trouble sustaining force due

to sensory impairment

• DTR unobtainable
• EMG/NCS shows loss of sensory nerve action

potentials but normal motor

Sensory Neuronopathy

Donofrio et al. Muscle & Nerve 1990

Case 2-Differential Diagnosis

Multifocal axonal sensory neuropathy or neuronopathy

• Paraneoplastic
• Carinomatous sensory neuronopathy
• Lymphomatous sensory

neuronopathy

• Paraproteinemias
• Sjogren’s syndrome
• Chronic gluten enteropathy
• Crohn’s disease
• Nonsystemic vasculitic neuropathy
• Primary Biliary Cirrosis
• Fisher variant Guillain Barre

Syndrome

• Cis-platinum toxicity
• Thalidomide toxicity
• Pyridoxine toxicity
• Vitamin E deficiency
• Styrene-induced peripheral

neuropathy

• Hereditary sensory neuropathy types

I-IV

• Friedreich’s ataxia
• Spinocerebellar degeneration
• Abetalipoproteinemia
• Idiopathic sensory neuronopathy

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Normal studies: CBC with differential, paraneoplastic ab panel, fasting glucose, BUN, creatinine, TSH, FT4, vitamin B12, methylmalonic acid, vitamin E, copper, SPEP, serum IFE, NMO ab, MAG ab, TTG ab, FTA-ab, HIV, lyme WB titer, HTLV, ANCA, cryoglobulins, C reactive protein, DS DNA ab, ACE Abnormal studies: ESR 43, ANA positive, >640 speckled. CSF: WBC 12, RBC 2, TP 25, glucose 63, oligoclonal bands positive (14 bands). Cytology negative

Case 2-Laboratory Evaluation

• Right superficial radial sensory nerve biopsy: Moderate to

severe chronic axonal neuropathy with ongoing Wallerian

• degeneration. No inflammation or vasculitis.
• PETCT Whole Body Vertex to Toes: No FDG evidence of primary

malignancy or metastasis.

• MR Neurogram Lumbar Spine/Pelvis: Normal course and caliber
• f the bilateral lumbar plexus and sciatic nerves. No evidence of

nerve root enlargement or abnormal enhancement.

• MR Brain/MR Angiogram w/ and w/o Contrast, 04.2017:

IMPRESSION: 1. No acute intracranial abnormality. 2. Again identified are sequela of prior bifrontal craniotomies with multiple air susceptibility compatible with prior treatment aneurysms. 3. Limited MRA secondary to artifact from multiple aneurysm clips. No evidence of high-grade stenosis or vessel occlusion.

Case 2-Laboratory Evaluation

• Chronic inflammation of liver and obliteration of

intra-hepatic bile ducts with cholestasis

• Young women
• Associated with cutaneous xanthomata
• Can be associated with other autoimmune disorders

such as SLE, mixed connective tissue disorder, Sjogren’s syndrome or scleroderma

• Elevated antimitochondrial (Mi2) antibodies

Primary Biliary Cirrhosis

• Case reports of sensory neuronopathy
• CSF can have elevated gammaglobulins
• Elevated quantitative IgM
• Can have autonomic dysfunction
• One case reported of primary biliary cirrhosis,

Sjogren’s syndrome with PNS and CNS vasculitis

Primary Biliary Cirrhosis

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• Non length dependent, asymmetric sensory

neuronopathy due to primary biliary cirrhosis and/or Sjogren’s syndrome

• IVIg 0.4 gm/kg/day x 5 for 6 months
• Continued to worsen despite IVIg
• Next step mycophenolate and glucocorticoid

therapy

Diagnosis and Treatment

• Sensory neuronopathy has a unique clinical and

EMG/NCS pattern

• Patients with sensory neuronopathy can describe

weakness and appear weak on exam due to loss of sensory feedback

• Characterizing the neuropathy helps direct the

differential diagnosis (Donofrio article)

• Better treatments are needed for sensory

neuronopathy!

Case 2 Take Home Points

Questions?

• Charron L, Peyronnard JM, Marchand L. Sensory Neuropathy Associated with Primary Biliary
• Cirrhosis. Arch Neurol. 1980;37:84-87.
• Cho HJ, Yune S, Seok JM et al. Clinical Characteristics and Treatment Response of Peripheral

Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center. J Clin Neurol 2017;13:77-83.

• Cohen P1, Pagnoux C, Mahr A, Arène JP, et al. Churg-Strauss syndrome with poor-prognosis

factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arth Rheum 2007, 57(4):686-693

• Collins MP. The Vasculitic Neuropathies: an Update. Current Opinion 2012;25:573-585.
• Comarmond C, Pagnoux C, Khellaf M et al. Eosinophilc Granulomatosis with Polyangiitis (Churg-

Strauss). Arth Rheum 2013, 65(1):270-281

• Crowell A, Gwathmey K. Sensory Neuronopathies. Curr Neurol Neurosci Rep 2017;17:79
• Donofrio P, Albers J. Polyneuropathy: Classification by Nerve Conduction Studies and
• Electromyography. Muscle & Nerve 1990;13:889-903.
• Gwathmey K. Sensory Neuronopathies. Muscle & Nerve 2015;53-8-19.
• Hendrickse MT, Triger DR. Autonomic and Peripheral Neuropathy in Primary Biliary Cirrhosis. J

Hepatology 1993;19:401-407.

• Illa I, Graus F, Ferrer I et al. Sensory Neuropathy as the Initial Manifestation of Primary Biliary
• Cirrhosis. J Neurol Neurosurg Psychiatry 1989;52:1307.

References

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• Kaplan J, Rosenberg R, Reinitz E et al. Peripheral Neuropathy and Sjogren’s Syndrome. Muscle

& Nerve 1990;13:570-579.

• Keresztes K, Istenes I, Folhoffer A et al. Autonomic and Sensory Nerve Dysfunction in Primary

Biliary Cirrhosis. World J Gastroenterology. 2004;10:3039-3043.

• Knight RE, Bourne AJ, Newton M, et al. Neurologic syndrome associated with low levels of

vitamin E in primary biliary cirrhosis. Gastroenterology 1986;91(1):209-11.

• Min JK, Park KS, Yu WJ et al. Systemic Mononuclear Inflammatory Vasculopathy Associated with

Sjogren’s Syndrome in a Patient with Primary Biliary Cirrosis. Korean J Int Med. 2000;15:1-4.

• Mohammad AJ, Hot A, Arndt F et al. Rituximab for the treatment of eosinophilic granulomatosis

with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2016 Feb;75(2):396-401.

• Murata K, Ishiguchi H, Ando R et al. Chronic Inflammatory Demyelinating Polyneuropathy

Associated with Primary Biliary Cirrhosis. J Clin Neuroscience 2013;20:1799-1801.

• Oiwa H, Mokuda S, Matsubara T et al. Neurologic Compications of Eosinophilic Granulomatosis

with Polyangiitis (EGPA): The Roles of History and Physical Examinations in the Diagnosis of

• EGPA. Intern Med 2017;56:3003-3008.
• Pereira PR, Viala K, Masonobe T et al. Sjogren Sensory Neuronopathy (Sjogren Ganglionopathy).

Long term outcome and Treatment Response in a Series of 13 Cases. Medicine. 2016;95(19):e3632.

References

• Samson M1, Puechal X, Devilliers H, et al. Long-term outcomes of 118 patients with eosinophilic

granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun 2013, 43:60-69.

• Thiel J, Hässler F, Salzer U, et al. Rituximab in the treatment of refractory or relapsing

eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Arth Res Therapy 2013, 15:R133

References