Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.

The clinical electrophysiology and pathological characteristics of 15 cases of vasculitic neuropathy / 中华内科杂志

Objective To summarize the clinical features,electrophysiology and neuropathological characteristics of peripheral nerves in patients with vasculitic neuropathy.Methods We retrospectively analyzed the clinical,electrophysiology and neuropathological characteristics of 15 patients with vasculitic neuropathy who underwent electrophysiology and sural nerve biopsy in our department from January 2009 to June 2013.Results There were 8 males and 7 females,aged from 38 to 82 years old,with a peripheral neuropathy course ranged from 0.5 month to 60 months.In the total of 15 patients,3 patients were diagnosed as nonsystemic vasculitic neuropathy,while the other 12 patients were diagnosed as systemic vasculitis neuropathy (SVN) including 5 cases of primary systemic vasculitis and 7 cases of secondary systemic vasculitis.In patients diagnosed as primary systemic vasculitis,there were 2 cases of Churg-Strass syndrome (CSS) and 3 cases of ANCA associated vasculitis.In patients diagnosed as secondary systemic vasculitis,there were 1 case of systemic lupus erythematosus (SLE),2 cases of sicca syndrome (SS),3 cases of rheumatoid arthritis (RA),1 case of Behcet' s disease associated with thyroid papillary carcinoma,1 case of hepatitis B and 1 case of RA-associated SS.For the pathological features of vasculitic neuropathy,type 1 lesion was found in 4 patients,type 2 lesion in 2 patients,and type 3 lesion in 9 patients.Axon degeneration was observed in 8 patients,while 7 patients manifested as axon degeneration associated with demyelination and local thickening of the perineurium was found in 2 patients.Conclusion Multiple mononeuropathy and asymmetric polyneuropathy are the common clinical presentations of vasculitic neuropathy.Electrodiagnostic testing almost always reveals the evidence of a predominantly axonal and sensorimotor process with associated demyelination presented in some cases.Sural nerve biopsy showes changes indicative of an axonopathy.

A Case of Essential Cryoglobulinemic Neuropathy

Mixed cryoglobulinemia occurs predominantly in association with chronic liver disease, infections, autoimmune diseases, and malignancies. Mixed cryoglobulinemia is classified as essential if no primary disease is identified. We report a 42-year-old woman with a painful essential cryoglobulinemic neuropathy. She developed arthralgia, purpura, and subacute multifocal sensorimotor neuropathy. Laboratory studies did not show any associated conditions except for an elevated cryoglobulin level. An electrophysiological study and nerve biopsy were indicative of multifocal axonal neuropathy. The neuropathy was stabilized with immunosuppressive treatment.

Non-systemic Vasculitic Neuropathy Presenting as Ascending Paralysis

Typical vasculitic neuropathy commonly manifests as a subacute multiple mononeuropathy, symmetrical or asymmetrical sensori-motor polyneuropathy. Various clinical presentations of peripheral neuropathy may confuse the diagnostic approach sometimes. A 76-year old man presented progressive areflexic ascending paralysis. gait ataxia, severe vibration and position sense impairment for 1 month. We initially considered as subacute demyelinating polyneuropathy. Electrophysiologic studies showed mainly axonopathy with some evidence of demyelination. We performed sural nerve biopsy and diagnosed as vasculitic neuropathy. No laboratory data or clinical findings favored systemic vasculitic involvement. So we concluded vasculitis confined to the peripheral nerves. Vasculitic neuropathy rarely presented as large myelinated nerve fiber disease. It should be considered the clinical variability of vasculitic neuropathy so extensive studies are needed in the case of peripheral polyneuropathy with indefinite cause.