Patients without VF deficits or whose visual deficits are stable or improving can be managed expectantly without negative impact on outcomes. Clinical severity based on a PAS ≥ 4 appeared to influence management towards emergency surgical intervention.
Context Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are responsible for a subset of familial isolated pituitary adenoma (FIPA) cases and sporadic pituitary neuroendocrine tumors (PitNETs). Objective To compare prospectively diagnosed AIP mutation-positive (AIPmut) PitNET patients with clinically presenting patients and to compare the clinical characteristics of AIPmut and AIPneg PitNET patients. Design 12-year prospective, observational study. Participants & Setting We studied probands and family members of FIPA kindreds and sporadic patients with disease onset ≤18 years or macroadenomas with onset ≤30 years (n = 1477). This was a collaborative study conducted at referral centers for pituitary diseases. Interventions & Outcome AIP testing and clinical screening for pituitary disease. Comparison of characteristics of prospectively diagnosed (n = 22) vs clinically presenting AIPmut PitNET patients (n = 145), and AIPmut (n = 167) vs AIPneg PitNET patients (n = 1310). Results Prospectively diagnosed AIPmut PitNET patients had smaller lesions with less suprasellar extension or cavernous sinus invasion and required fewer treatments with fewer operations and no radiotherapy compared with clinically presenting cases; there were fewer cases with active disease and hypopituitarism at last follow-up. When comparing AIPmut and AIPneg cases, AIPmut patients were more often males, younger, more often had GH excess, pituitary apoplexy, suprasellar extension, and more patients required multimodal therapy, including radiotherapy. AIPmut patients (n = 136) with GH excess were taller than AIPneg counterparts (n = 650). Conclusions Prospectively diagnosed AIPmut patients show better outcomes than clinically presenting cases, demonstrating the benefits of genetic and clinical screening. AIP-related pituitary disease has a wide spectrum ranging from aggressively growing lesions to stable or indolent disease course.
We present the highest reported frequency of brain biopsy for cryptogenic neurological disease. The risk associated with the procedure was low and the biopsy results impacted significantly upon diagnosis and management. We therefore propose that the procedure should no longer be considered one of last resort.
DIANA BECK (1902-1956) WAS probably the first female neurosurgeon in the world and played a large part in establishing neurosurgery in the United Kingdom. She served as the consultant advisor in neurosurgery for the Emergency Medical Service in the southwest region during the Second World War and subsequently set up the neurosurgical unit at Frenchay Hospital in Bristol before becoming a consultant at The Middlesex Hospital in London in 1947. There, she was the first woman to be given charge of men in a consultant capacity in a major London teaching hospital. During her 31-year career, she was the only female neurosurgeon in Western Europe and the United States. Miss Beck came to the attention of the general public when she operated on Alan Alexander Milne in 1952. However, she was better known in neurosurgical circles for her interest in intracranial hemorrhage. In this article, some of her original research, case reports, and her seminal paper on the surgical treatment of intracerebral hemorrhage are presented. They are a stark reminder of the limited investigations available at that time. In addition to her neurosurgical achievements, Miss Beck was a remarkable character and teacher. Her Saturday ward rounds proved so popular that they competed successfully with the students' weekend plans. In a specialty in which women continue to represent a disproportionately small percentage of the workforce, Miss Beck was one of our earliest role models.
Impulse control disorders (ICDs) constitute socially disruptive behaviors such as pathological gambling, impulsive eating, compulsive shopping, and hypersexuality. These conditions are well recognized in patients on dopamine agonist (DA) therapy for Parkinson disease. Dopamine agonists are widely used as first-line agents in the treatment of prolactinomas, but ICDs in this group of patients are relatively rare, perhaps because of lower therapeutic doses used. A review of the literature yielded only a few cases of ICDs in patients on DA treatment for prolactinomas. These symptoms are perhaps underreported because of lack of awareness among patients and health care professionals. Impulse control disorders are recognized psychiatric disorders that have significant psychological and social implications, and patients need to be counselled about this rare possibility when embarking on prolonged DA therapy. We describe a young patient with severe, socially disruptive impulsivity manifesting with pathological gambling who had been on long-term bromocriptine therapy for a macroprolactinoma.
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