Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review

Vakrakou, Aigli G.; Evangelopoulos, Maria-Eleftheria; Boutzios, Georgios; Tzanetakos, Dimitrios; Tzartos, John; Velonakis, Georgios; Toulas, Panagiotis; Anagnostouli, Maria; Andreadou, Elisabeth; Stefanis, Leonidas; Fragoulis, G.; Kilidireas, Constantinos

doi:10.1007/s00296-019-04502-6

Cited by 11 publications

(8 citation statements)

References 40 publications

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“…109 Spinal cord compression from nearby pachymeningeal involvement can occur, but parenchymal involvement of the spinal cord is extraordinarily rare. 110 CSF and MRI findings are nonspecific in these cases (►Fig. 7).…”

Section: Immunoglobulin G4-related Diseasementioning

confidence: 99%

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

Galetta

Bhattacharyya

2021

Semin Neurol

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Systemic autoimmune diseases can affect the peripheral and central nervous system. In this review, we outline the common inpatient consultations for patients with neurological symptoms from rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, immunoglobulin G4–related disease, Behçet's disease, giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis, polyarteritis nodosa, and ankylosing spondylitis. We discuss the symptoms, diagnostic strategies, and treatment options.

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Section: Immunoglobulin G4-related Diseasementioning

confidence: 99%

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

Galetta

Bhattacharyya

2021

Semin Neurol

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“…However, other conditions were extensively excluded. To the best of our knowledge, there are only two previous descriptions of parenchymal spinal cord involvement associated with IgG4‐related disease, one of them is LETM 4,5 . Being neuromyelitis optica spectrum disorders (NMOSD) a recognized cause of LETM, 6 we tested the serum for anti‐AQP4 and anti‐MOG antibodies several times during the patient history, with persistently negative results.…”

Section: Introductionmentioning

confidence: 99%

“…To the best of our knowledge, there are only two previous descriptions of parenchymal spinal cord involvement associated with IgG4-related disease, one of them is LETM. 4,5 Being neuromyelitis optica spectrum disorders (NMOSD) a recognized cause of LETM, 6 we tested the serum for anti-AQP4 and anti-MOG antibodies several times during the patient history, with persistently negative results. This patient also exhibits ocular inflammation in the form of uveitis, a manifestation also associated with IgG4-related disease.…”

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confidence: 99%

Longitudinal extensive transverse myelitis: A presentation of IgG4‐related disease

Oliveira

Moura

Pinto

et al. 2022

Neurology & Clinical Neurosc

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The IgG4-related disease is an immune-mediated multisystemic disorder, with nervous system involvement being increasingly recognized. 1,2 The most common manifestations are pachymeningitis, hypophysitis, and orbital disease.We report a 65-year-old man with no relevant past medical history (namely allergies). By the age 39 (1984), he developed a progressive gait instability and weakness in the lower limbs. Cerebrospinal fluid (CSF) showed 5 lymphocytes and proteins of 0.65 g/dL. Multiple sclerosis was considered as the cause of cerebellar and pyramidal syndromes, and the patient received adrenocorticotropic hormone for 3 days. He achieved a gradual and almost complete recovery and eventually lost to follow-up.In 2008, at age 54, he developed weakness in the lower limbs associated with urinary retention. The neurological examination revealed paraparesis and bilateral pyramidal syndrome, accompanied by urinary retention, superficial sensory loss (T4-level), and hypopallesthesia. The brain MRI revealed frontal parasagittal clastic lesions secondary to a previous car accident. Spinal MRI (Figure 1) documented a longitudinal extensive transverse myelitis (LETM).CSF analysis revealed pleocytosis-88 lymphocytes/μL, 38 eosinophils/μL-and elevated proteins (0.82 g/dL), with absent oligoclonal bands. Blood analysis was relevant for eosinophilia (0.64 × 10 3 /μL), elevated IgE (370 U/mL), and IgG4 231 mg/dL (>2 times above the upper limit of normal). Anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were persistently negative on live cell-based assays. Parasitological and serological analysis were unremarkable. The immune study was otherwise normal, and he had no systemic auto-immune disorder. A full-body CT revealed mediastinal lymphadenopathies. He was treated with high-dose methylprednisolone for 5 days and maintained on oral

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“…This disease can involve virtually any organ, and pancreas, salivary glands, and lacrimal glands are most commonly affected. In addition, IgG4-related central nervous system (CNS) involvement is a rare and distinct entity of IgG4-related disease ( 1 ), with hypertrophic pachymeningitis and hypophysitis being the most common manifestations ( 2 ). It can also involve parenchyma, peri-ventricular area, and cranial nerves ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

“…In addition, IgG4-related central nervous system (CNS) involvement is a rare and distinct entity of IgG4-related disease ( 1 ), with hypertrophic pachymeningitis and hypophysitis being the most common manifestations ( 2 ). It can also involve parenchyma, peri-ventricular area, and cranial nerves ( 2 ). However, IgG4-related disease manifesting as spinal pachymeningitis, which may represent a distinct subtype of CNS involvement, is relatively rare ( 3 – 5 ).…”

Section: Introductionmentioning

confidence: 99%

A Case With IgG4-Related Spinal Pachymeningitis Causing Spinal Cord Compression

et al. 2020

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Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by sclerosing lesions and an increased serum IgG4 level. This condition can involve any organ, but IgG4-related spinal pachymeningitis is relatively rare. In the current study, we report a case of spinal cord compression caused by IgG4-related spinal pachymeningitis. A 39-year-old man presented to us with a 15-day history of back pain and a 3-day history of dysuresia, exacerbated by weakness in the lower extremities for 2 days. Cervical magnetic resonance imaging (MRI) showed strip-shaped abnormal signals along the anterior and posterior borders of the spinal cord at the C5–T4 levels. The IgG level in cerebrospinal fluid was 718.0 mg/L. Thoracic MRI revealed strip-shaped abnormal signals with remarkable enhancement along the anterior and posterior borders of the dural sac at the T1–T6 levels. Histopathological examination confirmed IgG4-related spinal pachymeningitis. The symptoms worsened rapidly, and surgical resection of the space-occupying lesion in the vertebral canal was performed for spinal decompression. Corticosteroid therapy was administered, and the patient's motor functions were mildly improved. IgG4-related disease can manifest as spinal pachymeningitis and cause spinal cord compression. Clinicians should be aware of this rare condition, and early diagnosis, timely surgical decompression, and appropriate corticosteroid therapy should be highlighted.

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Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review

Cited by 11 publications

References 40 publications

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

Longitudinal extensive transverse myelitis: A presentation of IgG4‐related disease

A Case With IgG4-Related Spinal Pachymeningitis Causing Spinal Cord Compression

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