Sarcoid polyneuropathy masquerading as chronic inflammatory demyelinating polyneuropathy

Singhal, Neel S.; Irodenko, Viktoriya S.; Margeta, Marta; Layzer, Robert B.

doi:10.1002/mus.24652

Cited by 17 publications

(5 citation statements)

References 15 publications

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“…Rare manifestations include subacute demyelinating polyneuropathy mimicking Guillain-Barré syndrome, or a chronic demyelinating inflammatory polyneuropathy. 39,[41][42][43] There appears to be a high frequency of granulomatous myositis in nearby muscle. 39,41 The mechanism of peripheral nerve sarcoidosis is usually granulomatous epi-or perineuritis, or vasculitis.…”

Section: Peripheral Nervesmentioning

confidence: 99%

Neurosarcoidosis

Neto

Moss

Willis

et al. 2017

Semin Respir Crit Care Med

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Neurosarcoidosis is an uncommon but clinically significant manifestation that will be routinely encountered in sarcoidosis specialty clinics. Overall, neurologic involvement is recognized in 5 to 10% of individuals with sarcoidosis. Neurologic symptoms will be the presenting manifestation of sarcoidosis in approximately one-half of those with neurosarcoidosis. The clinical and imaging features of neurosarcoidosis vary widely, largely depending on the anatomic distribution of the disease. The likelihood of spontaneous resolution is lower than for sarcoidosis in general, and residual functional deficits are not uncommon. Therefore, most patients with neurosarcoidosis require immunosuppressive therapy. Small fiber neuropathy, a recently recognized nongranulomatous parasarcoidosis syndrome, is prevalent in chronic sarcoidosis. The variety of neurologic manifestations, broad differential diagnosis, and complexity of management render neurosarcoidosis an area best served by multidisciplinary teams. Sarcoidologists, neurologists, radiologists, neurosurgeons, endocrinologists, urologists, physiatrists, and physical/occupational therapists all potentially have important roles.

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Section: Peripheral Nervesmentioning

confidence: 99%

Neurosarcoidosis

Neto

Moss

Willis

et al. 2017

Semin Respir Crit Care Med

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“…Sarcoidosis of the PNS is very rare, since our experience found sarcoid granulomas in 11 cases out of 3475 nerve biopsies throughout 11 years in our tertiary center [8]. Reviewing the literature, SAR-CIDP has been reported in 12 cases of chronic CIDP, and 15 cases of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] with only 4 definite NS cases [8,11]. CTD-CIDP has been reported in only 25 cases in the literature [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41], with the largest description being about 6CIDP associated with SLE [39].…”

Section: Discussionmentioning

confidence: 99%

Red Flags for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Sarcoidosis or Connective Tissue Diseases

Pémille

Noël

Adam

et al. 2023

JCM

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system. Diagnosis relies on clinical and electrophysiological criteria. Various disorders requiring specific treatment regimens may be associated with CIDP, including sarcoidosis (SAR-CIDP) and connective tissue disease (CTD-CIDP). Therefore, it is important to distinguish between CIDP, SAR-CIDP and CTD-CIDP. In this retrospective monocentric study, we analyzed 16 patients with SAR-CIDP and 11 with CTD-CIDP and compared them with a group of 17 patients with idiopathic CIDP. SAR-CIDP patients had a frequently acute or subacute CIDP onset. CTD-CIDPs were mostly Sjögren’s syndrome and lupus, and patients had a chronic onset. An older age at onset (64.5 vs. 54 years, p = 0.04), more atypical presentation (19/25 (76%) vs. 6/17 (35%), p = 0.008), acute/subacute onset of symptoms (15/25 (60%) vs. 1/17 (6%), p = 0.0004) and more frequent weight loss (7/16 (44%) vs. 0/17 (0%), p = 0.017) were identified SAR-CIDP and CTD-CIDP groups. Response to intravenous immunoglobulin therapy was lower in the combined SAR-CIDP and CTD-CIDP group (44% versus 82%, p = 0.005). As sarcoidosis and CTDs might be associated with CIDP and require specific management, the “red flags” mentioned above should be kept in mind by clinicians managing patients with CIDP.

