Thoracic arachnoiditis, arachnoid cyst and syrinx formation secondary to myelography with Myodil, 30 years previously

Spinal arachnoiditis can rarely occur following irritation from foreign body substances, including certain oil based contrast agents used for myelography. We describe a patient with thoracic arachnoiditis, arachnoid cyst and syringomyelia, 30 years following a myelogram with Myodil. A 62-year-old female presented with chronic thoraco-lumbar back pain, a spastic paraparesis and sphincter disturbance. She had undergone a myelogram with Myodil, 30 years previously for investigation of back pain. A MRI scan revealed evidence of arachnoiditis, thoracic syringomyelia (T6–T8) and an anteriorly placed, extramedullary, arachnoid cyst at T10–T12, compressing the cord. At surgery, T7–T10 thoracic laminectomies were carried out and syringo- and cysto-subarachnoid shunts were inserted. At 12 months follow-up, the sphincter disturbance, lower limb weakness and mobility problems had almost resolved. Although, the use of oil based contrast agents such as Myodil has been discontinued, the present case illustrates some of the rare sequelae of its use, manifesting decades later. Aggressive surgical intervention produced symptomatic benefit

Suspected idiopathic sclerosing orbital inflammation presenting as immunoglobulin G4-related disease: a case report

<p>Abstract</p> <p>Introduction</p> <p>Idiopathic sclerosing orbital inflammation is a rare and ill-defined heterogeneous entity, and a distinct subset of orbital inflammation. Recently, attention has been focused on immunoglobulin G4-related disease complicated with fibrotic changes in some other organs with high serum immunoglobulin G4 levels. This report presents a case of suspected idiopathic sclerosing orbital inflammation complicated with high serum immunoglobulin G4 levels.</p> <p>Case presentation</p> <p>An 82-year-old Japanese woman had a 30-year history of chronic thyroiditis. She experienced right ptosis and eyelid swelling. These symptoms gradually developed over five years. The clinical and radiographic findings suggested that our patient had idiopathic sclerosing orbital inflammation. We were unable to obtain our patient's consent to perform a biopsy. While the serum immunoglobulin G level was within the normal limits, the serum immunoglobulin G4 level was significantly elevated. The serum immunoglobulin G4 levels decreased after the administration of oral prednisolone at a daily dose of 20 mg. In addition, the swelling and ptosis of the right upper eyelid disappeared gradually after four weeks. Our patient was then suspected to have idiopathic sclerosing orbital inflammation complicated with immunoglobulin G4-related disease and chronic thyroiditis.</p> <p>Conclusion</p> <p>An orbital pseudotumor of this type is indicative of idiopathic sclerosing orbital inflammation immunoglobulin G4-related disease. Immunoglobulin G4 may thus be considered a subclass of immunoglobulin G when the serum immunoglobulin G level is within normal limits.</p

Hyper-IgG4 disease: report and characterisation of a new disease

BACKGROUND: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. METHODS: We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS: Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION: We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good

Identifying the deficiencies of current diagnostic criteria for neurofibromatosis 2 using databases of 2777 individuals with molecular testing

Purpose We have evaluated deficiencies in existing diagnostic criteria for neurofibromatosis 2 (NF2). Methods Two large databases of individuals fulfilling NF2 criteria (n = 1361) and those tested for NF2 variants with criteria short of diagnosis (n = 1416) were interrogated. We assessed the proportions meeting each diagnostic criterion with constitutional or mosaic NF2 variants and the positive predictive value (PPV) with regard to definite diagnosis. Results There was no evidence for usefulness of old criteria “glioma“ or “neurofibroma.” “Ependymoma” had 100% PPV and high levels of confirmed NF2 diagnosis (67.7%). Those with bilateral vestibular schwannoma (VS) alone aged ≥60 years had the lowest confirmation rate (6.6%) and reduced PPV (80%). Siblings as a first-degree relative, without an affected parent, had 0% PPV. All three individuals with unilateral VS and an affected sibling were proven not to have NF2. The biggest overlap was with LZTR1-associated schwannomatosis. In this category, seven individuals with unilateral VS plus ≥2 nondermal schwannomas reduced PPV to 67%. Conclusions The present study confirms important deficiencies in NF2 diagnostic criteria. The term “glioma” should be dropped and replaced by “ependymoma.” Similarly “neurofibroma” should be removed. Dropping “sibling” from first-degree relatives should be considered and testing of LZTR1 should be recommended for unilateral VS

The role of MR angiography in the pretreatment assessment of intracranial aneurysms:a comparative study

rysms are being treated increasingly by the endovascular route. Endovascular treatment of aneurysms requires an accurate depiction of the aneurysm neck and its relation to parent and branch vessels preoperatively. Our goal was to estimate the clinical efficacy of MR angiography (MRA) in the pretreatment assessment of ruptured and unruptured intracranial aneurysms. We compared MRA source data (axial acquired partitions), multiplanar reconstruction (MPR) of these data, as well as maximum intensity projection (MIP) and 3D-isosurface images with intraarterial digital subtraction angiography (IA-DSA). METHODS: The study was performed in 29 patients with 42 intracerebral aneurysms. The MRA data were examined in four different forms—as axial source data, MPR images of the source data, and MIP and 3D isosurface–rendered images. A composite standard of reference for each aneurysm was then constructed using this information together with the IA-DSA findings by looking at aneurysm detection rate, aneurysm morphology, neck interpretation, and branch vessel relationship to the aneurysm. All techniques, including conventional IA-DSA, were then scored independently on a five-point scale from 1 (non diagnostic) to 5 (ex-cellent correlation with the standard of reference) for each of the aneurysm components a

Time of Flight 3D Magnetic Resonance Angiography in the Follow-up of Coiled Cerebral Aneurysms

The use of Guglielmi Detachable Coil (CDC) for the endovascular treatment of intracerebral aneurysms is increasing, particularly in those aneurysms for which there is a high surgical morbidity and mortality. However, the long-term efficacy of GDC is not known. Until the natural history of GDC treatment is established long-term follow-up in this cohort of patients is required, of necessity involving repeated intraarterial angiography (IA DSA) with its known attendant risks and exposure to ionising radiation. Three dimensional time-of-flight magnetic resonance angiography (3D TOF MRA) is now readily accepted as a non-invasive screening tool for familial aneurysmal disease and has been used as an alternative to IA DSA in the surgical management of aneurysmal subarachnoid haemorrhage. MRA in patients treated with GDC is safe, imparts no radiation dose and provides acceptable image quality. The aim of this study was to assess 3D TOP MRA source data, maximum intensity projection (MIP) and 3D isosurface reconstruction in comparison to IA DSA in the follow-up of 25 patients treated with GDC. Images were assessed for parent and branch artery flow, the presence of neck recurrence and aneurysm regrowth. There was good correlation for all these features when 3D isosurface MRA and source data were compared with IA DSA. The correlation between MIP MRA and IA DSA was less robust. Additional confidence can be obtained by performing plain films of the skull to demonstrate change in coil ball configuration. MRA has the potential to replace IA DSA in the follow-up of GDC treated cerebral aneurysms. </jats:p