Neuromuscular complications of connective tissue diseases

Rosenbaum, Richard B.

doi:10.1002/1097-4598(200102)24:2<154::aid-mus20>3.0.co;2-3

Cited by 80 publications

(83 citation statements)

References 146 publications

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“…The most common presentation is sensory motor or sensory axonal neuropathy. Less commonly, mononeuritis multiplex, autonomic neuropathy, cranial nerve mononeuropathies, and Guillain-Barré syndrome or CIDP can occur in patients with SLE [11]. PN is also a frequent manifestation of DM, especially type I.…”

Section: Discussion ____________________________mentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus

et al. 2017

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Peripheral nervous system involvement frequently occurs in systemic lupus erythematosus (SLE) patients. However, chronic inflammatory demyelinating polyneuropathy (CIDP) is an unusual presentation that can develop before, after, or simultaneously with the onset of SLE. This paper reports the case of a 20-year-old man with diabetes mellitus (DM) and CIDP accompanied by SLE. The patient complained of progressive weakness in the bilateral upper and lower extremities that had begun 2 months prior to this visit. He was diagnosed with CIDP and treated with intravenous immunoglobulin (IVIG), but had little improvement. A plasma exchange was then scheduled, but it was not helpful either. The patient developed polyarthritis, oral ulcer, and a worsening of his muscle weakness two weeks later. A neurologic examination revealed 3/5 muscle strength in the upper and lower extremities, absent deep tendon reflex (DTR), and impaired position sense. The patient was diagnosed with SLE because of pancytopenia, lymphopenia, pleuropericardial effusion, proteinuria, high titer anti-nuclear antibody (ANA), and anti-dsDNA. A kidney biopsy revealed stage IV lupus nephritis. The patient received 3 pulses of methyl prednisolone, 6 months of cyclophosphamide, and a high daily dose of prednisolone. His proteinuria improved, and he regained the ability to ambulate with a normal gait after about 2.5 months. To the best of the authors" knowledge, the concurrency of CIDP with SLE and DM has not been previously reported.

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Section: Discussion ____________________________mentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus

et al. 2017

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“…Although a corneal reflex was absent in two patients at the time of neurological examination, the facial paresthesias and sensory deficit were more apparent in the perioral region. The motor division of the trigeminal nerve was spared in all patients 3,4,7,8 . BRS is the standard test to assess trigeminal function.…”

Section: Discussionmentioning

confidence: 93%

“…The sensory abnormalities evolve slowly and usually spread contralaterally in an asymmetric pattern. The numbness may be accompanied by burning dysesthesia that is distinct from trigeminal neuralgia 3 . TSN is an infrequent complication of SSc.…”

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confidence: 99%

Trigeminal sensory neuropathy associated with systemic sclerosis: report of three Brazilian cases

Ribeiro

Fialho

Souza

et al. 2009

Arq. Neuro-Psiquiatr.

