Valsalva-triggered pseudotumor cerebri syndrome: Case series and pathogenetic implications.

OBJECTIVE: To report the clinical and radiologic features of 3 patients in whom prolonged vigorous coughing/Valsalva triggered the development of pseudotumor cerebri syndrome (PTCS) that persisted even after the coughing attacks had stopped and to discuss how this novel proximate trigger supports a "hydraulic model" of PTCS pathogenesis. METHODS: This was a retrospective case series of 3 patients seen between January 2011 and July 2017 in a tertiary care neuro-ophthalmology clinic. RESULTS: Three female patients (ages 13, 28, and 21 years) were asymptomatic until developing prolonged fits of profuse coughing. Two patients had upper respiratory tract infections, and 1 patient had a prolonged asthma exacerbation. Symptoms typical of PTCS began 2 weeks into the coughing attacks, and patients were examined 1 to 2 weeks after coughing had ceased. One patient was overweight and 2 were obese. All 3 patients had papilledema, and 1 of 3 patients had lateral rectus palsy. Two of 3 patients had visual field defects, and all 3 patients had dural venous sinus stenoses (DVSS) on venous imaging. Lumbar puncture (LP) confirmed elevated opening pressures in 2 of 3 patients and brought immediate and sustained relief of symptoms; LP attempts failed in the third patient because of body habitus (body mass index 68 kg/m2). All patients were given acetazolamide and advised to work on weight loss. At follow-up 3 to 6 months later, all 3 patients had complete remission of their clinical syndromes and resolution of their DVSS on neuroimaging. CONCLUSIONS: In susceptible individuals, prolonged coughing/Valsalva can trigger PTCS in the setting of collapsible (nonfixed) DVSS. This new observation supports a hydraulic model of PTCS pathogenesis.

Multivariable prediction model for suspected giant cell arteritis: development and validation.

PURPOSE: To develop and validate a diagnostic prediction model for patients with suspected giant cell arteritis (GCA). METHODS: A retrospective review of records of consecutive adult patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at seven university centers. The pathologic diagnosis was considered the final diagnosis. The predictor variables were age, gender, new onset headache, clinical temporal artery abnormality, jaw claudication, ischemic vision loss (VL), diplopia, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelet level. Multiple imputation was performed for missing data. Logistic regression was used to compare our models with the non-histologic American College of Rheumatology (ACR) GCA classification criteria. Internal validation was performed with 10-fold cross validation and bootstrap techniques. External validation was performed by geographic site. RESULTS: There were 530 complete TABx records: 397 were negative and 133 positive for GCA. Age, jaw claudication, VL, platelets, and log CRP were statistically significant predictors of positive TABx, whereas ESR, gender, headache, and temporal artery abnormality were not. The parsimonious model had a cross-validated bootstrap area under the receiver operating characteristic curve (AUROC) of 0.810 (95% CI =0.766-0.854), geographic external validation AUROC's in the range of 0.75-0.85, calibration pH-L of 0.812, sensitivity of 43.6%, and specificity of 95.2%, which outperformed the ACR criteria. CONCLUSION: Our prediction rule with calculator and nomogram aids in the triage of patients with suspected GCA and may decrease the need for TABx in select low-score at-risk subjects. However, misclassification remains a concern.

Quantitative evaluation of vision-related and health-related quality of life after endoscopic transsphenoidal surgery for pituitary adenoma.

OBJECTIVE Endoscopic resection of pituitary adenomas has been reported to improve vision function in up to 80%-90% of patients with visual impairment due to these adenomas. It is unclear how these reported rates translate into improvement in visual outcomes and general health as perceived by the patients. The authors evaluated self-assessed health-related quality of life (HR-QOL) and vision-related QOL (VR-QOL) in patients before and after endoscopic resection of pituitary adenomas. METHODS The authors prospectively collected data from 50 patients who underwent endoscopic resection of pituitary adenomas. This cohort included 32 patients (64%) with visual impairment preoperatively. Twenty-seven patients (54%) had pituitary dysfunction, including 17 (34%) with hormone-producing tumors. Patients completed the National Eye Institute Visual Functioning Questionnaire and the 36-Item Short Form Health Survey preoperatively and 6 weeks and 6 months after surgery. RESULTS Patients with preoperative visual impairment reported a significant impact of this condition on VR-QOL preoperatively, including general vision, near activities, and peripheral vision; they also noted vision-specific impacts on mental health, role difficulties, dependency, and driving. After endoscopic resection of adenomas, patients reported improvement across all these categories 6 weeks postoperatively, and this improvement was maintained by 6 months postoperatively. Patients with preoperative pituitary dysfunction, including hormone-producing tumors, perceived their general health and physical function as poorer, with some of these patients reporting improvement in perceived general health after the endoscopic surgery. All patients noted that their ability to work or perform activities of daily living was transiently reduced 6 weeks postoperatively, followed by significant improvement by 6 months after the surgery. CONCLUSIONS Both VR-QOL and patient's perceptions of their ability to do work and perform other daily activities as a result of their physical health significantly improved by 6 months after endoscopic resection of pituitary adenoma. The use of multidimensional QOL questionnaires provides a precise assessment of perceived outcomes after endoscopic surgery.

