IgG4-related hypophysitis: a retrospective cohort study.

PURPOSE: IgG4-related hypophysitis (IgG4-RH) is a rare chronic inflammatory condition of the pituitary gland. This study reports the presentation, management and outcomes for patients with histologically proven IgG4-related hypophysitis. METHODS: A prospectively maintained electronic database was searched over a 14-year period from 1 January 2007 to 31 December 2020 at a single academic centre to identify all patients with a histological diagnosis of IgG4-RH. A retrospective case note review from electronic health records was conducted for each case to extract data on their presentation, management and outcomes. RESULTS: A total of 8 patients (5 male) with a median age of 51 years were identified. The most common presenting symptoms were headache (4/8; 50%), fatigue (3/8; 37.5%) and visual impairment (2/8; 25%). Three patients were initially treated with high-dose steroids aiming for reduction of the pituitary mass. However, ultimately all patients underwent transsphenoidal surgery. Post-operative changes included radiological reduction in pituitary mass in all patients that had imaging (7/7; 100%), improvement in vision (1/2; 50%), residual thick pituitary stalk (5/7; 71.4%), persistent anterior hypopituitarism (4/8; 50%) and panhypopopituitarism including diabetes insipidus (3/8; 37.5%). CONCLUSIONS: IgG4-RH is an increasingly recognised entity presenting with a variety of symptoms and signs. Clinical presentation is similar to other forms of hypophysitis. It is therefore important to consider IgG4-RH as a differential and to have a low threshold for pituitary biopsy, the diagnostic gold standard. The diagnosis of IgG4-RH will guide decisions for additional workup for IgG4-related disease, multi-disciplinary team involvement and follow-up.

Management strategy for treatment of vasospasm following transsphenoidal excision of craniopharyngioma.

We describe two cases of clinical and radiographic vasospasm after transsphenoidal resection of a craniopharyngioma. We review the literature on the association of vasospasm and craniopharyngioma and examine management options. Given the lack of evidence for the optimal management of these patients, treatment in concordance with protocols for vasospasm due to subarachnoid haemorrhage is recommended.

Contrast-enhanced MR angiography of intracranial giant aneurysms.

BACKGROUND AND PURPOSE: Intravoxel phase dispersion and flow saturation often prevent adequate depiction of intracranial giant aneurysms on 3D time-of-flight (3D-TOF) MR angiography (MRA). Additional diagnostic difficulties may arise from T1 contamination artifact of an associated blood clot. Our aim was to assess whether contrast-enhanced MRA could improve the evaluation of giant aneurysms and to compare two different types of contrast-enhanced MRA. METHODS: We studied 11 aneurysms in 10 patients (age range, 31-77 years) with giant aneurysms of the anterior (n = 9) and posterior (n = 2) cerebral circulation by comparing 3D-TOF, first-pass dynamic contrast-enhanced MRA, and steady-state contrast-enhanced 3D-TOF sequences. Additional comparison with digital subtraction angiography (DSA) was performed in eight aneurysms. RESULTS: In nine of 11 aneurysms, 3D-TOF did not adequately show the lumen and exiting vessels. Contrast-enhanced 3D-TOF and dynamic contrast-enhanced MRA showed the aneurysm sac and exiting vessels in all of these cases. Dynamic contrast-enhanced MRA showed a better intravascular contrast than did contrast-enhanced 3D-TOF, which led to better delineation of the aneurysms. T1 contamination artifact from intra- or extraluminal blood clot was evident on the 3D-TOF images in four cases. The artifact was less marked on the contrast-enhanced 3D-TOF image and was completely eliminated on the dynamic contrast-enhanced MRA image by subtraction of precontrast images. The diagnostic information provided by dynamic contrast-enhanced MRA was comparable to that provided by DSA. CONCLUSION: Precontrast 3D-TOF is inadequate for the assessment of giant cerebral aneurysms. Both contrast-enhanced 3D-TOF and dynamic contrast-enhanced MRA reliably show the aneurysm sac and connected vessels. Dynamic MRA provides a superior contrast between flow and background and eliminates T1 contamination artifact. It should therefore be considered as the MRA sequence of choice.

Risk factors for the formation of multiple intracranial aneurysms.