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Systemic sclerosis (SSc) is a connective tissue disease manifest by fibrotic tissue changes, microvascular disease, and autoimmune abnormalities. The prevalence of different neurological manifestations in SSc has ranged from 0.8% 1 to 18.5% 2 according to the adopted criteria. Trigeminal sensory neuropathy (TSN) causes numbness in the mandibular or maxillary divisions of the nerve in about 2/3 of the cases, and in the distribution of all divisions in the remaining cases. The sensory abnormalities evolve slowly and usually spread contralaterally in an asymmetric pattern. The numbness may be accompanied by burning dysesthesia that is distinct from trigeminal neuralgia 3 . TSN is an infrequent complication of SSc. Although epidemiological studies are scarce, the prevalence of TSN associated with SSc in the largest series available was 4% 4 . A fairly diligent review of the literature revealed no previous report of TSN as a complication of SSc in Brazil. We present three cases of such association. CASES Case 1A 42-year-old male had presented progressive facial hypoesthesia and dysesthesia for the last three years and was diagnosed with the diffuse form of SSc two years ago. At his first neurological evaluation, he had sclerodermic fascies, sclerodactyly, Raynaud's phenomenon, bilateral facial and lingual hypoesthesia, left hemifacial dysesthesia, and an absent left corneal reflex. Nailfold capillaroscopy was performed and showed devascularization and ectasias. ANA was positive (1/640) and anti-RNP was also positive. Nerve conduction studies (NCS) revealed sensory myelinic polyneuropathy in all limbs and face. Blink reflex studies (BRS) demonstrated absent responses on both sides to left supraorbital nerve stimulation. Electromyography (EMG) was normal. He was on methotrexate and prednisone without improvement of the TSN. Gabapentin 800 mg daily and nortryptilin 100 mg daily were introduced with only a partial response at their maximum tolerable doses. Case 2A 47-year-old female had presented Raynaud' s phenomenon and arthritis in her hands for the last three years and was feeling progressive numbness in the inferior half of the face for the last two years, without any previous diagnosis. At her first neurological evaluation, she had sclerodermic fascies, sclerodactyly, hypoesthesia in the maxillary and mandibular divisions territory bilaterally. ANA was positive (1/80), an esophagogram showed an increased esophageal caliber and a thoracic tomography confirmed this finding and revealed signs of pulmonary fibrosis. All electrophysiological studies performed (NCS, BRS, EMG) were normal. She had never been on medication for TSN or SSc yet. Case 3A 50-year-old female had presented hypoesthesia only in the maxillary division of the right trigeminal nerve about three years before her first neurological evaluation. Six months later, she started presenting hypoesthesia in the mandibullary division, effort dyspnea, Raynaud' s phenomenon and myalgia. She was diagnosed with SSc in overlap with myositis 10 months before our firs...

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“…As in our study, progressive idiopathic axonal neuropathy can be characterised as a sensorimotor neuropathy of the arms and legs, and may show asymmetric and proximal deficits, but a gamut of other possible clinical features has been observed including cranial neuropathy, sensory ataxia, small fiber neuropathy (see Table 1). Such a diversity of clinical manifestations has also been described in vasculitic neuropathy [8,9,11,23,25,32,34,35,46,48,49], but not in CIAP [22,27,43,59,60]. Thus, especially if other clinical signs or laboratory findings do not provide evidence for a systemic autoimmune disease (i. e. systemic vasculitis), patients with progressive idiopathic axonal neuropathy are clinically indistinguishable from patients with vasculitic neuropathy.…”

Section: Discussionmentioning

confidence: 97%

Progressive idiopathic axonal neuropathy

Vrancken

Notermans

Jansen

et al. 2004

Journal of Neurology

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Patients with a progressive disabling idiopathic axonal neuropathy could have a potentially treatable immune mediated neuropathy. To evaluate whether progressive idiopathic axonal neuropathy could be a pathologically difficult to prove vasculitic neuropathy pathologically difficult to prove or if it could be a separate clinical entity (i. e. with the axon as the primary immunological target), we performed a comparative clinical and histopathological study in 10 patients with progressive idiopathic axonal neuropathy, 10 patients with vasculitic neuropathy, and 12 patients with chronic idiopathic axonal polyneuropathy (CIAP). The clinical features and disease course in patients with progressive idiopathic axonal neuropathy and patients with vasculitic neuropathy were similar. Six patients with progressive idiopathic axonal neuropathy had been treated with prednisone and/or intravenous immunoglobulin. Disability decreased in all these six patients, but also in two of the four non-treated patients. Upon reviewing the sural nerve biopsy specimens, vasculitis was found in one patient with progressive idiopathic axonal neuropathy. Vasculitis-associated signs of ischemic injury or inflammation (most notably: large variation in fascicular axonal degeneration, perivascular inflammation, inflammation of the blood vessel wall without lumen obstruction) were found in four patients with progressive idiopathic axonal neuropathy, in all patients with vasculitic neuropathy, but were absent in patients with CIAP. The findings show that there is a small chance of finding sural nerve vasculitis upon scrutinising biopsy examination in progressive idiopathic axonal neuropathy. The presence of vasculitis-associated signs in progressive idiopathic axonal neuropathy suggests that some of these patients could have vasculitic neuropathy, even if vasculitic lesions cannot be demonstrated. However, if inflammatory changes cannot be demonstrated this does not preclude an immune-mediated origin.

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Neuromuscular complications of connective tissue diseases

Cited by 80 publications

References 146 publications

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus

Trigeminal sensory neuropathy associated with systemic sclerosis: report of three Brazilian cases

Progressive idiopathic axonal neuropathy

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