Remission of nonparaneoplastic autoimmune retinopathy after minimal steroid treatment.

PURPOSE: To describe the clinical findings in a patient demonstrating recovery from nonparaneoplastic autoimmune retinopathy after a minimal course of steroid treatment. METHODS: Clinical presentation was documented, and paraclinical tests were obtained using Humphrey automated perimetry for visual fields, Western blotting for antiretinal antibodies, and electroretinography for evaluation of rod and cone function. RESULTS: Initial presentation revealed marked visual field deficits, electroretinographic dysfunction, and the presence of α-enolase autoantibodies. After a brief course of oral corticosteroids, the patient demonstrated improvement in visual fields, disappearance of α-enolase autoantibodies, partial recovery of the cone on-response, and complete recovery of the rod response. CONCLUSION: This case is distinguished from previous reports by the rapidity and degree of recovery, the brevity of treatment required, and the unique electroretinographic recovery pattern with concomitant disappearance of α-enolase autoantibodies. These findings suggest a pathologic role for α-enolase autoantibodies in autoimmune rod bipolar cell dysfunction. Identification of other cases exhibiting such improvements and associated autoantibody activity may expand our understanding of nonparaneoplastic autoimmune retinopathy disease pathogenesis.

Sellar and parasellar intravascular lymphoma mimicking pituitary apoplexy.

BACKGROUND: Intravascular lymphoma (IVL) is a rare subtype of large-cell non-Hodgkin lymphoma, characterized by proliferation of lymphoma cells within the lumina of small vessels. There are no previously reported cases of IVL involving the pituitary gland presenting with neuro-ophthalmic findings. METHODS: A 68-year-old female presented with headache, right third nerve palsy, and Horner syndrome. MRI showed a 1.4-cm sellar mass consistent with a pituitary macroadenoma. Two weeks later, despite treatment with dexamethasone, the patient developed complete bilateral ophthalmoplegia and ptosis. Repeat MRI showed invasion of the clivus and cavernous sinuses, and a transsphenoidal pituitary biopsy was undertaken. RESULTS: The preliminary histopathology was consistent with bland pituitary apoplexy, but subsequent examination of an incidentally biopsied nasal polyp revealed endovascular malignant lymphoid cells that, on further scrutiny, were also present in the pituitary tissue. The diagnosis of IVL was confirmed, and the patient had an excellent clinical and radiological response to cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (CHOP-R) chemotherapy. CONCLUSION: IVL may involve the pituitary gland, causing sellar mass effect, cavernous sinus infiltration, and pituitary ischemia, mimicking pituitary apoplexy with neuro-ophthalmic features. It can be effectively treated with CHOP-R chemotherapy.

Disorders of the optic tract, radiation, and occipital lobe.

Disorders of the optic tract, lateral geniculate nucleus, optic radiation, and occipital lobe - collectively called the retrochiasmal visual pathways - are commonly encountered in neurological practice, and may result from a number of causes. The major visual morbidity of retrochiasmal disease is the homonymous visual field defect, which is found in approximately 8% of stroke patients. A homonymous visual field defect may have profound legal, occupational, and financial consequences for patients, with many patients unable to read, drive, or return to work after sustaining retrochiasmal damage. Some homonymous hemianopias may improve, usually within days of a cerebral infarction, but remain stable after 3 months. Although treatment options are limited to those of the underlying cause, appropriate counseling and low-vision rehabilitation may be helpful.

Risk factors for idiopathic intracranial hypertension in men: a case-control study.

OBJECTIVE: To identify risk factors for idiopathic intracranial hypertension (IIH) in men. DESIGN: Case-control study. A 96-item telephone questionnaire, answered retrospectively, with cases recalling at the age of their diagnosis and controls recalling at the age of their corresponding case's diagnosis. SETTING: Outpatient clinics in two US tertiary care centers. PARTICIPANTS: The characteristics of 24 men with IIH were compared to those of 48 controls matched for sex, age, race, and World Health Organization body mass index (BMI) category. MAIN OUTCOME MEASURES: Two previously validated questionnaires: the ADAM (Androgen Deficiency in Aging Males) questionnaire for testosterone deficiency and the Berlin questionnaire for obstructive sleep apnea (OSA), embedded within the telephone questionnaire. Analysis with Mantel-Haenszel odds ratios and mixed-effects logistic regression models accounted for matching. RESULTS: Cases and controls had similar enrollment matching characteristics. Although matching was successful by BMI category, there was a small difference between BMI values of cases and controls (cases: median 31.7, controls: median 29.9; p=0.03). After adjustment by BMI value, men with IIH were significantly more likely than controls to have a positive ADAM questionnaire for testosterone deficiency (OR: 17.4, 95% CI: 5.6-54.5; p<0.001) and significantly more likely to have either a positive Berlin questionnaire for OSA or history of diagnosed OSA (OR: 4.4, 95% CI: 1.5-12.9; p=0.03). CONCLUSIONS: Men with IIH are more likely than controls to have symptoms associated with testosterone deficiency and OSA. These associations suggest a possible role for sex hormones and OSA in the pathogenesis of IIH in men.