OBJECT: Several factors are known to increase the risk of subarachnoid hemorrhage (SAH) and spontaneous intracerebral hematoma. However, information on the roles of these same factors in the formation of multiple aneurysms is less well defined. The purpose of this study was to examine factors associated with an increased risk of multiple aneurysm formation. METHODS: A retrospective review of the medical records of all patients with a diagnosis of SAH and intracranial aneurysms who were admitted to a single institution between 1985 and 1997 was undertaken. The authors examined associations between risk factors (patient age and sex, menopausal state of female patients, hypertension, cigarette smoking, alcohol consumption, history of cardiovascular disease or diabetes mellitus, and family history of cerebrovascular disease) and the presence of multiple aneurysms by using the Fisher exact test and logistic regression analysis. Of 400 patients admitted with a diagnosis of cerebral aneurysms, 392 were included in the study (287 women and 105 men). Two hundred eighty-four patients harbored a single aneurysm and 108 harbored multiple aneurysms (2 aneurysms in 68 patients, three aneurysms in 22 patients, four aneurysms in 13 patients, and five aneurysms in five patients). CONCLUSIONS: Statistical analysis revealed that, as opposed to the occurrence of a single aneurysm, there was a significant association between the presence of multiple aneurysms and hypertension (p < 0.001), cigarette smoking (p < 0.001), family history of cerebrovascular disease (p < 0.001), female sex (p < 0.001), and postmenopausal state in female patients (p < 0.001).

Giant temporo-occipital sinus pericranii. A case report.

A rare case of a giant, temporo-occipital sinus pericranii is presented. A 38-year-old male presented with minor symptoms of headache and heaviness over an enlarging temporo-occipital bone defect. Within the defect a soft, compressible, mass lesion was observed, which varied in size with changes in intracranial pressure. Radiological imaging demonstrated bone erosion around a fluid filled mass, which on angiography communicated via a series of channels with the transverse sinus. A diagnosis of sinus pericranii was made. Due to the risk of future complication the patient elected to undergo surgery, which successfully resected the mass and obliterated the venous communications with the diploic veins and transverse sinus. The classification, aetiology, differential diagnosis, radiological characteristics and management options relating to sinus pericranii are discussed.

Palatal trauma: an unusual cause of a parotid sinus.

A case is described of a 3-year-old boy who presented with a seemingly trivial injury to his soft palate, who went on to develop a parotid sinus as a result of a retained foreign body. This is a rare clinical problem and it highlights the difficulty in the clinical assessment of a palatal injury--especially in children. The child had the foreign body removed successfully 5 months after the initial injury and made an uneventful recovery.

Advances in non-invasive imaging of intracranial vascular disease.

Intra-arterial catheter angiography has, in the past, been the mainstay for the investigation of intracranial vascular disease. It is, however, invasive, usually requires in-patients admission, and is associated with a rate of neurological complications between 1% and 3%. In recent years, magnetic resonance angiography (MRA) and CT angiography (CTA) have emerged as non-invasive alternatives for imaging blood vessels and have made a significant impact on neuroradiological investigations. It is the purpose of this article to explain the basic technical principles of these two methods and to give an overview of their current clinical applications.

Angiogram negative subarachnoid haemorrhage: outcomes and the role of repeat angiography.

BACKGROUND: Angiogram negative sub-arachnoid haemorrhage (SAH) is generally considered to have a more benign course than SAH of known cause. There is also variability from centre to centre as to what proportion of angiogram negative SAH patients undergo repeat Digital Subtraction Angiography (DSA). We performed a retrospective study looking at the last four years' of SAH patients at our institution in order to ascertain the clinical course, the nature and results of repeat imaging. METHODS: Retrospective analysis of clinical records and imaging of all patients presenting to our institution with non-traumatic SAH between April 2008 and February 2012 was performed. Results were analysed for presenting grades, blood distribution, complications, outcomes, repeat imaging modalities and findings. RESULTS: 459 patients with proven non-traumatic SAH of which 50 (11%) had no vascular cause identified on their initial angiogram were identified. The blood distribution was perimesencephalic in 17, non-perimesencephalic in 23, and 10 patients were computed tomography (CT) Negative with a positive lumbar puncture. Eight (16%) patients were complicated by hydrocephalus and 2 (4%) were complicated by vasospasm. Eight patients (16%) underwent repeat cranial DSA with a high suspicion in a multi-disciplinary team setting. None of the repeat angiograms showed an underlying aetiology for the SAH. 76% of patients had a Glasgow Outcome Score of 5 at 6 months. There were no rebleeds. CONCLUSIONS: While generally more benign, angiogram negative subarachnoid haemorrhage can have a complicated clinical course. In our experience repeat DSA should be reserved for cases in which there is significant suspicion of occult vascular lesion. However, evidence-based guidelines are needed to aid the development of management protocols for angiogram-negative SAH and ensuring optimal patient outcomes.

Dural infiltration of metastatic Hodgkin's lymphoma.

Hodgkin's disease is a neoplasm of clonal Reed-Sternberg cells. Intracranial Hodgkin's lymphoma is rare. We present a unique case of a 57-year-old woman with secondary intracranial lymphoma infiltrating the dura. Her past medical history included Hodgkin's lymphoma from which she was deemed to be in remission at the time of presentation. Following an acute onset of seizures, she underwent radiological investigations that demonstrated an enhancing right-sided temporal dural-based space occupying lesion. Histopathological findings revealed nodular sclerosing Hodgkin's lymphoma with pathognomonic Reed-Sternberg cells. In cases of intracranial lesions with dural infiltration, Hodgkin's lymphoma should be considered in the diagnosis.