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“…Ducray et al [4] reported a case of a 36-year-old-man who first presented two relapses of chronic inflammatory demyelinating polyneuropathy (CIDP) before the diagnosis of sarcoidosis was made. In 2015, Singhal et al [5], reported also the clinical, electrophysiological, and pathological findings of a patient who carried a diagnosis of sensory-predominant CIDP for over a decade but was ultimately found to have sarcoidosis. In cases of suspected peripheral nerve sarcoidosis, biopsy of both nerve and muscle tissue may increase the diagnostic yield and help rule out other rare inflammatory, infectious, or neoplastic causes of neuropathies.…”

Section: Discussionmentioning

confidence: 99%

“…Several explanations for the pathogenesis of neuropathy associated with sarcoidois have been suggested: such as endoneural granuloma, perivascular granuloma and necrotizing epineural vasculitis [5].…”

Section: Discussionmentioning

confidence: 99%

Chronic Demyelinating Inflammatory Polyradiculoneuropathy Associated With Sarcoidosis

Mansour¹,

Souissi²,

Beyrouti³

et al. 2017

J Neurol Disord

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Sarcoidosis is an inflammatory multisystem disorder, usually involving the lung, the skin, the lymph nodes, and the eyes. The prevalence of clinical involvement of the nervous system is estimated to be about 5% to 15% [1]. Both central and peripheral nervous system can be affected. Sarcoid polyneuropathy is a rare and clinically heterogeneous disorder that may be the initial presentation of sarcoidosis [2]. Only a few cases have been reported of chronic demyelinating inflammatory polyradiculoneuropathy (CIDP) associated with sarcoidosis. We report a clinical course of a patient presented with a sensorimotor demyelinating polyneuropathy with secondary axonal loss few years after being diagnosed with lung sarcoidosis. Keywords: Sarcoidosis; Polyneuropathy; CIDP; Nerve biopsy Case ReportA 60-year-old woman, with medical history of lung sarcoidosis diagnosed in 2003 treated by 40 mg prednisolone daily, was admitted in our neurological department in March 2011 for paresthesia and burning sensation in her two hands evolving for five months.Neurological examination revealed mild distal limb weakness, generalized hyporeflexia, impairment of touch, pain, and temperature sensations in both hands. Vibration sense was severely affected in the higher limbs, especially in the left side. Patient was ataxic with a positive Romberg's sign.Electrophysiological study demonstrated bilateral elongated distal latencies, reduced nerve conduction velocities, and conduction blocks respectively in the median, the ulnar and the peroneal nerves (Table 1). It also objectified delayed F response in the median, the ulnar and the tibial nerves (Table 2) fulfilling the EFNS/PNS diagnostic criteria for CIDP.Cerebrospinal fluid (CSF) analysis revealed an elevated protein level (0.54 g/L; normal range 0.16 -0.4 g/L) without pleocytosis. Magnetic resonance imaging (MRI) of the brain and the spinal cord was normal. Nerve biopsy was not performed because of patient's refusal. Serologies of Lyme, hepatitis B and C and human immunodeficiency virus were negatives. Levels of thyroid hormones, glycaemia and Vitamin B12 were within normal limits. Screening tests for autoantibodies, including antinuclear antibody (Ab), anti-SS A/SS B Ab, anticardiolipin Ab, antineutrophil cytoplasmic Abs (P-ANCA and C-ANCA) and tumor markers were also unrevealing.The patient received initially IV methylprednisolone for 3 days (1gr/ day), then oral prednisolone of 60 mg/day for 2 months followed by a very gradual reduction. Thereafter, the patient was maintained on oral prednisolone (40 mg daily) in association with azathioprine (100 mg daily). Slight improvement in the muscle strength was observed.

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Sarcoid polyneuropathy masquerading as chronic inflammatory demyelinating polyneuropathy

Abstract: Introduction: Sarcoid polyneuropathy is a rare and clinically heterogeneous disorder that

Cited by 17 publications

References 15 publications

Neurosarcoidosis

Neurosarcoidosis

Red Flags for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Sarcoidosis or Connective Tissue Diseases

Chronic Demyelinating Inflammatory Polyradiculoneuropathy Associated With Sarcoidosis

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