Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-term Outcomes.

BACKGROUND AND OBJECTIVES: Meningeal inflammation is one of the most common manifestations of neurosarcoidosis, occurring in 16%-69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Our primary aim was to study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment. METHODS: Patients with a diagnosis of neurosarcoidosis seen at Emory University (January 2011-August 2021) were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a CNS or non-CNS site). RESULTS: Twenty-six of 215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathologic confirmation came from CNS tissue in 50%. The median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium cerebelli were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). The median clinical follow-up was 48 months. The median modified Rankin scale score at last follow-up improved to 1.0 from 2.0 at presentation. DISCUSSION: Pachymeningitis due to sarcoidosis often presents with headaches, visual dysfunction, and seizures; it usually affects the dura of the falx cerebri, anterior and middle cranial fossae, and tentorium cerebelli and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is good.

Differences in single gland and multigland disease are seen in low biochemical profile primary hyperparathyroidism.

BACKGROUND: Primary hyperparathyroidism is characterized by increased levels of serum calcium and parathyroid hormone. Recently, 2 additional mild biochemical profiles have emerged, normocalcemic and normohormonal primary hyperparathyroidism. We reviewed our surgical experience of mild biochemical profile patients and compared them with classic primary hyperparathyroidism patients. METHODS: This is a single institution, retrospective cohort review of all patients who underwent parathyroidectomy for primary hyperparathyroidism from 2006-2012. Preoperative and intraoperative variables were analyzed. Univariable analysis was performed with analysis of variance and the χ2 test. A logistic regression was performed to identify significantly independent predictor variables for multigland disease. RESULTS: A total of 573 patients underwent parathyroidectomy for primary hyperparathyroidism (classic, n = 405; normohormonal, n = 96; normocalcemic, n = 72). Normocalcemic primary hyperparathyroidism was associated with multigland disease in 43 (45%, P < .001) patients as compared with the normohormonal (7, 10%) and classic (36, 9%) groups. On logistic regression, significant predictors for multigland disease were the normocalcemic subtype and positive family history. Twelve month biochemical normalization rates after operative treatment were >98% in all 3 groups. CONCLUSION: Our series shows that normocalcemic primary hyperparathyroidism is associated with a high incidence of multigland disease. Normohormonal disease is similar to classic disease patients with >90% presenting with single adenomas. Excellent rates of biochemical normalization can be obtained by operative treatment in all 3 groups.