Robust processing of intracranial CT angiograms for 3D volume rendering.

The goal of this study was to develop a robust and simple technique for processing of cranial CT angiograms (CTA) in the clinical setting. The method described in this paper involves segmentation of the bone, then dilation of the skull by adding three or four layers of voxels. This dilated skull is subtracted from the vessels object on a voxel-by-voxel basis, allowing segmentation and subsequent display of the vessels only. For evaluation of the technique, three groups of operators processed one CTA, and the quality of the 3D views obtained and the times taken were compared. One group was given training by an expert and a "recipe" for guidance, the second was given only the "recipe," and the third group consisted of expert operators. All operators were able to produce good or acceptable shaded-surface displays when compared with digital subtraction angiography, within 10 min for experienced users, an average of 17 min for trained operators and 26 min for those using only the recipe sheet. Using a simple scoring system for the appearance of feeding vessels and draining veins, no significant differences were found between the three levels of training and experience. This technique simplifies the processing of CTAs and is quick enough to make such examinations part of a routine clinical service.

Neurenteric sinus dorsal to the thoracic spine with an associated cutaneous haemangioma: case report.

The case of an infant born with a large cutaneous haemangioma overlying the thoracic spine is presented. A small midline pit lay within the haemangioma, histological examination of which confirmed it to be a neurenteric sinus. The possible embryological origins of this rare lesion are discussed.

A retrospective study of surgical and conservative treatment for spinal extradural abscess.

The management of spinal extradural abscess (SEA), particularly the choice between surgical and conservative treatment, is controversial. We therefore undertook a retrospective study of patients admitted with this diagnosis: Details of presentation, treatment and outcome were obtained by review of inpatient notes and radiology. Twenty-five individuals with SEA aged 10-79 years were admitted between 1989 and 1995. Nine were treated non-operatively with antibiotics, of which three also underwent diagnostic CT-guided biopsy, and 16 underwent surgical drainage. There was no significant difference in the neurological features at presentation between the two groups. Two patients (8%) died; 11 (44%) patients remained the same and 12 (48%) improved following treatment. Four patients treated conservatively (44%) and seven treated surgically (43%) were capable of return to work or school, whilst a further seven patients (three treated conservatively and four treated surgically) were able to lead independent lives. No significant difference in outcome was demonstrated between patients treated non-operatively and those treated surgically. We conclude that certain carefully selected patients can be treated conservatively, but that surgery should remain the mainstay of management.

Results of posterior cervical foraminotomy for treatment of cervical spondylitic radiculopathy.

We evaluated the results of posterior cervical foraminotomy for spondylitic radiculopathy using a questionnaire sent to all 77 patients who had undergone surgery between 1990 and 1995 at our institution. Sixty-two patients (40 male) returned their questionnaires, one of whom had undergone two procedures (dealt with as separate events). Sixty patients complained of pre-operative arm pain; of these 42 (70%) had complete or > 75% resolution of their pain, 14 (23%) had < 75% improvement in their pain and four (7%) had the same or worsened pain at the time of the questionnaire. Sixteen patients (27%) reported initial improvement in symptoms with subsequent deterioration. The mean patient satisfaction score using a linear analogue scale from 0 to 10 was 7.5. Main postoperative complaints were neck pain (22%), persisting motor deficit (6%) and persisting sensory deficit (9%). One patient suffered nerve root damage at surgery. For unilateral and, in some cases, multi-level degenerative disease causing cervical radiculopathy, posterior cervical foraminotomy is a useful technique with the advantage of avoiding fusion, immobilization and the long-term risk of instability.

Artefact on MRA following aneurysm clipping: an in vitro study and prospective comparison with conventional angiography.

Using both an experimental model and clinical cases, we looked at the artefact produced by Aesculap titanium-alloy aneurysm clips on MRA. Experimentally, the volume affected by artefact was 50 % less when the clip was imaged lying parallel to the main ferromagnetic field than when lying perpendicular to it. Clinically, MRA was prospectively compared with digital subtraction angiography (DSA) in nine patients who had undergone aneurysm clipping. One patient with a non-diagnostic MRA due to movement artefact was excluded. In all other cases there was an area of signal loss surrounding the clips, obscuring the immediately adjacent vessel segments. There was good demonstration of the adjacent bifurcations in five cases and the contralateral circulation was seen well in all patients. In three cases in which the adjacent bifurcations were not seen, considerable vasospasm was suggested by MRA and confirmed with DSA. In one patient an unclipped contralateral ophthalmic artery aneurysm was identified using both modalities. In this series there were no adverse events relating to clips in either static or time-varying magnetic fields.